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Cardiovascular disease (CVD) is excessively prevalent and premature in bipolar disorder (BD), even after controlling for traditional cardiovascular risk factors. The increased risk of CVD in BD may be subserved by microvascular dysfunction. We examined coronary microvascular function in relation to youth BD.
Participants were 86 youth, ages 13–20 years (n = 39 BD, n = 47 controls). Coronary microvascular reactivity (CMVR) was assessed using quantitative T2 magnetic resonance imaging during a validated breathing-paradigm. Quantitative T2 maps were acquired at baseline, following 60-s of hyperventilation, and every 10-s thereafter during a 40-s breath-hold. Left ventricular structure and function were evaluated based on 12–15 short- and long-axis cardiac-gated cine images. A linear mixed-effects model that controlled for age, sex, and body mass index assessed for between-group differences in CMVR (time-by-group interaction).
The breathing-paradigm induced a significant time-related increase in T2 relaxation time for all participants (i.e. CMVR; β = 0.36, p < 0.001). CMVR was significantly lower in BD v. controls (β = −0.11, p = 0.002). Post-hoc analyses found lower T2 relaxation time in BD youth after 20-, 30-, and 40 s of breath-holding (d = 0.48, d = 0.72, d = 0.91, respectively; all pFDR < 0.01). Gross left ventricular structure and function (e.g. mass, ejection fraction) were within normal ranges and did not differ between groups.
Youth with BD showed evidence of subclinically impaired coronary microvascular function, despite normal gross cardiac structure and function. These results converge with prior findings in adults with major depressive disorder and post-traumatic stress disorder. Future studies integrating larger samples, prospective follow-up, and blood-based biomarkers are warranted.
Children who develop coronary artery aneurysms after Kawasaki disease are at risk for cardiovascular morbidity, requiring health care transition and lifelong follow-up with an adult specialist. Follow-up losses after health care transition have been reported but without outcome and patient experience evaluation.
The Theoretical Domains Framework underpinned our aim to explore the required self-care behaviours and experiences of young adults’ post-health care transition.
A qualitative description approach was used for virtual, 1:1 interviews with 11 participants, recruited after health care transition from a regional cardiac centre in Ontario. Directed content analysis was employed.
Health, psychosocial, and lifestyle challenges were compounded by a sense of loss. Six themes emerged within the Theoretical Domains Framework categories. Participants offered novel health care transition programme recommendations.
The realities of health care transition involve multiple, overlapping stressors for young adults with Kawasaki disease and coronary artery aneurysms. Our findings will inform a renewed health care transition programme and will include outcome evaluation.
Post-traumatic stress disorder occurs in parents of infants with CHD, contributing to psychological distress with detrimental effects on family functioning and well-being. We sought to determine the prevalence and factors associated with post-traumatic stress disorder symptoms in parents whose infants underwent staged palliation for single ventricle heart disease.
Materials and methods:
A large longitudinal multi-centre cohort study evaluated 215 mothers and fathers for symptoms of post-traumatic stress disorder at three timepoints, including post-Norwood, post-Stage II, and a final study timepoint when the child reached approximately 16 months of age, using the self-report questionnaire Impact of Event Scale – Revised.
The prevalence of probable post-traumatic stress disorder post-Norwood surgery was 50% of mothers and 39% of fathers, decreasing to 27% of mothers and 24% of fathers by final follow-up. Intrusive symptoms such as flashbacks and nightmares and hyperarousal symptoms such as poor concentration, irritability, and sudden physical symptoms of racing heart and difficulty breathing were particularly elevated in parents. Higher levels of anxiety, reduced coping, and decreased satisfaction with parenting were significantly associated with symptoms of post-traumatic stress disorder in parents. Demographic and clinical variables such as parent education, pre-natal diagnosis, medical complications, and length of hospital stay(s) were not significantly associated with symptoms of post-traumatic stress disorder.
Parents whose infants underwent staged palliation for single ventricle heart disease often reported symptoms of post-traumatic stress disorder. Symptoms persisted over time and routine screening might help identify parents at-risk and prompt referral to appropriate supports.
Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials.
We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design.
Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field.
Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.
Recent years have seen an exponential increase in the variety of healthcare data captured across numerous sources. However, mechanisms to leverage these data sources to support scientific investigation have remained limited. In 2013 the Pediatric Heart Network (PHN), funded by the National Heart, Lung, and Blood Institute, developed the Integrated CARdiac Data and Outcomes (iCARD) Collaborative with the goals of leveraging available data sources to aid in efficiently planning and conducting PHN studies; supporting integration of PHN data with other sources to foster novel research otherwise not possible; and mentoring young investigators in these areas. This review describes lessons learned through the development of iCARD, initial efforts and scientific output, challenges, and future directions. This information can aid in the use and optimisation of data integration methodologies across other research networks and organisations.
Anomalous aortic origin of the coronary arteries is associated with exercise-induced ischaemia, leading some physicians to restrict exercise in patients with this condition. We sought to determine whether exercise restriction was associated with increasing body mass index over time. From 1998 to 2015, 440 patients ⩽30 years old were enrolled into an inception cohort. Exercise-restriction status was documented in 143 patients. Using linear mixed model repeated-measures regression, factors associated with increasing body mass index z-score over time, including exercise restriction and surgical intervention as time-varying covariates, were investigated. The 143 patients attended 558 clinic visits for which exercise-restriction status was recorded. The mean number of clinic visits per patient was 4, and the median duration of follow-up was 1.7 years (interquartile range (IQR) 0.5–4.4). The median age at first clinic visit was 10.3 years (IQR 7.1–13.9), and 71% (101/143) were males. All patients were alive at their most recent follow-up. At the first clinic visit, 54% (78/143) were exercise restricted, and restriction status changed in 34% (48/143) during follow-up. The median baseline body mass index z-score was 0.2 (IQR 0.3–0.9). In repeated-measures analysis, neither time-related exercise restriction nor its interaction with time was associated with increasing body mass index z-score. Surgical intervention and its interaction with time were associated with decreasing body mass index z-score. Although exercise restriction was not associated with increasing body mass index over time, surgical intervention was associated with decreasing body mass index z-score over time in patients with anomalous aortic origin of the coronary arteries.
A few studies have evaluated the impact of clinical trial results on practice in paediatric cardiology. The Infant Single Ventricle (ISV) Trial results published in 2010 did not support routine use of the angiotensin-converting enzyme inhibitor enalapril in infants with single-ventricle physiology. We sought to assess the influence of these findings on clinical practice.
A web-based survey was distributed via e-mail to over 2000 paediatric cardiologists, intensivists, cardiothoracic surgeons, and cardiac advance practice nurses during three distribution periods. The results were analysed using McNemar’s test for paired data and Fisher’s exact test.
The response rate was 31.5% (69% cardiologists and 65% with >10 years of experience). Among respondents familiar with trial results, 74% reported current practice consistent with trial findings versus 48% before trial publication (p<0.001); 19% used angiotensin-converting enzyme inhibitor in this population “almost always” versus 36% in the past (p<0.001), and 72% reported a change in management or improved confidence in treatment decisions involving this therapy based on the trial results. Respondents familiar with trial results (78%) were marginally more likely to practise consistent with the trial results than those unfamiliar (74 versus 67%, p=0.16). Among all respondents, 28% reported less frequent use of angiotensin-converting enzyme inhibitor over the last 3 years.
Within 5 years of publication, the majority of respondents was familiar with the Infant Single Ventricle Trial results and reported less frequent use of angiotensin-converting enzyme inhibitor in single-ventricle infants; however, 28% reported not adjusting their clinical decisions based on the trial’s findings.
Background: Foetuses with simple tetralogy of Fallot almost universally have a patent ductus arteriosus. Two recently identified cases had an absent patent ductus arteriosus, requiring emergent intervention at birth. The objective of this study was to determine whether foetuses diagnosed with tetralogy of Fallot and no patent ductus arteriosus have poorer outcomes compared with those with tetralogy of Fallot+patent ductus arteriosus. Methods: All foetal cases of tetralogy of Fallot between January, 2000 and 2012 were retrospectively identified from The Hospital for Sick Children (Toronto, Canada) database. Cases – tetralogy of Fallot+no patent ductus arteriosus confirmed on postnatal echo – and controls – tetralogy of Fallot+patent ductus arteriosus, matched for gestational age – were identified from prenatal records, and both clinical and echocardiographic data were reviewed. Optimal outcome was defined as valve-sparing repair with no residual lesions. Student’s t-tests and Fisher’s exact χ2 were used to compare groups. Results: n=115 foetuses were diagnosed with tetralogy of Fallot: 11 (9%) had no patent ductus arteriosus, and were matched to 22 controls – mean gestational age at diagnosis 23.2±4.2 weeks, 23.4±6.6 weeks, respectively. Cases had a higher proportion of right aortic arches (64% versus 14%, p<0.001). Foetal and postnatal echocardiographic data did not reveal significant differences in branch pulmonary artery sizes, pulmonary valve sizes, or ventricular function. No differences were identified for cyanosis at birth (2/10 versus 7/20, p=0.67), or catheter intervention (5/10 versus 4/22, p=0.12). Optimal outcome rates were similar between cases and controls (4/11 (36%) versus 5/21 (24%), p=0.68). Conclusions: The patent ductus arteriosus does not appear to have an impact on clinical outcome in foetuses with tetralogy of Fallot.
Purpose: The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease. Methods: This was a cross-sectional observational study involving 325 single ventricle patients, aged 10–18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables were compared. Results: From the generic assessment, patients perceived marginally lower physical functioning (p = 0.05) but greater freedom from bodily pain compared with a normal population (p < 0.001). The equivalent physical functioning/limitations domain of the generic instrument, compared with the disease-specific instrument, had similar associations (higher multi-variable model R2) with medical history variables (R2 = 0.14 versus R2 = 0.12, respectively) and stronger associations with exercise testing variables (R2 = 0.22 versus R2 = 0.06). Similarly, the corresponding freedom from bodily pain/symptoms domains from both questionnaires showed a greater association for the generic instrument with medical history variables (R2 = 0.15 versus R2 = 0.09, respectively) and non-cardiac conditions (R2 = 0.13 versus R2 = 0.06). The associations of each questionnaire with echocardiographic results, cardiac magnetic resonance imaging results, and serum brain natriuretic peptide levels were uniformly weak (R2 range <0.01 to 0.04). Conclusions: Assessment of the physical functional health status using generic and disease-specific instruments yields few differences with regard to associations between conceptually similar domains and patient and clinical characteristics for adolescents after Fontan procedure.
A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.
We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114).
A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome.
After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.
We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles.
Foetuses were included who had a left ventricle that was 2–4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models.
From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean −3.8 versus −4.2 intervention group, p = 0.04, odds ratio 2.8).
The need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography.
To determine the gross motor skills of school-aged children after the Fontan procedure and compare the locomotor and object control skills with normative data.
This study followed a cross-sectional design.
This study was based on hospital outpatient visit, with accelerometry conducted at home.
This study included 55 patients, including 22 girls in the age group of 6–10 years, 5.1 years after Fontan.
Main outcome measures
Test of Gross Motor Development – Version 2, daily activity by accelerometer, medical history review, child and parent perceptions of activity.
Being involved in active team sports increased locomotor percentile score by 10.3 points (CI: 4.4, 16.1). Preference for weekend outdoor activities (6.9, CI: 2.0, 11.8), performing at least 30 minutes of moderate-to-vigorous physical activity daily (24.5, CI: 7.3, 41.8), and reporting that parents seldom criticise the child's physical activity (21.8, CI: 8.9, 34.8) were also associated with higher locomotor percentile scores (p < 0.01). Object control percentile scores were higher (p < 0.03) with involvement in formal instruction (5.9, CI: 1.1, 10.6) and being restricted to “activities within comfortable limits” (27.6, CI: 7.7, 47.5). Older chronological age (r = 0.28), a more complicated medical history (r = 0.36), and older age at Fontan (r = 0.28) were associated with greater skill delay (p < 0.04).
Children after Fontan attain basic motor skills at a later age than their peers, and deficits continue for more complex skills as age increases, suggesting a need for longitudinal monitoring of gross motor skill development through the elementary school years. Future research might investigate whether a gross motor skill rehabilitation programme can provide these children with the motor skills needed to successfully participate in a physically active lifestyle with peers.
Obstruction of the reconstructed aortic arch, or the neoaortic arch, is now known to be an important factor increasing mortality after the Norwood operation for hypoplastic left heart syndrome. Transcatheter balloon angioplasty has been shown to provide effective relief of both native aortic coarctation and obstructions of the aortic arch occurring subsequent to therapeutic intervention. We sought to determine the outcomes of balloon angioplasty used as an initial treatment for obstruction of the neoaortic arch occurring after the Norwood operation. We gathered the characteristics of 58 patients with such obstruction from 8 institutions, noting procedural factors and outcomes of initial balloon dilation. Obstruction occurred at a median interval of 4 months, with a range from 1.5 months to 6.3 years, after a Norwood operation. Ventricular dysfunction was present before dilation in 13 patients. Mean peak to peak systolic pressure gradients were acutely reduced from 31±20 mm Hg to 6±9 mmHg (p<0.001), with outcome subjectively judged to be successful in 89%- Three patients with pre-existing ventricular dysfunction died within 48 hours of dilation. There were 10 additional deaths during the period of followup, with Kaplan Meier estimates of survival after intervention of 87% at 1 month, 77% at 12 months, and 72% after 15 months. In addition, 9 patients required re-intervention during the period of follow-up, with Kaplan Meier estimates of freedom from re-intervention after dilation of 87% at 6 months, 78% at 12 months and 74% after 18 months. Although transcatheter dilation of neoaortic arch obstructions after Norwood operation is successful, there is a high risk of re-intervention and ongoing mortality in this subgroup of patients. Close follow-up is recommended.
To identify child and parental factors associated with screen time in 3-year-old children.
Participants were recruited from a large primary-care paediatric group practice in Toronto, Canada.
Healthy 3-year-old children were included. A questionnaire was completed by their parents on screen time. Descriptive statistics and linear regression models were used to assess associations between child screen time and selected factors. Multivariable models included factors from the univariate analysis with P < 0·1. Estimated effects and 95 % CI are reported.
A total of 157 children were enrolled (91 % recruitment). The mean screen time per weekday was 104 min (similar for weekend day). In all, 10 % of children had a television (TV) in their bedroom; 59 % consumed at least one meal while watching TV; and 81 % of parents had household rules about screen time. Controlling for maternal education and age, eating lunch and dinner in front of the screen and mother being employed were associated with an increase in child weekday screen time of 96 (95 % CI 30, 192), 42 (95 % CI 12, 90) and 36 (95 % CI 6, 72) min/d, respectively. Eating lunch in front of the screen and an increase of 1 h of parental screen time were associated with an increase of 78 (95 % CI 36, 132) and 12 (95 % CI 6, 18) min/d in child weekend screen time. Family rules decreased child weekend screen time by 30 (95 % CI 6, 54) min/d.
Interventions that include these important parental factors should be evaluated for their effectiveness in reducing screen time.
Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level. We sought to assess the impact of increasing altitude on functional capacity in patients with Fontan palliation.
A retrospective review of 22 patients at low altitude (1602 metres) and 119 patients at sea level with Fontan circulation, as well as age-, gender-, and altitude-matched controls, underwent maximal cardiopulmonary exercise testing. Linear regression models were created to determine the influence of altitude on differences in exercise variables between Fontan patients and their matched controls.
Peak oxygen consumption was 28.4 millilitres per kilogram per minute (72% predicted) for the sea-level cohort and 24.2 millilitres per kilogram per minute (63% predicted) for the moderate altitude cohort. The matched case–control differences for patients at moderate altitude were greater for peak oxygen consumption (−29% against −13%, p = 0.04), anaerobic threshold (−36% against −5%, p = 0.001), and oxygen pulse (−35% against −18%, p = 0.007) when compared with patients living at sea level. When compared to institution-matched controls, the same parameters fell by 3%, 8.9%, and 4.2%, respectively, for each increase of 1000 feet in residential altitude (p = 0.03, p = 0.001, and p = 0.05, respectively).
Patients with Fontan circulation at a higher altitude have impairment in aerobic capacity when compared with patients at sea level. Reduction in exercise capacity is associated with a reduction in stroke volume, likely related to increased pulmonary vascular resistance.
Functional health is becoming an important part of outcome assessment following congenital heart surgery.
The Child Health Questionnaire was used to evaluate self-reported functional health in a cohort of children operated on for congenital heart disease between 1996 and 2002, now aged 10–20 years. A total of 288 schoolchildren served as controls. The association between demographic and clinical factors such as the Risk Adjusted Classification for Congenital Heart Surgery, the Aristotle Basic Complexity Score, physical and psycho-social domains was explored by multivariate analysis.
In total 239 children who were operated on (response rate 68%, mean age at assessment 13.1 years, 50% male children) participated. There were no differences between children operated on for congenital heart disease and controls in nine out of thirteen domains. In multivariate analysis, male gender was positively associated with physical, mental and general health. Higher education of the parents was also associated with better scores for family activities, physical, emotional and general health. In contrast, living with a single parent was negatively associated with mental health. Category 4 in the Risk Adjusted Classification for Congenital Heart Surgery was associated with worse scores in all behaviour domains. The Aristotle Basic Complexity Score was not associated with any domain.
Functional health in children operated for congenital heart disease was overall similar to other children of the same age. Male gender of the child, education of the parents, living with a single parent, and category 4 in the Risk Adjusted Classification for Congenital Heart Surgery were important factors for functional health.
Knowledge of normal aortic dimensions is important in the management of children with aortic disease. So as to define such dimensions, we undertook a retrospective review of clinical data and aortic cineangiograms from 167 subjects without aortic disease having a mean age of 3.67 years, with a range from 0.01 to 14.95 years. Amongst the patients, 56 were without detectable cardiac lesions, 66 patients had mild pulmonary stenosis, 30 were seen with Kawasaki disease, and 15 with small interatrial defects within the oval fossa. Aorto-grams were available in all. No patient had any hemodynamic derangement that would have affected the aorta during intrauterine life or childhood. Systolic dimensions were measured in the ascending and descending aorta at the level of the carina, at the transverse aortic arch distal to the brachiocephalic, of the left common carotid artery at its origin, at the transverse aortic arch distal to the left common carotid artery, at the aortic isthmus, and of the aorta at the level of the diaphragm. A regression analysis model was used to establish the range of predicted normal values, with their confidence limits, standardizing the values to height as the biophysical parameter having the highest correlation to aortic dimensions. Normal ranges were established for all the levels of measurement. The data should prove useful in identifying abnormalities of the thoracic aorta during childhood, and when assessing the outcomes of interventions.
Challenges inherent in researching rare congenital cardiac lesions led to creation of the Congenital Heart Surgeons’ Society Data Center (Data Center) two decades ago. The Data Center pools experiences from up to 60 institutions, and over 4,700 children have been prospectively recruited within nine diagnostic inception cohorts. This report describes the operations of our research database, with particular focus on analytic strategies employed.
Methods and results
A procedural log is created of all investigations and interventions, and reports from enrolling institutions are subsequently obtained. Cross-sectional follow-up is undertaken annually by the Data Center. All data are linked to the individual child, and quality control mechanisms ensure that completeness and accuracy are maximised. Specific advantages of Data Center analytic approaches include multi-phase parametric hazard analysis, re-sampling techniques for reliable risk factor identification, competing risks methodology, and propensity-adjusted comparisons. Virtues of applying these techniques to a research database are illustrated by clinically pertinent questions that have been addressed in place of what would be difficult through randomised trials.
The Data Center is a cost-effective, versatile tool for researching congenital cardiac surgical outcomes. Research databases are ideally suited to in-depth investigations of survival and functional outcomes. Multi-center propensity-adjusted analyses represent efficient surrogates for randomised trials. Well-designed observational prospective studies should remain a principle mode of researching congenital cardiac disease.
Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16).
While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.
To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies.
Childhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival.
We included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study.
We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure.
Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.