158 results on '"Ralph S. Mosca"'
Search Results
2. Procalcitonin as a biomarker of bacterial infection in pediatric patients after congenital heart surgery
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Sujata B Chakravarti, Diane A Reformina, Timothy M Lee, Sunil P Malhotra, Ralph S Mosca, and Puneet Bhatla
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Cardiac surgery ,cardiopulmonary bypass (CPB) ,infection ,pediatric ,congenital heart surgery ,procalcitonin (PCT) ,Medicine ,Pediatrics ,RJ1-570 ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
Background: Bacterial infection (BI) after congenital heart surgery (CHS) is associated with increased morbidity and is difficult to differentiate from systemic inflammatory response syndrome caused by cardiopulmonary bypass (CPB). Procalcitonin (PCT) has emerged as a reliable biomarker of BI in various populations. Aim: To determine the optimal PCT threshold to identify BI among children suspected of having infection following CPB. Setting and Design: Single-center retrospective observational study. Setting and Design: Single-center retrospective observational study. Statistical Analysis: The Wilcoxon rank-sum test was used for nonparametric variables. The diagnostic performance of PCT was evaluated using a receiver operating characteristic (ROC) curve. Results: Ninety-eight patients were included. The median age was 2 months (25th and 75th interquartile of 0.1-7.5 months). Eleven patients were included in the BI group. The median PCT for the BI group (3.42 ng/mL, 25th and 75th interquartile of 2.34-5.67) was significantly higher than the median PCT for the noninfected group (0.8 ng/mL, 25th and 75th interquartile 0.38-3.39), P = 0.028. The PCT level that yielded the best compromise between the sensitivity (81.8%) and specificity (66.7%) was 2 ng/mL with an area under the ROC curve of 0.742. Conclusion: A PCT less than 2 ng/mL makes BI unlikely in children suspected of infection after CHS.
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- 2016
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3. Modified Blalock-Thomas-Taussig Shunt Using Femoral Artery Homograft
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Sandy Zhang, Suneet Bhansali, Jaclyn McKinstry, Prema Ramaswamy, Kristen Thomas, Michael Martinez, Ralph S. Mosca, and T.K. Susheel Kumar
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- 2023
4. Improving Longitudinal Outcomes, Efficiency, and Equity in the Care of Patients With Congenital Heart Disease
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Khanh H. Nguyen, Steven A Kamenir, Stephen Cook, Kathleen E. Walsh-Spoonhower, Edward L. Hannan, Robert Vincent, Brett R. Anderson, Erin A Paul, John Billings, David B. Meyer, Joyce Woo, New York State Congenital Heart Surgery Collaborative for Longitudinal Outcomes, Jane W. Newburger, Sarah Crook, Neil Devejian, Michael F. Swartz, Kacie Dragan, George M. Alfieris, Ralph S. Mosca, Emile A Bacha, and Marshall L. Jacobs
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Heart Defects, Congenital ,medicine.medical_specialty ,Adolescent ,Heart disease ,Social Determinants of Health ,medicine.medical_treatment ,New York ,Pharmacy ,Efficiency ,Severity of Illness Index ,Health Services Accessibility ,Insurance Claim Review ,Outpatients ,Match rate ,medicine ,Humans ,Longitudinal Studies ,Registries ,Social determinants of health ,Healthcare Disparities ,Child ,Rehabilitation ,Health Equity ,Medicaid ,business.industry ,Infant, Newborn ,Equity (finance) ,Infant ,medicine.disease ,United States ,Health equity ,Treatment Outcome ,Child, Preschool ,Family medicine ,Cardiology and Cardiovascular Medicine ,business ,Algorithms ,Follow-Up Studies - Abstract
Longitudinal follow-up, resource utilization, and health disparities are top congenital heart research and care priorities. Medicaid claims include longitudinal data on inpatient, outpatient, emergency, pharmacy, rehabilitation, home health utilization, and social determinants of health-including mother-infant pairs.The New York Congenital Heart Surgeons Collaborative for Longitudinal Outcomes and Utilization of Resources linked robust clinical details from locally held state and national registries from 10 of 11 New York congenital heart centers to Medicaid claims, building a novel, statewide mechanism for longitudinal assessment of outcomes, expenditures, and health inequities.The authors included all children 18 years of age undergoing cardiac surgery in The Society of Thoracic Surgeons Congenital Heart Surgery Database or the New York State Pediatric Congenital Cardiac Surgery Registry from 10 of 11 New York centers, 2006 to 2019. Data were linked via iterative, ranked deterministic matching on direct identifiers. Match rates were calculated and compared. Proportions of the linked cohort trackable over 3, 5, and 10 years were described.Of 14,097 registry cases, 59% (n = 8,322) reported Medicaid use. Of these, 7,414 were linked to New York claims, at an 89% match rate. Of matched cases, the authors tracked 79%, 74%, and 65% of children over 3, 5, and 10 years when requiring near-continuous Medicaid enrollment. Allowing more lenient enrollment criteria, the authors tracked 86%, 82%, and 76%, respectively. Mortality over this time was 7.7%, 8.4%, and 10.0%, respectively. Manual validation revealed ∼100% true matches.This establishes a novel statewide data resource for assessment of longitudinal outcome, health expenditure, and disparities for children with congenital heart disease.
- Published
- 2021
5. Periscopic technique in Norwood operation is associated with better preservation of early ventricular function
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Sujata Chakravarti, David M. Williams, Puneet Bhatla, Brandon Winston, Catherine Bull, Luv Makadia, Richard G. Ohye, T.K. Susheel Kumar, Ralph S. Mosca, and James C. Nielsen
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,RV-PA-C, right ventricle to pulmonary artery conduit ,periscopic technique ,PT, periscope technique ,RV function ,Ventriculotomy ,Hypoplastic left heart syndrome ,symbols.namesake ,Norwood ,Internal medicine ,medicine.artery ,EDA, end diastolic area ,medicine ,echocardiography ,Congenital: Norwood Procedure ,Fisher's exact test ,RV to PA conduit ,Ejection fraction ,business.industry ,HLHS, hypoplastic left heart syndrome ,MBTT, modified Blalock-Taussig-Thomas ,FAC, fraction area change ,medicine.disease ,Norwood Operation ,TT, traditional technique ,RV, right ventricle ,medicine.anatomical_structure ,Ventricle ,Pulmonary artery ,symbols ,Cardiology ,Surgery ,Tricuspid Valve Regurgitation ,business - Abstract
Objective Although the right ventricle (RV) to pulmonary artery conduit in stage 1 Norwood operation results in improved interstage survival, the long-term effects of the ventriculotomy used in the traditional technique remain a concern. The periscopic technique (PT) of RV to pulmonary artery conduit placement has been described as an alternative technique to minimize RV injury. A retrospective study was performed to compare the effects of traditional technique and PT on ventricular function following Norwood operation. Methods A retrospective study of all patients who underwent Norwood operation from 2012 to 2019 was performed. Patients with baseline RV dysfunction and significant tricuspid valve regurgitation were excluded. Prestage 2 echocardiograms were reviewed by a blinded experienced imager for quantification of RV function (sinus and infundibular RV fractional area change) as well as for regional conduit site wall dysfunction (normal or abnormal, including hypokinesia, akinesia, or dyskinesia). Wilcoxon rank-sum tests were used to assess differences in RV infundibular and RV sinus ejection fraction and the Fisher exact test was used to assess differences in regional wall dysfunction. Results Twenty-two patients met inclusion criteria. Eight underwent traditional technique and 14 underwent PT. Median infundibular RV fractional area change was 49% and 37% (P = .02) and sinus RV fractional area change was 50% and 41% for PT and traditional technique (P = .007) respectively. Similarly qualitative regional RV wall function was better preserved in PT (P = .002). Conclusions The PT for RV to pulmonary artery conduit in Norwood operation results in better preservation of early RV global and regional systolic function. Whether or not this benefit translates to improved clinical outcome still needs to be studied.
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- 2021
6. Surgical Management of Isolated Congenital Tricuspid Valve Regurgitation in an Adult
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Stacey Chen, Sergey Ayzenberg, Ralph S. Mosca, and Mikhail Vaynblat
- Abstract
Isolated tricuspid valve regurgitation can be caused by either primary valvular abnormalities or secondary to annular dilation. Congenital tricuspid valve regurgitation in the adult is rare and encompasses a heterogenous group of anatomical variants that is often associated with or is a sequela of other congenital heart diseases that poses a unique challenge to the surgical management of these patients. We present a case of primary isolated congenital tricuspid valve regurgitation and successful tricuspid valve repair in an adult.
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- 2022
7. Femoral artery homograft for coronary artery plasty following arterial switch operation
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Charles Ma, Dan G. Halpern, Jodi L. Feinberg, David Y.T. Chen, Ralph S. Mosca, Puneet Bhatla, and T.K. Susheel Kumar
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,medicine.anatomical_structure ,business.industry ,Internal medicine ,medicine.artery ,Cardiology ,Congenital: Transposition of the Great Arteries: Case Report ,Medicine ,Surgery ,Femoral artery ,business ,Artery - Published
- 2020
8. Surgical Management of Giant Intrapericardial Teratoma Encasing the Coronary Artery
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James C. Nielsen, Michelle Ramirez, Emina Hodzic, Madhu Sharma, Tk Susheel Kumar, Jason C. Fisher, Ralph S. Mosca, Puneet Bhatla, Prashant K. Minocha, and Margret Magid
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Surgical resection ,medicine.medical_specialty ,business.industry ,Infant, Newborn ,Teratoma ,General Medicine ,Coronary Vessels ,Surgery ,Heart Neoplasms ,medicine.anatomical_structure ,Intrapericardial teratoma ,Pediatrics, Perinatology and Child Health ,Medicine ,Humans ,Cardiology and Cardiovascular Medicine ,business ,Pericardium ,Artery - Abstract
Intrapericardial teratomas are rare, predominantly benign tumors that warrant surgical resection in the neonatal period because of their potential detrimental effects on the cardiorespiratory system. Surgical resection can be a challenge when the tumor encases and obscures a coronary artery. Adherence to certain operative principles is necessary to achieve successful outcomes.
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- 2021
9. Abstract 12806: Anatomic Correlates of Systolic Anterior Motion in D-looped Transposition of the Great Arteries Post Atrial Switch
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Dan G. Halpern, Mark V. Sherrid, TK Kumar, Ralph S. Mosca, Norman Aiad, Jodi L. Feinberg, Michael Argilla, and Leon Axel
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medicine.medical_specialty ,Great arteries ,business.industry ,Physiology (medical) ,Internal medicine ,medicine ,Transposition (telecommunications) ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Atrial switch - Abstract
Introduction: A paucity of data exists of the anatomic-physiological mechanisms for sub-pulmonic systolic anterior motion (SAM) of the mitral valve (MV) in d-looped transposition of the great arteries post atrial switch (d-TGA/AtS). Hypothesis: The development of SAM may be inherent to the anatomy of the MV or affected by external factors such as a dilated systemic right ventricle (RV) or pectus deformity. Methods: Clinical and cardio-imaging analysis of 18 adult patients with d-TGA/AtS (Age 42±6 years old, 56% Male) between 2015-2019 was performed. Echocardiography data included mitral apparatus anatomy (Figure) and CT/MRI data included biventricular dimensions, function, and Haller index (HI). Results: Patients with leaflet SAM (n=5) compared to patients without SAM (n=13) had significantly higher MV protrusion height (2.1 ± 0.4 vs 1.5 ± 0.4 cm p ≤0.01, respectively, Table) and longer anterior MV leaflet length (3.0 ± 0.24 vs 2.6 ± 0.34 cm p ≤0.05, respectively, Table) when compared to those without. CT/MRI showed higher sub-pulmonic left ventricular ejection fraction (LVEF) in the SAM group relative to No SAM (68 ± 5 vs 54 ± 7 % p ≤0.005, respectively, Table). RV size and function, chest deformity (HI>3.5), presence of a ventricular lead pacemaker, and septal thickness did not play a role in development of SAM (Table). Conclusions: An elongated mitral apparatus is associated with the development of SAM in d-TGA/AtS and the development of LVOT obstruction. Increased sub-pulmonic LVEF contributes to SAM, whereas the systemic RV dimensions, septal thickness, and degree of chest deformity do not correlate with SAM.
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- 2020
10. An unusual case of a solitary cardiac myofibroma causing severe right ventricular outflow tract obstruction in an infant
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Achiau Ludomirsky, Fei Chen, T.K. Susheel Kumar, Joseph J. Maleszewski, Ralph S. Mosca, and Prashant K. Minocha
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Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Skin Neoplasms ,Myofibroma ,Infantile myofibromatosis ,Context (language use) ,030204 cardiovascular system & hematology ,Right ventricular outflow tract obstruction ,Ventricular Outflow Obstruction ,Heart Neoplasms ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Child ,Unusual case ,business.industry ,Infant ,Myofibromatosis ,General Medicine ,medicine.disease ,030228 respiratory system ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Radiology ,Cardiology and Cardiovascular Medicine ,business - Abstract
Cardiac tumours are relatively uncommon, particularly in children. Myofibroma is an extremely rare variety of cardiac tumour, which nearly always arises in the context of infantile myofibromatosis. Herein, we present a case of a solitary cardiac myofibroma causing right ventricular outflow tract obstruction in a 2-month-old male infant.
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- 2020
11. Commentary: Tetralogy of Fallot: Will time tell?
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Ralph S. Mosca
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,business.industry ,General surgery ,medicine ,Surgery ,Cardiology and Cardiovascular Medicine ,medicine.disease ,business ,Tetralogy of Fallot - Published
- 2020
12. Altering management decisions with gained anatomical insight from a 3D printed model of a complex ventricular septal defect
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Ralph S. Mosca, Puneet Bhatla, and Justin T. Tretter
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Heart Septal Defects, Ventricular ,Models, Anatomic ,Cardiac Catheterization ,medicine.medical_specialty ,3d printed ,Decision Making ,Computed tomography ,Cardiac catheterisation ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,0502 economics and business ,Humans ,Medicine ,Medical physics ,Cardiac Surgical Procedures ,Surgical repair ,medicine.diagnostic_test ,business.industry ,05 social sciences ,Infant, Newborn ,Disease Management ,General Medicine ,Infant newborn ,Surgery ,Tomography x ray computed ,Echocardiography ,Printing, Three-Dimensional ,Pediatrics, Perinatology and Child Health ,050211 marketing ,Tomography, X-Ray Computed ,Cardiology and Cardiovascular Medicine ,business - Abstract
Rapid prototyping is quickly gaining utility in various complex forms of CHD. In properly selected cases, these printed models provide detailed anatomical understanding that help guide potential surgical and cardiac catheterisation interventions. We present a case of a tunnel-like ventricular septal defect referred for surgical repair, where the decision to obtain a three-dimensional printed model helped in better understanding of the anatomy, leading to delaying, and hopefully avoiding altogether, surgical repair.
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- 2016
13. Utility and Scope of Rapid Prototyping in Patients with Complex Muscular Ventricular Septal Defects or Double-Outlet Right Ventricle: Does it Alter Management Decisions?
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Piers Barker, Nicole Wake, Sujata Chakravarti, Shi Joon Yoo, Puneet Bhatla, Ralph S. Mosca, Achi Ludomirsky, Justin T. Tretter, Michael Argilla, Doff B. McElhinney, and Larry A. Latson
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Heart Septal Defects, Ventricular ,Male ,Models, Anatomic ,Rapid prototyping ,medicine.medical_specialty ,Heart disease ,Decision Making ,030204 cardiovascular system & hematology ,Surgical planning ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Imaging, Three-Dimensional ,0302 clinical medicine ,Double outlet right ventricle ,medicine ,Humans ,Cardiac Surgical Procedures ,Child ,medicine.diagnostic_test ,business.industry ,Infant ,Heart ,Magnetic resonance imaging ,Vascular surgery ,medicine.disease ,Magnetic Resonance Imaging ,Double Outlet Right Ventricle ,Surgery ,Cardiac surgery ,medicine.anatomical_structure ,Ventricle ,Child, Preschool ,Printing, Three-Dimensional ,Pediatrics, Perinatology and Child Health ,Female ,Tomography, X-Ray Computed ,Cardiology and Cardiovascular Medicine ,business - Abstract
Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.
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- 2016
14. Understanding the spectrum of sinus venosus interatrial communications
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Ralph S. Mosca, Robert H. Anderson, Justin T. Tretter, Carl L. Backer, Diane E. Spicer, Sathish Chikkabyrappa, and Puneet Bhatla
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Male ,0301 basic medicine ,Superior sinus venosus defect ,medicine.medical_specialty ,Vena Cava, Superior ,Adolescent ,Vascular Malformations ,Autopsy ,030204 cardiovascular system & hematology ,Heart Septal Defects, Atrial ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,Abnormalities, Multiple ,Child ,Normal heart ,Retrospective Studies ,Sinus venosus ,Sinus venosus defect ,Anomalous pulmonary venous connection ,business.industry ,General Medicine ,medicine.disease ,030104 developmental biology ,medicine.anatomical_structure ,Pulmonary Veins ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Cardiac magnetic resonance ,Echocardiography, Transesophageal ,Interatrial septum - Abstract
BackgroundIt is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects.MethodsWe reviewed our experience with patients suspected of having a sinus venosus defect from August, 2011, through October, 2015, analysing the findings in light of the current hypotheses used to explain the development of the defects, along with correlations made by inspection of autopsy specimens.ResultsWe evaluated findings from 16 patients, with a mean age of 7.7 years, ranging from 2.7 to 15 years. Of the group, 13 were ultimately diagnosed with a superior sinus venosus defect, two with an inferior defect, and one with isolated anomalous pulmonary venous connection in the absence of an interatrial communication. Initially, two patients were thought to have oval fossa defects, one from each subtype, but were correctly diagnosed following cardiac magnetic resonance interrogation. Anomalous pulmonary venous connections were present in all cases.ConclusionAppreciation of the changes occurring during normal cardiac development helps in understanding the anatomical substrate underscoring the spectrum of sinus venosus defects. The lesions are veno-venous connections due to partially anomalous pulmonary venous connections, producing interatrial communications outside the confines of the interatrial septum.
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- 2016
15. Surgical planning for a complex double-outlet right ventricle using 3D printing
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Sathish Chikkabyrappa, Ralph S. Mosca, Justin T. Tretter, Sujata Chakravarti, and Puneet Bhatla
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Models, Anatomic ,medicine.medical_specialty ,Heart Ventricles ,medicine.medical_treatment ,3D printing ,030204 cardiovascular system & hematology ,Surgical planning ,Preoperative care ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Imaging, Three-Dimensional ,0302 clinical medicine ,Double outlet right ventricle ,Preoperative Care ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Medical physics ,Cardiac catheterization ,Surgical repair ,Surgical approach ,business.industry ,Models, Cardiovascular ,Equipment Design ,medicine.disease ,Double Outlet Right Ventricle ,Surgery ,Printing, Three-Dimensional ,Computer-Aided Design ,Surgical simulation ,Cardiology and Cardiovascular Medicine ,business - Abstract
Rapid prototyping may be beneficial in properly selected cases of complex congenital heart disease, providing detailed anatomical understanding that helps to guide potential surgical and cardiac catheterization interventions. We present a case of double-outlet right ventricle, where the decision to obtain a three-dimensional printed model helped for better understanding of the anatomy, with the additional advantage of surgical simulation in planning the surgical approach and type of surgical repair.
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- 2017
16. From Eye Wash to Cardiac Modeling
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Ralph S. Mosca
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Pulmonary and Respiratory Medicine ,Heart Septal Defects, Ventricular ,medicine.medical_specialty ,Heart septal defect ,business.industry ,MEDLINE ,General Medicine ,030204 cardiovascular system & hematology ,medicine.disease ,03 medical and health sciences ,0302 clinical medicine ,030228 respiratory system ,Internal medicine ,medicine ,Cardiology ,Humans ,Surgery ,Cardiology and Cardiovascular Medicine ,business - Published
- 2018
17. Ubiquitin C-Terminal Hydrolase 1 and Phosphorylated Axonal Neurofilament Heavy Chain in Infants Undergoing Cardiac Surgery: Preliminary Assessment as Potential Biomarkers of Brain Injury
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Ralph S. Mosca, Sathish Chikkabyrappa, Sunil P. Malhotra, Amanda Mastrippolito, Timothy Lee, Sujata Chakravarti, Diane Reformina, and Gerry Shaw
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Male ,medicine.medical_specialty ,Neurofilament ,Ubiquitin C-Terminal Hydrolase ,Enzyme-Linked Immunosorbent Assay ,Pilot Projects ,030204 cardiovascular system & hematology ,Pharmacology ,law.invention ,03 medical and health sciences ,0302 clinical medicine ,Postoperative Complications ,Ubiquitin ,law ,Neurofilament Proteins ,Hydrolase ,Cardiopulmonary bypass ,medicine ,Humans ,Prospective Studies ,Cardiopulmonary Bypass ,Spectroscopy, Near-Infrared ,biology ,business.industry ,Infant, Newborn ,Infant ,General Medicine ,Cardiac surgery ,Circulatory Arrest, Deep Hypothermia Induced ,Brain Injuries ,Pediatrics, Perinatology and Child Health ,biology.protein ,Deep hypothermic circulatory arrest ,Phosphorylation ,Surgery ,Female ,Cardiology and Cardiovascular Medicine ,business ,Ubiquitin Thiolesterase ,030217 neurology & neurosurgery ,Biomarkers - Abstract
Background: There are no reliable markers to assess brain injury in neonates following cardiac surgery. We examine ubiquitin C-terminal hydrolase 1 (UCHL1) and phosphorylated axonal neurofilament heavy chain (pNF-H), neuronal-specific biomarkers released following axonal and cortical injury, in neonates undergoing cardiac surgery involving cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). Methods: Twenty-six patients younger than three months were prospectively enrolled (CPB only, n = 12 and DHCA, n = 14). Healthy newborns (n = 22) served as the control. Blood samples were collected preoperatively and postoperatively upon intensive care unit admission (hour 0) and subsequently at 12, 24, 36, and 48 hours. Serum was tested for UCHL1 and pNF-H using enzyme-linked immunosorbent assay. Concomitant arterial blood gas, lactate, and cerebral near-infrared spectroscopy (NIRS) monitoring were performed. Results: Ubiquitin C-terminal hydrolase 1 showed a significant rise at 0 hours in the DHCA group compared to baseline (74.9 ± 13.7 pg/mL vs 33.9 ± 37.3 pg/mL, P < .0001). Levels returned to baseline at 12 hours. There was an early rise in UCHL1 at 0 hours in the CPB group, P = .09. Phosphorylated axonal neurofilament heavy chain was decreased at 0 hours in both the CPB and DHCA groups compared to baseline, P = .06. There was no difference between control and baseline levels of UCHL1 ( P = .9) or pNF-H ( P = .77). Decreased NIRS was observed in the DHCA group at 0 hours (57.3 ± 10.5) versus baseline (64.2 ± 12.3), but not significant ( P = .21). There was no correlation between biomarkers and NIRS at 0 hours. Conclusion: A rapid rise in UCHL1 levels was observed in the DHCA group, suggesting that it may be a marker for acute brain injury. Follow-up with neurodevelopmental studies is ongoing.
- Published
- 2018
18. Commentary: The Achilles' heel of the stage 1 palliation
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Ralph S. Mosca
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Heel ,business.industry ,medicine.medical_treatment ,Femoral vein ,MEDLINE ,Surgery ,medicine.anatomical_structure ,medicine.artery ,Pulmonary artery ,medicine ,Norwood procedure ,Stage (cooking) ,Cardiology and Cardiovascular Medicine ,business - Published
- 2019
19. Retrograde transcatheter device closure of a complex paravalvular leak after bioprosthetic pulmonary valve replacement in a pediatric patient
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Sathish Chikkabyrappa, Doff B. McElhinney, and Ralph S. Mosca
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medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,medicine.medical_treatment ,General Medicine ,030204 cardiovascular system & hematology ,eye diseases ,Surgery ,03 medical and health sciences ,Pediatric patient ,0302 clinical medicine ,Internal medicine ,Congenital pulmonary valve stenosis ,Angiography ,medicine ,Cardiology ,Radiology, Nuclear Medicine and imaging ,sense organs ,030212 general & internal medicine ,Paravalvular leak ,Cardiology and Cardiovascular Medicine ,business ,Bioprosthetic pulmonary valve replacement ,Cardiac catheterization - Abstract
We report a case of retrograde transcatheter device closure of a complex paravalvular leak (PVL) after bioprosthetic pulmonary valve replacement (PVR) in a 13-year-old patient with congenital pulmonary valve stenosis. There are prior reports of pulmonary PVL closure after PVR in adults (Seery and Slack, Congenit Heart Dis 2014;9:E19-F22), but indications for and technical considerations in PVL closure after bioprosthetic PVR, particularly in children, are not well defined. © 2015 Wiley Periodicals, Inc.
- Published
- 2015
20. Anomalous aortic origin of a coronary artery: 90% of optimal therapy is 50% improved technology
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Ralph S. Mosca
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Aorta ,business.industry ,Coronary Vessel Anomalies ,Coronary Vessel Anomaly ,MEDLINE ,030204 cardiovascular system & hematology ,medicine.disease ,03 medical and health sciences ,0302 clinical medicine ,Text mining ,Internal medicine ,Anomalous aortic origin of a coronary artery ,medicine.artery ,medicine ,Cardiology ,Humans ,Surgery ,030212 general & internal medicine ,Prospective Studies ,Cardiology and Cardiovascular Medicine ,business ,Prospective cohort study - Published
- 2017
21. Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair
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Jennifer Tingo, Betul Yilmaz, Paul J. Chai, Stéphanie Levasseur, Jan M. Quaegebeur, Jeffrey D. Dayton, Emile A. Bacha, Ralph S. Mosca, Marc Najjar, and Elizabeth H. Stephens
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Cardiac function curve ,Male ,medicine.medical_specialty ,Time Factors ,Heart Ventricles ,030204 cardiovascular system & hematology ,03 medical and health sciences ,Primary repair ,0302 clinical medicine ,Internal medicine ,Medicine ,Humans ,Ventricular Function ,Cardiac Surgical Procedures ,Tetralogy of Fallot ,Retrospective Studies ,business.industry ,Heart Septal Defects ,Infant ,Mitral Valve Insufficiency ,General Medicine ,medicine.disease ,Pulmonary Valve Insufficiency ,Treatment Outcome ,030228 respiratory system ,Echocardiography ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Cardiology ,Atrioventricular canal ,Surgery ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background: Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) is a challenging operation increasingly being performed as a complete, primary repair in infancy. Previous studies have focused on perioperative outcomes; however, midterm valve function, ventricular function, and residual obstruction have received little attention. Methods: We retrospectively reviewed 20 patients who underwent CAVC/TOF repair (January 2005 to December 2014). A two-patch repair was used in all patients to correct the CAVC defect. Tetralogy of Fallot repair included transannular patch in 11 (65%) patients and valve-sparing in 6 (35%) patients. Results: The average age at surgery was 72 ± 122 weeks, 40% were male, and 80% had trisomy 21. Mean echo follow-up was 3.0 ± 3.0 years. There were no in-hospital or late mortalities. The rate of reoperation was 20%. At the latest follow-up, moderate left atrioventricular valve regurgitation was present in three (15%) patients and mild stenosis present in seven (35%) patients. One (5%) patient had moderate right ventricular outflow tract (RVOT) obstruction. The valve-sparing population was smaller at the time of surgery than the non-valve-sparing cohort (body surface area: 0.28 ± 0.04 vs 0.42 ± 0.11, P = .002) and less likely to have had a previous shunt (0% vs 64%, P = .01). Among the valve-sparing patients (six), at the latest follow-up, moderate pulmonary insufficiency was present in two (33%) patients. Conclusion: Repair of CAVC concomitant with TOF can be performed with low mortality and acceptable perioperative morbidity. Management of the RVOT remains a challenge for the long term.
- Published
- 2017
22. Shunt right or left? Decision 2016
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Ralph S. Mosca
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Fistula ,business.industry ,Heart Ventricles ,MEDLINE ,030204 cardiovascular system & hematology ,Pulmonary Artery ,medicine.disease ,Shunt (medical) ,Surgery ,03 medical and health sciences ,0302 clinical medicine ,030228 respiratory system ,medicine.artery ,Pulmonary artery ,Medicine ,Humans ,Cardiology and Cardiovascular Medicine ,business - Published
- 2016
23. Super Glenn: Able to Defeat Recalcitrant Pulmonary Vascular Adversaries?
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Ralph S. Mosca
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,MEDLINE ,General Medicine ,030204 cardiovascular system & hematology ,Fontan procedure ,03 medical and health sciences ,0302 clinical medicine ,Text mining ,030228 respiratory system ,Medicine ,Surgery ,Cardiology and Cardiovascular Medicine ,business ,Intensive care medicine - Published
- 2018
24. TRANSTHORACIC TRANSCATHETER APPROACH FOR THE EXCLUSION OF A PSEUDOANEURYSM IN A FONTAN PATIENT
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Ralph S. Mosca, Puneet Bhatla, Hannah L. Tredway, Nicole Perry, Michael Argilla, Xingchen Mai, and Dan G. Halpern
- Subjects
congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,business.industry ,Status post ,medicine.disease ,Transcatheter approach ,Surgery ,Pseudoaneurysm ,Great arteries ,cardiovascular system ,Medicine ,cardiovascular diseases ,Tricuspid atresia ,Cardiology and Cardiovascular Medicine ,business - Abstract
Surgically corrected congenital heart defects may result in unique long-term complications. An 18 year old man with tricuspid atresia, normally related great arteries, and ventricular septal defect (VSD), status post a staged surgical palliation culminating in an extra-cardiac Fontan, presented
- Published
- 2019
25. Periscope Modification of Right Ventricle-to-Pulmonary Artery Shunt in Norwood Operation
- Author
-
Masaki Tsukashita M.D. and Ralph S. Mosca
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Heart Ventricles ,Hemodynamics ,Pulmonary Artery ,Norwood Procedures ,Hypoplastic left heart syndrome ,Prosthesis Implantation ,Pseudoaneurysm ,Electrical conduit ,medicine.artery ,Internal medicine ,Hypoplastic Left Heart Syndrome ,Humans ,Medicine ,cardiovascular diseases ,business.industry ,Anastomosis, Surgical ,Suture Techniques ,medicine.disease ,Norwood Operation ,Surgery ,Shunt (medical) ,surgical procedures, operative ,medicine.anatomical_structure ,Ventricle ,Pulmonary artery ,cardiovascular system ,Cardiology ,Cardiology and Cardiovascular Medicine ,business - Abstract
Given the purported hemodynamic advantages of the right ventricle (RV) to pulmonary artery (PA) conduit, many surgeons have adopted it as their preferred source of pulmonary blood flow during stage I palliation for hypoplastic left heart syndrome. Potential disadvantages of the RV-PA shunt include ventricular dysfunction, pseudoaneurysm formation, arrhythmia, and conduit obstruction, which can lead to a higher rate of unplanned reinterventions. The "dunk" technique was described to reduce the RV incision and proximal conduit obstruction; however, insertion of the ringed graft from the epicardium can be cumbersome and risk RV injury. We introduce a simplified, alternative method of placing the conduit, which we call the periscope technique, whereby the graft is withdrawn from within the RV cavity.
- Published
- 2014
26. Utility of rapid prototyping in Complex DORV: does it alter management decisions?
- Author
-
Daniel K. Sodickson, Ralph S. Mosca, Sujata Chakravarti, Larry A. Latson, Puneet Bhatla, and Nicole Wake
- Subjects
Medicine(all) ,Rapid prototyping ,medicine.medical_specialty ,Radiological and Ultrasound Technology ,business.industry ,030204 cardiovascular system & hematology ,03 medical and health sciences ,Engineering management ,0302 clinical medicine ,030225 pediatrics ,Poster Presentation ,Medicine ,Radiology, Nuclear Medicine and imaging ,Medical physics ,Cardiology and Cardiovascular Medicine ,business - Published
- 2016
27. Potential Uses of Cord Blood in Cardiac Surgery
- Author
-
Ralph S. Mosca
- Subjects
medicine.medical_specialty ,Heart disease ,business.industry ,Review Article ,Disease ,medicine.disease ,Umbilical cord ,Public healthcare ,Cardiac surgery ,Surgical therapy ,medicine.anatomical_structure ,Cord blood ,medicine ,Stem cell ,Intensive care medicine ,business ,Biomedical engineering - Abstract
Despite advances in the fields of prevention, medical intervention and surgical therapy, cardiovascular disease remains a major public healthcare issue. A promising area of research is the potential application of regenerative therapies with pluripotential stem cells to reduce the burden of heart disease and its sequelae. Umbilical cord blood, a rich source of multiple populations of nonembryonic stem cells, will be a valuable resource and has the potential to advance therapeutic options for patients with acquired and congenital heart disease.
- Published
- 2012
28. Outcomes in pediatric cardiac transplantation with a positive HLA cross-match
- Author
-
Linda J. Addonizio, Marc E. Richmond, Daphne T. Hsu, Jan M. Quaegebeur, Ralph S. Mosca, Jonathan M. Chen, and Jacqueline M. Lamour
- Subjects
Heart transplantation ,Transplantation ,Pediatrics ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Cardiomyopathy ,Human leukocyte antigen ,Single Center ,medicine.disease ,Pediatrics, Perinatology and Child Health ,medicine ,Overall survival ,In patient ,Transplant patient ,business - Abstract
Richmond ME, Hsu DT, Mosca RS, Chen J, Quaegebeur JM, Addonizio LJ, Lamour JM. Outcomes in pediatric cardiac transplantation with a positive HLA cross-match. Pediatr Transplantation 2012: 16: 29–35. © 2011 John Wiley & Sons A/S. Abstract: Previous studies have shown poor outcomes in pediatric heart transplant recipients with a high PRA or a positive direct donor–recipient cross-match. This study describes outcomes in patients with a positive cross-match at a large pediatric program. Pediatric heart transplant patients at a large single center between January 1993 and July 2009 were reviewed; those with cross-match data were analyzed. Cross-match data were available in 242/262 (92.4%) patients. Indications for transplant were cardiomyopathy (58%), CHD (32%), and retransplant (7%). PRA was ≥10% in 31/213 (14.6%) patients. A retrospective cross-match was positive in 17/31 (55%) patients with PRA ≥10% and 0/182 with PRA
- Published
- 2011
29. Listing and Transplanting Adults With Congenital Heart Disease
- Author
-
Jonathan Yang, Ralph S. Mosca, Jan M. Quaegebeur, Mark J. Russo, Jonathan M. Chen, and Ryan R. Davies
- Subjects
Adult ,Heart Defects, Congenital ,Male ,Reoperation ,medicine.medical_specialty ,Waiting Lists ,Heart disease ,medicine.medical_treatment ,Kaplan-Meier Estimate ,Risk Assessment ,Young Adult ,Postoperative Complications ,Risk Factors ,Physiology (medical) ,Internal medicine ,medicine ,Humans ,Young adult ,Proportional Hazards Models ,Heart transplantation ,Proportional hazards model ,business.industry ,Middle Aged ,medicine.disease ,Ventricular assistance ,Transplantation ,Waiting list ,Multivariate Analysis ,Cardiology ,Heart Transplantation ,Female ,Cardiology and Cardiovascular Medicine ,business ,Body mass index - Abstract
Background— An increasing number of patients with congenital heart disease (CHD) are reaching adulthood and may require heart transplantation. The survival of these patients after listing and transplantation has not been evaluated. Methods and Results— A total of 41 849 patients (aged >18 years) were listed for primary transplantation during 1995–2009. Patients with a history of CHD (n=1035; 2.5%) were compared with those with other causes (non-CHD group) (n=40 814; 97.5%); 26 055 (62.3%) reached transplantation and were subdivided into those with (reoperation group; n=10 484; 40.2%) and without (nonreoperation group; n=15 571; 59.8%) a previous sternotomy. Survival on the waiting list was similar between groups, but mechanical ventricular assistance was not associated with superior survival to transplantation among CHD patients. CHD patients were more likely to have body mass index P P P Conclusions— Adults with CHD have high 30-day mortality but better late survival after heart transplantation. Mechanical circulatory assistance does not improve waiting list survival in these patients. This may be due to a combination of highly complex reoperative surgery and often poor preoperative systemic health.
- Published
- 2011
30. USE OF A BIDIRECTIONAL GLENN SHUNT TO ENABLE CLOSURE OF AN ATRIAL SEPTAL DEFECT IN THE SETTING OF RIGHT VENTRICULAR HYPOPLASIA
- Author
-
Jason Costa, Dan G. Halpern, Michael Argilla, Ralph S. Mosca, and Rachel Smaldone
- Subjects
medicine.medical_specialty ,business.industry ,Glenn shunt ,Right ventricular hypoplasia ,Hypoxia (medical) ,Atrial septal defects ,Internal medicine ,mental disorders ,Split S2 ,medicine ,Cardiology ,Physical exam ,In patient ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business - Abstract
Management of atrial septal defects (ASD) in patients with abnormal RV anatomy or function is challenging, as simple ASD closure may not be hemodynamically tolerated. A 34-year-old woman presented with progressive dyspnea. Physical exam revealed hypoxia (SpO2 72%), fixed split S2, and digital
- Published
- 2018
31. Repair of Truncus Arteriosus With Interrupted Aortic Arch
- Author
-
Ralph S. Mosca
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,business.industry ,Internal medicine ,Interrupted aortic arch ,cardiovascular system ,Cardiology ,medicine ,Surgery ,Cardiology and Cardiovascular Medicine ,medicine.disease ,business ,Truncus arteriosus - Published
- 2010
32. Surgical repair in neonatal life of cardiac haemangiomas diagnosed prenatally
- Author
-
Ralph S. Mosca, Yasutaka Hirata, and Jayendra Sharma
- Subjects
Adult ,Male ,medicine.medical_specialty ,Pediatrics ,Prenatal diagnosis ,Pericardial effusion ,Ultrasonography, Prenatal ,Resection ,Diagnosis, Differential ,Heart Neoplasms ,Young Adult ,Pregnancy ,Fetal hydrops ,Humans ,Medicine ,Cardiac Surgical Procedures ,Surgical repair ,business.industry ,General Medicine ,Antenatal ultrasound ,medicine.disease ,Surgery ,Fetal Diseases ,Neonatal life ,Pediatrics, Perinatology and Child Health ,Female ,Good prognosis ,Hemangioma ,Cardiology and Cardiovascular Medicine ,business - Abstract
Although cardiac tumours are rare, such tumours are increasingly being diagnosed with increasing frequency and great accuracy by antenatal ultrasound. Cardiac haemangiomas account for less than one-twentieth of all primary cardiac tumours, with most being diagnosed in the neonatal period. We report 3 instances of successful neonatal resection of cardiac haemangioma subsequent to prenatal diagnosis. Such diagnosis is important in perinatal management, since early surgical intervention provides a good prognosis.
- Published
- 2009
33. Children undergoing heart transplant are at increased risk for postoperative vasodilatory shock*
- Author
-
James Killinger, Charles L. Schleien, Daphne T. Hsu, George E. Hardart, and Ralph S. Mosca
- Subjects
Male ,medicine.medical_specialty ,medicine.medical_treatment ,Critical Care and Intensive Care Medicine ,law.invention ,Postoperative Complications ,Risk Factors ,law ,Intensive care ,Internal medicine ,medicine ,Cardiopulmonary bypass ,Humans ,Prospective Studies ,Child ,Prospective cohort study ,Blood urea nitrogen ,Heart transplantation ,business.industry ,Incidence (epidemiology) ,Infant, Newborn ,Infant ,Shock ,Intensive care unit ,surgical procedures, operative ,Child, Preschool ,Relative risk ,Pediatrics, Perinatology and Child Health ,Cardiology ,Heart Transplantation ,Female ,business - Abstract
OBJECTIVE: To determine the incidence of vasodilatory shock (VDS) in children after cardiopulmonary bypass (CPB), and to describe this syndrome of post-CPB VDS in children. DESIGN: Prospective, observational. SETTING: Pediatric and neonatal intensive care units in a tertiary care, children's hospital. PATIENTS: Three hundred children undergoing CPB. INTERVENTION: None. MEASUREMENTS AND MAIN RESULTS: Three hundred subjects undergoing CPB were evaluated for clinical evidence of VDS following CPB. The incidence of post-CPB VDS was 3%. Characteristics of children who developed VDS: higher peak lactate (6.2 +/- 2.6 vs. 3.0 +/- 2.1 mmol/L; p = 0.0002), higher peak serum blood urea nitrogen (18.5 +/- 4.6 vs. 15.6 +/- 7.2 mg/dL; p = 0.04), lower urine output (1.7 +/- 0.8 vs. 2.6 +/- 0.2 mL/kg/hr; p = 0.04), and fewer intensive care unit free days (14.9 +/- 9.0 vs. 21.1 +/- 7.2 days; p = 0.01). Univariate predictors for the development of post-CPB VDS included children who had heart transplantation (HT) (relative risk [RR], 9.8; 95% confidence interval [CI], 2.7-35.2) or ventricular assist device (VAD) placed (RR, 17.9; 95% CI, 3.8-84.1), a cardiomyopathy diagnosis (RR, 8.5; 95% CI, 2.3-31), age >12 years (RR, 4.5; 95% CI, 1.2-17.0), CPB time >180 minutes (RR, 7.1; 95% CI, 1.9-26.2), and preoperative ventricular dysfunction (RR, 3.7; 95% CI, 1.0-13.4). By stratified analysis, the only independent predictor for the development of VDS was undergoing HT/VAD. CONCLUSIONS: Post-CPB VDS is uncommon in children. However, children who undergo HT or VAD placement are at high risk for developing post-CPB VDS. Recognition that the overall incidence of post-CPB is low-except in the HT/VAD population-may help guide therapy in the pediatric post-CPB patient.
- Published
- 2009
34. Direction of Preoperative Ventricular Shunting Affects Ventricular Mechanics After Tetralogy of Fallot Repair
- Author
-
Jason P. Van Batavia, Joshua P. Kanter, Henry M. Spotnitz, Santos E. Cabreriza, Ralph S. Mosca, Jan M. Quaegebeur, Marc E. Richmond, Alan D. Weinberg, and T. Alexander Quinn
- Subjects
Heart Septal Defects, Ventricular ,medicine.medical_specialty ,Volume overload ,Myocardial Reperfusion Injury ,Article ,law.invention ,Electrocardiography ,Ventricular Dysfunction, Left ,law ,Physiology (medical) ,Internal medicine ,medicine ,Cardiopulmonary bypass ,Humans ,Intraoperative Complications ,Tetralogy of Fallot ,Heart septal defect ,Cardiopulmonary Bypass ,medicine.diagnostic_test ,business.industry ,Infant ,medicine.disease ,Biomechanical Phenomena ,Shunting ,Preload ,medicine.anatomical_structure ,Echocardiography ,Ventricle ,Anesthesia ,Heart Function Tests ,Cardiology ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background— Tetralogy of Fallot (TOF) typically results in clinical cyanosis or volume overload of the left ventricle (LV), depending on the direction and magnitude of shunting across the ventricular septal defect (VSD). The present study examines the effects of surgical TOF repair on LV mechanics and compares these changes between patients with VSD shunts that are predominantly right-to-left (R-L; “blue TOF”) and those with VSD shunts that are predominantly left-to-right (L-R; “pink TOF”). Methods and Results— Eleven patients (6 R-L and 5 L-R) 4.3 to 18.4 months old (median 7.1 months old) were studied. LV end-diastolic area (EDA) was calculated from transesophageal echocardiograms obtained during initiation and weaning of cardiopulmonary bypass. LV end-diastolic pressure was measured by micromanometer. Compliance was assessed by end-diastolic pressure-area curves. Contractility was assessed from preload recruitable stroke work by the stroke work–versus–LV EDA relation. VSD shunt direction was determined by preoperative Doppler echocardiography. Changes in LV function at the conclusion of cardiopulmonary bypass included decreased stroke area (from 6.6±0.9 to 4.1±0.4 cm 2 /m 2 , P =0.012) and ejection fraction (from 55±2% to 41±3%, P 2 /m 2 , P =0.003), which suggests a decrease in ventricular compliance. Additionally, the end-diastolic pressure-area curves shifted to the left in all patients. Preload recruitable stroke work decreased (from 34.8±2.4 to 21.8±2.6 mm Hg, P =0.007), which demonstrates a decrease in ventricular contractility. When separated by preoperative shunt direction, LV EDA increased in R-L patients by 0.9±0.5 cm 2 /m 2 postoperatively but decreased in L-R patients by 4.3±0.8 cm 2 /m 2 ( P Conclusions— LV diastolic and systolic function are depressed after TOF repair. Mechanical effects of the VSD patch and myocardial depressant effects of ischemia and reperfusion during surgery probably contribute to the observed changes in LV mechanics. Different effects of surgical repair on LV preload in pink and blue TOF also contribute to the spectrum of clinical results observed after surgery.
- Published
- 2008
35. Genomic Profiling of Left and Right Ventricular Hypertrophy in Congenital Heart Disease
- Author
-
Sushma Reddy, Ralph S. Mosca, Beth D. Kaufman, Manisha Desai, Seema Mital, Jonathan M. Chen, Juan C Osorio, and Anthony W. Ferrante
- Subjects
Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Adolescent ,Heart disease ,Adrenergic ,Hemodynamics ,Muscle hypertrophy ,Young Adult ,Ventricular hypertrophy ,Internal medicine ,Renin–angiotensin system ,Gene expression ,Humans ,Medicine ,Child ,Oligonucleotide Array Sequence Analysis ,Hypertrophy, Right Ventricular ,business.industry ,Gene Expression Profiling ,Infant, Newborn ,Infant ,Genomics ,medicine.disease ,Echocardiography, Doppler ,medicine.anatomical_structure ,Ventricle ,Child, Preschool ,Cardiology ,Female ,Hypertrophy, Left Ventricular ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background The right ventricle (RV) has a lower ability than the left ventricle (LV) to adapt to systemic load. The molecular basis of these differences is not known. We compared hypertrophy-signaling pathways between the RV and the LV in patients with congenital heart disease (CHD). Methods Gene expression was measured using DNA microarrays in myocardium from children with CHD with LV or RV obstructive lesions undergoing surgery. The expression of 175 hypertrophy-signaling genes was compared between the LV (n = 7) and the RV (n = 11). Hierarchic clustering was performed. Results Seventeen genes (10%) were differentially expressed between the LV and the RV. Expression of genes for angiotensin, adrenergic, G-proteins, cytoskeletal, and contractile components was lower ( P 05) and expression of maladaptive factors (fibroblast growth factors, transforming growth factor-β, caspases, ubiquitin) was higher in the RV compared with the LV ( P 05). Five of 7 LV samples clustered together. Only 4 of 11 RV samples clustered with the LV. Genes critical to adaptive remodeling correlated with the degree of LV hypertrophy but not RV hypertrophy. Conclusion The transcription of pathways of adaptive remodeling was lower in the RV compared with the LV. This may explain the lower ability of the RV to adapt to hemodynamic load in CHD.
- Published
- 2008
36. Twenty-Year Experience With Heart Transplantation for Infants and Children With Restrictive Cardiomyopathy: 1986–2006
- Author
-
Ralph S. Mosca, J. C. Schwarzenberger, Seema Mital, Adam J. Bograd, Jonathan M. Chen, Jacqueline M. Lamour, Jan M. Quaegebeur, Linda J. Addonizio, and Daphne T. Hsu
- Subjects
Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Cardiomyopathy ,Diastole ,Internal medicine ,medicine.artery ,medicine ,Humans ,Immunology and Allergy ,Pharmacology (medical) ,Child ,Retrospective Studies ,Heart transplantation ,Cardiomyopathy, Restrictive ,Transplantation ,business.industry ,Restrictive cardiomyopathy ,Infant ,medicine.disease ,Treatment Outcome ,medicine.anatomical_structure ,Cardiothoracic surgery ,Child, Preschool ,Pulmonary artery ,Cardiology ,Vascular resistance ,Heart Transplantation ,Female ,business - Abstract
Idiopathic restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children notable for severe diastolic dysfunction and progressive elevation of pulmonary vascular resistance (PVR). Traditionally, those with pulmonary vascular resistance indices (PVRI)6 W.U. x m(2) have been precluded from heart transplantation (HTX). The clinical course of all patients transplanted for RCM between 1986 and 2006 were reviewed. Preoperative, intraoperative and postoperative variables were evaluated. A total of 23 patients underwent HTX for RCM, with a mean age of 8.8 +/- 5.6 years and a mean time from listing to HTX of 43 +/- 60 days. Preoperative and postoperative (114 +/- 40 days) PVRI were 5.9 +/- 4.4 and 2.9 +/- 1.5 W.U. x m(2), respectively. At time of most recent follow-up (mean = 5.7 +/- 4.6 years), the mean PVRI was 2.0 +/- 1.0 W.U. x m(2). Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI6 W.U. x m(2) (chi(2)= 7.4, p0.01) were associated with the requirement of ECMO postoperatively. Neither PVRI nor mean PA pressure was associated with posttransplant mortality; 30-day and 1-year actuarial survivals were 96% and 86%, respectively. Five of the seven patients with preoperative PVRI6 W.U. x m(2) survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high-risk patients.
- Published
- 2008
37. Persistent Antegrade Pulmonary Blood Flow Post-Glenn Does Not Alter Early Post-Fontan Outcomes in Single-Ventricle Patients
- Author
-
Karen Altmann, Ralph S. Mosca, Jan M. Quaegebeur, Ashwin Prakash, Robert G. Gray, Ismee A. Williams, and Jonathan M. Chen
- Subjects
Heart Defects, Congenital ,Pulmonary and Respiratory Medicine ,Thorax ,Cardiac Catheterization ,Pulmonary Circulation ,Heart Ventricles ,medicine.medical_treatment ,Hemodynamics ,Fontan Procedure ,medicine.artery ,medicine ,Humans ,Retrospective Studies ,Cardiac catheterization ,Lung ,business.industry ,Respiratory disease ,Infant, Newborn ,Infant ,Blood flow ,medicine.disease ,medicine.anatomical_structure ,Echocardiography ,Ventricle ,Child, Preschool ,Anesthesia ,Pulmonary artery ,Surgery ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background The bidirectional Glenn cavopulmonary anastomosis (BDG) represents the standard interim procedure in treatment of patients with single-ventricle physiology. Anterograde pulmonary blood flow (APBF) maintained after BDG has been shown both to improve and to complicate postoperative clinical course. We studied its effects on outcome after BDG and eventual Fontan completion. Methods From November 1995 to November 2005, 60 patients underwent BDG and Fontan. All patients had APBF from the ventricle to the pulmonary artery at time of BDG. In group 1 (n = 39) APBF was maintained after BDG, whereas APBF was interrupted at BDG in group 2 (n = 21). Cardiac catheterization data, interstage morbidity, and postoperative outcome variables were recorded. Results Pre-BDG hemodynamics differed only in that the mean pulmonary artery pressure was higher in group 2 (17.0 ± 4.4 mm Hg) than in group 1 (13.8 ± 4.5 mm Hg; p = 0.03). There were no differences between groups 1 and 2 in BDG outcome variables. At pre-Fontan catheterization, group 1 had higher mean pulmonary artery pressure (13.3 versus 10.9 mm Hg, p = 0.01), arterial oxygen saturation (85.8 versus 80.9%, p = 0.0001), and fewer collateral vessels were coil embolized than in group 2 (0.9 versus 1.6, p = 0.02). Mean ventricular end-diastolic pressure was similar between groups. The Nakata index in group 1 remained stable from pre-BDG to pre-Fontan (348 versus 391, p = 0.24), but it decreased in group 2 (375 versus 227, p = 0.046). Conclusions Patients with anterograde pulmonary blood flow after BDG had a modest increase in pulmonary artery growth and arterial oxygen saturations, and decreased collateral vessel formation. This did not, however, confer additional benefit on outcome after BDG or on eventual Fontan completion.
- Published
- 2007
38. 180 ml and less: Cardiopulmonary bypass techniques to minimize hemodilution for neonates and small infants
- Author
-
Ralph S. Mosca, Adam J. Bograd, Kevin Charette, Jan M. Quaegebeur, Yasutaka Hirata, Linda B. Mongero, and Jonathan M. Chen
- Subjects
Heart Defects, Congenital ,Oxygenators ,Blood volume ,030204 cardiovascular system & hematology ,Prime (order theory) ,law.invention ,Venous line ,03 medical and health sciences ,Postoperative Complications ,0302 clinical medicine ,law ,Cardiopulmonary bypass ,Body Size ,Humans ,Medicine ,Radiology, Nuclear Medicine and imaging ,Polyvinyl Chloride ,Oxygenator ,Infusion Pumps ,Retrospective Studies ,Advanced and Specialized Nursing ,Hemodilution ,Blood Volume ,Cardiopulmonary Bypass ,business.industry ,Infant, Newborn ,Infant ,General Medicine ,030228 respiratory system ,Anesthesia ,Arterial line ,Cardiology and Cardiovascular Medicine ,Packed red blood cells ,business ,Safety Research - Abstract
Objective. To determine the efficacy of decreasing cardiopulmonary bypass (CPB) prime volume for neonates and small infants by using low prime oxygenators, small diameter polyvinyl chloride (PVC) tubing and removing the arterial line filter (ALF) in an effort to reduce intraoperative exposure to multiple units of packed red blood cells (PRBC). Methods. Two retrospective database studies comparing neonatal CPB prime volume were undertaken: Study 1 — A CPB circuit consisting of a 1/8 inch arterial line, a 3/16 inch venous line and a low prime oxygenator with 172 ml total circuit prime ( n = 74) was compared to a circuit with a 3/16 inch arterial line, a 1/4 inch venous line and a higher prime oxygenator with a 350 ml total circuit prime ( n = 74). Study 2 — The 172 ml circuit ( n = 389) was compared to a circuit that included an ALF and had a total circuit prime volume of 218 ml ( n = 389). Results. Study 1— of the 74 neonates and small infants whose CPB prime volume was 350 ml, 19 were exposed to two or more intraoperative exogenous PRBC units while only 3 neonates and small infants in the 172 ml prime group ( n = 74) received two or more units ( p = 0.0002). Study 2 — of the 389 neonates and small infants where an ALF was used (prime volume 218 ml), 54 were exposed to two or more exogenous PRBC units while only 36 of the 389 patients where an ALF was not used (prime volume 172 ml) received two or more units of intraoperative PRBCs ( p = 0.0436). Conclusion. Decreasing the neonatal and small infant extracorporeal circuit prime volume by as little as 46 ml resulted in significantly fewer multiple exposures to exogenous PRBC units. Perfusion (2007) 22, 327—331.
- Published
- 2007
39. Bidirectional Cavopulmonary Anastomosis: Impact on Diastolic Ventricular Function Indices
- Author
-
Jan M. Quaegebeur, Karen Altmann, Julie S. Glickstein, David E. Solowiejczyk, Charles S. Kleinman, E. S. Selamet Tierney, Beth F. Printz, and Ralph S. Mosca
- Subjects
Male ,medicine.medical_specialty ,Diastole ,law.invention ,law ,Internal medicine ,Heart rate ,Image Processing, Computer-Assisted ,medicine ,Cardiopulmonary bypass ,Humans ,Ventricular Function ,Postoperative Period ,Echocardiography, Doppler, Pulsed ,Ventricular function ,business.industry ,Heart Bypass, Right ,Infant ,Cavopulmonary Anastomosis ,Cardiac surgery ,medicine.anatomical_structure ,Ventricle ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Cardiology ,End-diastolic volume ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Systolic ventricular function has been demonstrated to remain unchanged following bidirectional cavopulmonary anastomosis (BCPA). The effects of BCPA on diastolic ventricular performance have not been critically assessed. The objective of this study was to evaluate the changes in diastolic ventricular function indices early after BCPA. Nineteen patients were enrolled prospectively. Transthoracic echocardiograms were performed at a median of 4 days prior to and 5 days subsequent to BCPA. Diastolic and systolic echocardiographic indices of ventricular performance were measured for the dominant ventricle. End diastolic volume decreased postoperatively (71.1 +/- 21.1 vs 68.08 +/- 17.9 ml/m2, p = 0.05). Tei index increased postoperatively (0.51 +/- 0.2 vs 0.62 +/- 0.1, p = 0.002), whereas inflow Doppler E velocity (70.3 +/- 13 vs 56.3 +/- 24.7 cm/sec, p = 0.04), E/A ratio (1.18 +/- 0.52 vs 0.84 +/- 0.2, p = 0.02), tissue Doppler E' velocity (9.5 +/- 2.5 vs 6.4 +/- 3.2 cm/sec, p = 0.03) and diastolic flow propagation velocity (56.5 +/- 12 vs 52.8 +/- 11 cm/sec, p = 0.04) all decreased. There was no change in ventricular mass, area change fraction, heart rate, or inflow Doppler A or tissue Doppler A' and S' velocities. This study demonstrated that diastolic indices of ventricular performance are altered indicating decreased diastolic function early following BCPA. Whether this observation is a result of a change in ventricular mass:volume ratio, loading conditions of the ventricle, ventricular geometry, or the effects of cardiopulmonary bypass remains to be determined.
- Published
- 2007
40. Cardiac retransplantation in high risk pediatric patients
- Author
-
Seema Mital, Daphne T. Hsu, Jonathan M. Chen, Jan M. Quaegebeur, Linda J. Addonizio, Ralph S. Mosca, Jacqueline M. Lamour, and Marc E. Richmond
- Subjects
Heart transplantation ,Transplantation ,medicine.medical_specialty ,Allograft failure ,business.industry ,medicine.medical_treatment ,Panel reactive antibody ,Retrospective cohort study ,Surgery ,Impaired renal function ,El Niño ,Pediatrics, Perinatology and Child Health ,medicine ,Graft survival ,business - Abstract
Cardiac retransplantation is often the only therapy to treat GV or other causes of allograft failure. Previous reports of retransplantation have conflicting results. In this series of 18 re-transplants in 16 patients from 1984-2005, indications for retransplantation were: GV (67%); GV with cellular rejection (28%); acute graft failure (2.5%); and chronic graft failure (2.5%). Mean age at retransplantation was 12.3 (range: 0.7-22) years with a mean primary graft survival of 5.3 years (range: 8 days-10.5 years). There was no short-term mortality with only three deaths at 4, 10, and 16 years post-retransplantation. Fourteen of 18 patients had risk factors for adverse outcomes following retransplantation: ECMO support in one patient prior to retransplantation; impaired renal function in three patients; elevated panel reactive antibody screen in seven patients; a history of PTLD in five patients; and a recent episode of rejection (13-36 days) in four patients. One-, five- and ten-year survival after retransplantation was 100%, 83% and 66%, respectively, comparable to survival after primary transplantation. Freedom from rejection was not significantly different between primary and retransplantations. All patients who underwent treatment for PTLD had excellent results after retransplantation with one recurrence 16 months after retransplant. Overall, patients had excellent survival after retransplantation even in those with risk factors for poor outcome.
- Published
- 2007
41. Hybrid therapy for hypoplastic left heart syndrome: Myth, alternative, or standard--neither Minotaur nor Midas
- Author
-
Ralph S. Mosca
- Subjects
Pulmonary and Respiratory Medicine ,Pediatrics ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,030204 cardiovascular system & hematology ,medicine.disease ,Fontan Procedure ,Norwood Procedures ,Hypoplastic left heart syndrome ,Surgery ,Fontan procedure ,03 medical and health sciences ,0302 clinical medicine ,030228 respiratory system ,Hypoplastic Left Heart Syndrome ,Medicine ,Humans ,Norwood procedure ,Cardiology and Cardiovascular Medicine ,business - Published
- 2015
42. Anomalous Aortic Origin of a Coronary Artery Is Not Always a Surgical Disease
- Author
-
Colin K.L. Phoon and Ralph S. Mosca
- Subjects
medicine.medical_specialty ,Coronary Vessel Anomalies ,Disease ,030204 cardiovascular system & hematology ,Sudden cardiac death ,Lesion ,03 medical and health sciences ,Incomplete knowledge ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,030212 general & internal medicine ,Sinus (anatomy) ,business.industry ,Patient Selection ,Sinus of Valsalva ,medicine.disease ,Surgery ,Natural history ,medicine.anatomical_structure ,Death, Sudden, Cardiac ,Anomalous aortic origin of a coronary artery ,Pediatrics, Perinatology and Child Health ,Cardiology ,Stents ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Artery - Abstract
Anomalous aortic origin of the coronary artery (AAOCA) from the opposite sinus of Valsalva with an interarterial course has become a high-profile lesion as a result of its association with sudden cardiac death in otherwise young and healthy individuals. Despite our incomplete knowledge of its pathophysiology and natural history, surgical intervention is often recommended. Evidence now shows AAOCA to be relatively common, with lower than previously suspected rates of sudden cardiac death. Analysis of this information reveals that AAOCA is not always a surgical disease. Future multi-institutional studies will continue to define those subgroups best served by observation or surgery.
- Published
- 2015
43. Pulmonary valve replacement after repair of tetralogy of Fallot: Evolving strategies
- Author
-
Ralph S. Mosca
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Palliative care ,Treatment outcome ,MEDLINE ,030204 cardiovascular system & hematology ,Prosthesis Design ,03 medical and health sciences ,0302 clinical medicine ,Pulmonary Valve Replacement ,medicine ,Prosthesis design ,Humans ,Cardiac Surgical Procedures ,Tetralogy of Fallot ,Prosthetic valve ,Heart Valve Prosthesis Implantation ,Pulmonary Valve ,business.industry ,Palliative Care ,medicine.disease ,Pulmonary Valve Insufficiency ,Surgery ,Treatment Outcome ,030228 respiratory system ,Heart Valve Prosthesis ,Cardiology and Cardiovascular Medicine ,business - Published
- 2015
44. TGA/VSD/LVOTO: Evolution of surgical therapy
- Author
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Ralph S. Mosca
- Subjects
Pulmonary and Respiratory Medicine ,Heart Septal Defects, Ventricular ,Heart Valve Prosthesis Implantation ,medicine.medical_specialty ,business.industry ,Transposition of Great Vessels ,Surgery ,Blood Vessel Prosthesis ,Pulmonary Valve Stenosis ,Surgical therapy ,Blood Vessel Prosthesis Implantation ,Heart Valve Prosthesis ,Medicine ,Humans ,Abnormalities, Multiple ,Cardiology and Cardiovascular Medicine ,business - Published
- 2015
45. Adult-age donors offer acceptable long-term survival to pediatric heart transplant recipients: An analysis of the United Network of Organ Sharing database
- Author
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Mark J. Russo, Robert Sorabella, Ralph S. Mosca, Faisal H. Cheema, Timothy P. Martens, Seema Mital, Jonathan M. Chen, Ryan R. Davies, and Isaac George
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Adult ,Male ,Pulmonary and Respiratory Medicine ,United Network for Organ Sharing ,Thorax ,medicine.medical_specialty ,Pediatrics ,Adolescent ,MEDLINE ,Adult age ,Long term survival ,medicine ,Humans ,Child ,Intensive care medicine ,Survival analysis ,business.industry ,Patient Selection ,Age Factors ,Middle Aged ,Survival Analysis ,Tissue Donors ,Transplantation ,El Niño ,Heart Transplantation ,Female ,Surgery ,Cardiology and Cardiovascular Medicine ,business - Abstract
ObjectivesA critical shortage of donor organs has caused many centers to use less restrictive donor criteria, including the use of adult-age donors for pediatric recipients. The purpose of this study is (1) to describe the supply of pediatric (0-18 years) heart donors, (2) to explore the relationship between donor age and long-term survival, and (3) to define threshold age ranges associated with decreased long-term survival.MethodsThe United Network of Organ Sharing provided deidentified patient-level data. Primary analysis focused on 1887 heart transplant recipients aged 9 to 18 years undergoing transplantation from October 1, 1987, to September 25, 2005. Kaplan-Meier analysis and log-rank tests were used in time-to-event analysis. Receiver operating characteristic curves and stratum-specific likelihood ratios were generated to compare survival at various donor age thresholds.ResultsThe number of pediatric donors decreased (P < .001) over the study period, particularly from 1993 (n = 640) through 2004 (n = 432). Among recipients aged 9 to 18 years, univariate analysis demonstrated a statistically significant (P < .001) inverse relationship between donor age and survival. Stratum-specific likelihood ratio analysis generated 3 strata for donor age: the low-risk, intermediate-risk, and high-risk groups consisted of donors aged 13 years or younger (n = 611, 32.41%), 14 to 51 years (n = 1258, 66.7%), and 52 years and older (n = 16, 0.85%), respectively. In the low-risk, intermediate-risk, and high-risk groups median survival was 4069 days (11.1 years), 3495 days (9.57 years), and 1197 days (3.28 years), respectively.ConclusionsAlthough donors aged 13 years or less offer pediatric recipients the best chance for achieving long-term survival, donors aged 14 to 51 years offer good outcomes to pediatric recipients. Consideration should be given to expanded use of well-selected adult-age donors for pediatric recipients.
- Published
- 2006
46. Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome
- Author
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Jan M. Quaegebeur, Ralph S. Mosca, Julie S. Glickstein, William E. Hellenbrand, Jonathan M. Chen, Renee Margossian, and Michelle L. Mercando
- Subjects
Male ,Reoperation ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,medicine.medical_treatment ,Pulmonary insufficiency ,Angioplasty ,medicine.artery ,Humans ,Medicine ,Tetralogy of Fallot ,Pulmonary Valve ,Respiratory distress ,business.industry ,Infant, Newborn ,Infant ,Recovery of Function ,Perioperative ,medicine.disease ,Survival Analysis ,Surgery ,Catheter ,Treatment Outcome ,medicine.anatomical_structure ,Child, Preschool ,Pulmonary valve ,Pulmonary artery ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Objective Primary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle–pulmonary artery continuity. Methods Anatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality). Results Twenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 ± 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function. Conclusions We report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle–pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.
- Published
- 2006
47. Truncus Arteriosus Associated with Interrupted Aortic Arch in 50 Neonates: A Congenital Heart Surgeons Society Study
- Author
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Andrew S. Mackie, Gary K. Lofland, Christopher A. Caldarone, Brian W. McCrindle, Eugene H. Blackstone, Tara Karamlou, William G. Williams, Ralph S. Mosca, and Igor E. Konstantinov
- Subjects
Heart Septal Defects, Ventricular ,Male ,Reoperation ,Pulmonary and Respiratory Medicine ,Aortic arch ,medicine.medical_specialty ,Persistent truncus arteriosus ,Aorta, Thoracic ,Comorbidity ,Constriction, Pathologic ,Pulmonary Artery ,Cohort Studies ,Ductus arteriosus ,Internal medicine ,medicine.artery ,Prevalence ,medicine ,Humans ,Abnormalities, Multiple ,Prospective Studies ,Ductus Arteriosus, Patent ,Societies, Medical ,Subclavian artery ,Aorta ,Arterial trunk ,business.industry ,Interrupted aortic arch ,Infant, Newborn ,Infant ,Thoracic Surgery ,medicine.disease ,Combined Modality Therapy ,Heart Valves ,Respiration, Artificial ,Survival Analysis ,Truncus Arteriosus, Persistent ,Surgery ,Survival Rate ,Transplantation ,Treatment Outcome ,medicine.anatomical_structure ,Prostaglandins ,Cardiology ,Heart Transplantation ,Female ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Background. Patients with both interrupted aortic arch (IAA) and truncus arteriosus (TA) have worse outcomes than those with either lesion in isolation. We determined outcomes and associated factors in this rare group. Methods. From 1987 to 1997, 50 (11%) of 472 neonates with IAA were identified with TA. Site of aortic arch interruption was distal to the left subclavian artery in 16% and between the left common carotid and subclavian artery in 84%. From the common arterial trunk, the pulmonary arteries arose from a main pulmonary trunk in 46%, common orifice in 22%, and separate orifices in 32%. At presentation, truncal valve stenosis was present in 12% and regurgitation in 22%. Results. There were 34 deaths, with a single early hazard phase. Overall survival from admission was 44%, 39%, and 31% at 6 months, 1 year, and 10 years, respectively. One patient had primary cardiac transplantation and 4 died without any intervention. The IAA repair alone was performed in 7 patients, with single stage repair of both IAA and TA in 38 patients. Associated factors for overall time-related death include female gender (p < 0.001), type III TA (p < 0.001) and one institution (low-risk; p < 0.001). Results improved somewhat over time (p < 0.001). At 5 years after IAA repair only 28% were alive without arch repair intervention, and at 5 years after TA repair only 18% were alive without conduit reoperation. Conclusions. The combination of IAA and TA carries high early mortality, with high risk of reinterventions in survivors. One stage repair of both TA and IAA is the optimal management.
- Published
- 2006
48. Early Complete Atrioventricular Canal Repair Yields Outcomes Equivalent to Late Repair
- Author
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Anjali Chelliah, Paul J. Chai, Elizabeth H. Stephens, Emile A. Bacha, Ralph S. Mosca, Halit Yerebakan, Jan M. Quaegebeur, Ali Ibrahimiye, Betul Yilmaz, Stéphanie Levasseur, and Jonathan M. Chen
- Subjects
Pulmonary and Respiratory Medicine ,Male ,medicine.medical_specialty ,Time Factors ,New York ,Ventricular outflow tract obstruction ,law.invention ,Postoperative Complications ,law ,medicine ,Cardiopulmonary bypass ,Humans ,Cardiac Surgical Procedures ,Retrospective Studies ,Atrioventricular valve ,business.industry ,Mortality rate ,Incidence ,Infant, Newborn ,Infant ,Retrospective cohort study ,Perioperative ,medicine.disease ,Surgery ,Survival Rate ,Treatment Outcome ,Echocardiography ,Atrioventricular canal ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,Trisomy ,business ,Endocardial Cushion Defects ,Follow-Up Studies - Abstract
Background Repair of complete atrioventricular canal early in infancy has traditionally carried greater morbidity and mortality than repair performed later. However, an individualized anatomy-based repair may give young infants outcomes that are equivalent to older patients. Methods We retrospectively reviewed 139 patients who underwent complete atrioventricular canal repair from January 2005 to December 2012. An individualized approach was used: 2-patch repair was performed in 98 patients for large ventricular septal defects and a modified single-patch ("Australian technique") was used in 41 for "shallow" ventricular septal defects. Results The average age was 25.5 ± 3.9 weeks, 50% were boys, and 78% had trisomy 21. Mean follow-up was 5.1 ± 0.2 years, with 100% completeness of data. There were 3 in-hospital deaths (2.1%) and 1 late death (0.7%). A permanent pacemaker was required in 2 patients (1.4%). The rate for left atrioventricular valve reoperation was 8% at a mean of 211 ± 238 days after the original repair (range, 6 to 682 days). Compared with patients aged older than 3 months, the 39 patients (28%) who were younger than 3 months had similar perioperative courses and rate of reoperation. Compared with patients with an Australian repair, the 98 patients (71%) with a 2-patch repair were more likely to have trisomy 21 and had slightly increased cardiopulmonary bypass and cross-clamp times but similar outcomes. Multivariate analysis showed postoperative left atrioventricular valve regurgitation greater than 2 and left ventricular outflow tract obstruction were significant risk factors for reoperation on the left atrioventricular valve (both p Conclusions Repair of complete atrioventricular canal using an individualized surgical approach yields reoperation and early mortality rates similar for younger infants compared with older infants, obviating the need to delay operation in symptomatic patients.
- Published
- 2014
49. Pulmonary position cryopreserved homografts: Durability in pediatric Ross and non-Ross patients
- Author
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Karen Altmann, Welton M. Gersony, Chava Khan, David E. Solowiejczyk, Ralph S. Mosca, Ehud Krongrad, Elif Seda Selamet Tierney, Jan M. Quaegebeur, Howard D. Apfel, and Laura Bevilacqua
- Subjects
Pulmonary and Respiratory Medicine ,Thorax ,medicine.medical_specialty ,Ventricular Outflow Obstruction ,Blood Vessel Prosthesis Implantation ,Risk Factors ,Blood vessel prosthesis ,parasitic diseases ,Humans ,Medicine ,In patient ,Child ,Lung ,Potential risk ,business.industry ,Graft Survival ,Graft Occlusion, Vascular ,Infant, Newborn ,Infant ,Implant failure ,Blood Vessel Prosthesis ,Prosthesis Failure ,Surgery ,body regions ,Treatment Outcome ,surgical procedures, operative ,medicine.anatomical_structure ,El Niño ,Child, Preschool ,Cardiology and Cardiovascular Medicine ,business ,Artery - Abstract
Objective The purpose of this study was to evaluate the outcome and risk factors for implant failure in pediatric patients who underwent pulmonary position homograft placement for right ventricular outflow tract obstruction compared with conduit placement as a component of the Ross operation. Actuarial 5-year survivals for cryopreserved right ventricle-to-pulmonary artery homografts range from 55% to 94% at all ages. It is not known whether there is a difference in homograft durability when utilized for right ventricular outflow tract obstruction or as part of the Ross operation. Methods The records of all pediatric patients receiving a right ventricle-to-pulmonary artery homograft from July 1989 through October 2003 were reviewed. Ninety-eight consecutive patients were studied (26 Ross, 72 non-Ross). In addition to Ross versus non-Ross comparisons, other potential risk factors for homograft failure analyzed included age at operation, follow-up time, type of surgery, and homograft type and size. Results Ross and non-Ross patients were comparable in age at the time of the operation and follow-up time. Homograft failure rates were 12% and 51% for Ross and non-Ross patients, respectively. Freedom from reintervention was 93% in the Ross and 66% in the non-Ross group at 5 years (P = .019). On multivariate analysis, non-Ross operation and age less than 2 years were significant predictors of homograft failure. Conclusions 1. Pediatric patients undergoing the Ross operation have longer homograft survival than pediatric patients treated for right ventricular outflow tract obstruction, independent of age. 2. Homografts placed in patients less than 2 years of age have shorter homograft survival.
- Published
- 2005
50. The effect of repair technique on postoperative right-sided obstruction in patients with truncus arteriosus
- Author
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Ralph S. Mosca, Jan M. Quaegebeur, Ryan R. Davies, Michelle L. Mercando, Jonathan M. Chen, William E. Hellenbrand, and Julie S. Glickstein
- Subjects
Thorax ,Reoperation ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Persistent truncus arteriosus ,Pulmonary Artery ,law.invention ,law ,Hypothermia, Induced ,medicine.artery ,Cardiopulmonary bypass ,medicine ,Ventricular outflow tract ,Humans ,Cardiac Surgical Procedures ,Retrospective Studies ,Cardiopulmonary Bypass ,business.industry ,Infant, Newborn ,Infant ,Retrospective cohort study ,Perioperative ,medicine.disease ,Truncus Arteriosus, Persistent ,Surgery ,Catheter ,Treatment Outcome ,Pulmonary Veins ,Anesthesia ,Pulmonary artery ,business ,Cardiology and Cardiovascular Medicine - Abstract
Objectives We reviewed our experience with repair of truncus arteriosus to assess the effect of type of right ventricular outflow tract reconstruction on perioperative morbidity, survival, and freedom from catheter-based interventions and reoperation. Methods Patients undergoing repair of truncus arteriosus from June 1990 through February 2004 were evaluated on the basis of operative procedure regarding preoperative and postoperative variables, the need for postoperative catheter-based intervention or reoperation, and survival on the basis of univariate, multivariable, and actuarial analyses. Results Of 54 study patients, 15 (28%) received a valved homograft, and 39 (72%) received a direct connection with a variety of hood materials. Five (9.1%) patients died. Valved homograft recipients were more likely to require reoperation than patients receiving direct connections (40% vs 15%, P = .046); however, valved homograft and direct connection recipients had a similar incidence of the combined end point of reoperation or catheter-based intervention (40.0% vs 37.5%, P = .865). Univariate and multivariable modeling demonstrated use of valved homografts or direct connections with an autologous pericardial hood to be predictive of the need for later catheter-based intervention or reoperation. Actuarial analysis demonstrated a trend toward improved outcomes in the direct connection group and within the direct connection cohort, a statistically significant difference on the basis of hood type. Conclusions Although the direct connection technique might not prevent later catheter-based intervention, it does reduce the need for reoperation. Outcomes among direct connection recipients were associated with hood type: polytetrafluoroethylene hoods (W. L. Gore & Associates, Inc, Tempe, Ariz) had the lowest rate of reintervention, and untreated autologous pericardial hoods had the highest rate of reintervention. We report excellent outcomes with primary repair of truncus arteriosus. Where anatomically appropriate, we advocate the direct connection technique.
- Published
- 2005
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