76 results on '"Derek G. Human"'
Search Results
2. Physiological Responses to Exercise in Pediatric Heart Transplant Recipients
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George G.S. Sandor, Derek G. Human, Astrid M. De Souza, Anita T. Cote, Joshua M. Bovard, Martin C.K. Hosking, Kevin C. Harris, Kathryn Armstrong, and James E. Potts
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Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Cardiac index ,Hemodynamics ,Physical Therapy, Sports Therapy and Rehabilitation ,Ventricular Function, Left ,Fick principle ,Oxygen Consumption ,Heart Rate ,Internal medicine ,Arteriovenous oxygen difference ,Heart rate ,medicine ,Humans ,Orthopedics and Sports Medicine ,Child ,Exercise ,Retrospective Studies ,Heart transplantation ,Body surface area ,business.industry ,Stroke Volume ,Stroke volume ,Transplant Recipients ,Echocardiography ,Case-Control Studies ,Exercise Test ,Cardiology ,Heart Transplantation ,Female ,business - Abstract
INTRODUCTION Pediatric heart transplant (HTx) recipients have reduced exercise capacity typically two-thirds of predicted values, the mechanisms of which are not fully understood. We sought to assess the cardiorespiratory responses to progressive exercise in HTx relative to controls matched for age, sex, body size, and work rate. METHODS Fourteen HTx recipients and matched controls underwent exercise stress echocardiography on a semisupine cycle ergometer. Hemodynamics, left ventricular (LV) dimensions, and volumes were obtained and indexed to body surface area. Oxygen consumption (V˙O2) was measured, and arteriovenous oxygen difference was estimated using the Fick Principle. RESULTS At rest, LV mass index (P = 0.03) and volumes (P < 0.001) were significantly smaller in HTx, whereas wall thickness (P < 0.01) and LV mass-to-volume ratio (P = 0.01) were greater. Differences in LV dimensions and stroke volume persisted throughout exercise, but the pattern of response was similar between groups as HR increased. As exercise progressed, heart rate and cardiac index increased to a lesser extent in HTx. Despite this, V˙O2 was similar (P = 0.82) at equivalent work rates as HTx had a greater change in arteriovenous oxygen difference (P < 0.01). CONCLUSIONS When matched for work rate, HTx had similar metabolic responses to controls despite having smaller LV chambers and an attenuated increase in hemodynamic responses. These findings suggest that HTx may increase peripheral O2 extraction as a compensatory mechanism in response to reduced cardiovascular function.
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- 2019
3. 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
4. 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.
- Published
- 2018
5. Using Text Messaging to Communicate with Adolescent Heart Transplant Patients
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Tim F. Oberlander, Steven L. Rathgeber, Derek G. Human, Tom Blydt-Hansen, J.E. Potts, Richard T. Lester, Kathryn Armstrong, Claire R Galvin, A.M. De Souza, and Sarah M. Hutchison
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Pulmonary and Respiratory Medicine ,Transplantation ,medicine.medical_specialty ,business.industry ,Patient response ,Quality of life ,Intervention (counseling) ,Health care ,Physical therapy ,medicine ,Text messaging ,Vulnerable population ,Surgery ,Transplant patient ,Cognitive skill ,Cardiology and Cardiovascular Medicine ,business - Abstract
Purpose Pediatric heart transplant (PHTx) patients require life-long medication and follow up. Adolescents are a vulnerable population with multifactorial reasons for non-adherence to their medical needs. Adherence may depend on the cognitive skills required to plan and to self-regulate, referred to as executive function (EF). Deficits in EF are associated with medication non-adherence. Facilitating consistent communication between adolescents and their health care providers (HCP) via a short messaging service (SMS) platform allows for regular communication and may improve quality of life (QoL) and medication compliance. Methods In collaboration with WelTel, we have implemented a secure, healthcare-specific SMS platform for PHTx aged 12-18 years. At enrollment, EF was assessed by both patient and parent reports using the BRIEF2 (reported global executive composite (GEC) T-score). PedsQL questionnaires and surveys to assess patient engagement with the SMS platform were measured at enrollment and will be reassessed after 1 year. Change in serum immunosuppression levels from baseline to 1 year post SMS intervention will be used as a surrogate measure of medication compliance. Patient response rate and number of care conversations (≥3 messages) were reviewed. Results We report preliminary data on 17 eligible PHTx. Eight have enrolled, 9 in total did not due to a lack of interest (n=2), no access to a cellular phone (n=5), and developmental delay (n=2). At baseline the BRIEF2 did not demonstrate any clinically significant abnormalities in EF, with all scores below the threshold of 70 (mean patient GEC T-score = 52.4, range 39-68; mean parent GEC T-score = 50, range 39-61). Median baseline QoL score was 85 (Range 70-98; healthy adolescents= 83+/-15). Patients have been connected to the SMS network for a median duration of 3.4 months (1.2 - 8.0 months) and there have been a total of 551 messages with 89 that have consisted of a conversation of ≥3 messages. Conclusion We present a novel mode of communication between adolescent PHTx and their healthcare providers. At baseline there is no impairment in EF and QoL is consistent with healthy adolescents. Patients that are enrolled have demonstrated frequent communication using the SMS platform since its introduction. The long-term impact of increased communication on QoL and medication compliance will be reviewed after 1 year.
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- 2020
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. 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
10. 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
11. 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
12. 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
13. 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.
- Published
- 2017
14. 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
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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
15. A novel treadmill protocol for exercise testing in children: the British Columbia Children's Hospital protocol
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Astrid M. De Souza, D. Kathryn Duff, James E. Potts, Kevin C. Harris, and Derek G. Human
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medicine.medical_specialty ,pediatrics ,Physical Therapy, Sports Therapy and Rehabilitation ,030204 cardiovascular system & hematology ,cardiorespiratory function ,Metabolic equivalent ,Broadcast control channel ,03 medical and health sciences ,0302 clinical medicine ,Bruce protocol ,Heart rate ,medicine ,Orthopedics and Sports Medicine ,treadmill protocol ,Treadmill ,Respiratory exchange ratio ,Original Research ,Protocol (science) ,validation ,business.industry ,030229 sport sciences ,Physical therapy ,business ,human activities ,Respiratory minute volume - Abstract
Background Exercise testing in children is widely recommended for a number of clinical and prescriptive reasons. Many institutions continue to use the Bruce protocol for treadmill testing; however, with its incremental changes in speed and grade, it has challenges for practical application in children. We have developed a novel institutional protocol (British Columbia Children’s Hospital (BCCH)), which may have better utility in paediatric populations. Aim To determine if our institutional protocol yields similar peak responses in minute ventilation (VE), oxygen consumption (VO 2 ), carbon dioxide production (VCO 2 ), respiratory exchange ratio (RER), metabolic equivalents (METS) and heart rate (HR) when compared with the traditional Bruce protocol. Methods On two different occasions, 70 children (boys=33; girls=37) aged 10–18 years completed an exercise test on a treadmill using each of the protocols. During each test, metabolic gas exchange parameters were measured. HR was monitored continuously during exercise using an HR monitor. Results Physiological variables were similar between the two protocols (median (IQR); r s ): VE (L/min) (BCCH=96.7 (72.0–110.2); Bruce=99.2 (75.6–120.0); r s =0.95), peak VO 2 (mL/min) (BCCH=2897 (2342–3807); Bruce=2901 (2427–3654); r s =0.94) and METS (BCCH=16.2 (14.8–17.7); Bruce=16.4 (14.7–17.9); r s =0.89). RERs were similar (BCCH=1.00 (0.96–1.02); Bruce=1.03 (0.99–1.07); r s =0.48). Total exercise time (in seconds) was longer for the BCCH protocol: BCCH=915 (829–1005); Bruce=810 (750-919); r s =0.67. Conclusion The BCCH protocol produces similar peak exercise responses to the Bruce protocol and provides an alternative for clinical exercise testing in children.
- Published
- 2017
16. THROMBOEMBOLISM IN THE ADULT FONTAN POPULATION: A MARKER OF FONTAN CIRCULATORY DYSFUNCTION?
- Author
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M. Riahi, V. Tony, N. Johal, J. Grewal, Jonathan Leipsic, Gnalini Sathananthan, Derek G. Human, S. Sandhu, Marla Kiess, and S. Chakrabarti
- Subjects
medicine.medical_specialty ,education.field_of_study ,business.industry ,Internal medicine ,Circulatory system ,Population ,Cardiology ,medicine ,Cardiology and Cardiovascular Medicine ,business ,education - Published
- 2019
17. Physical Activity, Exercise Capacity and Quality of Life in Adolescent Heart Transplant Patients
- Author
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Claire R Galvin, A.M. De Souza, Samantha Lui, Derek G. Human, Anita T. Cote, T. Sueyoshi, N. Arora, and Kathryn Armstrong
- Subjects
Pulmonary and Respiratory Medicine ,National Physical Activity Guidelines ,Transplantation ,medicine.medical_specialty ,business.industry ,Gold standard ,Physical activity ,Exercise capacity ,humanities ,Test (assessment) ,Quality of life ,Interquartile range ,Physical therapy ,Medicine ,Surgery ,Cardiology and Cardiovascular Medicine ,business ,Aerobic capacity - Abstract
Purpose The primary goal of pediatric heart transplantation (PHTx) is to improve the quality of life (QoL) of patients. National physical activity guidelines for adolescents recommend at least 60-90 minutes of moderate to vigorous physical activity per day. The Physical Activity Questionnaire (PAQ-C/A) for children and adolescents provides general estimates of physical activity levels. Measurement of peak aerobic capacity (VO2peak) using cardiopulmonary exercise testing (CPX) is the gold standard for assessing exercise capacity. Both physical activity and exercise capacity may be important factors that influence reporting a normal QoL. Our aim was to determine if there was a relationship between physical activity, exercise capacity, and QoL in a PHTx adolescent population. Methods This is a single centre retrospective chart review of adolescent (11-18 yrs) PHTx patients. Those who completed a maximal CPX test (RER>1.0), self-report physical activity (PAQ-C or PAQ-A) and QoL (PedsQL and PedsQL-Transplant) questionnaires, as part of their annual assessment, were eligible for inclusion. Results were compared to published age- and sex-matched healthy control data used in our laboratory. VO2peak and QoL were deemed to be normal if results were within two standard deviations of the mean. Pearson correlation coefficients were calculated. Medians and interquartile ranges are reported. Results Thirteen PHTx (10 males) completed a CPX and both QoL questionnaires. The median age was 14.9 yrs (12.3-17.5 yrs). VO2peak=34.5 mL/min/kg (30.2-43.4 mL/min/kg. Only 1 of 13 had an abnormal VO2peak. Total Peds QL score=84.8 (68.5-92.4) with 10 out 13 reporting a normal QoL. The Peds QL-Transplant score=90.5 (84.8-95.1). There was no significant correlation between VO2peak and QoL. The PAQ-C/PAQ-A was completed in 10 HTx. PAQ scores=2.38 (1.59-3.01). Only 2 of 13 met the cut-off point (>2.87) for adequate physical activity. There was a significant correlation between the Peds QL-Transplant QoL and the PAQ (r=0.69; p=0.04) Conclusion PHTx adolescents do not meet their required physical activity recommendations. Despite this, they have low normal exercise capacity and report a normal QoL. Efforts to engage adolescents to increase their physical activity should be encouraged.
- Published
- 2019
18. 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
19. 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
20. Response to: Comment on 'Assessment of Liver Stiffness in Pediatric Fontan Patients Using Transient Elastography'
- Author
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Orlee R. Guttman, Richard A. Schreiber, Becky Chen, James E. Potts, and Derek G. Human
- Subjects
medicine.medical_specialty ,Hepatology ,business.industry ,Gastroenterology ,General Medicine ,030204 cardiovascular system & hematology ,03 medical and health sciences ,Elasticity Imaging Techniques ,0302 clinical medicine ,Text mining ,Liver ,Liver stiffness ,medicine ,Humans ,lcsh:Diseases of the digestive system. Gastroenterology ,030211 gastroenterology & hepatology ,Radiology ,lcsh:RC799-869 ,business ,Transient elastography ,Letter to the Editor - Published
- 2017
21. Left Ventricle Pseudoaneurysm After Aortic Valvuloplasty
- Author
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Martin C.K. Hosking, Derek G. Human, and Abdullah Alhuzaimi
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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
22. Serial measurements of exercise performance in pediatric heart transplant patients using stress echocardiography
- Author
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James E. Potts, Astrid M. De Souza, Derek G. Human, George G.S. Sandor, and Joanne P. Yeung
- Subjects
Cardiac function curve ,Heart transplantation ,Transplantation ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Hemodynamics ,Immunosuppression ,Surgery ,Contractility ,Blood pressure ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Stress Echocardiography ,medicine ,Cardiology ,business - Abstract
Yeung JP, Human DG, Sandor GGS, De Souza AM, Potts JE. Serial measurements of exercise performance in pediatric heart transplant patients using stress echocardiography. Pediatr Transplantation 2011: 15: 265–271. © 2011 John Wiley & Sons A/S. Abstract: Heart transplantation is an increasingly acceptable therapeutic option for children with end-stage and complex congenital heart disease. With advances in surgery, immunosuppression, and follow-up care, functional outcomes need to be evaluated. We report the results of serial exercise testing performed using stress echocardiography in a cohort of pediatric HTP. HTP (n = 7) exercised on a semi-recumbent ergometer to volitional fatigue. Echocardiography-Doppler measurements, HR, and blood pressure were taken at rest and during staged exercise. Results were compared with healthy CON (n = 12). HTP did significantly less work during exercise (940 vs. 1218 J/kg, p
- Published
- 2011
23. ATRIAL ARRHYTHMIAS AND THROMBOEMBOLIC COMPLICATIONS IN ADULTS WITH FONTAN CIRCULATION
- Author
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Marla Kiess, Derek G. Human, J. Grewal, S. Chakrabarti, Andrew D. Krahn, Darryl Wan, A. Barlow, and M. Riahi
- Subjects
medicine.medical_specialty ,business.industry ,Internal medicine ,Cardiology ,Medicine ,Atrial arrhythmias ,Cardiology and Cardiovascular Medicine ,business ,Fontan circulation - Published
- 2018
24. 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
25. 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
26. 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
27. 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
28. PAN-CANADIAN SURVEY OF MODELS OF CARE OF PATIENTS WITH THORACIC AORTIC DISEASE
- Author
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D.Y. Tam, Christopher A. Caldarone, G. Horne, Ismail El-Hamamsy, Mark E. Peterson, Derek G. Human, Timothy J. Bradley, Maral Ouzounian, Richard D. Weisel, and Nanette Alvarez
- Subjects
medicine.medical_specialty ,business.industry ,medicine ,Thoracic aortic disease ,Cardiology and Cardiovascular Medicine ,business ,Surgery - Published
- 2016
29. 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
30. Clipped tube fenestration after extracardiac Fontan allows for simple transcatheter coil occlusion
- Author
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Michael W.H. Patterson, Jacques G. LeBlanc, Derek G. Human, J. A. Gordon Culham, Timothy J. Bradley, Suvro S. Sett, and Walter J. Duncan
- Subjects
Heart Defects, Congenital ,Pulmonary and Respiratory Medicine ,Cardiac Catheterization ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Fontan Procedure ,Surgery ,Child, Preschool ,Complete occlusion ,cardiovascular system ,medicine ,Coil occlusion ,Humans ,Tube (fluid conveyance) ,cardiovascular diseases ,Transcutaneous oxygen ,Child ,Cardiology and Cardiovascular Medicine ,business ,Fenestration ,Shunt (electrical) ,Retrospective Studies ,Cardiac catheterization - Abstract
Background. Expensive devices are increasingly used to close a patent fenestration after a modified Fontan operation. We report our 5-year institutional experience of clipped tube fenestration after extracardiac Fontan operation, which allows for simple transcatheter coil occlusion. Methods. We retrospectively reviewed 30 children, median age of 4.0 years (range, 2.4 to 8.8 years) who underwent extracardiac Fontan operation between May 1996 and May 2001, and were fenestrated using a 4- to 8-mm diameter clipped tube graft. Results. Ten children had a patent fenestration occluded by transcatheter placement of 15 detachable coils (5- to 8-mm diameter). Aortic oxygen saturations increased on average by 5.5% (2% to 14%) and mean pressures in the Fontan circuit by 2.5 mm Hg (0 to 3 mm Hg). Four had immediate complete occlusion angiographically and 6 had trivial residual shunt, but complete occlusion by echocardiography at follow-up. There have been no immediate complications, late coil embolizations, thromboembolic events, or documented hemolysis within a follow-up after coil implantation of 1.7 years (0.4 to 4.5 years). Spontaneous fenestration closure was documented in 8 patients at cardiac catheterization and 9 patients by echocardiography with consistent improvement in resting transcutaneous oxygen saturation. Two children with a patent fenestration have been considered inappropriate for closure, and there was one early surgical death. There have been no complications related to the tube fenestration modification within a follow-up postoperation of 2.6 years (0.1 to 5.5 years). Conclusions. Clipped tube fenestration after extracardiac Fontan operation is a useful surgical modification that allows for simple transcatheter coil occlusion.
- Published
- 2003
31. 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
32. 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
33. WITHDRAWN: Presentation, Diagnosis, and Medical Management of Heart Failure in Children: Canadian Cardiovascular Society Guidelines
- Author
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Aamir Jeewa, Reeni Soni, Derek G. Human, Kenny K. Wong, Paul F. Kantor, Michael McGillion, Lindsay M. Ryerson, Steven C. Greenway, Jane Lougheed, Kristen George, Carol Chan, Adrian Dancea, J. Conway, S. Lucy Roche, Holger Buchholz, Nicole Barbosa, Joseph Atallah, Robert D. Ross, Jack F. Price, Catherine Chant-Gambacort, Letizia Gardin, Rejane Dillenburg, and Judith Wilson
- Subjects
medicine.medical_specialty ,Presentation ,business.industry ,media_common.quotation_subject ,Pediatrics, Perinatology and Child Health ,Management of heart failure ,Alternative medicine ,Medicine ,Canadian Cardiovascular Society ,business ,Intensive care medicine ,media_common - Published
- 2014
34. 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
35. Presentation, diagnosis, and medical management of heart failure in children: Canadian Cardiovascular Society guidelines
- Author
-
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
36. ATRIAL ARRHYTHMIAS AND THROMBOEMBOLIC EPISODES IN ADULTS WITH REPAIRED TETROLOGY OF FALLOT: LONG TERM FOLLOW-UP
- Author
-
Marla Kiess, Derek G. Human, Christian Steinberg, Andrew D. Krahn, Clara Tsui, A. Barlow, J. Grewal, Darryl Wan, and S. Chakrabarti
- Subjects
medicine.medical_specialty ,Long term follow up ,business.industry ,Internal medicine ,Cardiology ,Medicine ,Atrial arrhythmias ,Cardiology and Cardiovascular Medicine ,business - Published
- 2016
37. Serial measurements of exercise performance in pediatric heart transplant patients using stress echocardiography
- Author
-
Joanne P, Yeung, Derek G, Human, George G S, Sandor, Astrid M, De Souza, and James E, Potts
- Subjects
Adult ,Male ,Exercise Tolerance ,Adolescent ,Heart Diseases ,Hemodynamics ,Blood Pressure ,Ultrasonography, Doppler ,Pediatrics ,Ventricular Function, Left ,Treatment Outcome ,Echocardiography ,Exercise Test ,Heart Transplantation ,Humans ,Female ,Child ,Exercise ,Immunosuppressive Agents ,Echocardiography, Stress - Abstract
Heart transplantation is an increasingly acceptable therapeutic option for children with end-stage and complex congenital heart disease. With advances in surgery, immunosuppression, and follow-up care, functional outcomes need to be evaluated. We report the results of serial exercise testing performed using stress echocardiography in a cohort of pediatric HTP. HTP (n = 7) exercised on a semi-recumbent ergometer to volitional fatigue. Echocardiography-Doppler measurements, HR, and blood pressure were taken at rest and during staged exercise. Results were compared with healthy CON (n = 12). HTP did significantly less work during exercise (940 vs. 1218 J/kg, p0.03). Their SVI (33 vs. 49 mL/m(2), p0.003), CI (5.16 vs. 9.25 L/min/m(2), p0.0005), and HR (162 vs. 185 bpm, p0.02) were lower at peak exercise. HTP had a lower SF at peak exercise (48% vs. 52%, p0.03) and an abnormal relationship between the MVCFc and σPS. During follow-up, hemodynamics and left ventricular function remained relatively constant in HTP. HTP are able to exercise safely; however, their exercise tolerance is reduced, and hemodynamics and contractility are diminished. Over time, their hemodynamics and left ventricular function have remained relatively constant.
- Published
- 2011
38. Doppler echocardiographic and morphologic evaluation of patients following operative repair of aortic coarctation
- Author
-
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
39. 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
-
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
40. A case of horseshoe lung and complex congenital heart disease in a term newborn
- Author
-
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
41. Progression of organ manifestations upon enzyme replacement therapy in a patient with mucopolysaccharidosis type I/Hurler
- Author
-
Saadet Mercimek-Mahmutoglu, Sylvia Stoeckler-Ipsiroglu, Derek G. Human, Christopher W. Reilly, and Paula J. Waters
- Subjects
Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Pediatrics ,medicine.medical_treatment ,Hearing Loss, Sensorineural ,Mucopolysaccharidosis I ,Hematopoietic stem cell transplantation ,Mucopolysaccharidosis type I ,Iduronidase ,Pediatric surgery ,medicine ,Humans ,Enzyme Replacement Therapy ,Glycosaminoglycans ,Maternal and child health ,business.industry ,LARONIDASE ,Hematopoietic Stem Cell Transplantation ,nutritional and metabolic diseases ,Enzyme replacement therapy ,Surgery ,surgical procedures, operative ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Disease Progression ,business - Abstract
Enzyme replacement therapy (ERT) has been increasingly used as an interim treatment in severe mucopolysaccharidosis type I (MPSI)/Hurler patients prior to hematopoietic stem cell transplantation (HSCT).We present the outcome of a patient with MPSI/Hurler after 14 months of ERT prior to HSCT.Urinary glucosaminoglycan excretion decreased by 70% after one month of ERT. Liver volume decreased by 14% of baseline after 12 months of ERT. Pre-existing thoracolumbar kyphosis progressed to thoracolumbar dislocation with complete displacement of facets after 12 months of ERT. New development of mitral valve thickening was found by echocardiography and mild hearing loss progressed to severe sensorineural hearing loss after 13 months of ERT.ERT over a period of 14 months did not prevent progression of organ manifestations in our patient. Patients should be monitored every 6 months for cardiac, skeletal and audiological involvement on ERT.
- Published
- 2008
42. Noninvasive Estimation of Aortic Valve Areas in Children with Aortic Stenosis
- Author
-
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
43. Validating a new Treadmill Protocol for Clinical Exercise Testing in Children
- Author
-
Kathryn Duff, James E. Potts, Kevin M. Harris, Derek G. Human, and Astrid M. De Souza
- Subjects
Protocol (science) ,Broadcast control channel ,medicine.medical_specialty ,Bruce protocol ,Physical medicine and rehabilitation ,business.industry ,Physical therapy ,Medicine ,Physical Therapy, Sports Therapy and Rehabilitation ,Orthopedics and Sports Medicine ,Treadmill ,business - Published
- 2015
44. 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
45. 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
46. Balloon Angioplasty for Congenital Aortic Valve Stenosis
- Author
-
Derek G. Human, George G.S. Sandor, and James E. Potts
- Subjects
Male ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Aortic Valve Stenosis ,Balloon ,Catheterization ,Internal medicine ,Angioplasty ,medicine ,Cardiology ,Congenital aortic valve stenosis ,Humans ,Female ,Cardiology and Cardiovascular Medicine ,business - Published
- 2012
47. 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
48. 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
49. 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
50. 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
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