56 results on '"Giglia TM"'
Search Results
2. Recommendations for training in pediatric cardiology. Task Force 5: requirements for pediatric cardiac critical care.
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Kulik T, Giglia TM, Kocis KC, Mahoney LT, Schwartz SM, Wernovsky G, Wessel DL, American College of Cardiology Foundation, American Heart Association, and American Academy of Pediatrics
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- 2005
3. Critical treatment strategies for acute pulmonary hypertension in infants and children: Pediatric Cardiac Intensive Care Society Scientific Statement.
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Giglia TM, Bronicki R, Checchia PA, and Laussen PC
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- 2010
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4. Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation?
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Giglia TM and Humpl T
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- 2010
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5. Outcomes Associated with Giant Coronary Artery Aneurysms after Kawasaki Disease: A Single-Center United States Experience.
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Elias MD, Brothers JA, Hogarty AN, Martino J, O'Byrne ML, Patel C, Stephens P, Tingo J, Vetter VL, Ravishankar C, and Giglia TM
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Objectives: To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAAs) at a single US center., Study Design: Medical records for all patients with KD and giant CAAs at a pediatric academic institution were reviewed. Primary outcomes included major adverse cardiovascular events (MACE) and normalization of CA luminal diameter, using Kaplan-Meier analyses., Results: There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with a median age at diagnosis of 0.9 years (range, 0.2-13.3 years). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 patients (21.7%) at a median of 1.4 years (range, 0.04-22.6 years) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z scores of ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to a normal luminal diameter at a median of 3.6 years (range, 0.6-12.0 years). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%., Conclusions: Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z scores of ≥20. Despite regression to a normal luminal diameter in >25% of CAAs, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)
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- 2024
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6. Incidence, Management, and Outcomes of Pulmonary Embolism at Tertiary Pediatric Hospitals in the United States.
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Rastogi R, Okunowo O, Faerber JA, Mavroudis CD, Whitworth H, Giglia TM, Witmer C, Raffini LJ, and O'Byrne ML
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Background: Pediatric pulmonary embolism (PE) is rare and potentially life-threatening. Though thrombolysis and thrombectomy are increasingly used in adult PE, trends in pediatric treatment and outcomes remain incompletely described., Objectives: The purpose of this study was to describe the incidence of PE, proportion of cases treated with anticoagulation alone, systemic thrombolysis, and directed therapy (local thrombolysis and thrombectomy), clinical outcomes, and total costs., Methods: A multicenter observational study was performed using administrative data from the Pediatric Health Information System database to study PE treated at U.S. pediatric hospitals from 2015 to 2021. Outcomes by treatment were evaluated using multivariable generalized linear mixed effects models., Results: Of 3,136 subjects, 70% were at least 12 years of age, and 46% were male. Sixty-two percent had at least 1 comorbidity, and congenital heart disease of any kind was the most prevalent (20%). Eighty-eight percent of subjects received anticoagulation alone, 7% received systemic thrombolysis, and 5% received directed therapy. Overall in-hospital mortality was 7.5%. Treatment approach did not change over time ( P = 0.98). After adjusting for patient characteristics, directed therapy was associated with a lower risk of mortality (adjusted percentage -3%, [95% CI: -5% to 0%]) than anticoagulation alone. Systemic thrombolysis was associated with a greater total cost of hospitalization ($113,043 greater [95% CI: $62,866, $163,219]). Length of hospital stay did not differ by treatment., Conclusions: Pediatric patients with PE have a high incidence of underlying chronic disease. Anticoagulation alone remains the mainstay of treatment, with thrombolysis and thrombectomy rarely being used. Given the relative rarity of pediatric PE, additional research requiring innovative study designs is paramount., Competing Interests: The current study used resources from the Children's Hospital of Philadelphia Cardiac Center Clinical Research Core. Dr Raffini has received consulting fees from Boehringer Ingelheim, Genentech, and CSL Behring. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.PERSPECTIVESCOMPETENCY IN MEDICAL KNOWLEDGE 1: Most pediatric patients with PE have at least 1 chronic disease, of which cardiac disease is the most common. COMPETENCY IN MEDICAL KNOWLEDGE 2: Most pediatric patients with PE are treated with anticoagulation, though the choice of anticoagulation has shifted over time. COMPETENCY IN PATIENT CARE: Though most pediatric patients with PE are treated with anticoagulation, thrombolysis and thrombectomy must also be considered as treatment modalities. TRANSLATIONAL OUTLOOK: Though anticoagulation is the mainstay of pediatric PE treatment, better delineation of which patients may benefit from thrombolysis or thrombectomy is necessary., (© 2024 The Authors.)
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- 2024
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7. Kawasaki Disease in the Time of COVID-19 and MIS-C: The International Kawasaki Disease Registry.
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Harahsheh AS, Shah S, Dallaire F, Manlhiot C, Khoury M, Lee S, Fabi M, Mauriello D, Tierney ESS, Sabati AA, Dionne A, Dahdah N, Choueiter N, Thacker D, Giglia TM, Truong DT, Jain S, Portman M, Orr WB, Harris TH, Szmuszkovicz JR, Farid P, and McCrindle BW
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- Child, Humans, SARS-CoV-2, Registries, COVID-19 epidemiology, Mucocutaneous Lymph Node Syndrome diagnosis, Mucocutaneous Lymph Node Syndrome epidemiology, Mucocutaneous Lymph Node Syndrome therapy, Systemic Inflammatory Response Syndrome
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Background: Patients with multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) have overlapping clinical features. We compared demographics, clinical presentation, management, and outcomes of patients according to evidence of previous SARS-CoV-2 infection., Methods: The International Kawasaki Disease Registry (IKDR) enrolled KD and MIS-C patients from sites in North, Central, and South America, Europe, Asia, and the Middle East. Evidence of previous infection was defined as: Positive (household contact or positive polymerase chain reaction [PCR]/serology), Possible (suggestive clinical features of MIS-C and/or KD with negative PCR or serology but not both), Negative (negative PCR and serology and no known exposure), and Unknown (incomplete testing and no known exposure)., Results: Of 2345 enrolled patients SARS-CoV-2 status was Positive for 1541 (66%) patients, Possible for 89 (4%), Negative for 404 (17%) and Unknown for 311 (13%). Clinical outcomes varied significantly among the groups, with more patients in the Positive/Possible groups presenting with shock, having admission to intensive care, receiving inotropic support, and having longer hospital stays. Regarding cardiac abnormalities, patients in the Positive/Possible groups had a higher prevalence of left ventricular dysfunction, and patients in the Negative and Unknown groups had more severe coronary artery abnormalities., Conclusions: There appears to be a spectrum of clinical features from MIS-C to KD with a great deal of heterogeneity, and one primary differentiating factor is evidence for previous acute SARS-CoV-2 infection/exposure. SARS-CoV-2 Positive/Possible patients had more severe presentations and required more intensive management, with a greater likelihood of ventricular dysfunction but less severe coronary artery adverse outcomes, in keeping with MIS-C., (Copyright © 2023 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)
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- 2024
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8. Cardiac Biomarkers Aid in Differentiation of Kawasaki Disease from Multisystem Inflammatory Syndrome in Children Associated with COVID-19.
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Walton M, Raghuveer G, Harahsheh A, Portman MA, Lee S, Khoury M, Dahdah N, Fabi M, Dionne A, Harris TH, Choueiter N, Garrido-Garcia LM, Jain S, Dallaire F, Misra N, Hicar MD, Giglia TM, Truong DT, Tierney ESS, Thacker D, Nowlen TT, Szmuszkovicz JR, Norozi K, Orr WB, Farid P, Manlhiot C, and McCrindle BW
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Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022. The study population included 118 KD patients who met American Heart Association KD criteria and compared them to 946 MIS-C patients who met 2020 Centers for Disease Control and Prevention case definition. All included patients had at least one measurement of amino-terminal prohormone brain natriuretic peptide (NTproBNP) or cardiac troponin I (TnI), and echocardiography. Regression analyses were used to determine associations between cardiac biomarker levels, diagnosis, and cardiac involvement. Higher NTproBNP (≥ 1500 ng/L) and TnI (≥ 20 ng/L) at presentation were associated with MIS-C versus KD with specificity of 77 and 89%, respectively. Higher biomarker levels were associated with shock and intensive care unit admission; higher NTproBNP was associated with longer hospital length of stay. Lower left ventricular ejection fraction, more pronounced for MIS-C, was also associated with higher biomarker levels. Coronary artery involvement was not associated with either biomarker. Higher NTproBNP and TnI levels are suggestive of MIS-C versus KD and may be clinically useful in their differentiation. Consideration might be given to their inclusion in the routine evaluation of both conditions., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
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- 2023
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9. Feasibility of Digital Stethoscopes in Telecardiology Visits for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.
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Stagg A, Giglia TM, Gardner MM, Shustak RJ, Natarajan SS, Hehir DA, Szwast AL, Rome JJ, Ravishankar C, and Preminger TJ
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- Infant, Humans, Feasibility Studies, Heart Murmurs diagnosis, Stethoscopes, Heart Defects, Congenital diagnosis, Heart Defects, Congenital therapy, Hypoplastic Left Heart Syndrome surgery
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Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. Interstage telecardiology visits (TCV) have been effective in identifying clinical concerns and preventing unnecessary emergency department visits in this high-risk population. We aimed to assess the feasibility of implementing auscultation with digital stethoscopes (DSs) during TCV and the potential impact on interstage care in our Infant Single Ventricle Monitoring & Management Program. In addition to standard home-monitoring practice for TCV, caregivers received training on use of a DS (Eko CORE attachment assembled with Classic II Infant Littman stethoscope). Sound quality of the DS and comparability to in-person auscultation were evaluated based on two providers' subjective assessment. We also evaluated provider and caregiver acceptability of the DS. From 7/2021 to 6/2022, the DS was used during 52 TCVs in 16 patients (median TCVs/patient: 3; range: 1-8), including 7 with hypoplastic left heart syndrome. Quality of heart sounds and murmur auscultation were subjectively equivalent to in-person findings with excellent inter-rater agreement (98%). All providers and caregivers reported ease of use and confidence in evaluation with the DS. In 12% (6/52) of TCVs, the DS provided additional significant information compared to a routine TCV; this expedited life-saving care in two patients. There were no missed events or deaths. Use of a DS during TCV was feasible in this fragile cohort and effective in identifying clinical concerns with no missed events. Longer term use of this technology will further establish its role in telecardiology., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
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- 2023
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10. Neighborhood Social Vulnerability and Interstage Weight Gain: Evaluating the Role of a Home Monitoring Program.
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Shustak RJ, Huang J, Tam V, Stagg A, Giglia TM, Ravishankar C, Mercer-Rosa L, Guevara JP, and Gardner MM
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- Infant, Humans, Retrospective Studies, Social Vulnerability, Logistic Models, Weight Gain, Hypoplastic Left Heart Syndrome diagnosis, Hypoplastic Left Heart Syndrome surgery, Univentricular Heart
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Background Poor interstage weight gain is a risk factor for adverse outcomes in infants with hypoplastic left heart syndrome. We sought to examine the association of neighborhood social vulnerability and interstage weight gain and determine if this association is modified by enrollment in our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP). Methods and Results We performed a retrospective single-center study of infants with hypoplastic left heart syndrome before (2007-2010) and after (2011-2020) introduction of the ISVMP. The primary outcome was interstage weight gain, and the secondary outcome was interstage growth failure. Multivariable linear and logistic regression models were used to examine the association between the Social Vulnerability Index and the outcomes. We introduced an interaction term into the models to test for effect modification by the ISVMP. We evaluated 217 ISVMP infants and 111 pre-ISVMP historical controls. The Social Vulnerability Index was associated with interstage growth failure ( P =0.001); however, enrollment in the ISVMP strongly attenuated this association ( P =0.04). Pre-ISVMP, as well as high- and middle-vulnerability infants gained 4 g/d less and were significantly more likely to experience growth failure than low-vulnerability infants (high versus low: adjusted odds ratio [aOR], 12.5 [95% CI, 2.5-62.2]; middle versus low: aOR, 7.8 [95% CI, 2.0-31.2]). After the introduction of the ISVMP, outcomes did not differ by Social Vulnerability Index tertile. Infants with middle and high Social Vulnerability Index scores who were enrolled in the ISVMP gained 4 g/d and 2 g/d more, respectively, than pre-ISVMP controls. Conclusions In infants with hypoplastic left heart syndrome, high social vulnerability is a risk factor for poor interstage weight gain. However, enrollment in the ISVMP significantly reduces growth disparities.
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- 2023
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11. Association of Home Monitoring and Unanticipated Interstage Readmissions in Infants With Hypoplastic Left Heart Syndrome.
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Shustak RJ, Faerber JA, Stagg A, Hehir DA, Natarajan SS, Preminger TJ, Szwast A, Rome JJ, Giglia TM, Ravishankar C, Mercer-Rosa L, and Gardner MM
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- Humans, Infant, Patient Readmission, Retrospective Studies, Cohort Studies, Treatment Outcome, Risk Factors, Hypoplastic Left Heart Syndrome diagnosis, Hypoplastic Left Heart Syndrome surgery, Univentricular Heart complications
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Background The impact of home monitoring on unanticipated interstage readmissions in infants with hypoplastic left heart syndrome has not been previously studied. We sought to examine the association of our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP) with readmission frequency, cumulative readmission days, and readmission illness severity and to identify patient-level risk factors for readmission. Methods and Results We performed a retrospective single-center cohort study comparing infants with hypoplastic left heart syndrome enrolled in ISVMP (December 2010-December 2019) to historical controls (January 2007-November 2010). The primary outcome was number of readmissions per interstage days. Secondary outcomes were cumulative interstage readmission days and occurrence of severe readmissions. Inverse probability weighted and multivariable generalized linear models were used to examine the association between ISVMP and the outcomes. We compared 198 infants in the ISVMP to 128 historical controls. Infants in the ISVMP had more than double the risk of interstage readmission compared with controls (adjusted incidence rate ratio, 2.38 [95% CI, 1.50-3.78]; P =0.0003). There was no difference in cumulative interstage readmission days (adjusted incidence rate ratio, 1.02 [95% CI, 0.69-1.50]; P =0.90); however, infants in the ISVMP were less likely to have severe readmissions (adjusted odds ratio, 0.28 [95% CI, 0.11-0.68]; P =0.005). Other factors independently associated with number of readmissions included residing closer to our center, younger gestational age, genetic syndrome, and discharge on exclusive enteral feeds. Conclusions Infants in the ISVMP had more frequent readmissions but comparable readmission days and fewer severe unanticipated readmissions. These findings suggest that home monitoring can reduce interstage morbidity without increasing readmission days.
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- 2023
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12. Neurologic complications of infective endocarditis in children.
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Jacobwitz M, Favilla E, Patel A, Giglia TM, Taing K, Ravishankar C, Gaynor JW, Licht DJ, McGuire JL, and Beslow LA
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- Humans, Child, Adolescent, Retrospective Studies, Brain Ischemia complications, Stroke, Endocarditis, Bacterial complications, Endocarditis, Bacterial diagnosis, Endocarditis, Bacterial epidemiology, Endocarditis complications, Endocarditis diagnosis, Nervous System Diseases etiology, Nervous System Diseases complications
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Objectives: To define the frequency and characteristics of acute neurologic complications in children hospitalised with infective endocarditis and to identify risk factors for neurologic complications., Study Design: Retrospective cohort study of children aged 0-18 years hospitalised at a tertiary children's hospital from 1 January, 2008 to 31 December, 2017 with infective endocarditis., Results: Sixty-eight children met Duke criteria for infective endocarditis (43 definite and 25 possible). Twenty-three (34%) had identified neurologic complications, including intracranial haemorrhage (25%, 17/68) and ischaemic stroke (25%, 17/68). Neurologic symptoms began a median of 4.5 days after infective endocarditis symptom onset (interquartile range 1, 25 days), though five children were asymptomatic and diagnosed on screening neuroimaging only. Overall, only 56% (38/68) underwent neuroimaging during acute hospitalisation, so additional asymptomatic neurologic complications may have been missed. Children with identified neurologic complications compared to those without were older (48 versus 22% ≥ 13 years old, p = 0.031), more often had definite rather than possible infective endocarditis (96 versus 47%, p < 0.001), mobile vegetations >10mm (30 versus 11%, p = 0.048), and vegetations with the potential for systemic embolisation (65 versus 29%, p = 0.004). Six children died (9%), all of whom had neurologic complications., Conclusions: Neurologic complications of infective endocarditis were common (34%) and associated with mortality. The true frequency of neurologic complications was likely higher because asymptomatic cases may have been missed without screening neuroimaging. Moving forward, we advocate that all children with infective endocarditis have neurologic consultation, examination, and screening neuroimaging. Additional prospective studies are needed to determine whether early identification of neurologic abnormalities may direct management and ultimately reduce neurologic morbidity and overall mortality.
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- 2023
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13. Examination of Adverse Reactions After COVID-19 Vaccination Among Patients With a History of Multisystem Inflammatory Syndrome in Children.
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Elias MD, Truong DT, Oster ME, Trachtenberg FL, Mu X, Jone PN, Mitchell EC, Dummer KB, Sexson Tejtel SK, Osakwe O, Thacker D, Su JA, Bradford TT, Burns KM, Campbell MJ, Connors TJ, D'Addese L, Forsha D, Frosch OH, Giglia TM, Goodell LR, Handler SS, Hasbani K, Hebson C, Krishnan A, Lang SM, McCrindle BW, McHugh KE, Morgan LM, Payne RM, Sabati A, Sagiv E, Sanil Y, Serrano F, Newburger JW, and Dionne A
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- United States epidemiology, Child, Humans, Male, Child, Preschool, Female, COVID-19 Vaccines adverse effects, BNT162 Vaccine, Cross-Sectional Studies, Vaccination adverse effects, COVID-19 epidemiology, COVID-19 prevention & control, Connective Tissue Diseases
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Importance: Data are limited regarding adverse reactions after COVID-19 vaccination in patients with a history of multisystem inflammatory syndrome in children (MIS-C). The lack of vaccine safety data in this unique population may cause hesitancy and concern for many families and health care professionals., Objective: To describe adverse reactions following COVID-19 vaccination in patients with a history of MIS-C., Design, Setting, and Participants: In this multicenter cross-sectional study including 22 North American centers participating in a National Heart, Lung, and Blood Institute, National Institutes of Health-sponsored study, Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children (MUSIC), patients with a prior diagnosis of MIS-C who were eligible for COVID-19 vaccination (age ≥5 years; ≥90 days after MIS-C diagnosis) were surveyed between December 13, 2021, and February 18, 2022, regarding COVID-19 vaccination status and adverse reactions., Exposures: COVID-19 vaccination after MIS-C diagnosis., Main Outcomes and Measures: The main outcome was adverse reactions following COVID-19 vaccination. Comparisons were made using the Wilcoxon rank sum test for continuous variables and the χ2 or Fisher exact test for categorical variables., Results: Of 385 vaccine-eligible patients who were surveyed, 185 (48.1%) received at least 1 vaccine dose; 136 of the vaccinated patients (73.5%) were male, and the median age was 12.2 years (IQR, 9.5-14.7 years). Among vaccinated patients, 1 (0.5%) identified as American Indian/Alaska Native, non-Hispanic; 9 (4.9%) as Asian, non-Hispanic; 45 (24.3%) as Black, non-Hispanic; 59 (31.9%) as Hispanic or Latino; 53 (28.6%) as White, non-Hispanic; 2 (1.1%) as multiracial, non-Hispanic; and 2 (1.1%) as other, non-Hispanic; 14 (7.6%) had unknown or undeclared race and ethnicity. The median time from MIS-C diagnosis to first vaccine dose was 9.0 months (IQR, 5.1-11.9 months); 31 patients (16.8%) received 1 dose, 142 (76.8%) received 2 doses, and 12 (6.5%) received 3 doses. Almost all patients received the BNT162b2 vaccine (347 of 351 vaccine doses [98.9%]). Minor adverse reactions were observed in 90 patients (48.6%) and were most often arm soreness (62 patients [33.5%]) and/or fatigue (32 [17.3%]). In 32 patients (17.3%), adverse reactions were treated with medications, most commonly acetaminophen (21 patients [11.4%]) or ibuprofen (11 [5.9%]). Four patients (2.2%) sought medical evaluation, but none required testing or hospitalization. There were no patients with any serious adverse events, including myocarditis or recurrence of MIS-C., Conclusions and Relevance: In this cross-sectional study of patients with a history of MIS-C, no serious adverse events were reported after COVID-19 vaccination. These findings suggest that the safety profile of COVID-19 vaccination administered at least 90 days following MIS-C diagnosis appears to be similar to that in the general population.
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- 2023
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14. Time to Move Forward on Pediatric Atrial Standstill.
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Tan RBM, Giglia TM, and Cecchin F
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- Humans, Child, Heart Atria surgery, Heart Block, Cardiomyopathies
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Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
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- 2023
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15. Initial Experience with Telemedicine for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.
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Stagg A, Giglia TM, Gardner MM, Offit BF, Fuller KM, Natarajan SS, Hehir DA, Szwast AL, Rome JJ, Ravishankar C, Laskin BL, and Preminger TJ
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- Infant, Newborn, Infant, Humans, Pandemics, Patient Discharge, COVID-19, Heart Defects, Congenital surgery, Telemedicine
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Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality; home monitoring programs have mitigated these risks. In 2019, we instituted telemedicine (TM) in our established Infant Single Ventricle Monitoring Program. All consecutive patients discharged following neonatal operation/intervention were monitored until subsequent stage 2 surgical palliation. We offered TM (synchronous video) visits as part of regularly scheduled follow-up, replacing at least one in-person primary care visit with a TM cardiologist visit. We tracked emergency department (ED) visits, hospitalizations, how TM identified clinical concerns, and whether use of TM prevented unnecessary ED visits or expedited in-person assessment. We assessed caregiver and clinician satisfaction. Between 8/2019 and 5/2020, we conducted 60 TM visits for 29 patients. Of 31 eligible patients, 2 families (6.9%) declined. Median monitoring time was 199 days (range 75-264) and median number of TM visits/patient was 2 (range 1-5). In 6 visits (10%), significant clinical findings were identified which avoided an ED visit. Five TM visits led to expedited outpatient assessments, of which 1 patient required hospitalization. There were no missed events or deaths. Median ED visits/patient/month were significantly lower compared to the same calendar period of the prior year (0.0 (0-2.5) vs. 0.4 (0-3.7), p = 0.0004). Caregivers and clinicians expressed high levels of satisfaction with TM. TM for this high-risk population is feasible and effective in identifying clinical concerns and preventing unnecessary ED visits. TM was particularly effective during the COVID-19 pandemic, allowing for easy adaptation of care to ensure patient safety in this fragile cohort., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
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- 2023
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16. Multi-disciplinary collaborative consensus guidance statement on the assessment and treatment of postacute sequelae of SARS-CoV-2 infection (PASC) in children and adolescents.
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Malone LA, Morrow A, Chen Y, Curtis D, de Ferranti SD, Desai M, Fleming TK, Giglia TM, Hall TA, Henning E, Jadhav S, Johnston AM, Kathirithamby DRC, Kokorelis C, Lachenauer C, Li L, Lin HC, Locke T, MacArthur C, Mann M, McGrath-Morrow SA, Ng R, Ohlms L, Risen S, Sadreameli SC, Sampsel S, Tejtel SKS, Silver JK, Simoneau T, Srouji R, Swami S, Torbey S, Gutierrez MV, Williams CN, Zimmerman LA, and Vaz LE
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- Child, Humans, Adolescent, Consensus, SARS-CoV-2, Disease Progression, COVID-19
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- 2022
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17. Successful treatment of intracardiac thrombosis in the presence of fulminant myocarditis requiring ECMO associated with COVID-19.
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Mejia EJ, O'Connor MJ, Samelson-Jones BJ, Mavroudis CD, Giglia TM, Keashen R, Rossano J, Naim MY, and Maeda K
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- Humans, COVID-19 complications, Extracorporeal Membrane Oxygenation, Heart Diseases complications, Heart Diseases therapy, Myocarditis complications, Myocarditis diagnosis, Myocarditis therapy, Thrombosis complications, Thrombosis therapy
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- 2022
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18. Clinically Suspected Myocarditis Temporally Related to COVID-19 Vaccination in Adolescents and Young Adults: Suspected Myocarditis After COVID-19 Vaccination.
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Truong DT, Dionne A, Muniz JC, McHugh KE, Portman MA, Lambert LM, Thacker D, Elias MD, Li JS, Toro-Salazar OH, Anderson BR, Atz AM, Bohun CM, Campbell MJ, Chrisant M, D'Addese L, Dummer KB, Forsha D, Frank LH, Frosch OH, Gelehrter SK, Giglia TM, Hebson C, Jain SS, Johnston P, Krishnan A, Lombardi KC, McCrindle BW, Mitchell EC, Miyata K, Mizzi T, Parker RM, Patel JK, Ronai C, Sabati AA, Schauer J, Sexson Tejtel SK, Shea JR, Shekerdemian LS, Srivastava S, Votava-Smith JK, White S, and Newburger JW
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- Adolescent, Child, Electrocardiography methods, Female, Humans, Magnetic Resonance Imaging, Cine methods, Male, Myocarditis blood, Myocarditis etiology, Retrospective Studies, Time Factors, Young Adult, COVID-19 prevention & control, COVID-19 Vaccines adverse effects, Myocarditis diagnostic imaging, Myocarditis physiopathology
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Background: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth., Methods: We retrospectively collected data on patients <21 years old presenting before July 4, 2021, with suspected myocarditis within 30 days of COVID-19 vaccination. Lake Louise criteria were used for cardiac MRI findings. Myocarditis cases were classified as confirmed or probable on the basis of the Centers for Disease Control and Prevention definitions., Results: We report on 139 adolescents and young adults with 140 episodes of suspected myocarditis (49 confirmed, 91 probable) at 26 centers. Most patients were male (n=126, 90.6%) and White (n=92, 66.2%); 29 (20.9%) were Hispanic; and the median age was 15.8 years (range, 12.1-20.3; interquartile range [IQR], 14.5-17.0). Suspected myocarditis occurred in 136 patients (97.8%) after the mRNA vaccine, with 131 (94.2%) after the Pfizer-BioNTech vaccine; 128 (91.4%) occurred after the second dose. Symptoms started at a median of 2 days (range, 0-22; IQR, 1-3) after vaccination. The most common symptom was chest pain (99.3%). Patients were treated with nonsteroidal anti-inflammatory drugs (81.3%), intravenous immunoglobulin (21.6%), glucocorticoids (21.6%), colchicine (7.9%), or no anti-inflammatory therapies (8.6%). Twenty-six patients (18.7%) were in the intensive care unit, 2 were treated with inotropic/vasoactive support, and none required extracorporeal membrane oxygenation or died. Median hospital stay was 2 days (range, 0-10; IQR, 2-3). All patients had elevated troponin I (n=111, 8.12 ng/mL; IQR, 3.50-15.90) or T (n=28, 0.61 ng/mL; IQR, 0.25-1.30); 69.8% had abnormal ECGs and arrhythmias (7 with nonsustained ventricular tachycardia); and 18.7% had left ventricular ejection fraction <55% on echocardiogram. Of 97 patients who underwent cardiac MRI at a median 5 days (range, 0-88; IQR, 3-17) from symptom onset, 75 (77.3%) had abnormal findings: 74 (76.3%) had late gadolinium enhancement, 54 (55.7%) had myocardial edema, and 49 (50.5%) met Lake Louise criteria. Among 26 patients with left ventricular ejection fraction <55% on echocardiogram, all with follow-up had normalized function (n=25)., Conclusions: Most cases of suspected COVID-19 vaccine myocarditis occurring in persons <21 years have a mild clinical course with rapid resolution of symptoms. Abnormal findings on cardiac MRI were frequent. Future studies should evaluate risk factors, mechanisms, and long-term outcomes.
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- 2022
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19. Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades.
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Osborne J, Friedman K, Runeckles K, Choueiter NF, Giglia TM, Dallaire F, Newburger JW, Low T, Mathew M, Mackie AS, Dahdah N, Yetman AT, Harahsheh AS, Raghuveer G, Norozi K, Burns JC, Jain S, Mondal T, Portman MA, Szmuszkovicz JR, Crean A, and McCrindle BW
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- Adolescent, Anticoagulants administration & dosage, Aspirin administration & dosage, Child, Coronary Aneurysm etiology, Coronary Aneurysm therapy, Female, Humans, Male, Mucocutaneous Lymph Node Syndrome complications, Registries, Retrospective Studies, Warfarin administration & dosage, Guideline Adherence, Mucocutaneous Lymph Node Syndrome therapy, Venous Thromboembolism prevention & control
- Abstract
In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) for long-term management are defined by both maximal and current coronary artery (CA) dimensions normalized as z-scores. We sought to determine the degree to which current recommended practice differs from past actual practice, highlighting areas for knowledge translation efforts. The International KD Registry (IKDR) included 1651 patients with CA aneurysms (z-score > 2.5) from 1999 to 2016. Patients were classified by AHA RL using maximum CA z-score (RL 3 = small, RL 4 = medium, RL 5 = large/giant) and subcategorized based on decreases over time. Medical management provided was compared to recommendations. Low-dose acetylsalicylic acid (ASA) use ranged from 86 (RL 3.1) to 95% (RL 5.1) for RLs where use was "indicated." Dual antiplatelet therapy (ASA + clopidogrel) use ranged from 16% for RL 5.2 to 9% for RL 5.4. Recommended anticoagulation (warfarin or low molecular weight heparin) use was 65% for RL 5.1, while 12% were on triple therapy (anticoagulation + dual antiplatelet). Optional statin use ranged from 2 to 8% depending on RL. Optional beta-blocker use was 2-25% for RL 5, and 0-5% for RLs 3 and 4 where it is not recommended. Generally, past practice was consistent with the latest AHA guidelines, taking into account the flexible wording of recommendations based on the limited evidence, as well as unmeasured patient-specific factors. In addition to strengthening the overall evidence base, knowledge translation efforts may be needed to address variation in thromboprophylaxis management.
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- 2021
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20. Evidence of thrombotic microangiopathy in children with SARS-CoV-2 across the spectrum of clinical presentations.
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Diorio C, McNerney KO, Lambert M, Paessler M, Anderson EM, Henrickson SE, Chase J, Liebling EJ, Burudpakdee C, Lee JH, Balamuth FB, Blatz AM, Chiotos K, Fitzgerald JC, Giglia TM, Gollomp K, Odom John AR, Jasen C, Leng T, Petrosa W, Vella LA, Witmer C, Sullivan KE, Laskin BL, Hensley SE, Bassiri H, Behrens EM, and Teachey DT
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- Acute Kidney Injury etiology, Acute Kidney Injury pathology, Adolescent, Antibodies, Viral blood, Biomarkers metabolism, COVID-19 pathology, COVID-19 virology, Child, Child, Preschool, Cluster Analysis, Complement Membrane Attack Complex metabolism, Creatinine blood, Female, Humans, Male, RNA, Viral metabolism, Reverse Transcriptase Polymerase Chain Reaction, SARS-CoV-2 genetics, SARS-CoV-2 isolation & purification, Severity of Illness Index, Thrombotic Microangiopathies complications, COVID-19 diagnosis, Thrombotic Microangiopathies diagnosis
- Abstract
Most children with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection have mild or minimal disease, with a small proportion developing severe disease or multisystem inflammatory syndrome in children (MIS-C). Complement-mediated thrombotic microangiopathy (TMA) has been associated with SARS-CoV-2 infection in adults but has not been studied in the pediatric population. We hypothesized that complement activation plays an important role in SARS-CoV-2 infection in children and sought to understand if TMA was present in these patients. We enrolled 50 hospitalized pediatric patients with acute SARS-CoV-2 infection (n = 21, minimal coronavirus disease 2019 [COVID-19]; n = 11, severe COVID-19) or MIS-C (n = 18). As a biomarker of complement activation and TMA, soluble C5b9 (sC5b9, normal 247 ng/mL) was measured in plasma, and elevations were found in patients with minimal disease (median, 392 ng/mL; interquartile range [IQR], 244-622 ng/mL), severe disease (median, 646 ng/mL; IQR, 203-728 ng/mL), and MIS-C (median, 630 ng/mL; IQR, 359-932 ng/mL) compared with 26 healthy control subjects (median, 57 ng/mL; IQR, 9-163 ng/mL; P < .001). Higher sC5b9 levels were associated with higher serum creatinine (P = .01) but not age. Of the 19 patients for whom complete clinical criteria were available, 17 (89%) met criteria for TMA. A high proportion of tested children with SARS-CoV-2 infection had evidence of complement activation and met clinical and diagnostic criteria for TMA. Future studies are needed to determine if hospitalized children with SARS-CoV-2 should be screened for TMA, if TMA-directed management is helpful, and if there are any short- or long-term clinical consequences of complement activation and endothelial damage in children with COVID-19 or MIS-C., (© 2020 by The American Society of Hematology.)
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- 2020
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21. Management of Multisystem Inflammatory Syndrome in Children Associated With COVID-19: A Survey From the International Kawasaki Disease Registry.
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Elias MD, McCrindle BW, Larios G, Choueiter NF, Dahdah N, Harahsheh AS, Jain S, Manlhiot C, Portman MA, Raghuveer G, Giglia TM, and Dionne A
- Abstract
Background: Since April 2020, there have been numerous reports of children presenting with systemic inflammation, often in critical condition, and with evidence of recent infection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This condition, since defined as the multisystem inflammatory syndrome in children (MIS-C), is assumed to be a delayed immune response to coronavirus disease 2019 (COVID-19), and there are frequently cardiac manifestations of ventricular dysfunction and/or coronary artery dilation., Methods: We surveyed the inpatient MIS-C management approaches of the members of the International Kawasaki Disease Registry across 38 institutions and 11 countries., Results: Among the respondents, 56% reported using immunomodulatory treatment for all MIS-C patients, regardless of presentation. Every respondent reported use of intravenous immunoglobulin (IVIG), including 53% administering IVIG in all patients. Steroids were most often used for patients with severe clinical presentation or lack of response to IVIG, and only a minority used steroids in all patients (14%). Acetylsalicylic acid was frequently used among respondents (91%), including anti-inflammatory and/or antiplatelet dosing. Respondents reported use of prophylactic anticoagulation, especially in patients at higher risk for venous thromboembolism, and therapeutic anticoagulation, particularly for patients with giant coronary artery aneurysms., Conclusions: There is variation in management of MIS-C patients, with suboptimal evidence to assess superiority of the various treatments; evidence-based gaps in knowledge should be addressed through worldwide collaboration to optimize treatment strategies., (© 2020 Canadian Cardiovascular Society. Published by Elsevier Inc.)
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- 2020
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22. Echocardiographic Findings in Pediatric Multisystem Inflammatory Syndrome Associated With COVID-19 in the United States.
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Matsubara D, Kauffman HL, Wang Y, Calderon-Anyosa R, Nadaraj S, Elias MD, White TJ, Torowicz DL, Yubbu P, Giglia TM, Hogarty AN, Rossano JW, Quartermain MD, and Banerjee A
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- Adolescent, Betacoronavirus, COVID-19, Child, Coronavirus Infections diagnostic imaging, Coronavirus Infections physiopathology, Female, Humans, Male, Mucocutaneous Lymph Node Syndrome diagnostic imaging, Mucocutaneous Lymph Node Syndrome physiopathology, Pandemics, Pneumonia, Viral diagnostic imaging, Pneumonia, Viral physiopathology, Retrospective Studies, SARS-CoV-2, Systemic Inflammatory Response Syndrome physiopathology, Coronavirus Infections complications, Echocardiography, Heart physiopathology, Pneumonia, Viral complications, Systemic Inflammatory Response Syndrome diagnostic imaging
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Background: Centers from Europe and United States have reported an exceedingly high number of children with a severe inflammatory syndrome in the setting of coronavirus disease 2019, which has been termed multisystem inflammatory syndrome in children (MIS-C)., Objectives: This study aimed to analyze echocardiographic manifestations in MIS-C., Methods: A total of 28 MIS-C, 20 healthy control subjects and 20 classic Kawasaki disease (KD) patients were retrospectively reviewed. The study reviewed echocardiographic parameters in the acute phase of the MIS-C and KD groups, and during the subacute period in the MIS-C group (interval 5.2 ± 3 days)., Results: Only 1 case in the MIS-C group (4%) manifested coronary artery dilatation (z score = 3.15) in the acute phase, showing resolution during early follow-up. Left ventricular (LV) systolic and diastolic function measured by deformation parameters were worse in patients with MIS-C compared with KD. Moreover, MIS-C patients with myocardial injury were more affected than those without myocardial injury with respect to all functional parameters. The strongest parameters to predict myocardial injury in MIS-C were global longitudinal strain, global circumferential strain, peak left atrial strain, and peak longitudinal strain of right ventricular free wall (odds ratios: 1.45 [95% confidence interval (CI): 1.08 to 1.95], 1.39 [95% CI: 1.04 to 1.88], 0.84 [95% CI: 0.73 to 0.96], and 1.59 [95% CI: 1.09 to 2.34], respectively). The preserved LV ejection fraction (EF) group in MIS-C showed diastolic dysfunction. During the subacute period, LVEF returned to normal (median from 54% to 64%; p < 0.001) but diastolic dysfunction persisted., Conclusions: Unlike classic KD, coronary arteries may be spared in early MIS-C; however, myocardial injury is common. Even preserved EF patients showed subtle changes in myocardial deformation, suggesting subclinical myocardial injury. During an abbreviated follow-up, there was good recovery of systolic function but persistence of diastolic dysfunction and no coronary aneurysms., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)
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- 2020
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23. Variation in the use of pulmonary vasodilators in children and adolescents with pulmonary hypertension: a study using data from the MarketScan® insurance claims database.
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O'Byrne ML, Faerber JA, Katcoff H, Frank DB, Davidson A, Giglia TM, and Avitabile CM
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Despite progress in pharmacotherapy in pediatric pulmonary hypertension, real-world patterns of directed pulmonary hypertension therapy have not been studied in the current era. A retrospective observational study of children (≤18 years) with pulmonary hypertension was performed using data from the MarketScan® Commercial and Medicaid claims databases. Associations between etiology of pulmonary hypertension and pharmaceutical regimen were evaluated, as were the associations between subject social and geographic characteristics (insurance-type, race, and/or census region) and regimen. Annualized costs of single- and multi-class regimens were calculated. In total, 873 subjects were studied, of which 94% received phosphodiesterase-5 inhibitors, 31% endothelin receptor antagonist, 9% prostacyclin analogs, and 7% calcium channel blockers. Monotherapy was used in 72% of subjects. Phosphodiesterase-5 inhibitors monotherapy was the most common regimen (93%). Subjects with idiopathic pulmonary hypertension, congenital heart disease, and unclassified pulmonary hypertension receive more than one agent and were more likely to receive both endothelin receptor antagonist and prostacyclin analogs than other forms of pulmonary hypertension. Compared to recipients of public insurance, subjects with commercial insurance were more likely to receive more intense therapy ( p = 0.003), which was confirmed in multivariable analysis (OR: 1.4, p = 0.03). Receipt of commercial insurance was also associated with increased annual costs across all subjects ( p < 0.001) and for the most common specific regimens. The majority of children with pulmonary hypertension receive phosphodiesterase monotherapy, followed by phosphodiesterase-endothelin receptor antagonist two drug regimens, and finally the addition of prostacyclin analogs for three-drug therapy. However, even after adjustment for measurable confounders, commercial insurance was associated with higher intensity care and higher costs (even within specific classes of pulmonary vasodilators). The effect of these associations on clinical outcome cannot be discerned from the current data set, but patterns of treatment deserve further attention., (© The Author(s) 2020.)
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- 2020
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24. Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience.
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Kaplinski M, Ittenbach RF, Hunt ML, Stephan D, Natarajan SS, Ravishankar C, Giglia TM, Rychik J, Rome JJ, Mahle M, Kennedy AT, Steven JM, Fuller SM, Nicolson SC, Spray TL, Gaynor JW, and Mascio CE
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- Age Factors, Birth Weight, Child, Child, Preschool, Female, Gestational Age, Humans, Hypoplastic Left Heart Syndrome mortality, Infant, Length of Stay, Logistic Models, Male, Retrospective Studies, Risk Factors, Hypoplastic Left Heart Syndrome surgery, Norwood Procedures mortality
- Abstract
Background The superior cavo-pulmonary connection was introduced at our institution in 1988 for infants undergoing surgery for hypoplastic left heart syndrome. Patients with hypoplastic left heart syndrome remain at high risk for mortality in the time period between the Norwood procedure and the superior cavo-pulmonary connection. The primary objectives of this study were to compare interstage mortality across 4 eras and analyze factors that may impact interstage mortality. Methods and Results Patients with hypoplastic left heart syndrome who underwent the Norwood procedure, were discharged from the hospital, and were eligible for superior cavo-pulmonary connection between January 1, 1988, and December 31, 2017, were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated with 95% CIs. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality. There were 1111 patients who met the inclusion criteria. Overall, interstage mortality was 120/1111 (10.8%). Interstage mortality was significantly lower in era 4 relative to era 1 (4.6% versus 13.4%; P =0.02) during the time that age at the superior cavo-pulmonary connection was the lowest (135 days; P <0.01) and the interstage monitoring program was introduced. In addition, use of the right ventricle to pulmonary artery shunt was associated with decreased interstage mortality ( P =0.02) and was more routinely practiced in era 4. Conclusions During this 30-year experience, the risk of interstage mortality decreased significantly in the most recent era. Factors that coincide with this finding include younger age at superior cavo-pulmonary connection, introduction of an interstage monitoring program, and increased use of the right ventricle to pulmonary artery shunt.
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- 2020
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25. Low-Molecular-Weight Heparin vs Warfarin for Thromboprophylaxis in Children With Coronary Artery Aneurysms After Kawasaki Disease: A Pragmatic Registry Trial.
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Manlhiot C, Newburger JW, Low T, Dahdah N, Mackie AS, Raghuveer G, Giglia TM, Dallaire F, Mathew M, Runeckles K, Pahl E, Harahsheh AS, Norozi K, de Ferranti SD, Friedman K, Yetman AT, Kutty S, Mondal T, and McCrindle BW
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- Anticoagulants administration & dosage, Anticoagulants adverse effects, Canada epidemiology, Child, Preschool, Female, Hemorrhage chemically induced, Hemorrhage epidemiology, Humans, Incidence, Male, Registries statistics & numerical data, Risk Adjustment, United States epidemiology, Chemoprevention methods, Chemoprevention statistics & numerical data, Coronary Aneurysm complications, Coronary Aneurysm drug therapy, Heparin, Low-Molecular-Weight administration & dosage, Heparin, Low-Molecular-Weight adverse effects, Mucocutaneous Lymph Node Syndrome complications, Mucocutaneous Lymph Node Syndrome epidemiology, Thrombosis epidemiology, Thrombosis etiology, Thrombosis prevention & control, Warfarin administration & dosage, Warfarin adverse effects
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Background: The substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-weight heparin [LMWH] or warfarin) for thromboprophylaxis in large CAAs., Methods: Data from 383 patients enrolled in the International KD Registry (IKDR) were used. Time-to-event analysis was used to account for differences in treatment duration and follow-up., Results: From diagnosis onward (96% received acetylsalicylic acid concomitantly), 114 patients received LMWH (median duration 6.2 months, interquartile range [IQR] 2.5-12.7), 80 warfarin (median duration 2.2 years, IQR 0.9-7.1), and 189 no anticoagulation. Cumulative incidence of coronary artery thrombosis with LMWH was 5.7 ± 3.0%, with warfarin 6.7 ± 3.7%, and with no anticoagulation 20.6 ± 3.0% (P < 0.001) at 2.5 years after the start of thromboprophylaxis (LMWH vs warfarin HR 1.5, 95% confidence interval [CI] 0.4-5.1; P = 0.56). A total of 51/63 patients with coronary artery thrombosis received secondary thromboprophylaxis (ie, thromboprophylaxis after a previous thrombus): 27 LMWH, 24 warfarin. There were no differences in incidence of further coronary artery thrombosis between strategies (HR 2.9, 95% CI 0.6-13.5; P = 0.19). Severe bleeding complications were generally rare (1.6 events per 100 patient-years) and were noted equally for patients on LMWH and warfarin (HR 2.3, 95% CI 0.6-8.9; P = 0.25)., Conclusions: LMWH and warfarin appear to have equivalent effectiveness for preventing thrombosis in large CAAs after KD, although event rates for secondary thromboprophylaxis and safety outcomes were low. Based on our findings, all patients with CAA z-score ≥ 10 should receive anticoagulation, but the choice of agent might be informed by secondary risk factors and patient preferences., (Copyright © 2020 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)
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- 2020
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26. Standardization of the Perioperative Management for Neonates Undergoing the Norwood Operation for Hypoplastic Left Heart Syndrome and Related Heart Defects.
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Natarajan SS, Stagg A, Taylor AM, Griffis HM, Bosler CK, Cates M, Dewitt AG, Giglia TM, Mascio CE, Ravishankar C, Rossano JW, Taylor LC, Ware EP, Nicolson SC, and Rome JJ
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- Child, Humans, Infant, Newborn, Reference Standards, Risk Factors, Treatment Outcome, Heart Defects, Congenital, Hypoplastic Left Heart Syndrome surgery, Norwood Procedures adverse effects
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Objectives: In-hospital complications after the Norwood operation for single ventricle heart defects account for the majority of morbidity and mortality. Inpatient care variation occurs within and across centers. This multidisciplinary quality improvement project standardized perioperative management in a large referral center., Design: Quality improvement project., Setting: High volume cardiac center, tertiary care children's hospital., Patients: Neonates undergoing Norwood operation., Interventions: The quality improvement team developed and implemented a clinical guideline (preoperative admission to 48 hr after surgery). The composite process metric, Guideline Adherence Score, contained 13 recommendations in the guideline that reflected consistent care for all patients., Measurements and Main Results: One-hundred two consecutive neonates who underwent Norwood operation (January 1, 2013, to July 12, 2016) before guideline implementation were compared with 50 consecutive neonates after guideline implementation (July 13, 2016, to May 4, 2018). No preguideline operations met the goal Guideline Adherence Score. In the first 6 months after guideline implementation, 10 of 12 operations achieved goal Guideline Adherence Score and continued through implementation, reaching 100% for the last 10 operations. Statistical process control analysis demonstrated less variability and decreased hours of postoperative mechanical ventilation and cardiac ICU length of stay during implementation. There were no statistically significant differences in major hospital complications or in 30-day mortality. A higher percentage of patients were extubated by postoperative day 2 after guideline implementation (67% [30/47] vs 41% [41/99], respectively; p = 0.01). Of these patients, reintubation within 72 hours of extubation significantly decreased after guideline implementation (0% [0/30] vs 17% [7/41] patients, respectively; p = 0.02)., Conclusions: This initiative successfully implemented a standardized perioperative care guideline for neonates undergoing the Norwood operation at a large center. Positive statistical process control centerline shifts in Guideline Adherence Score, length of postoperative mechanical ventilation, and cardiac ICU length of stay were demonstrated. A higher percentage were successfully extubated by postoperative day 2. Establishment of standard processes can lead to best practices to decrease major adverse events.
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- 2020
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27. Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry.
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McCrindle BW, Manlhiot C, Newburger JW, Harahsheh AS, Giglia TM, Dallaire F, Friedman K, Low T, Runeckles K, Mathew M, Mackie AS, Choueiter NF, Jone PN, Kutty S, Yetman AT, Raghuveer G, Pahl E, Norozi K, McHugh KE, Li JS, De Ferranti SD, and Dahdah N
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- Child, Child, Preschool, Coronary Aneurysm pathology, Coronary Vessels pathology, Female, Humans, Infant, Male, Retrospective Studies, Risk Assessment, Coronary Aneurysm complications, Mucocutaneous Lymph Node Syndrome complications, Registries
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Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA Z score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA Z scores ≥10, the cumulative incidence of luminal narrowing (>50% of lumen diameter), coronary artery thrombosis, and composite major adverse cardiovascular complications at 10 years was 20±3%, 18±2%, and 14±2%, respectively. No complications were observed in patients with a CAA Z score <10. Higher CAA Z score and a greater number of coronary artery branches affected were associated with increased risk of all types of complications. At 10 years, normalization of luminal diameter was noted in 99±4% of patients with small (2.5≤ Z <5.0), 92±1% with medium (5.0≤ Z <10), and 57±3% with large CAAs ( Z ≥10). CAAs in the left anterior descending and circumflex coronary artery branches were more likely to normalize. Risk factor analysis of coronary artery branch level outcomes was performed with a total of 893 affected branches with Z score ≥10 in 440 patients. In multivariable regression models, hazards of luminal narrowing and thrombosis were higher for patients with CAAs of the right coronary artery and left anterior descending branches, those with CAAs that had complex architecture (other than isolated aneurysms), and those with CAAs with Z scores ≥20. Conclusions For patients with CAA after KD, medium-term risk of complications is confined to those with maximum CAA Z scores ≥10. Further risk stratification and close follow-up, including advanced imaging, in patients with large CAAs is warranted.
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- 2020
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28. Bleeding and Thrombosis With Pediatric Extracorporeal Life Support: A Roadmap for Management, Research, and the Future From the Pediatric Cardiac Intensive Care Society: Part 2.
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Penk JS, Reddy S, Polito A, Cisco MJ, Allan CK, Bembea M, Giglia TM, Cheng HH, Thiagarajan RR, and Dalton HJ
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- Anticoagulants administration & dosage, Anticoagulants adverse effects, Anticoagulants pharmacology, Blood Coagulation drug effects, Child, Extracorporeal Membrane Oxygenation methods, Extracorporeal Membrane Oxygenation trends, Factor Xa Inhibitors administration & dosage, Factor Xa Inhibitors adverse effects, Factor Xa Inhibitors pharmacology, Hemorrhage prevention & control, Humans, Thrombosis prevention & control, von Willebrand Factor administration & dosage, von Willebrand Factor adverse effects, von Willebrand Factor pharmacology, Extracorporeal Membrane Oxygenation adverse effects
- Abstract
Objectives: To make recommendations on improving understanding of bleeding and thrombosis with pediatric extracorporeal life support including future research directions., Data Sources: Evaluation of literature and consensus conferences of pediatric critical care and extracorporeal life support experts., Study Selection: A team of 10 experts with pediatric cardiac and extracorporeal membrane oxygenation experience and expertise met through the Pediatric Cardiac Intensive Care Society to review current knowledge and make recommendations for future research to establish "best practice" for anticoagulation management related to extracorporeal life support., Data Extraction/data Synthesis: This white paper focuses on clinical understanding and limitations of current strategies to monitor anticoagulation. For each test of anticoagulation, limitations of current knowledge are addressed and future research directions suggested., Conclusions: No consensus on best practice for anticoagulation monitoring exists. Structured scientific evaluation to answer questions regarding anticoagulation monitoring and bleeding and thrombotic events should occur in multicenter studies using standardized approaches and well-defined endpoints. Outcomes related to need for component change, blood product administration, healthcare outcome, and economic assessment should be incorporated into studies. All centers should report data on patient receiving extracorporeal life support to a registry.
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- 2019
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29. Bleeding and Thrombosis With Pediatric Extracorporeal Life Support: A Roadmap for Management, Research, and the Future From the Pediatric Cardiac Intensive Care Society: Part 1.
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Penk JS, Reddy S, Polito A, Cisco MJ, Allan CK, Bembea MM, Giglia TM, Cheng HH, Thiagarajan RR, and Dalton HJ
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- Anticoagulants administration & dosage, Anticoagulants adverse effects, Anticoagulants pharmacology, Antithrombins administration & dosage, Antithrombins adverse effects, Antithrombins pharmacology, Blood Coagulation drug effects, Child, Critical Illness therapy, Extracorporeal Membrane Oxygenation methods, Extracorporeal Membrane Oxygenation trends, Humans, Platelet Aggregation Inhibitors administration & dosage, Platelet Aggregation Inhibitors adverse effects, Platelet Aggregation Inhibitors pharmacology, Extracorporeal Membrane Oxygenation adverse effects, Hemorrhage prevention & control, Thrombosis prevention & control
- Abstract
Objectives: To make practical and evidence-based recommendations on improving understanding of bleeding and thrombosis with pediatric extracorporeal life support and to make recommendations for research directions., Data Sources: Evaluation of literature and consensus conferences of pediatric critical care and extracorporeal life support experts., Study Selection: A team of 10 experts with pediatric cardiac and extracorporeal membrane oxygenation experience and expertise met through the Pediatric Cardiac Intensive Care Society to review current knowledge and make recommendations for future research to establish "best practice" for anticoagulation management related to extracorporeal life support., Data Extraction/synthesis: The first of a two-part white article focuses on clinical understanding and limitations of medications in use for anticoagulation, including novel medications. For each medication, limitations of current knowledge are addressed and research recommendations are suggested to allow for more definitive clinical guidelines in the future., Conclusions: No consensus on best practice for anticoagulation exists. Structured scientific evaluation to answer questions regarding anticoagulant medication and bleeding and thrombotic events should occur in multicenter studies using standardized approaches and well-defined endpoints. Outcomes related to need for component change, blood product administration, healthcare outcome, and economic assessment should be incorporated into studies. All centers should report data on patients receiving extracorporeal life support to a registry. The Extracorporeal Life Support Organization registry, designed primarily for quality improvement purposes, remains the primary and most successful data repository to date.
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- 2019
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30. Association of a Home Monitoring Program With Interstage and Stage 2 Outcomes.
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Gardner MM, Mercer-Rosa L, Faerber J, DiLorenzo MP, Bates KE, Stagg A, Natarajan SS, Szwast A, Fuller S, Mascio CE, Fleck D, Torowicz DL, Giglia TM, Rome JJ, and Ravishankar C
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- Biomarkers blood, Body Weight, Female, Heart Ventricles abnormalities, Heart Ventricles physiopathology, Humans, Hypoplastic Left Heart Syndrome diagnosis, Hypoplastic Left Heart Syndrome mortality, Hypoplastic Left Heart Syndrome physiopathology, Infant, Infant, Newborn, Male, Oxygen blood, Patient Readmission, Recovery of Function, Retrospective Studies, Risk Assessment, Risk Factors, Time Factors, Treatment Outcome, Anthropometry, Cardiac Surgical Procedures adverse effects, Cardiac Surgical Procedures mortality, Heart Ventricles surgery, Home Care Services, Hypoplastic Left Heart Syndrome surgery, Oximetry, Palliative Care, Predictive Value of Tests, Ventricular Function
- Abstract
Background In shunt-dependent, single-ventricle patients, mortality remains high in the interstage period between discharge after neonatal surgery and stage 2 operation. We sought to evaluate the impact of our infant single-ventricle management and monitoring program ( ISVMP ) on interstage mortality and stage 2 outcomes. Methods and Results This retrospective single-center cohort study compared patients enrolled in ISVMP at hospital discharge with historical controls. The relationship of ISVMP to interstage mortality was determined with a bivariate probit model for the joint modeling of both groups, using an instrumental variables approach. We included 166 ISVMP participants (December 1, 2010, to June 30, 2015) and 168 controls (January 1, 2007, to November 30, 2010). The groups did not differ by anatomy, gender, race, or genetic syndrome. Mortality was lower in the ISVMP group (5.4%) versus controls (13%). An ISVMP infant compared with a historical control had an average 29% lower predicted probability of interstage death (adjusted probability: -0.29; 95% CI , -0.52 to -0.057; P=0.015). On stratified analysis, mortality was lower in the hypoplastic left heart syndrome subgroup undergoing Norwood operation (4/84 [4.8%] versus 12/90 [14%], P=0.03) but not in those with initial palliation of shunt only ( P=0.90). ISVMP participants were younger at the time of the stage 2 operation (138 versus 160 days, P<0.001), with no difference in postoperative mortality or length of stay. Conclusions In this single-center study, we report significantly lower interstage mortality for participants with hypoplastic left heart syndrome enrolled in ISVMP . Younger age at stage 2 operation was not associated with postoperative mortality or longer length of stay.
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- 2019
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31. Evaluating the utility of routine screening catheterisation before interstage discharge of infants with single-ventricle physiology.
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Bates KE, Glatz AC, Giglia TM, Natarajan SS, Ravishankar C, Stagg A, and Rome JJ
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- Echocardiography, Female, Heart Ventricles abnormalities, Heart Ventricles surgery, Humans, Infant, Infant, Newborn, Male, Monitoring, Ambulatory methods, Palliative Care methods, Philadelphia, Postoperative Complications etiology, ROC Curve, Retrospective Studies, Risk Factors, Treatment Outcome, Catheterization adverse effects, Hypoplastic Left Heart Syndrome mortality, Hypoplastic Left Heart Syndrome surgery, Norwood Procedures, Patient Discharge
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Introduction: Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes., Methods: Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves., Results: There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64., Conclusion: Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.
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- 2019
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32. Pseudoaneurysm of the mitral-aortic intervalvular fibrosa in a healthy child.
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Bhatt SM, Giglia TM, Gleason MM, and Banerjee A
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- Aneurysm, False congenital, Child, Preschool, Diagnosis, Differential, Echocardiography, Electrocardiography, Female, Heart Aneurysm congenital, Humans, Aneurysm, False diagnosis, Aortic Valve diagnostic imaging, Heart Aneurysm diagnosis, Heart Atria diagnostic imaging, Mitral Valve diagnostic imaging
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- 2017
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33. Bleeding and Thrombosis in Pediatric Cardiac Intensive Care.
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Giglia TM and Witmer C
- Subjects
- Child, Coronary Care Units, Hemostasis physiology, Humans, Intensive Care Units, Pediatric, Risk Factors, Hemorrhage therapy, Thrombosis therapy
- Abstract
Objectives: The objective of this article is to review the particular tendencies as well as specific concerns of bleeding and clotting in children with critical cardiac disease., Data Source: MEDLINE and PubMed., Conclusion: Children with critical heart disease are at particular risk for bleeding and clotting secondary to intrinsic as well as extrinsic factors. We hope that this review will aid the clinician in managing the unique challenges of bleeding and clotting in this patient population, and serve as a springboard for much needed research in this area.
- Published
- 2016
- Full Text
- View/download PDF
34. Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care Anticoagulation and Thrombolysis.
- Author
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Giglia TM, Witmer C, Procaccini DE, and Byrnes JW
- Subjects
- Child, Coronary Care Units, Fibrinolytic Agents therapeutic use, Heart Failure drug therapy, Humans, Intensive Care Units, Pediatric, Platelet Aggregation Inhibitors therapeutic use, Anticoagulants therapeutic use, Critical Care standards, Heart Defects, Congenital drug therapy
- Abstract
Objective: Thrombotic complications are increasingly being recognized as a significant cause of morbidity and mortality in pediatric and congenital heart disease. The objective of this article is to review the medications currently available to prevent and treat such complications., Data Sources: Online searches were conducted using PubMed., Study Selection: Studies were selected for inclusion based on their scientific merit and applicability to the pediatric cardiac population., Data Extraction: Pertinent information from each selected study or scientific review was extracted for inclusion., Data Synthesis: Four classes of medications were identified as potentially beneficial in this patient group: anticoagulants, antiplatelet agents, thrombolytic agents, and novel oral anticoagulants. Data on each class of medication were synthesized into the follow sections: mechanism of action, pharmacokinetics, dosing, monitoring, reversal, considerations for use, and evidence to support., Conclusions: Anticoagulants, antiplatelet agents, and thrombolytic agents are routinely used successfully in the pediatric patient with heart disease for the prevention and treatment of a wide range of thrombotic complications. Although the novel oral anticoagulants have been approved for a limited number of indications in adults, studies on the safety and efficacy of these agents in children are pending.
- Published
- 2016
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35. Outcomes using a clinical practice pathway for the management of pulse loss following pediatric cardiac catheterization.
- Author
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Glatz AC, Keashen R, Chang J, Balsama LA, Dori Y, Gillespie MJ, Giglia TM, Raffini L, and Rome JJ
- Subjects
- Age Factors, Anticoagulants adverse effects, Arterial Occlusive Diseases diagnostic imaging, Arterial Occlusive Diseases etiology, Arterial Occlusive Diseases mortality, Arterial Occlusive Diseases physiopathology, Cardiac Catheterization mortality, Child, Child, Preschool, Drug Administration Schedule, Fibrinolytic Agents adverse effects, Hemorrhage chemically induced, Hospitals, Pediatric, Humans, Infant, Infant, Newborn, Philadelphia, Predictive Value of Tests, Retrospective Studies, Risk Factors, Thrombosis diagnostic imaging, Thrombosis etiology, Thrombosis mortality, Thrombosis physiopathology, Time Factors, Treatment Outcome, Ultrasonography, Anticoagulants administration & dosage, Arterial Occlusive Diseases drug therapy, Cardiac Catheterization adverse effects, Critical Pathways, Fibrinolytic Agents administration & dosage, Outcome Assessment, Health Care, Pulse, Thrombosis drug therapy
- Abstract
Objective: Objectives To describe the results of a clinical practice pathway (CPP) for the management of postcatheterization pulse loss in a children's hospital., Background: Standardized approaches to the diagnosis and management of postcatheterization arterial thrombus are lacking. As a result, substantial practice variation exists., Methods: Data collected prospectively for quality improvement purposes were retrospectively reviewed., Results: Since initiation of the CPP, 93/1,672 (5.4%) catheterizations resulted in pulse loss at a median patient age and weight of 73 days (1 day-5.8 years) and 4.8 kg (2-14.1 kg). Arterial thrombus was documented by ultrasound (US) in 85. Of these, 66 resolved by 12 weeks of therapy, seven patients died, and four were lost to follow-up before completing treatment. Eight patients had persistent thrombus despite a full treatment course (89% success rate in those able to complete treatment). Of patients treated with unfractionated heparin as initial therapy, 46% (17/37) achieved a therapeutic partial thromboplastin time within 12 hr with 19% (67/343) of all levels therapeutic. As a result, the CPP was modified to use enoxaparin as first line agent, of which 57% (41/72) had a therapeutic anti-Xa level after the 2nd dose and 88% by the 4th dose. No bleeding complications were observed. A priori established process metrics were achieved., Conclusions: A CPP utilizing early initiation of anticoagulation and US to aid diagnosis of postcatheterization arterial thrombus and response to therapy is feasible and effective. In those able to complete up to 12 weeks of treatment, resolution occurs in nearly 90%. © 2014 Wiley Periodicals, Inc., (© 2014 Wiley Periodicals, Inc.)
- Published
- 2015
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36. Stroke in children with cardiac disease: report from the International Pediatric Stroke Study Group Symposium.
- Author
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Sinclair AJ, Fox CK, Ichord RN, Almond CS, Bernard TJ, Beslow LA, Chan AK, Cheung M, deVeber G, Dowling MM, Friedman N, Giglia TM, Guilliams KP, Humpl T, Licht DJ, Mackay MT, and Jordan LC
- Subjects
- Child, Heart Diseases epidemiology, Heart Diseases therapy, Humans, Stroke diagnosis, Stroke epidemiology, Stroke therapy, Heart Diseases physiopathology, Stroke physiopathology
- Abstract
Background: Cardiac disease is a leading cause of stroke in children, yet limited data support the current stroke prevention and treatment recommendations. A multidisciplinary panel of clinicians was convened in February 2014 by the International Pediatric Stroke Study group to identify knowledge gaps and prioritize clinical research efforts for children with cardiac disease and stroke., Results: Significant knowledge gaps exist, including a lack of data on stroke incidence, predictors, primary and secondary stroke prevention, hyperacute treatment, and outcome in children with cardiac disease. Commonly used diagnostic techniques including brain computed tomography and ultrasound have low rates of stroke detection, and diagnosis is frequently delayed. The challenges of research studies in this population include epidemiologic barriers to research such as small patient numbers, heterogeneity of cardiac disease, and coexistence of multiple risk factors. Based on stroke burden and study feasibility, studies involving mechanical circulatory support, single ventricle patients, early stroke detection strategies, and understanding secondary stroke risk factors and prevention are the highest research priorities over the next 5-10 years. The development of large-scale multicenter and multispecialty collaborative research is a critical next step. The designation of centers of expertise will assist in clinical care and research., Conclusions: There is an urgent need for additional research to improve the quality of evidence in guideline recommendations for cardiogenic stroke in children. Although significant barriers to clinical research exist, multicenter and multispecialty collaboration is an important step toward advancing clinical care and research for children with cardiac disease and stroke., (Copyright © 2015 Elsevier Inc. All rights reserved.)
- Published
- 2015
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37. Challenges and priorities for research: a report from the National Heart, Lung, and Blood Institute (NHLBI)/National Institutes of Health (NIH) Working Group on thrombosis in pediatric cardiology and congenital heart disease.
- Author
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McCrindle BW, Li JS, Manlhiot C, Tweddell JS, Giglia TM, Massicotte MP, Monagle P, Krishnamurthy R, Mahaffey KW, Michelson AD, Verdun N, Almond CS, Newburger JW, Brandão LR, Esmon CT, Manco-Johnson MJ, Ichord R, Ortel TL, Chan AK, Portman R, Rose M, Strony J, and Kaltman JR
- Subjects
- Cardiology, Child, Heart Defects, Congenital diagnosis, Heart Defects, Congenital surgery, Heart Diseases diagnosis, Heart Diseases surgery, Humans, Pediatrics, Thrombosis diagnosis, Thrombosis surgery, United States, Heart Defects, Congenital therapy, Heart Diseases therapy, National Heart, Lung, and Blood Institute (U.S.), Research, Thrombosis therapy
- Published
- 2014
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38. Prevention and treatment of thrombosis in pediatric and congenital heart disease: a scientific statement from the American Heart Association.
- Author
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Giglia TM, Massicotte MP, Tweddell JS, Barst RJ, Bauman M, Erickson CC, Feltes TF, Foster E, Hinoki K, Ichord RN, Kreutzer J, McCrindle BW, Newburger JW, Tabbutt S, Todd JL, and Webb CL
- Subjects
- Adolescent, American Heart Association, Anticoagulants therapeutic use, Child, Child, Preschool, Humans, Infant, Thrombectomy, Thrombolytic Therapy, United States, Coronary Thrombosis prevention & control, Coronary Thrombosis therapy, Heart Diseases complications, Heart Diseases congenital
- Published
- 2013
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39. Bleeding and thrombotic emergencies in pediatric cardiac intensive care: unchecked balances.
- Author
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Giglia TM, Dinardo J, Ghanayem NS, Ichord R, Niebler RA, Odegard KC, Massicotte MP, Yates AR, Laussen PC, and Tweddell JS
- Abstract
Children in the cardiac intensive care unit (CICU) with congenital or acquired heart disease are at risk for hematologic complications, both hemorrhage and thrombosis. The overall incidence of hematologic complications in the CICU is unknown, but risk factors and target groups have been identified where the essential physiologic balance between bleeding and clotting has been disrupted. Although the best management of life-threatening bleeding and clotting is prevention, the cardiac intensivist is often faced with managing life-threatening hematologic events involving patients from within the unit or those who present from outside. Part I of this review deals with the propensity of children with congenital and acquired heart disease to complications of both bleeding and clotting, and includes discussions of perioperative bleeding, thromboses in single-ventricle patients, clotting of Blalock-Taussig shunts and thrombotic complications of mechanical valves. Part II deals with the subject of stroke in children with heart disease. Part III reviews monitoring the effectiveness of anticoagulation and thrombolysis in the CICU. Currently available diagnostics modalities, medications and management strategies are reviewed and future directions discussed.
- Published
- 2012
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40. Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics.
- Author
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Pierpont ME, Basson CT, Benson DW Jr, Gelb BD, Giglia TM, Goldmuntz E, McGee G, Sable CA, Srivastava D, and Webb CL
- Subjects
- Chromosome Aberrations, Cytogenetic Analysis methods, DNA Mutational Analysis, Humans, Mutation, Heart Defects, Congenital diagnosis, Heart Defects, Congenital genetics
- Abstract
The intent of this review is to provide the clinician with a summary of what is currently known about the contribution of genetics to the origin of congenital heart disease. Techniques are discussed to evaluate children with heart disease for genetic alterations. Many of these techniques are now available on a clinical basis. Information on the genetic and clinical evaluation of children with cardiac disease is presented, and several tables have been constructed to aid the clinician in the assessment of children with different types of heart disease. Genetic algorithms for cardiac defects have been constructed and are available in an appendix. It is anticipated that this summary will update a wide range of medical personnel, including pediatric cardiologists and pediatricians, adult cardiologists, internists, obstetricians, nurses, and thoracic surgeons, about the genetic aspects of congenital heart disease and will encourage an interdisciplinary approach to the child and adult with congenital heart disease.
- Published
- 2007
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41. ACC/AHA/AAP recommendations for training in pediatric cardiology.
- Author
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Allen HD, Bricker JT, Freed MD, Hurwitz RA, McQuinn TC, Schieken RM, Strong WB, Zahka KG, Sanders SP, Colan SD, Cordes TM, Donofrio MT, Ensing GJ, Geva T, Kimball TR, Sahn DJ, Silverman NH, Sklansky MS, Weinberg PM, Beekman RH 3rd, Hellenbrand WE, Lloyd TR, Lock JE, Mullins CE, Rome JJ, Teitel DF, Vetter VL, Silka MJ, Van Hare GF, Walsh EP, Kulik T, Giglia TM, Kocis KC, Mahoney LT, Schwartz SM, Wernovsky G, Wessel DL, Murphy DJ Jr, Foster E, Benson DW Jr, Baldwin HS, Mahoney LT, and McQuinn TC
- Subjects
- Cardiology education, Education, Medical standards, Fellowships and Scholarships standards, Pediatrics education
- Published
- 2005
- Full Text
- View/download PDF
42. ACCF/AHA/AAP recommendations for training in pediatric cardiology. A report of the American College of Cardiology Foundation/American Heart Association/American College of Physicians Task Force on Clinical Competence (ACC/AHA/AAP Writing Committee to Develop Training Recommendations for Pediatric Cardiology).
- Author
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Graham TP Jr, Beekman RH 3rd, Allen HD, Bricker JT, Freed MD, Hurwitz RA, McQuinn TC, Schieken RM, Strong WB, Zahka KG, Sanders SP, Colan SD, Cordes TM, Donofrio MT, Ensing GJ, Geva T, Kimball TR, Sahn DJ, Silverman NH, Sklansky MS, Weinberg PM, Hellenbrand WE, Lloyd TR, Lock JE, Mullins CE, Romes JJ, Teitel DF, Vetter VL, Silka MJ, Van Hare GF, Walsh EP, Kulik T, Giglia TM, Kocis KC, Mahoney LT, Schwartz SM, Wernovsky G, Wessel DL, Murphy D Jr, Foster E, Benson DW Jr, Baldwin HS, Hirshfeld JW Jr, Kugler JD, Moskowitz WB, Creager MA, Lorell BH, Merli G, Rodgers GP, Rutherford JD, Tracy CM, and Weitz HH
- Subjects
- Child, Humans, United States, American Hospital Association, Cardiology standards, Pediatrics standards, Societies, Medical
- Published
- 2005
- Full Text
- View/download PDF
43. ACCF/AHA/AAP recommendations for training in pediatric cardiology. Task force 5: requirements for pediatric cardiac critical care.
- Author
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Kulik T, Giglia TM, Kocis KC, Mahoney LT, Schwartz SM, Wernovsky G, and Wessel DL
- Subjects
- United States, Cardiology education, Critical Care standards, Pediatrics education
- Published
- 2005
- Full Text
- View/download PDF
44. Case report: pulmonary vein stenosis following RF ablation of paroxysmal atrial fibrillation: successful treatment with balloon dilation.
- Author
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Moak JP, Moore HJ, Lee SW, Giglia TM, Sable CA, Furbush NC, and Ringel RR
- Subjects
- Adolescent, Angiography, Atrial Fibrillation diagnosis, Catheter Ablation methods, Electrocardiography methods, Follow-Up Studies, Humans, Male, Pulmonary Veno-Occlusive Disease diagnostic imaging, Pulmonary Veno-Occlusive Disease therapy, Risk Assessment, Tachycardia, Paroxysmal diagnosis, Treatment Outcome, Ultrasonography, Interventional, Atrial Fibrillation surgery, Catheter Ablation adverse effects, Catheterization methods, Pulmonary Veno-Occlusive Disease etiology, Tachycardia, Paroxysmal surgery
- Abstract
Paroxysmal atrial fibrillation and atrial tachycardia may originate from a focal source in one or multiple pulmonary veins. A focal origin facilitates a potential cure amendable to radiofrequency ablation. Herein we report the case of a 16 year old adolescent male with a tachycardia induced cardiomyopathy who presented with very frequent paroxysmal episodes of atrial fibrillation, atrial flutter and atrial tachycardia. The origin of the arrhythmia was mapped to the secondary branches of the left lower pulmonary vein using an octapolar micro-mapping catheter. Immediately following application of three radiofrequency lesions, angiography of the left lower pulmonary vein revealed a region of focal stenosis at the site of energy application, with delayed pulmonary venous emptying. Attempts to relieve any element of spasm using direct administration of nitroglycerin were unsuccessful. Three months later repeat catheterization revealed an unchanged region of tight anatomical stenosis. Balloon dilation of two stenotic areas resulted in dramatic relief of the obstruction and improved venous drainage. Recatheterization 6 months later revealed mild restenosis that was successfully redilated. Intracardiac ultrasound demonstrated focal constriction. Care should be exercised in attempting RF ablation in distal arborization sites of the pulmonary veins in children, because of the small caliber compared to adult subjects. Radiofrequency induced focal areas of stenosis may be amenable to balloon catheter dilation.
- Published
- 2000
- Full Text
- View/download PDF
45. Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies.
- Author
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Gentles TL, Colan SD, Giglia TM, Mandell VS, Mayer JE Jr, and Sanders SP
- Subjects
- Child, Preschool, Cineangiography, Coronary Vessel Anomalies physiopathology, Echocardiography, Follow-Up Studies, Heart Defects, Congenital complications, Heart Defects, Congenital physiopathology, Humans, Infant, Infant, Newborn, Myocardial Ischemia etiology, Postoperative Complications etiology, Time Factors, Coronary Vessel Anomalies complications, Heart Defects, Congenital surgery, Pulmonary Valve abnormalities, Ventricular Function, Left physiology
- Abstract
Background: Right ventricular decompression (RVD) may cause myocardial ischemia in patients with pulmonary atresia with intact ventricular septum and associated coronary artery abnormalities. Although we have previously shown that mortality is very high when two or more coronary arteries are obstructed, the effects of lesser degrees of coronary abnormalities are unknown. We therefore evaluated the effect of RVD on left ventricular (LV) function in those with less extensive coronary artery abnormalities., Methods and Results: Preoperative cineangiograms demonstrated fistulas with or without one coronary artery stenosis in 12 of 24 patients aged 2 days to 33 months at the time of RVD. Preoperative and postoperative two-dimensional echocardiograms were analyzed for global and regional LV function. One infant with fistulas involving two coronary arteries and stenosis of the right coronary artery died from severe global LV dysfunction after RVD. Despite this, mean LV end-diastolic volume (66 +/- 17 mL/m2) and mean LV ejection fraction (60 +/- 9%) were similar in patients with and without coronary artery abnormalities before and after RVD. Before RVD, regional LV dysfunction was seen in 8 of 132 (6%) regions in those with coronary artery abnormalities and in 3 of 132 (2%) in those without coronary artery abnormalities. After RVD, there were 16 of 132 (12%) abnormal regions in those with coronary artery abnormalities and 1 of 132 (< 1%) in those without coronary artery abnormalities. In regions with normal wall motion before RVD, the presence of coronary artery abnormalities was related to regional LV dysfunction after RVD (P < .001)., Conclusions: Regional LV dysfunction was rare in patients without coronary artery abnormalities. In those with less extensive coronary artery abnormalities not involving obstruction to multiple coronary arteries, regional LV dysfunction was common before and increased after RVD, but severe global LV dysfunction was unusual.
- Published
- 1993
46. Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum.
- Author
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Giglia TM, Jenkins KJ, Matitiau A, Mandell VS, Sanders SP, Mayer JE Jr, and Lock JE
- Subjects
- Blood Volume, Coronary Circulation, Humans, Infant, Newborn, Pulmonary Valve diagnostic imaging, Survival Analysis, Treatment Outcome, Tricuspid Valve diagnostic imaging, Ventricular Function, Right, Echocardiography, Heart Septal Defects, Ventricular diagnostic imaging, Heart Septal Defects, Ventricular surgery, Pulmonary Valve abnormalities, Pulmonary Valve surgery
- Abstract
Background: Neonates with pulmonary atresia and intact ventricular septum (PA-IVS) are frequently born with hypoplastic right heart structures that must grow after right ventricular decompression (RVD) procedures for a complete two-ventricle physiology to be achieved. Previous authors have asserted that neonatal right heart size or morphology will predict right heart growth potential. Since 1983, our bias has favored early RVD regardless of initial right heart size. In 1986, we recognized a subset of patients with coronary artery abnormalities associated with poor outcome after RVD and have defined these patients as having a right ventricular-dependent coronary circulation (RVDCC)., Methods and Results: To assess the influence of right heart size on outcome independent of the presence of RVDCC, we measured echocardiographic right ventricular (RV) dimensions in 37 neonates with adequate studies presenting between 1983 and 1990. Coronary artery anatomy was adequately assessed by angiography in 36. RV volume and tricuspid valve (TV) diameter were significantly smaller in patients with RVDCC than in those without. However, there was no statistically significant association between RV volume or TV diameter and survival among patients with or without RVDCC: Among 29 patients without RVDCC, 23 of 24 (95.8%) who achieved RVD are alive compared with 1 of 5 (20%) who did not achieve RVD (P = .001). Twenty-one of the 23 survivors have a complete two-ventricle physiology with low right atrial pressure. Among 7 patients with RVDCC, 2 patients who underwent RVD died early of left ventricular failure, whereas 4 of 5 who did not undergo RVD have survived single ventricular palliation., Conclusions: Small right heart size is associated with RVDCC but is not associated with survival in PA-IVS. Patients without RVDCC have improved survival after RVD regardless of neonatal right heart size.
- Published
- 1993
- Full Text
- View/download PDF
47. Use of inhaled nitric oxide and acetylcholine in the evaluation of pulmonary hypertension and endothelial function after cardiopulmonary bypass.
- Author
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Wessel DL, Adatia I, Giglia TM, Thompson JE, and Kulik TJ
- Subjects
- Administration, Inhalation, Child, Preschool, Cyclic GMP blood, Humans, Hypertension, Pulmonary blood, Infant, Methemoglobin analysis, Postoperative Period, Acetylcholine pharmacology, Cardiopulmonary Bypass, Endothelium, Vascular physiopathology, Hypertension, Pulmonary physiopathology, Hypertension, Pulmonary surgery, Nitric Oxide pharmacology
- Abstract
Background: Increased pulmonary vascular resistance is common in congenital heart disease and is exacerbated by cardiopulmonary bypass (CPB). We investigated whether CPB is responsible for pulmonary endothelial dysfunction and contributes to postoperative pulmonary hypertension., Methods and Results: We infused the endothelium-dependent vasodilator acetylcholine (ACH) into the pulmonary circulation of pulmonary hypertensive children with congenital heart disease either before (n = 12) or after (n = 22) surgical repair on CPB. The dose response to ACH (10(-9) to 10(-6) M) was recorded for all hemodynamic variables. Nine additional postoperative patients were studied with ACH followed by inhalation of 80 ppm nitric oxide, an endothelium-independent smooth muscle relaxant. Plasma levels of cyclic GMP (cGMP) were measured before and after ACH and nitric oxide administration. Pulmonary vasodilation with 10(-6) M ACH was seen in all preoperative patients but was markedly attenuated in postoperative patients. Baseline pulmonary vascular resistance (5.6 +/- 1.0 U x m2) fell 46 +/- 5% in preoperative patients but declined only 11 +/- 4% from baseline (5.8 +/- 0.9 U x m2) in postoperative patients (P < .002). However, inhalation of 80 ppm nitric oxide after ACH infusion in postoperative patients lowered pulmonary vascular resistance by 33 +/- 4% (P < .0002 compared with postoperative ACH response) with minimal effects on the systemic circulation. This finding suggests that the capacity for smooth muscle relaxation and pulmonary vasodilation was present in postoperative patients but could not be induced by ACH. Plasma levels of cGMP in postoperative patients were unchanged after acetylcholine infusion but rose more than threefold during pulmonary vasodilation with nitric oxide (P < .0001). This finding is consistent with the purported role of cGMP as the second messenger effecting smooth muscle relaxation in this process., Conclusions: CPB may be responsible for postoperative dysfunction of the pulmonary endothelial cell and may contribute to postoperative pulmonary hypertension in children. Inhaled nitric oxide is a potent pulmonary vasodilator after CPB with minimal systemic circulatory effects. It may have important diagnostic and therapeutic applications in patients with congenital heart disease.
- Published
- 1993
- Full Text
- View/download PDF
48. Anomalous coronary arteries and coronary artery fistulas in infants and children.
- Author
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Karr SS and Giglia TM
- Subjects
- Child, Child, Preschool, Coronary Angiography, Coronary Vessel Anomalies mortality, Coronary Vessel Anomalies surgery, Echocardiography, Humans, Infant, Postoperative Complications mortality, Survival Rate, Coronary Vessel Anomalies diagnosis
- Published
- 1993
- Full Text
- View/download PDF
49. Course in the intensive care unit after 'preparatory' pulmonary artery banding and aortopulmonary shunt placement for transposition of the great arteries with low left ventricular pressure.
- Author
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Wernovsky G, Giglia TM, Jonas RA, Mone SM, Colan SD, and Wessel DL
- Subjects
- Cardiac Catheterization, Humans, Infant, Intensive Care Units, Respiration, Artificial, Time Factors, Transposition of Great Vessels physiopathology, Ventricular Function, Left physiology, Blood Vessel Prosthesis, Cardiac Output, Low epidemiology, Palliative Care methods, Postoperative Complications epidemiology, Pulmonary Artery surgery, Transposition of Great Vessels surgery
- Abstract
Background: In patients with transposition of the great arteries with low left ventricular pressure, pulmonary artery banding with aortopulmonary shunt placement has been advocated to "prepare" the left ventricle for systemic work before an arterial switch operation., Methods and Results: In 28 patients, this preparatory procedure was performed with one death. A successful arterial switch operation was performed at a median of 7 days later in 24 of 27 survivors; one child had a Senning performed, and two others died. During this interval period, the left ventricular-to-right ventricular pressure ratio increased from 48 +/- 8% to 98 +/- 19%, and left ventricular mass (indexed for body surface area) increased from 46 +/- 17 to 72 +/- 23 g/m2. After the preparatory procedure, the initial postoperative period was frequently characterized by a low-output syndrome of variable length and severity. Prolonged mechanical ventilation, extended inotropic support, and/or a significant metabolic acidosis was present in 21 of 28 patients in the immediate postoperative period., Conclusions: The low-output syndrome is most likely due to a combination of acute (fixed) right ventricular volume overload from the shunt and acute (transient) left ventricular dysfunction from the pulmonary artery band. This low-output syndrome should be anticipated following the preparatory procedure.
- Published
- 1992
50. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.
- Author
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Giglia TM, Mandell VS, Connor AR, Mayer JE Jr, and Lock JE
- Subjects
- Arteriovenous Fistula mortality, Coronary Angiography, Coronary Circulation physiology, Coronary Vessel Anomalies mortality, Humans, Infant, Newborn, Retrospective Studies, Treatment Outcome, Arteriovenous Fistula congenital, Arteriovenous Fistula surgery, Coronary Vessel Anomalies diagnostic imaging, Coronary Vessel Anomalies surgery, Heart Ventricles, Pulmonary Valve abnormalities
- Abstract
Background: Coronary artery anomalies including 1) right ventricle (RV)-to-coronary artery fistulas, 2) coronary artery stenoses, and 3) coronary occlusions occur in patients with pulmonary atresia with intact ventricular septum (PA-IVS). In some, a large part of the coronary blood supply may depend on the RV. This RV-dependent coronary circulation may determine survival after right ventricular decompression (RVD): RVD may cause RV "steal" in the presence of fistulas alone and ischemia, coronary isolation, or myocardial infarction in the presence of coronary stenoses., Methods and Results: Eighty-two patients with PA-IVS who presented between January 1979 and January 1990 were reviewed; 26 (32%) had RV-to-coronary artery fistulas. Of these 26, 23 had adequate preoperative coronary angiograms for analysis. RVD was achieved in 16. Seven of 16 had fistulas only; each survived RVD. Six of 16 had stenosis of a single coronary artery [left anterior descending coronary artery (LAD), four; right coronary artery (RCA), two]; four of six survived RVD. Three of 16 had stenoses and/or occlusion of both the RCA and LAD; all three died shortly after RVD of acute left ventricular dysfunction., Conclusions: 1) Potential RV steal alone does not preclude successful RVD. 2) Fistulas with stenoses to a single coronary artery may not preclude successful RVD. 3) RVD appears to be contraindicated in the presence of stenoses and/or occlusion involving both the right and left coronary systems. Nonsurvival after RVD seems to depend on the amount of the left ventricular myocardium at risk, i.e., that which is distal to coronary artery stenoses, especially when involvement of both coronary arteries limits effective collateralization. Precise definition of coronary arterial anatomy is mandatory in neonates with PA-IVS.
- Published
- 1992
- Full Text
- View/download PDF
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