57 results on '"Derek G. Human"'
Search Results
2. Increasing age and atrial arrhythmias are associated with increased thromboembolic events in a young cohort of adults with repaired tetralogy of Fallot
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J. Grewal, Amanda Barlow, Derek G. Human, Darryl Wan, Santabhanu Chakrabarti, Clara Tsui, and Marla Kiess
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thromboembolic events ,medicine.medical_specialty ,business.industry ,atrial arrhythmia ,Atrial arrhythmias ,Original Articles ,medicine.disease ,congenital heart disease ,Internal medicine ,RC666-701 ,Cohort ,medicine ,Cardiology ,Diseases of the circulatory (Cardiovascular) system ,Original Article ,cardiovascular diseases ,tetralogy of Fallot ,Cardiology and Cardiovascular Medicine ,business ,Tetralogy of Fallot - Abstract
Background Adults with repaired Tetralogy of Fallot (rTOF) comprise one of the largest cohorts among adults with congenital heart disease (ACHD). These patients have a higher burden of atrial arrhythmias (AA), leading to increased adverse events, including stroke and transient ischemic attack (TIA). However, the data on factors associated with stroke/TIA in rTOF are limited, and classic risk factors may not apply. We studied event rates and associated factors for thromboembolism in a rTOF cohort. Methods Retrospective cohort study of all adult patients age >18 years with rTOF followed at a single ACHD tertiary care center. AA of interest were atrial fibrillation (AF) and atrial flutter (AFL). Results Data from 260 patients were identified, mean age 37.6 SD 13.3 years, followed over 5108 patient‐years (mean 16.6 SD 8.2 years). 43 patients had AF and/or AFL, and 30 patients had thromboembolic events, of which 19 patients had stroke/TIA. The event rate for any thromboembolism was 3.39 per 100 patient‐years follow‐up in patients with AA, compared to 1.80 in patients without (P = .07). In univariate analysis, older age and diabetes were associated with thromboembolic events. In multivariate analysis, only older age was associated with thromboembolic events. Conclusions In our relatively young cohort of adults with rTOF, there was a high prevalence of AA, associated with nearly double the rate of thromboembolic events compared to patients without AA. Older age alone is independently associated with thromboembolic events. Further studies into assessment of silent AA are required, and routine assessments should be considered at an earlier age., There is a high prevalence of atrial arrhythmias in our relatively young cohort of adults with repaired Tetralogy of Fallot. Atrial arrhythmias are associated with twofold increase in risk of thromboembolic events. Older age was identified as a key risk factor, and traditional risk factors were not applicable.
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- 2021
3. Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic Disease
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Derek G. Human, Zachary Laksman, Amanda J. Barlow, Gnalini Sathananthan, Drake A. Comber, Praveen Indraratna, and Ashley DeGraaf
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medicine.medical_specialty ,Disease entity ,animal structures ,business.industry ,Overlap syndrome ,Case Report ,medicine.disease ,Aortic disease ,Sudden cardiac death ,Increased risk ,Internal medicine ,Concomitant ,medicine ,Cardiology ,cardiovascular system ,Mitral valve prolapse ,Mitral annulus ,cardiovascular diseases ,Cardiology and Cardiovascular Medicine ,business - Abstract
Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD. This case series describes 3 patients in an adult HAD clinic who have concomitant mitral valve prolapse, MAD, and malignant arrhythmias. These cases may represent a unique disease entity or overlap syndrome, and they introduce MAD as a potential arrhythmogenic risk marker in HAD.
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- 2021
4. Outcomes related to respiratory syncytial virus with an abbreviated palivizumab regimen in children with congenital heart disease: a descriptive analysis
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Lana Shaiba, Pascal M. Lavoie, Cheryl Christopherson, Derek G. Human, Constantin R. Popescu, Richard S. Taylor, Jennifer Claydon, and Alfonso Solimano
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Palivizumab ,education.field_of_study ,Pediatrics ,medicine.medical_specialty ,Heart disease ,business.industry ,Research ,Population ,General Medicine ,medicine.disease ,Regimen ,Interquartile range ,Lower respiratory tract infection ,Cohort ,medicine ,business ,education ,medicine.drug ,Cohort study - Abstract
Background It has been hypothesized that 4 doses of palivizumab, a neutralizing monoclonal antibody against respiratory syncytial virus (RSV), administered during a fixed-date RSV season may reduce hospital admissions comparably to the standard 5-dose schedule. We report outcomes in children with congenital heart disease approved to receive this 4-dose palivizumab schedule in British Columbia. Methods We performed a population-based descriptive cohort analysis of all 406 approved palivizumab courses over 4 seasons (2012/13 to 2015/16) in 325 children with hemodynamically significant congenital heart disease enrolled in the British Columbia RSV Immunoprophylaxis Program. The primary outcome was in-season hospital admission for potential RSV-related lower respiratory tract infection (LRTI). Secondary outcomes include timing of admission in relation to dosing. Analysis was by intention-to-treat. Results Of the 406 approved palivizumab courses, 391 were administered. In 33 cases (8.4%), an additional dose was given immediately after cardiac bypass surgery. There were 17 RSV-confirmed hospital admissions (median age of children 5.9 mo [interquartile range 4-10 mo]) and 8 admissions in which the child was not tested for RSV, for a maximum of 25 potential RSV-related admissions (6.2 per 100 approvals [95% confidence interval 4.0-9.0]). Twenty-four (96%) of the 25 admissions occurred within the 4-dose palivizumab dosing period, and the remaining admission occurred 52 days after the fourth dose. Sixty-four (72%) of 89 admissions were RSV-negative; the baseline clinical characteristics of these children were not different from those of children with RSV-confirmed admissions. Interpretation In infants with hemodynamically significant congenital heart disease, a 4-dose fixed-date palivizumab schedule over a 6-month season provided seasonal protection comparable to that in a clinical trial involving a standard 5-dose schedule. Because RSV was responsible for only 19% of admissions for LRTI in our cohort, it is critical to continue to emphasize other preventive measures, including family education toward proper hand hygiene, breast-feeding and limiting infectious exposures in children at high risk.
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- 2019
5. Coronary artery intimal thickening and ventricular dynamics in pediatric heart transplant recipients
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Derek G. Human, George G.S. Sandor, Martin C.K. Hosking, Christine Voss, Kevin C. Harris, and Anita T. Cote
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Male ,medicine.medical_specialty ,Adolescent ,Heart Ventricles ,medicine.medical_treatment ,Diastole ,Lumen (anatomy) ,Coronary Artery Disease ,030204 cardiovascular system & hematology ,Coronary Angiography ,Ventricular Function, Left ,Coronary artery disease ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,030212 general & internal medicine ,Child ,Retrospective Studies ,Heart transplantation ,business.industry ,Stroke Volume ,General Medicine ,Stroke volume ,medicine.disease ,Coronary Vessels ,Echocardiography, Doppler ,Transplant Recipients ,Transplantation ,medicine.anatomical_structure ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Cardiology ,Heart Transplantation ,Female ,Surgery ,Thickening ,Cardiology and Cardiovascular Medicine ,business ,Tomography, Optical Coherence ,Follow-Up Studies ,Artery - Abstract
Objective Pediatric heart transplant recipients are at risk of posttransplant coronary artery disease known as cardiac allograft vasculopathy (CAV), and also may develop diastolic dysfunction. As CAV begins with a process of progressive intimal thickening, these occult diffuse changes may be detected using optical coherence tomography (OCT). We hypothesized that the development of CAV, as identified via OCT, may be a mechanism of declining ventricular function. Accordingly, the purpose of this study was to assess coronary artery intimal thickening and LV strain in children who have undergone heart transplantation. Methods In 17 children, we analyzed OCT images for coronary intima and media thickness, and cross-sectional area (CSA). We also performed speckle tracking imaging (STI) of the LV to determine longitudinal strain and strain rate, in addition to standard echocardiographic measures. Results Longitudinal diastolic strain rate was associated with maximum intima thickness (r = -.497, P = .042), intima CSA, (r = -.489, P = .047), maximum media thickness (r = -.503, P = .039), and media CSA (r = -.614, P = .009). The intima maximum thickness, intima/media, and intima/lumen ratios were associated with stroke volume index (Std. β = -0.487, P = .023 and Std. β = -0.488, P = .022, respectively). Conclusions These findings suggest coronary artery intimal thickening may be mechanistically linked to changes in ventricular function following cardiac transplantation.
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- 2018
6. Clinical Importance of Fontan Circuit Thrombus in the Adult Population: Significant Association With Increased Risk of Cardiovascular Events
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Tony Verma, Jonathon Leipsic, Derek G. Human, S. Chakrabarti, M. Riahi, Gnalini Sathananthan, Jasmine Grewal, Niall Johal, and Sherry Sandhu
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Adult ,Heart Defects, Congenital ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Magnetic Resonance Imaging, Cine ,Kaplan-Meier Estimate ,030204 cardiovascular system & hematology ,Fontan Procedure ,Risk Assessment ,Fontan procedure ,Cohort Studies ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Internal medicine ,Cause of Death ,Thromboembolism ,medicine ,Ventricular Dysfunction ,Humans ,cardiovascular diseases ,030212 general & internal medicine ,Thrombus ,Cause of death ,Retrospective Studies ,Heart transplantation ,medicine.diagnostic_test ,British Columbia ,business.industry ,Magnetic resonance imaging ,Retrospective cohort study ,Middle Aged ,medicine.disease ,Prognosis ,Survival Analysis ,surgical procedures, operative ,Echocardiography ,Heart failure ,Circulatory system ,cardiovascular system ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Pulmonary Embolism ,human activities - Abstract
Background The impact of Fontan circuit thrombus is poorly understood. The objectives of this study were to determine (1) the incidence of Fontan circuit thrombus and proportion of silent thrombus; (2) any association between Fontan circuit thrombus and markers of Fontan circulatory dysfunction; and (3) the association of Fontan circuit thrombus with adverse cardiac outcomes. Methods We conducted a retrospective review of adult patients who underwent the Fontan procedure (aged > 18 years) followed at St. Paul’s Hospital who underwent cardiac computed tomography or magnetic resonance imaging assessment (n = 67). Fontan circulatory dysfunction markers included clinical heart failure, N-terminal pro-brain natriuretic peptide, ventricular dysfunction, atrioventricular valvular regurgitation, refractory arrhythmias, declining exercise capacity, and hepatic/renal dysfunction. Adverse cardiac outcomes were death, heart transplantation, or surgery for Fontan revision or atrioventricular valve replacement. Results Fontan circuit thrombus was present in 15 of 67 patients (22%): 41% (7/17) classic/modified Fontan and 16% (8/50) total cavopulmonary connection. Incidence was 36% among those suspected to have Fontan circuit thrombus; 14% in those with no clinical/echocardiographic suspicion; and clinically silent in 40% diagnosed with Fontan thrombus. The time from Fontan surgery to Fontan circuit thrombus diagnosis was 22 ± 6 years in the classic/modified group vs 14 ± 8 years in the total cavopulmonary connection group (P = 0.03. Fontan circuit thrombus was associated with adverse cardiac outcomes (27% [4/15] vs 8% [4/52], P = 0.02), but there was no difference in Fontan circulatory dysfunction markers. Conclusion Given the incidence of Fontan circuit thrombus and association with adverse cardiac outcomes, routine surveillance of the Fontan circuit should strongly be considered. The identification of thrombus should lead to anticoagulation implementation/optimization, along with screening/intervention for reversible Fontan circulatory issues in an attempt to prevent adverse cardiac outcomes.
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- 2019
7. Adults With Complex Congenital Heart Disease: Cerebrovascular Considerations for the Neurologist
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Thalia S. Field, Jonathan M.C. Smith, Derek G. Human, and Jason G. Andrade
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cognition ,Pediatrics ,medicine.medical_specialty ,Heart disease ,Population ,Review ,030204 cardiovascular system & hematology ,Vascular risk ,lcsh:RC346-429 ,03 medical and health sciences ,0302 clinical medicine ,Adults with Congenital Heart Disease (ACHD) ,Cardiovascular Disease ,medicine ,cardiovascular diseases ,Complex congenital heart disease ,Cerebrovascular disease ,education ,Stroke ,lcsh:Neurology. Diseases of the nervous system ,Subclinical infection ,education.field_of_study ,business.industry ,medicine.disease ,stroke ,fontan ,Increased risk ,Neurology ,cyanotic ,Stroke prevention ,Neurology (clinical) ,Congenital Heart Disease (CHD) ,business ,030217 neurology & neurosurgery - Abstract
As infant and childhood mortality has decreased in congenital heart disease, this population is increasingly reaching adulthood. Adults with congenital heart disease (ACHD) represent a group with increased risk of stroke, silent brain infarcts, and vascular cognitive impairment. Cyanotic and other complex cardiac lesions confer the greatest risk of these cerebrovascular insults. ACHD patients, in addition to having an increased risk of stroke from structural cardiac issues and associated physiological changes, may have an accelerated burden of conventional vascular risk factors, including hypertension and impaired glucose metabolism. Adult neurologists should be aware of the risks of clinically evident and subclinical cerebrovascular disease in this population. We review the existing evidence on primary and secondary stroke prevention in individuals with complex congenital heart disease, and identify knowledge gaps in need of further research, including treatment of acute stroke in this population. Multisystemic genetic syndromes are outside the scope of this review.
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- 2019
8. Atrial arrhythmias and thromboembolic complications in adults post Fontan surgery
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Amanda J. Barlow, Darryl Wan, Derek G. Human, Andrew D. Krahn, M. Riahi, Santabhanu Chakrabarti, Jasmine Grewal, and Marla Kiess
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Adult ,Heart Defects, Congenital ,Male ,lcsh:Diseases of the circulatory (Cardiovascular) system ,medicine.medical_specialty ,Time Factors ,Cirrhosis ,Multivariate analysis ,030204 cardiovascular system & hematology ,Fontan Procedure ,Risk Assessment ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Risk Factors ,Thromboembolism ,Atrial Fibrillation ,medicine ,Humans ,cardiovascular diseases ,Stroke ,Retrospective Studies ,business.industry ,Congenital Heart Disease ,Age Factors ,Atrial fibrillation ,Retrospective cohort study ,Atrial arrhythmias ,medicine.disease ,Pulmonary embolism ,Surgery ,Treatment Outcome ,lcsh:RC666-701 ,Cohort ,cardiovascular system ,Female ,Cardiology and Cardiovascular Medicine ,business ,arrhythmias ,030217 neurology & neurosurgery - Abstract
ObjectivePatients with Fontan surgery experience late complications in adulthood. We studied the factors associated with the development and maintenance of atrial arrhythmias and thromboembolic complications in an adult population with univentricuar physiology post Fontan surgery.MethodsSingle centre retrospective cohort study of patients ≥18 years of age with Fontan circulation followed at our quaternary care centre for more than 1 year were included. Univariate and multivariate regression models were used where applicable to ascertain clinically significant associations between risk factors and complications.Results93 patients were included (age 30.2±8.8 years, 58% men). 28 (30%) had atriopulmonary Fontan connection, 35 (37.6%) had lateral tunnel Fontan and 29 (31.1%) had extracardiac Fontan pathway. After a mean of 7.27±5.1 years, atrial arrhythmia was noted in 37 patients (39.8%), of which 13 developed had atrial fibrillation (14%). The presence of atrial arrhythmia was associated with the number of prior cardiac surgeries/procedures, increasing age and prior atriopulmonary Fontan operation. Thromboembolic events were present in 31 patients (33%); among them 14 had stroke (45%), 3 had transient ischaemic attack (9.7%), 7 had pulmonary embolism (22.6%) and 5 had atrial thrombus with imaging (16.1%). The presence of thromboembolic events was only associated with age and the presence of cirrhosis in multivariate analysis.ConclusionsAtrial arrhythmias are common in adults with Fontan circulation at an early age, and are associated with prior surgical history and increasing age. Traditional risk factors may not be associated with atrial arrhythmia or thromboembolism in this cohort.
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- 2020
9. Thromboembolic episodes related to atrial arrhythmias in adults with transposition of great arteries
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Clara Tsui, Jasmine Grewal, Darryl Wan, Amanda J. Barlow, Marla Kiess, Derek G. Human, Santabhanu Chakrabarti, and Andrew D. Krahn
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Tachycardia ,lcsh:Diseases of the circulatory (Cardiovascular) system ,medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,Heart disease ,Population ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,030212 general & internal medicine ,education ,Stroke ,Congenital heart disease ,education.field_of_study ,business.industry ,Atrial fibrillation ,Retrospective cohort study ,Atrial arrhythmia ,medicine.disease ,lcsh:RC666-701 ,Great arteries ,Cohort ,Cardiology ,medicine.symptom ,business - Abstract
Background: Atrial arrhythmias (AA) are common in adults with congenital heart disease (ACHD). Although Intra-atrial Reentrant Tachycardia (IART) is well described in ACHD, Atrial Fibrillation (AF) is uncommon, but increasingly recognized. Patients with Transposition of the Great Arteries (TGA) and congenitally corrected-TGA (CC-TGA) have a high burden of AA at a relatively young age. However, long-term data of AA and associated thromboembolic risk are lacking in these patients. The prevalence, associated clinical factors, and complications of AA in a longitudinal TGA cohort was studied. Methods: A retrospective cohort study of all TGA patients from a single tertiary care centre was conducted. Data regarding documented atrial arrhythmias, thromboembolic events, and factors associated with thromboembolism were extracted and analyzed. Mean values and standard deviations were calculated for normally distributed continuous variables. When frequencies and means were compared, the chi-squared test and student t-test were used, respectively. Results: One-hundred twenty-five patients with TGA (76 TGA, 49 CC-TGA) were followed for a mean of 20.8 ± 13.2 years. AF was confirmed in 20% (n = 25) and there were 5 (20%) thromboembolic complications within the AF population. AF was associated with an annual thromboembolic event rate of 2.7%/year (stroke/transient ischemic attack 1.7%, systemic embolism 1.0%). Conclusion: AF is relatively common in the TGA ACHD population in long-term follow up. Although annual risk of thromboembolism is low in this young group of patients, life-time cumulative risk is potentially high. TGA patients should be screened actively for AF and appropriate anticoagulation therapy initiated. It is unclear if established risk prediction scores in other non-valvular AF populations may be applicable to this cohort.
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- 2018
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10. SACUBUTRIL/VALSARTAN IS SAFE AND EFFECTIVE IN REDUCING HEART FAILURE SYMPTOMS IN ADULTS WITH CONGENITAL HEART DISEASE
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M. Riahi, J. Amon, Derek G. Human, Darryl Wan, D. Chua, Mustafa Toma, Marla Kiess, Hamed Nazzari, S. Chakrabarti, Andrew Ignaszewski, A. Barlow, and J. Grewal
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medicine.medical_specialty ,Heart disease ,Valsartan ,business.industry ,Internal medicine ,Heart failure ,Cardiology ,medicine ,Cardiology and Cardiovascular Medicine ,medicine.disease ,business ,medicine.drug - Published
- 2019
11. 40 Respiratory syncytial virus-related outcomes from an abbreviated palivizumab dose regimen in children with congenital heart disease
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Derek G. Human, Richard J. K. Taylor, Constantin R. Popescu, Cheryl Christopherson, Jennifer Claydon, Pascal M. Lavoie, Lana Shaibah, and Alfonso Solimano
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Palivizumab ,Pediatrics ,medicine.medical_specialty ,Heart disease ,business.industry ,medicine.disease ,Virus ,Regimen ,Pediatrics, Perinatology and Child Health ,medicine ,Abstract / Résumés ,Respiratory system ,business ,medicine.drug - Abstract
BACKGROUND: Respiratory Syncytial Virus (RSV) is a leading cause of hospital admission for acute lower respiratory tract infections (LRTI) in young children. Infants with congenital heart disease (CHD) are at a higher risk of severe infection, specifically those with hemodynamically significant intra-cardiac shunts, impaired cardiac function or pulmonary hypertension. Palivizumab prevents RSV-related hospitalizations in children with higher risk indications, including CHD, however the number of doses required to achieve optimal protection remains controversial. We report the clinical outcomes of children with CHD who received an abbreviated Palivizumab 4-dose schedule, with an increased inter-dose interval in our region. OBJECTIVES: To report hospitalization outcomes in children with CHD who received an abbreviated 4-dose Palivizumab dose schedule through the centrally managed regional RSV Immunoprophylaxis Program. DESIGN/METHODS: Retrospective population-based study of children with CHD enrolled into the regional RSV Program between 2012–2016. Children received palivizumab between November and April. Eligibility was defined according to our provincial guidelines and this defined our sample size. The primary outcome was in-season hospitalizations for RSV-confirmed or potentially RSV-related (unknown) LRTI determined by cross referencing the RSV Program database and the Canadian Institute of Health Informatics (CIHI) discharge abstract database (DAD) using seven RSV-related ICD-10 diagnostic codes. Children were categorized according to CHD subtype. Analysis was by intention-to-treat. RESULTS: A total of 325 children ([mean ± SD] gestational age 37.9±6.2 weeks; birth weight 2870±871 grams) were approved to receive 406 palivizumab courses. Between November 2012 and April 2016, 89 hospitalizations occurred in CHD patients. Of this, seventeen were RSV-confirmed hospitalizations (median age 5.9 [IQR 4 to 10]months); 8 additional cases were not tested for RSV, for a maximum rate of hospitalizationsfor RSV-confirmed or unknown of 6.2 per 100 approvals (95%CI: 4.0 to 9.0%). 24/25 RSV-confirmed or potentially RSV-related hospitalizations occurred before the 4(th)Palivizumab dose. Only one RSV-confirmed hospitalization occurred (52 days) after the 4th Palivizumab dose. Clinical characteristics of children with RSV-confirmed hospitalizations (n=17) were similar to those with RSV-unrelated hospitalizations (n=40). CONCLUSION: Our data provide population-based evidence of the protection achieved by a 4-dose Palivizumab dose schedule in infants with CHD. This clinical practice yields substantial benefits to children, their families, and to the health care system, such as reduced visits, injections, and costs, compared to a standard 5-dose schedule. Our results warrant confirmation in other geographic areas.
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- 2019
12. Rationale and Design of the Canadian Outcomes Registry Late After Tetralogy of Fallot Repair: The CORRELATE Study
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Camilla Kayedpour, Andrew E. Warren, Christopher A. Caldarone, Paul Khairy, Andrew N. Redington, Rachel M. Wald, Syed Najaf Nadeem, Tiscar Cavallé-Garrido, Adrienne H. Kovacs, Edythe B. Tham, David J. Patton, Camille L. Hancock Friesen, Brian W. McCrindle, Edward J. Hickey, Michael E. Farkouh, Judith Therrien, Bernd J. Wintersperger, Jasmine Grewal, Candice K. Silversides, Christian Drolet, Derek G. Human, Gerald Lebovic, Mustafa A Altaha, Nanette Alvarez, Frederic Dallaire, and Isabelle Vonder Muhll
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Adult ,Male ,Canada ,Pediatrics ,medicine.medical_specialty ,Time Factors ,Heart disease ,Magnetic Resonance Imaging, Cine ,Pilot Projects ,Severity of Illness Index ,Asymptomatic ,Ventricular Function, Left ,Severity of illness ,medicine ,Humans ,Prospective Studies ,Registries ,Cardiac Surgical Procedures ,Child ,Prospective cohort study ,Cause of death ,Tetralogy of Fallot ,business.industry ,Incidence ,Middle Aged ,Prognosis ,medicine.disease ,Pulmonary Valve Insufficiency ,Survival Rate ,Heart failure ,Quality of Life ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies ,Cohort study - Abstract
Background Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined. Methods Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated. Results To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults. Conclusions CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR.
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- 2014
13. Chronic kidney damage in the adult Fontan population
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Santabhanu Chakrabarti, A. Barlow, J. Grewal, Derek G. Human, G. Sexsmith, D. Lee, Adeera Levin, and Marla Kiess
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Adult ,Male ,medicine.medical_specialty ,End organ damage ,Population ,Urology ,Renal function ,Urine ,030204 cardiovascular system & hematology ,urologic and male genital diseases ,Fontan Procedure ,Kidney Function Tests ,03 medical and health sciences ,chemistry.chemical_compound ,Young Adult ,0302 clinical medicine ,medicine ,Humans ,030212 general & internal medicine ,Prospective Studies ,Treatment Failure ,Renal Insufficiency, Chronic ,education ,Prospective cohort study ,Creatinine ,education.field_of_study ,business.industry ,medicine.disease ,female genital diseases and pregnancy complications ,chemistry ,Population Surveillance ,Albuminuria ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Kidney disease ,Glomerular Filtration Rate - Abstract
Objectives 1) To determine the accuracy of estimated GFR (eGFR) as compared to directly measured GFR (mGFR) in the adult Fontan population; 2) to determine the true prevalence of chronic kidney damage (CKD) as determined by uACR AND eGFR. Methods Prospective study of 81 patients Fontan patients (≥18years) followed at St. Paul's Hospital, University of British Columbia. CKD-EPI and MDRD equations used to calculate eGFR, mGFR determined by 99m Tc-DTPA renal dynamic imaging and urine albumin to creatinine ratios were calculated. Results The mGFR was 93±27ml/min/1.73m 2 : 28 (53%) had an mGFR 2 and 1 (2%) had an mGFR 2 . There was a modest correlation between mGFR and eGFR (EPI/MDRD) (r=0.50, p 2 , p 2 , p 90ml/min/1.73m 2 , 50% and 46% respectively had an mGFR 2 . Significant albuminuria (>3mg/mmol) was present in 33% and upwards of 32% of patients with a normal eGFR (MDRD/EPI) had evidence of CKD with uACR >3mg/mmol. Using combined criteria of eGFR 2 and/or uACR >3mg/mmol, 46% of patients had evidence of CKD. Conclusions This study draws attention to the need for stringent CKD screening as an important proportion of CKD is currently not being detected. Mild undetected CKD, an early marker of end organ damage, may also be an early sign of Fontan failure that requires warrants further research.
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- 2017
14. Longitudinal sequential biventricular assessment in adults with transposition of the great arteries and relationship with adverse outcomes
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Jonathon Leipsic, S. Chakrabarti, M. Orgad, Marla Kiess, M. Riahi, Derek G. Human, A. Claman, and Jasmine Grewal
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Adult ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,medicine.medical_treatment ,Transposition of Great Vessels ,Ventricular Dysfunction, Right ,Population ,030204 cardiovascular system & hematology ,Cohort Studies ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Clinical endpoint ,medicine ,Humans ,030212 general & internal medicine ,Longitudinal Studies ,Mortality ,education ,Heart transplantation ,education.field_of_study ,business.industry ,Middle Aged ,medicine.disease ,medicine.anatomical_structure ,Treatment Outcome ,Great arteries ,Ventricle ,Heart failure ,Cohort ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Cohort study ,Follow-Up Studies - Abstract
Background In a cohort of congenitally corrected transposition of the great arteries (cc-TGA) and transposition of the great arteries after atrial switch procedure (d-TGA) the study objectives were: 1) to assess the change of quantitative systemic right ventricle (sRV) parameters over time and; 2) to examine the relationship of quantitative sRV parameters with adverse clinical outcomes. Methods and results Single-center cohort study that included 49 (39%) cc-TGA and 76 (61%) d-TGA patients >18years who had at least one MUGA sRV assessment, 18/39 had more than one respectively. The primary clinical endpoint was all-cause mortality, heart transplantation and/or heart failure hospitalization. At a median clinical follow-up of 7years following the first MUGA, the primary endpoint occurred more often in cc-TGA versus d-TGA patients (18 (36.7%) vs. 9 (11.8%), p=0.03). Median time between the MUGA assessments was 5.8 (cc-TGA) and 4.9years (d-TGA). At last MUGA follow-up: 6 (33%) cc-TGA/14 (36%) d-TGA patients showed a significant decline in sRVEF (>5%); 6 (33%) cc-TGA/17 (44%) d-TGA patients had a significant increase in sRVEDVi; and 7 (39%) cc-TGA/19 (49%) PA-TGA patients had a significant increase in sRVESVi. Baseline sRV parameters were not associated with the primary end point or sRV changes over time. Conclusions An important proportion of both patient cohorts demonstrated a significant change in sRV parameters over time and these are likely related to multiple factors that vary between individuals given population heterogeneity. The TGA patients have distinct clinical trajectories with increased adverse heart failure outcomes in the cc-TGA population and sRV parameters were not related to adverse heart failure events in either group.
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- 2017
15. Aspirin Dose and Prevention of Coronary Abnormalities in Kawasaki Disease
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Rosie Scuccimarri, Mathew Mathew, Anita Dhanrajani, Dania Basodan, Nagib Dahdah, Astrid M. De Souza, Derek G. Human, Joel Blanchard, Zoe Fortier-Morissette, Soha Rached-d'Astous, Audrey Dionne, Brian W. McCrindle, Claudia Renaud, Frederic Dallaire, Harrison Saulnier, Samuel Blais, and Kimberley Kaspy
- Subjects
Male ,medicine.medical_specialty ,Coronary Disease ,030204 cardiovascular system & hematology ,Mucocutaneous Lymph Node Syndrome ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Pharmacotherapy ,030225 pediatrics ,Internal medicine ,medicine ,Humans ,Retrospective Studies ,Aspirin ,Dose-Response Relationship, Drug ,business.industry ,Confounding ,Absolute risk reduction ,Immunoglobulins, Intravenous ,Infant ,Retrospective cohort study ,medicine.disease ,Confidence interval ,Surgery ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Kawasaki disease ,Drug Therapy, Combination ,Female ,business ,Cohort study ,medicine.drug - Abstract
BACKGROUND:Acetylsalicylic acid (ASA) is part of the recommended treatment of Kawasaki disease (KD). Controversies remain regarding the optimal dose of ASA to be used. We aimed to evaluate the noninferiority of ASA at an antiplatelet dose in acute KD in preventing coronary artery (CA) abnormalities.METHODS:This is a multicenter, retrospective, nonrandomized cohort study including children 0 to 10 years of age with acute KD between 2004 and 2015 from 5 institutions, of which 2 routinely use low-dose ASA (3–5 mg/kg per day) and 3 use high-dose ASA (80 mg/kg per day). Outcomes were CA abnormalities defined as a CA diameter with a z score ≥2.5. We assessed the risk difference of CA abnormalities according to ASA dose. All subjects received ASA and intravenous immunoglobulin within 10 days of fever onset.RESULTS:There were 1213 subjects included, 848 in the high-dose and 365 in the low-dose ASA group. There was no difference in the risk of CA abnormalities in the low-dose compared with the high-dose ASA group (22.2% vs 20.5%). The risk difference adjusted for potential confounders was 0.3% (95% confidence interval [CI]: −4.5% to 5.0%). The adjusted risk difference for CA abnormalities persisting at the 6-week follow-up was −1.9% (95% CI: −5.3% to 1.5%). The 95% CI of the risk difference of CA abnormalities adjusted for confounders was within the prespecified 5% margin considered to be noninferior.CONCLUSIONS:In conjunction with intravenous immunoglobulin, low-dose ASA in acute KD is not inferior to high-dose ASA for reducing the risk of CA abnormalities.
- Published
- 2017
16. Management of Adults With Congenital Heart Disease Using Videoconferencing Across Western Canada: A 3-Year Experience
- Author
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Isabelle Vonder Muhll, Joseph Atallah, Pamela Heggie, Derek G. Human, Nanette Alvarez, Michal J. Kantoch, Ivan M. Rebeyka, David B. Ross, Erik PausJenssen, James W. Tam, Dylan A. Taylor, Payam Dehghani, and Luis G. Quinonez
- Subjects
Adult ,Heart Defects, Congenital ,Male ,Pediatrics ,medicine.medical_specialty ,Heart disease ,Population ,MEDLINE ,Telehealth ,computer.software_genre ,Session (web analytics) ,Alberta ,Young Adult ,Health services ,Videoconferencing ,Outcome Assessment, Health Care ,medicine ,Humans ,education ,Aged ,Aged, 80 and over ,education.field_of_study ,British Columbia ,business.industry ,Manitoba ,Middle Aged ,Patient Acceptance of Health Care ,medicine.disease ,Saskatchewan ,Telemedicine ,Referral centre ,Feasibility Studies ,Female ,Medical emergency ,Cardiology and Cardiovascular Medicine ,business ,computer - Abstract
Background Management of adults with congenital heart disease (ACHD) requires quaternary centres with cardiologists and cardiac surgeons who have expertise in the diagnosis and management of this patient population. We report on the feasibility of the management of ACHD patients using videoconferencing and streaming through 1 regional referral centre covering 4 western Canadian provinces, roughly 30% of Canada's land and population. Methods Videoconferencing sessions from January 2008 to December 2010 were systematically reviewed. Case presentations were classified as successful or unsuccessful. All patients were followed to assess whether the recommendations were acted upon. The hosting institution used the Alberta Health Services internet protocol network, while other connections used integrated service digital network. The videoconference equipment at the different sites includes Polycom HDX 9000 (Polycom, San Jose, CA), Tandberg Edge 95 (Tandberg, San Jose, CA), and Tandberg 990 (Tandberg). Results From January 2008 to December 2010 there were 26 sessions, 213 case presentations, and 177 patients discussed with an average 8.2 case presentations per session. Thirty-two case presentations were deferred, 10 of which were because of transmission errors and the remainder were because of unavailability of staff or images. Of the 177 recommendations, 124 procedures (91 surgical, 29 percutaneous, and 4 electrophysiological) were booked directly at the regional referral centre. Only 6 recommendations were not carried out (4 because of premature deaths, and 2 because of patient logistic issues). Conclusions The results of this study illustrate that telehealth is a feasible medium for arriving at consensus recommendation in the management of ACHD patients living in a geographically diverse area.
- Published
- 2013
17. Canadian Cardiovascular Society/Canadian Pediatric Cardiology Association Position Statement on Pulse Oximetry Screening in Newborns to Enhance Detection of Critical Congenital Heart Disease
- Author
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Anne Fournier, Deborah Fruitman, Derek G. Human, Kenny K. Wong, Lisa Graves, Jennifer L. Russell, and Michael Narvey
- Subjects
Position statement ,Heart Defects, Congenital ,Pediatrics ,medicine.medical_specialty ,Canada ,Consensus ,Heart disease ,Cardiology ,Physical examination ,03 medical and health sciences ,Prenatal ultrasound ,0302 clinical medicine ,Neonatal Screening ,030225 pediatrics ,medicine ,Humans ,030212 general & internal medicine ,Oximetry ,Critical congenital heart disease ,Societies, Medical ,medicine.diagnostic_test ,business.industry ,Infant, Newborn ,Canadian Cardiovascular Society ,medicine.disease ,Pulse oximetry ,Cardiology and Cardiovascular Medicine ,business ,Pediatric cardiology - Abstract
Congenital heart disease is the most common congenital malformation and approximately 3 in 1000 newborns have critical congenital heart disease (CCHD). Timely diagnosis affects morbidity, mortality, and disability, and newborn pulse oximetry screening has been studied to enhance detection of CCHD. In this position statement we present an evaluation of the literature for pulse oximetry screening. Current detection strategies including prenatal ultrasound examination and newborn physical examination are limited by low diagnostic sensitivity. Pulse oximetry screening is safe, noninvasive, easy to perform, and widely available with a high specificity (99.9%) and moderately high sensitivity (76.5%). When an abnormal saturation is obtained, the likelihood of having CCHD is 5.5 times greater than when a normal result is obtained. The use of pulse oximetry combined with current strategies has shown sensitivities of up to 92% for detecting CCHD. False positive results can be minimized by screening after 24 hours, and testing the right hand and either foot might further increase sensitivity. Newborns with abnormal screening results should undergo a comprehensive assessment and echocardiography performed if a cardiac cause cannot be excluded. Screening has been studied to be cost neutral to cost effective. We recommend that pulse oximetry screening should be routinely performed in all healthy newborns to enhance the detection of CCHD in Canada.
- Published
- 2016
18. Assessment of Liver Stiffness in Pediatric Fontan Patients Using Transient Elastography
- Author
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Orlee R. Guttman, Derek G. Human, Richard A. Schreiber, Becky Chen, and James E. Potts
- Subjects
medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,Cirrhosis ,Article Subject ,Liver fibrosis ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,Patient age ,Liver stiffness ,Internal medicine ,medicine ,cardiovascular diseases ,lcsh:RC799-869 ,Hepatology ,business.industry ,Gastroenterology ,General Medicine ,medicine.disease ,Surgery ,surgical procedures, operative ,Cohort ,Cardiology ,cardiovascular system ,030211 gastroenterology & hepatology ,lcsh:Diseases of the digestive system. Gastroenterology ,business ,Complication ,Transient elastography ,Research Article - Abstract
Background. Hepatic fibrosis is a potential complication following Fontan surgery and heralds long-term risk for cirrhosis. Transient elastography (TE) is a rapid, noninvasive method to assess liver fibrosis by measuring liver stiffness.Objectives. To compare liver stiffness and liver biochemistries in pediatric Fontan patients with age- and sex-matched controls and to determine patients’ acceptance of TE.Methods. Patients were recruited from British Columbia Children’s Hospital. Twenty-two Fontan patients (15 males) were identified. Demographic information and cardiac data were collected. TE was measured using size-appropriate probes.Results. The median age of the Fontan cohort was 13.7 (5.9–16.8) years. Time from Fontan surgery to TE was 9.6 (1.0–12.9) years. The median Fontan circuit pressure was 13 (11–14) mmHg. TE values were higher in Fontan patients versus controls (18.6 versus 4.7 kPa,p<0.001). There was no association between TE values and patient age (r=0.41,p=0.058), time since Fontan surgery (r=0.40,p=0.062), or median Fontan circuit pressure (CVP) (r=0.35,p=0.111). Patients found TE to be nonpainful, convenient, and safe.Conclusions. TE is feasible to assess liver stiffness in children following Fontan surgery. Pediatric Fontan patients have markedly elevated liver stiffness values. TE may have important utility in liver care follow-up of pediatric Fontan patients.
- Published
- 2016
19. Left Ventricle Pseudoaneurysm After Aortic Valvuloplasty
- Author
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Martin C.K. Hosking, Derek G. Human, and Abdullah Alhuzaimi
- Subjects
Male ,medicine.medical_specialty ,Percutaneous ,Heart Ventricles ,medicine.medical_treatment ,Catheterization ,Pseudoaneurysm ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,Cardiac catheterization ,business.industry ,Infant, Newborn ,Aortic Valve Stenosis ,medicine.disease ,Cardiac surgery ,Aortic valvuloplasty ,Radiography ,Stenosis ,medicine.anatomical_structure ,Left Ventricular Aneurysm ,Ventricle ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Aneurysm, False - Abstract
Acquired left ventricular aneurysm is extremely rare in children. This report describes an infant with acquired left ventricular aneurysm after percutaneous aortic balloon valvuloplasty for critical aortic stenosis. The potential risk factors for myocardial injury during cardiac catheterization and potential complications are discussed.
- Published
- 2011
20. Fatal congenital hypertrophic cardiomyopathy and a pancreatic nodule morphologically identical to focal lesion of congenital hyperinsulinism in an infant with costello syndrome: case report and review of the literature
- Author
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Derek G. Human, Avash J. Singh, Millan S. Patel, Elizabeth D. Sherwin, Brandon S. Sheffield, Anna F. Lee, Christopher Dunham, Paul Brooks, Amitava Sur, Stephen Yip, and Eduardo Ruchelli
- Subjects
0301 basic medicine ,Male ,Pathology ,medicine.medical_specialty ,030105 genetics & heredity ,RASopathy ,medicine.disease_cause ,Proto-Oncogene Mas ,Pathology and Forensic Medicine ,Proto-Oncogene Proteins p21(ras) ,03 medical and health sciences ,Costello syndrome ,medicine ,Humans ,HRAS ,Hyperinsulinemic hypoglycemia ,Pancreas ,business.industry ,Costello Syndrome ,Hypertrophic cardiomyopathy ,Infant, Newborn ,Infant ,General Medicine ,Cardiomyopathy, Hypertrophic ,medicine.disease ,Congenital myopathy ,030104 developmental biology ,Pediatrics, Perinatology and Child Health ,Mutation ,Congenital hyperinsulinism ,Congenital Hyperinsulinism ,business ,Hyperinsulinism - Abstract
Costello syndrome is characterized by constitutional mutations in the proto-oncogene HRAS, causing dysmorphic features, multiple cardiac problems, intellectual disability, and an increased risk of neoplasia. We report a male infant with dysmorphic features, born prematurely at 32 weeks, who, during his 3-month life span, had an unusually severe and ultimately fatal manifestation of hypertrophic cardiomyopathy and hyperinsulinemic hypoglycemia. Molecular studies in this patient demonstrated the uncommon Q22K mutation in the HRAS gene, diagnostic of Costello syndrome. The major autopsy findings revealed hypertrophic cardiomyopathy, congenital myopathy, and a 1.4-cm pancreatic nodule that was positive for insulin expression and morphologically identical to a focal lesion of congenital hyperinsulinism. Sequencing of KCNJ11 and ABCC8, the 2 most commonly mutated genes in focal lesion of congenital hyperinsulinism, revealed no mutations. While hyperinsulinism is a recognized feature of RASopathies, a focal proliferation of endocrine cells similar to a focal lesion of hyperinsulinism is a novel pathologic finding in Costello syndrome.
- Published
- 2015
21. The Protean Manifestations of Blunt Cardiac Trauma in Children
- Author
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Derek G. Human, James E. Potts, Julie Milligan, and Shubhayan Sanatani
- Subjects
Male ,Resuscitation ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Heart disease ,business.industry ,General Medicine ,Wounds, Nonpenetrating ,medicine.disease ,Surgery ,Blunt ,Heart Injuries ,Child, Preschool ,Intensive care ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Emergency Medicine ,medicine ,Humans ,Female ,business - Abstract
We present a case series of pediatric blunt cardiac trauma with a variety of cardiac abnormalities, occurring immediately and after the initial insult. The range of complications and importance of serial evaluations are emphasized.
- Published
- 2005
22. Infant Repair of Massive Aortic Aneurysm With Prosthetic Valved Conduit
- Author
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Norbert Froese, Jake Hiebert, Derek G. Human, Melanie K. Ganshorn, Takashi Sasaki, Ben C. Auld, Sanjiv K. Gandhi, and Neil D. Casey
- Subjects
Male ,Pulmonary and Respiratory Medicine ,Marfan syndrome ,medicine.medical_specialty ,Aortic Valve Insufficiency ,macromolecular substances ,Valved conduit ,Risk Assessment ,Severity of Illness Index ,Marfan Syndrome ,Aortic aneurysm ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,Dilated aortic root ,Ultrasonography ,Bioprosthesis ,Heart Valve Prosthesis Implantation ,Aortic Aneurysm, Thoracic ,business.industry ,musculoskeletal, neural, and ocular physiology ,Infant ,Mitral Valve Insufficiency ,medicine.disease ,Combined Modality Therapy ,Surgery ,Treatment Outcome ,nervous system ,cardiovascular system ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
A 4-month-old child with severe infantile Marfan syndrome underwent successful repair of an extremely dilated aortic root and severe aortic valve insufficiency using a prosthetic valved conduit.
- Published
- 2013
23. Kawasaki disease at British Columbia's Children's Hospital
- Author
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Peter N. Malleson, Mark R Riley, Derek G. Human, Regan L. Ebbeson, and J.E. Potts
- Subjects
Coronary artery aneurysm ,education.field_of_study ,Pediatrics ,medicine.medical_specialty ,Aspirin ,Heart disease ,business.industry ,Population ,medicine.disease ,Pediatrics, Perinatology and Child Health ,medicine ,Original Article ,Kawasaki disease ,Vasculitis ,business ,education ,Exanthem ,Systemic vasculitis ,medicine.drug - Abstract
To describe the clinical features, diagnosis, treatment and outcome of children with Kawasaki disease (KD) treated at a large tertiary care Canadian paediatric hospital and to try to identify correlations between clinical features and the development of coronary artery abnormalities.The charts of 176 patients diagnosed with typical, atypical or incomplete KD between 1992 and 2000 at British Columbia's Children's Hospital were reviewed.The male to female ratio was 1.8:1. The median age was 2.5 years (range two months to 14 years), with 8% nine years or older (42% Caucasian, 43% Asian). Cases occurred steadily throughout the year. One hundred two (58%) patients had typical, 18 (10%) patients had atypical and 56 (32%) patients had incomplete KD. The median time from fever onset to first intravenous immunoglobulin (IVIG) was seven days (range two to 49 days), and treatment began within 10 days of fever onset in 134 (76%) patients. All patients received one or more doses of 2 g/kg IVIG. Forty-two (24%) patients received a second dose for nonresponsiveness, of whom 10 (6%) remained nonresponsive. Eight (5%) patients received intravenous methylprednisolone. Forty-eight (27%) patients developed coronary artery abnormalities, with 10 (6%) echogenic abnormalities, 25 (14%) dilatations and 13 (7%) aneurysms (seven giant). No patient with a normal echocardiogram at four to eight weeks developed an abnormality on subsequent study. Fourteen (8%) patients had persistent abnormalities at last follow-up (median 447 days, range 62 to 3272 days): seven dilations and seven aneurysms (six giant). Five of 13 children (39%) who developed aneurysms failed to meet diagnostic criteria for typical KD, and three of those five aneurysms were present at less than one year after diagnosis. Four of eight (50%) patients receiving intravenous methyl-prednisolone for IVIG nonresponsiveness had or developed aneurysms. One patient died.Some children diagnosed with KD who fail to meet the diagnostic description develop coronary artery abnormalities. There is a need for a more accurate means of diagnosis to more appropriately use IVIG, an expensive and increasingly scarce resource. The role of corticosteroids remains unclear and a randomized controlled clinical trial to determine their role is needed.
- Published
- 2004
24. ANTI-PLATELET DOSE IS NOT INFERIOR TO ANTI-INFLAMMATORY DOSES OF ASPIRIN IN PREVENTING PERSISTENT CORONARY ANEURYSM IN THE TREATMENT OF ACUTE KAWASAKI DISEASE IN CHILDREN
- Author
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D. Basodan, Nagib Dahdah, Rosie Scuccimarri, Derek G. Human, Audrey Dionne, A. Dhanrajani, Brian W. McCrindle, Frederic Dallaire, Z. Fortier-Morissette, Mathew Mathew, Samuel Blais, and A.M. De Souza
- Subjects
medicine.medical_specialty ,Aspirin ,business.industry ,medicine.drug_class ,medicine.disease ,Anti platelet ,Anti-inflammatory ,Aneurysm ,Internal medicine ,medicine ,Cardiology ,Kawasaki disease ,Cardiology and Cardiovascular Medicine ,business ,medicine.drug - Published
- 2016
25. Maternal Anti-Ro and Anti-La Antibody–Associated Endocardial Fibroelastosis
- Author
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Glenn Taylor, John Finley, Derek G. Human, Lisa K. Hornberger, Robert M. Hamilton, Yuk M. Law, Earl D. Silverman, Norman H. Silverman, Lynne E. Nield, Brendan Mullen, Jeffrey F. Smallhorn, and P. Gareth R. Seaward
- Subjects
Adult ,Male ,Anti-La Antibody ,Pediatrics ,medicine.medical_specialty ,Pathology ,Heart disease ,Heart block ,Hydrops Fetalis ,Cardiomyopathy ,Mothers ,Autoantigens ,Ultrasonography, Prenatal ,Cohort Studies ,Fetus ,Pregnancy ,Physiology (medical) ,RNA, Small Cytoplasmic ,Ventricular Dysfunction ,medicine ,Humans ,Autoantibodies ,Retrospective Studies ,Autoimmune disease ,business.industry ,Cardiac Pacing, Artificial ,Infant, Newborn ,Infant ,Endocardial fibroelastosis ,Endocardial Fibroelastosis ,medicine.disease ,Immunohistochemistry ,Heart Block ,Ribonucleoproteins ,Echocardiography ,Female ,Cardiology and Cardiovascular Medicine ,business ,Anti-SSA/Ro autoantibodies - Abstract
Background — Maternal anti-Ro and anti-La antibodies are associated with congenital heart block (CHB). Although endocardial fibroelastosis (EFE) has been described in isolated cases of autoantibody-mediated CHB, the natural history and pathogenesis of this disease are poorly understood. Methods and Results — We retrospectively reviewed the clinical history, echocardiography, and pathology of fetuses and children with EFE associated with CHB born to mothers positive for anti-Ro or anti-La antibodies at 5 centers. Thirteen patients were identified, 6 with a prenatal and 7 with a postnatal diagnosis. Six mothers were positive for anti-Ro and anti-La antibodies, and 7 were positive for anti-Ro antibodies only. Only 1 mother had autoimmune disease. Severe ventricular dysfunction was seen in all fetal and postnatal cases. Four fetal and 3 postnatal cases had EFE at initial presentation. However, 2 fetal and 4 postnatal cases developed EFE 6 to 12 weeks and 7 months to 5 years from CHB diagnosis, respectively, even despite ventricular pacing in 6 postnatal cases. Eleven (85%) either died (n=9) or underwent cardiac transplantation (n=2) secondary to the EFE. Pathologic assessment of the explanted heart, available in 10 cases, revealed moderate to severe EFE in 7 and mild EFE in 3 cases, predominantly involving the left ventricle. Immunohistochemistry in 4 cases (including 3 fetuses) demonstrated deposition of IgG in 4 and IgM in 3 and T-cell infiltrates in 3 cases, suggesting an immune response by the affected fetus or child. Conclusions — EFE occurs in the presence of autoantibody-mediated CHB despite adequate ventricular pacing. Autoantibody-associated EFE has a very high mortality rate, whether developing in fetal or postnatal life.
- Published
- 2002
26. CHADS2 SCORE ASSOCIATED WITH ATRIAL FIBRILLATION BUT NOT PREDICTIVE OF STROKE IN ADULTS WITH CONGENITAL HEART DISEASE
- Author
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Andrew D. Krahn, Janarthanan Sathananthan, J. Grewal, Clara Tsui, S. Chakrabarti, Marla Kiess, A. Barlow, Darryl Wan, and Derek G. Human
- Subjects
medicine.medical_specialty ,Heart disease ,business.industry ,Internal medicine ,medicine ,Cardiology ,Atrial fibrillation ,Cardiology and Cardiovascular Medicine ,medicine.disease ,business ,Stroke - Published
- 2017
27. Early and intermediate-term complications of self-expanding stents limit its potential application in children with congenital heart disease
- Author
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Maurice P. Leung, Shubhayan Sanatani, Yiu-fai Cheung, J.A.Gordan Culham, Adolphus K.T Chau, and Derek G. Human
- Subjects
Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Adolescent ,Heart disease ,medicine.medical_treatment ,Hemodynamics ,Constriction, Pathologic ,Pulmonary Artery ,Prosthesis Design ,medicine.artery ,medicine ,Humans ,Child ,Intermediate term ,medicine.diagnostic_test ,business.industry ,Angiography ,Stent ,medicine.disease ,Surgery ,Equipment Failure Analysis ,Treatment Outcome ,Child, Preschool ,Retreatment ,Pulmonary artery ,Balloon dilation ,Pulmonary Veno-Occlusive Disease ,Female ,Stents ,Cardiology and Cardiovascular Medicine ,business ,Angioplasty, Balloon ,Follow-Up Studies - Abstract
OBJECTIVESWe report on the early and intermediate-term follow-up results of self-expanding Wallstent (Schneider, Switzerland) implanted in children with congenital heart disease.BACKGROUNDThe inherent shortcomings of balloon-expandable stents prompted the trial of an alternative stent.METHODSTwenty patients underwent 22 implantations of 25 self-expanding Wallstents between December 1993 and June 1997 in two institutions. The mean age and weight were 10.8 ± 4.5 years and 30.5 ± 14.2 kg, respectively. The patients were divided into two groups: 1) Group I comprised 17 patients with pulmonary arterial stenoses, 2) Group II comprised four patients with venous stenoses (one belonged to both groups). Sixteen patients underwent recatheterization at a median of 5.8 months (range 0.5 to 31, mean 8.1 months) after stenting. Hemodynamic and angiographic changes after the interventional procedures and complications were documented.RESULTSAll the stents were successfully deployed in the intended position. In Group I, the narrowest diameter of the stented vessel increased from 4.1 ± 1.5 to 8 ± 2 mm (95% increase, p < 0.0001) while the systolic pressure gradient across decreased from 24.6 ± 15.8 to 12.1 ± 11.4 mm Hg (51% decrease, p = 0.001). In Group II, the dimensional changes of the narrowest segment increased from 4.3 ± 0.5 to 7.5 ± 0.4 mm (75% increase, p = 0.003), and the pressure gradient reduced from 5.0 ± 2.9 to 0.9 ± 1.0 mm Hg (82% decrease, p = 0.04) across the stented venous channel. Distal migration of two optimally positioned stents occurred within 24 h of implantation. At recatheterization, significant neointimal ingrowth (>30% of the expanded diameter) was noted in 7 (28%) of the 25 implanted stents. This responded poorly to balloon dilation. Predisposing factors for the neointimal ingrowth included stents of smaller diameter (
- Published
- 2000
28. Presentation, diagnosis, and medical management of heart failure in children: Canadian Cardiovascular Society guidelines
- Author
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Adrian Dancea, Kristen George, Joseph Atallah, Robert D. Ross, Michelle M. Graham, Carol Chan, Beth Kauffman, Thomasin Adams-Webber, Steven C. Greenway, Mark K. Friedberg, Derek G. Human, Jack F. Price, Sarah Bowdin, Catherine Chant-Gambacort, Reeni Soni, Melanie D. Everitt, Robert S. McKelvie, Kenny K. Wong, Ashok Kakadekar, Judith Wilson, Michael McGillion, Suryakant Shah, Lars Grosse-Wortmann, Paul F. Kantor, Elfriede Pahl, Lindsay M. Ryerson, S. Lucy Roche, J. Conway, Aamir Jeewa, Julian Raiman, J.E. Potts, Jane Lougheed, Holger Buchholz, Seema Mital, Christian Drolet, Nicole Barbosa, John L. Jefferies, Letizia Gardin, Daphne T. Hsu, Charles E. Canter, Rejane Dillenburg, and Elizabeth A. Stephenson
- Subjects
Heart Defects, Congenital ,medicine.medical_specialty ,Pediatrics ,Canada ,Cardiotonic Agents ,Heart disease ,Adolescent ,Vasopressins ,Vasodilator Agents ,Diagnostico diferencial ,Management of heart failure ,MEDLINE ,Angiotensin-Converting Enzyme Inhibitors ,Diagnosis, Differential ,Catecholamines ,Risk Factors ,Medicine ,Humans ,Intensive care medicine ,Grading (education) ,Child ,Diuretics ,Arrhythmogenic Right Ventricular Dysplasia ,Societies, Medical ,Heart Failure ,Evidence-Based Medicine ,business.industry ,Myocardium ,Infant ,Evidence-based medicine ,Canadian Cardiovascular Society ,medicine.disease ,Prognosis ,Combined Modality Therapy ,Magnetic Resonance Imaging ,Myocarditis ,Death, Sudden, Cardiac ,Echocardiography ,Heart failure ,Child, Preschool ,Electrocardiography, Ambulatory ,Cardiology and Cardiovascular Medicine ,business ,Cardiomyopathies ,Angiotensin II Type 1 Receptor Blockers ,Algorithms ,Biomarkers - Abstract
Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.
- Published
- 2012
29. Doppler echocardiographic and morphologic evaluation of patients following operative repair of aortic coarctation
- Author
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Derek G. Human, D. Teien, H. Wendel, and M A Nanton
- Subjects
Adult ,medicine.medical_specialty ,Time Factors ,Adolescent ,Coarctation of the aorta ,Aorta, Thoracic ,Blood Pressure ,Corrective surgery ,Aortic disease ,Aortic Coarctation ,symbols.namesake ,Recurrence ,Internal medicine ,medicine ,Humans ,Aorta, Abdominal ,Child ,medicine.diagnostic_test ,business.industry ,Age Factors ,Infant ,Magnetic resonance imaging ,General Medicine ,Middle Aged ,medicine.disease ,Echocardiography, Doppler ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Angiography ,symbols ,Cardiology ,Radiology ,business ,Doppler effect ,Blood Flow Velocity ,Follow-Up Studies ,Diastolic flow - Abstract
Sixty-six patients operated on previously for coarctation of the aorta were included in the study. There were 50 children and 16 adults. Invasive and non-invasive gradients were recorded. Anatomical obstruction was evaluated by angiography in 54 patients and the numbers and size of collaterals were quantified. The closest correlation was found between Doppler-estimated gradients and invasive peak-instantaneous gradients (r = 0.87). The relation between anatomical obstruction and invasive peak-to-peak gradients, Doppler gradients and diastolic flow ere discouraging (r = 0.64, 0.54 and 0.35). Liberal indications for angiography or magnetic resonance tomography are recommended.
- Published
- 1992
30. Assessment of blood pressures and gradients by automated blood pressure device compared to invasive measurements in patients previously operated on for coarctation of the aorta
- Author
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M A Nanton, H. Wendel, Derek G. Human, and D. Teien
- Subjects
Adult ,Male ,Supine position ,Adolescent ,Heart disease ,Physiology ,Coarctation of the aorta ,Blood Pressure ,Sphygmomanometer ,Aortic Coarctation ,medicine ,Humans ,In patient ,Low correlation ,Child ,Exercise ,Blood pressure device ,business.industry ,Infant ,Reproducibility of Results ,General Medicine ,Middle Aged ,medicine.disease ,Blood pressure ,Child, Preschool ,Anesthesia ,Regression Analysis ,Female ,business - Abstract
Summary. The aim of the study was to compare invasive and non-invasive blood pressure measurements and gradients. Twenty-two children and 16 adults previously operated for coarctation of the aorta were included. Invasive blood pressures were recorded proximally and distally close to the former operation site and non-invasive systolic blood pressures were recorded by an automated sphygmomanometer on right arm and leg. The adults were investigated at rest and during supine exercise. The correlation between invasive and non-invasive measurements of proximal blood pressures in adults at rest and children were the following, r= 0.92, SD 7.6 mmHg (n= 16) and r= 0.85, SD 11 mmHg (n= 22) respectively. The corresponding correlation for the distal blood pressures were the following for adults at rest 0.64, SD 11.9 mmHg and in children /- = 0.82, SD 9.2 mmHg. During exercise in adults we found a low correlation when comparing invasive and non-invasive proximal and distal blood pressures and a poor correlation regarding the gradients, r= 0.50, SD 16 mmHg, r= 0.45, SD 15.9 mmHg and r= 0.30, SD 22.9 mmHg respectively (n= 16). We also measured the time interval between cessation of exercise and completion of the blood pressure recordings, which gave a mean interval of 73 sec (range 45–115 sec). During that interval the mean fall in the proximal blood pressure was 37 mmHg (range 20–80 mmHg), and the mean fall of the gradient was from 41 mmHg (range 20–76 mmHg) to 23 mmHg (range 6–56 mmHg). We conclude that the non-invasive estimation of proximal blood pressures gave fair results but that the estimation of gradients are unsatisfactory. The use of this method to predict exercise induced blood pressures changes and gradients seems highly unreliable.
- Published
- 1992
31. A case of horseshoe lung and complex congenital heart disease in a term newborn
- Author
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J. A. Gordon Culham, Aamir Jeewa, and Derek G. Human
- Subjects
Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Hypoplastic left heart syndrome ,Internal medicine ,Medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Lung hypoplasia ,Abnormalities, Multiple ,Complex congenital heart disease ,Lung ,Neuroradiology ,medicine.diagnostic_test ,business.industry ,Angiography ,Infant, Newborn ,Left pulmonary artery ,respiratory system ,medicine.disease ,respiratory tract diseases ,Horseshoe lung ,Pediatrics, Perinatology and Child Health ,Cardiology ,Radiology ,business ,Chest radiograph ,Tomography, X-Ray Computed - Abstract
We describe and illustrate a rare association of hypoplastic left heart syndrome, absent hilar left pulmonary artery, and an unusual bronchopulmonary malformation. This case highlights the utility of combination imaging of echocardiography and CT angiography in diagnosing a cyanotic newborn with a chest radiograph that is suspicious for lung hypoplasia.
- Published
- 2009
32. Noninvasive Estimation of Aortic Valve Areas in Children with Aortic Stenosis
- Author
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Derek G. Human, D. Teien, M A Nanton, and H. Wendel
- Subjects
Male ,Aortic valve ,medicine.medical_specialty ,Adolescent ,business.industry ,Infant ,Aortic Valve Stenosis ,General Medicine ,medicine.disease ,Echocardiography, Doppler ,Stenosis ,Aortic valve area ,medicine.anatomical_structure ,Aortic Valve ,Child, Preschool ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Cardiology ,medicine ,Humans ,Regression Analysis ,Female ,Radiology ,Child ,business - Abstract
Fifteen children with aortic stenosis were examined with Echo-Doppler cardiography in order to evaluate the use of the continuity equation to calculate the aortic valve area. The results were compared to invasive estimates of valve area calculated with the Gorlin's equation. A close correlation between the two methods, r = 0.94, was found and it is concluded that even in children an accurate estimate of valve area can be expected with the use of the continuity equation.
- Published
- 1990
33. Pulmonary arterial and intracranial calcification in the recipient of a twin-twin transfusion
- Author
-
Derek G. Human, Olivia C. Y. Kei, and Walter J. Duncan
- Subjects
medicine.medical_specialty ,Poor prognosis ,Twins ,Computed tomography ,Pulmonary Artery ,Electrocardiography ,Pregnancy ,medicine.artery ,medicine ,Diseases in Twins ,Humans ,Infant, Very Low Birth Weight ,Cesarean Section, Repeat ,medicine.diagnostic_test ,business.industry ,Ultrasound ,Infant, Newborn ,Calcinosis ,General Medicine ,Fetofetal Transfusion ,medicine.disease ,Twin twin transfusion ,Cerebral Veins ,Arterial calcification ,Pediatrics, Perinatology and Child Health ,Pulmonary artery ,Female ,Radiology ,Intracranial Arterial Diseases ,Cardiology and Cardiovascular Medicine ,business ,Intracranial calcification ,Tomography, X-Ray Computed ,Calcification - Abstract
Pulmonary arterial and intracranial calcifications are rarely found in children. A female infant, the recipient of a twin–twin transfusion syndrome was found, by ultrasound and computed tomography, to have both pulmonary arterial and intracerebral calcification. A rare condition, termed idiopathic arterial calcification of infancy, is the likely cause. This condition carries a poor prognosis and is usually fatal.
- Published
- 2005
34. Abnormal pericyte recruitment as a cause for pulmonary hypertension in Adams-Oliver syndrome
- Author
-
Millan S. Patel, Derek G. Human, David Chitayat, Nouriya Al-Sanna'a, M. E. Suzanne Lewis, Ian Adatia, Simi Bharya, and Glenn Taylor
- Subjects
Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Pathology ,Hypertension, Pulmonary ,Cutis marmorata telangiectatica congenita ,Limb Deformities, Congenital ,Autopsy ,Skin Diseases, Vascular ,Fatal Outcome ,Internal medicine ,Medicine ,Humans ,Abnormalities, Multiple ,Genetics (clinical) ,Fetal Growth Retardation ,Scalp ,business.industry ,Vascular disease ,Coronary Stenosis ,Infant, Newborn ,Syndrome ,medicine.disease ,Pulmonary hypertension ,Stenosis ,Endocrinology ,medicine.anatomical_structure ,Pericyte ,business ,Pericytes ,Blood vessel ,Adams–Oliver syndrome - Abstract
Adams-Oliver syndrome (AOS) consists of congenital scalp defects with variable limb defects of unknown pathogenesis. We report on two children with AOS plus additional features including intrauterine growth retardation (IUGR), cutis marmorata telangiectatica congenita (CMTC), pulmonary hypertension (PH), intracranial densities shown in one case to be sites of active bleeding and osteopenia. Autopsy in one case revealed defective vascular smooth muscle cell/pericyte coverage of the vasculature associated with two blood vessel abnormalities. Pericyte absence correlated with vessel dilatation while hyperproliferation of pericytes correlated with vessel stenosis. These findings suggest a unifying pathogenic mechanism for the abnormalities seen in AOS. These and previously reported cases establish that a subset of AOS patients is at high risk for PH.
- Published
- 2004
35. Use of an implantable loop recorder in the evaluation of children with congenital heart disease
- Author
-
Derek G. Human, Alejandro Peirone, Shubhayan Sanatani, Robert M. Hamilton, Gil J. Gross, and Christine C. Chiu
- Subjects
Heart Defects, Congenital ,Male ,Pediatrics ,medicine.medical_specialty ,Canada ,Heart disease ,Neurological disorder ,Syncope ,Implantable loop recorder ,medicine ,Palpitations ,Humans ,Child ,Retrospective Studies ,medicine.diagnostic_test ,business.industry ,Retrospective cohort study ,medicine.disease ,Electrodes, Implanted ,Long QT Syndrome ,Child, Preschool ,Cohort ,Electrocardiography, Ambulatory ,Female ,Implant ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Electrocardiography - Abstract
Background A recently developed implantable loop recorder (ILR) has been used in adult patients whose syncope remains unexplained in spite of extensive investigations. Syncope in the patient with congenital heart disease presents a diagnostic challenge. We applied this technology to a cohort of pediatric patients. Methods We reviewed our experience with an ILR in patients with congenital heart disease with syncope or palpitations after conventional investigations failed to identify a cause for the symptoms. Results ILRs were implanted in 4 patients with congenital heart disease at 2 centers for investigation of syncope (n = 2), near-syncope (n = 1), and palpitations (n = 1). Implantations were performed at a mean age of 5.9 ± 0.9 years (4.2 to 7.6 years) and a mean weight of 26.7 ± 6.6 kg (15.7 to 42.5 kg) with patients under general anesthesia, with no complications. All patients experienced typical symptoms and activated the device appropriately at a median of 86 days (46 to 102) after implantation. Each patient had good-quality data that allowed interpretation of the rhythm. In 2 of 4 cases, a likely cause for the symptoms was identified, with exclusion of more malignant arrhythmic diagnoses in all patients. Escalation of therapy was avoidable in all patients on the basis of the data recorded by the ILR. Conclusions Recently developed loop recorder technology can be implanted in the young child without difficulty. The ILR proved to be very useful for excluding malignant arrhythmias as a cause of symptoms in these patients at high risk. (Am Heart J 2002;143:366-72)
- Published
- 2002
36. 735 Acute kidney injury in neonates following post cardiac surgery utilizing the akin definition: A retrospective study
- Author
-
D.G. Matsell, Derek G. Human, A. Campbell, P. Skippen, R. Milner, C. Mammen, and A.E. Alabbas
- Subjects
medicine.medical_specialty ,business.industry ,Anesthesia ,Acute kidney injury ,Medicine ,Retrospective cohort study ,Cardiology and Cardiovascular Medicine ,business ,medicine.disease ,Surgery ,Cardiac surgery - Published
- 2011
37. ATRIAL FIBRILLATION IN ADULTS WITH CONGENITAL HEART DISEASE: SAME WINE IN DIFFERENT BOTTLE?
- Author
-
J. Grewal, Darryl Wan, Derek G. Human, Andrew D. Krahn, A. Barlow, Marla Kiess, J Yeung Lai-Wah, S. Chakrabarti, and Elizabeth D. Sherwin
- Subjects
Wine ,medicine.medical_specialty ,business.product_category ,Heart disease ,business.industry ,Internal medicine ,Cardiology ,Bottle ,Medicine ,Atrial fibrillation ,Cardiology and Cardiovascular Medicine ,business ,medicine.disease - Published
- 2014
38. Balloon angioplasty of right ventricular outflow tract conduits
- Author
-
George G.S. Sandor, Michael W.H. Patterson, Derek G. Human, J. A. Gordon Culham, J.E. Potts, and Shubhayan Sanatani
- Subjects
Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Hemodynamics ,Balloon ,Ventricular Outflow Obstruction ,Electrical conduit ,Angioplasty ,Internal medicine ,medicine ,Ventricular outflow tract ,Humans ,Child ,business.industry ,Graft Occlusion, Vascular ,Infant ,Vascular surgery ,medicine.disease ,Surgery ,Cardiac surgery ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Cardiology ,Female ,Stents ,Cardiology and Cardiovascular Medicine ,business ,Angioplasty, Balloon ,Calcification ,Follow-Up Studies - Abstract
Palliation of complex congenital heart disease, requiring reconstruction of the right ventricular outflow tract (RVOT), is standard practice. Survival of the homograft is a limiting factor. We examined the role of balloon angioplasty (BAP) in prolonging conduit life. Twelve patients underwent 15 BAP procedures between February 1989 and October 1997. The median age at conduit insertion was 28 months with detection of a significant echo gradient 42 months later. Calcification of homografts, with attendant obstruction and valve dysfunction, was present in all patients. BAP was performed within 1 month of echocardiography and reduced the gradient from a median of 57 to 38 mmHg (p < 0.0005). Echocardiographic follow-up showed persistent gradients (median 68 mmHg) and 11/12 patients went on to conduit replacement after BAP. Only one patient had replacement deferred as a result of BAP. Complications requiring intervention occurred in 20% of the procedures and included bleeding and an unusual balloon fracture. Although BAP can reduce the pressure gradient across the RVOT conduit, the effect is transient and the delay of surgery is not due to improved hemodynamic function. Approximately 10% of cases will benefit from BAP alone, but given the high rate of complications, we do not recommend this procedure as a means of prolonging conduit life.
- Published
- 2001
39. 90. Progression of cardiac manifestation on enzyme replacement therapy in a 4–6/12-year-old patient with mucopolysaccharidosis type II, Hunter syndrome
- Author
-
Derek G. Human, Sylvia Stockler-Ipsiroglu, and Saadet Mercimek-Mahmutoglu
- Subjects
medicine.medical_specialty ,Pediatrics ,business.industry ,Endocrinology, Diabetes and Metabolism ,Hunter syndrome ,Enzyme replacement therapy ,medicine.disease ,Biochemistry ,Endocrinology ,Internal medicine ,Genetics ,medicine ,Mucopolysaccharidosis type II ,business ,Molecular Biology - Published
- 2009
40. Kawasaki disease in the older child
- Author
-
Shubhayan Sanatani, Tarek Momenah, George G.S. Sandor, J.E. Potts, Michael W.H. Patterson, and Derek G. Human
- Subjects
Male ,Pediatrics ,medicine.medical_specialty ,Disease ,Mucocutaneous Lymph Node Syndrome ,Age Distribution ,Older patients ,medicine ,Prevalence ,Humans ,Age of Onset ,Child ,Retrospective Studies ,British Columbia ,business.industry ,Infant ,Retrospective cohort study ,Length of Stay ,medicine.disease ,Coronary arteries ,medicine.anatomical_structure ,Treatment Outcome ,Cardiovascular Diseases ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Older child ,Kawasaki disease ,Female ,Age of onset ,business ,Artery - Abstract
Objectives. To determine the prevalence of Kawasaki disease in older children and to evaluate its clinical presentation, time to diagnosis, and outcome in comparison with younger patients with the disease. Methodology. A retrospective analysis of all patients discharged with a diagnosis of Kawasaki disease at a pediatric tertiary care hospital over a 12-year period. Results. A total of 133 patients were included in this study; 7.5% were 9 years of age or older at the time of illness. Patients were grouped by age: infants included children age 1 to 8 years of age and children 9 years of age or older. Older children had a higher frequency of abnormal cardiovascular physical examination (50%) versus children (6%) and infants (10%). The older age group and the infants had a higher prevalence of coronary artery abnormalities and poor left ventricular function than did the 1- to 8-year-olds. Eighty percent of the older children had coronary arteries that were either dilated or aneurysmal, and 30% demonstrated left ventricular dysfunction on initial echocardiography. The number of days to diagnosis after meeting the diagnostic criteria was 5.8 ± 2.3 for infants, 5.2 ± 1.5 for older children, and 1.9 ± 0.3 for children. Older children had a complicated course of Kawasaki disease compared with younger patients. Conclusion. We found a higher prevalence of older children with Kawasaki disease at our center than has previously been reported. Older patients, as well as infants, had a higher rate of coronary artery abnormalities than did the children between 1 and 8 years of age. Older age at the time of illness or a delay in treatment may be important factors in determining cardiac involvement in Kawasaki disease.
- Published
- 1998
41. The Contegra conduit: Late outcomes in right ventricular outflow tract reconstruction
- Author
-
Jacques G. LeBlanc, Derek G. Human, Steve Co, Anthony A Holmes, and Andrew Campbell
- Subjects
lcsh:Diseases of the circulatory (Cardiovascular) system ,medicine.medical_specialty ,pulmonary ,lcsh:Medicine ,Persistent truncus arteriosus ,Early death ,Electrical conduit ,Internal medicine ,Contegra ,medicine ,Ventricular outflow tract ,cardiovascular diseases ,Survival rate ,Right ventricle outflow tract ,reintervention ,business.industry ,lcsh:R ,lcsh:RJ1-570 ,lcsh:Pediatrics ,medicine.disease ,Surgery ,Stenosis ,risk factor ,lcsh:RC666-701 ,Pediatrics, Perinatology and Child Health ,outcome ,cardiovascular system ,Cardiology ,Original Article ,Implant ,Cardiology and Cardiovascular Medicine ,business - Abstract
Objectives: To report the clinical outcomes (early death, late death, and rate of reintervention) and performance of the Contegra conduit as a right ventricle outflow tract implant and to determine the risk factors for early reintervention. Methods: Forty-nine Contegra conduits were implanted between January 2002 and June 2009. Data collection was retrospective. The mean age and follow-up duration of Contegra recipients was 3.5 ± 4.6 years and 4.2 ± 2.0 years, respectively. Results: There were three deaths (two early, one late), giving a survival rate of 93.9%. The rate of conduit-related reintervention was 19.6% and was most often due to distal conduit stenosis. Age at implantation of 22 mm) remains the first choice of implant in older children. The rates of reintervention are significantly higher with a diagnosis of truncus arteriosus, age at implantation of
- Published
- 2012
42. Economic Evaluation of Palivizumab in Children With Congenital Heart Disease: A Canadian Perspective
- Author
-
James E. Potts, Kevin C. Harris, Derek G. Human, Laurie M. Cender, Marsha C. Crosby, and Aslam H. Anis
- Subjects
Heart Defects, Congenital ,Palivizumab ,Pediatrics ,medicine.medical_specialty ,Heart disease ,Cost-Benefit Analysis ,Population ,Respiratory Syncytial Virus Infections ,Antibodies, Monoclonal, Humanized ,Indirect costs ,medicine ,Humans ,Child ,education ,health care economics and organizations ,education.field_of_study ,British Columbia ,business.industry ,Antibodies, Monoclonal ,Infant ,medicine.disease ,Bronchiolitis ,Child, Preschool ,Cohort ,Economic evaluation ,Cardiology and Cardiovascular Medicine ,business ,Historical Cohort ,medicine.drug - Abstract
Respiratory syncytial virus (RSV) is a common cause of bronchiolitis in infants. In children with congenital heart disease (CHD), it is associated with significant morbidity and mortality. Palivizumab is a monoclonal antibody that reduces the number of RSV-associated hospitalizations in children with CHD. We sought to assess cost savings and cost-effectiveness of palivizumab in children2 years old with hemodynamically significant CHD in a provincially administered RSV prophylaxis program.A cohort of children who received palivizumab (N = 292) from 2003-2007 was compared to a historical cohort of children (N = 412) from 1998-2003 who met the eligibility criteria for palivizumab prior to initiation of the prophylaxis program. Direct and indirect costs and benefits were determined.The direct and indirect costs in the historical cohort were $838 per patient season compared to $9130 per patient season in the palivizumab cohort. Risk of admission was reduced by 42%, and days in hospital were reduced by 83%. The incremental cost of the RSV prophylaxis program was $8292 per patient for 1 RSV season. The incremental cost to prevent 1 day of hospitalization was $15,514. The cost of palivizumab accounted for 87.9% of the cost of prophylaxis.Palivizumab is clinically effective; however, the cost was exceptionally high relative to the outcomes in this population. Given the financial constraints in a public health care setting, more strict criteria for patient selection or reduced drug costs would improve the cost-effectiveness of RSV prophylaxis.
- Published
- 2011
43. Kingella kingae endocarditis: A rare case of mitral valve perforation
- Author
-
Tawny Hung, Anthony A Holmes, Andrew Campbell, and Derek G. Human
- Subjects
lcsh:Diseases of the circulatory (Cardiovascular) system ,medicine.medical_specialty ,medicine.medical_treatment ,Perforation (oil well) ,lcsh:Medicine ,Case Report ,Kingella kingae ,Haemophilus parainfluenzae ,Mitral valve ,Medicine ,Endocarditis ,Mitral valve repair ,biology ,business.industry ,lcsh:R ,lcsh:RJ1-570 ,lcsh:Pediatrics ,biology.organism_classification ,medicine.disease ,Surgery ,pediatric ,medicine.anatomical_structure ,lcsh:RC666-701 ,Bacteremia ,Pediatrics, Perinatology and Child Health ,mitral regurgitation ,Cardiology and Cardiovascular Medicine ,business ,Cardiobacterium hominis - Abstract
Kingella kingae , a HACEK (Haemophilus parainfluenzae, Aggregatibacter actinomycetemcomitans, Aggregatibacter aphrophilus, Cardiobacterium hominis, Eikenella corrodens, Kingella kingae) organism, is a common resident of the upper airway in children; it has been associated with endocarditis in children with pre-existing heart conditions. This case report describes K. kingae endocarditis leading to valvular damage in a previously healthy 18-month-old child. Our patient developed a K. kingae bacteremia that was later complicated by meningitis, septic embolic stroke, and endocarditis of the mitral valve, leading to perforation of the posterolateral leaflet. The patient was initially treated conservatively with cefotaxime but, subsequently, required a mitral valve repair with a pericardial patch and annuloplasty. This report draws attention to the need for clinicians to be aware of the potentially serious complications of K. kingae infection in young children. If K. kingae infection is suspected then therapy should be initiated promptly with a β-lactam, followed by early echocardiographic assessment. This case also highlights the lack of specific guidelines available for K. kingae endocarditis.
- Published
- 2011
44. Quantification of left to right shunts by echo Doppler cardiography in patients with ventricular septal defects
- Author
-
Derek G. Human, M A Nanton, K. Karp, D. Teien, and H. Wendel
- Subjects
Adult ,Heart Septal Defects, Ventricular ,Male ,medicine.medical_specialty ,Cardiac Catheterization ,Adolescent ,medicine.medical_treatment ,Right-to-left shunt ,Hemodynamics ,symbols.namesake ,Radionuclide angiography ,Internal medicine ,medicine.artery ,medicine ,Ventricular outflow tract ,Humans ,cardiovascular diseases ,Radionuclide Angiography ,Child ,Cardiac catheterization ,Heart septal defect ,medicine.diagnostic_test ,business.industry ,Infant ,General Medicine ,medicine.disease ,Echocardiography, Doppler ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Angiography ,cardiovascular system ,Cardiology ,symbols ,Female ,business ,Doppler effect - Abstract
Thirty-one patients with ventricular septal defect were evaluated with Echo Doppler cardiography and colour flow mapping. The ventricular septal defect area was estimated by measurement of the jet diameter by colour flow mapping. The ventricular septal defect flow was calculated as the product of the cross-sectional area of the ventricular septal defect and the velocity time integral of flow through the defect. In ten patients the diameter obtained by colour flow mapping was compared with the diameter obtained at angiography, r = 0.88 (SD = 0.14 cm). The aortic flow was calculated as the product of left ventricular outflow tract cross-sectional area and the corresponding velocity time integral. QP/QS ratio was estimated as (ventricular defect flow + aortic flow)/aortic flow and compared to estimates by a radionuclide or oximetric method. A correlation of r = 0.95 (SD = 0.23) was found.
- Published
- 1991
45. Left ventricular thrombosis following a Norwood procedure
- Author
-
Derek G. Human, Walter J. Duncan, and Andrew Campbell
- Subjects
Reoperation ,medicine.medical_specialty ,Heart Ventricles ,medicine.medical_treatment ,MEDLINE ,Hypoplastic left heart syndrome ,Postoperative Complications ,Internal medicine ,Hypoplastic Left Heart Syndrome ,Ventricular Dysfunction ,medicine ,Humans ,Aortic atresia ,Cardiac Surgical Procedures ,Ventricular thrombosis ,business.industry ,Infant, Newborn ,Follow up studies ,Thrombosis ,General Medicine ,medicine.disease ,Echocardiography ,Pediatrics, Perinatology and Child Health ,Cardiology ,Norwood procedure ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Published
- 2007
46. 119 Indications for Use of Palivizumab for RSV Prophylaxis in Infants and Children with Congenital Heart Disease: A Guide for Physicians
- Author
-
Bosco Paes, P Kantor, B Simmons, Upton Allen, K-J Lee, Karel O'Brien, J Finley, M Blaney, M Giuffre, and Derek G. Human
- Subjects
Palivizumab ,medicine.medical_specialty ,Pediatrics ,Heart disease ,business.industry ,Pediatrics, Perinatology and Child Health ,Medicine ,business ,Intensive care medicine ,medicine.disease ,medicine.drug - Published
- 2004
47. Left ventricular thrombus following repair of truncus arterosus
- Author
-
C. Anthony Ryan, Derek G. Human, Christian M. Sodar, and Eli Zalzstein
- Subjects
congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Heart Diseases ,Heart Ventricles ,Antithrombin III ,Truncus arteriosus ,Postoperative Complications ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,business.industry ,Infant ,Thrombosis ,Vascular surgery ,Left ventricular thrombus ,medicine.disease ,Truncus Arteriosus, Persistent ,Surgery ,Cardiac surgery ,Truncus ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,circulatory and respiratory physiology - Abstract
A 6-month-old infant developed a left ventricular thrombus in association with low antithrombin-III activity following the repair of a truncus arteriosus. Conservative management was associated with spontaneous resolution of the clot. The significance of this association is discussed.
- Published
- 1990
48. Mitral valve replacement in children
- Author
-
C.N. Barnard, Derek G. Human, H.S. Joffe, and Fraser Cb
- Subjects
Pulmonary and Respiratory Medicine ,Mitral regurgitation ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Mitral valve replacement ,medicine.disease ,Surgery ,Stenosis ,medicine.anatomical_structure ,Calcinosis ,Mitral valve ,medicine ,Etiology ,Cardiology and Cardiovascular Medicine ,business ,Complication ,Cardiac catheterization - Abstract
Mitral valve replacement (MVR) is associated with higher mortality and morbidity rates in children than in adults, and the use of heterograft valves has been encouraged. The results of MVR in 56 consecutive patients, aged 2 to 12 years, presenting between January, 1972, and January 1979, were reviewed to test these beliefs. The etiology of mitral valve disease was rheumatic in 46, congenital in eight, and acute bacterial endocarditis in two. All children were seriously disabled (NYHA Classes III and IV). Cardiac catheterization in 36 patients revealed mixed valve disease in 26, pure mitral regurgitation in seven, and pure mitral stenosis in three. Seventeen Starr-Edwards (SE), five Lillehei (L), Bjork-Shiley (BS), eight Hancock (H), and 25 Carpentier-Edwards (CE) mitral prosthesis were inserted. Operative mortality was 2% (1 BS) and late mortality was 10% (three SE, one L, two CE). Serious late complications occurred in 30% of survivors, including 11 instances of calcific valve stenosis (five H, six CE), one case of valve thrombosis (1 L), and two embolic episodes (1 SER, 1 H). Survival curves were similar for patients with heterograft and mechanical valves (92% and 77% at 5 years). Event-free curves showed heterograft valves to have a far higher complication rate than mechanical valves (10% complication free at 4 years compared to 84% free at 5 years). Early operative results in children are excellent, and the overall mortality (10%) compares favorably with figures for MVR in adults. However the long-term durability and choice of prostheses remain problematical, since less than 10% of heterograft valves survive beyond 5 years.
- Published
- 1982
49. Infective Endocarditis in South African Children
- Author
-
Derek G. Human, C. T. Hugo-Hamman, and M. M. A. De Moor
- Subjects
Heart Defects, Congenital ,Male ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Heart disease ,Heart malformation ,South Africa ,Risk Factors ,Case fatality rate ,medicine ,Humans ,Endocarditis ,Cardiac Surgical Procedures ,Child ,Retrospective Studies ,biology ,business.industry ,Infant, Newborn ,Infant ,Endocarditis, Bacterial ,medicine.disease ,biology.organism_classification ,Surgery ,Cardiac surgery ,Infectious Diseases ,Viridans streptococci ,Child, Preschool ,Infective endocarditis ,Pediatrics, Perinatology and Child Health ,Rheumatic fever ,Female ,business - Abstract
The clinical and laboratory findings in 29 children with infective endocarditis over the past 10 years are reviewed retrospectively. Twenty children (70 per cent) had congenital heart disease, five (16 per cent) had rheumatic heart disease, and four (14 per cent) had no underlying cardiac abnormality prior to presentation. Twelve (41 per cent) patients had undergone cardiac surgery prior to developing endocarditis. The most common causative organisms were Staphylococcus aureus, Staphylococcus epidermidis, and viridans streptococci none of which was associated with a significantly greater mortality. There was a high case fatality rate of 35 per cent of children below the age of 6 months and children without an underlying cardiac abnormality were particularly at risk. There is a need for standardization of diagnostic criteria for infective endocarditis to facilitate accurate collaborative epidemiological investigation.
- Published
- 1989
50. Frontonasal dysplasia associated with tetralogy of Fallot
- Author
-
Derek G. Human, M. M. A. De Moor, and R. Baruch
- Subjects
Male ,medicine.medical_specialty ,Nose ,Genetics ,Humans ,Medicine ,Abnormalities, Multiple ,Frontonasal dysplasia ,Hypertelorism ,Child ,Facial anomaly ,Genetics (clinical) ,Tetralogy of Fallot ,Bone Diseases, Developmental ,business.industry ,Infant ,Anatomy ,medicine.disease ,Nasal tip ,Surgery ,Mental deficiency ,Median cleft face syndrome ,Multifactorial Inheritance ,Female ,medicine.symptom ,business ,Research Article - Abstract
Three children with frontonasal dysplasia associated with tetralogy of Fallot are reported. All cases had true hypertelorism and a median nasal groove with absence of the nasal tip. There was no mental deficiency. The facial anomaly is a sporadic, non-genetic interference of the normal development of the face. This is the first report of frontonasal dysplasia associated with a cardiac defect. Multifactorial inheritance of this syndrome is proposed.
- Published
- 1987
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