Counting on random number generation: Uncovering mild executive dysfunction in congenital heart disease.

Congenital heart disease (CHD) is associated with various neurocognitive deficits, particularly targeting executive functions (EFs), of which random number generation (RNG) is one indicator. RNG has, however, never been investigated in CHD. We administered the Mental Dice Task (MDT) to 67 young adults with CHD and 55 healthy controls. This 1-minute-task requires the generation of numbers 1 to 6 in a random sequence. RNG performance was correlated with a global EF score. Participants underwent MRI to examine structural-volumetric correlates of RNG. Compared to controls, CHD patients showed increased backward counting, reflecting deficient inhibition of automatized behavior. They also lacked a small-number bias (higher frequency of small relative to large numbers). RNG performance was associated with global EF scores in both groups. In CHD patients, MRI revealed an inverse association of counting bias with most of the volumetric measurements and the amount of small numbers was positively associated with corpus callosum volume, suggesting callosal involvement in the "pseudoneglect in number space". In conclusion, we found an impaired RNG performance in CHD patients, which is associated with brain volumetric measures. RNG, reportedly resistant to learning effects, may be an ideal task for the longitudinal assessment of EFs in patients with CHD.

Palliative Care in Children With Advanced Heart Disease in a Tertiary Care Environment: A Mini Review.

Palliative care for children continues to evolve. More recently, this has also been true in the field of pediatric cardiology, particularly for children with advanced heart disease. In these children, similarly to children with cancer, treatment successes are offset by the risks of long-term morbidities, including premature death. This mini review aims to provide an overview of current knowledge on children suffering from advanced heart disease, their medical care during various phases of illness (including the palliative and end-of-life phase), symptom burden, experiences of parents, prognostic understanding of parents and physicians, and current status of the involvement of pediatric palliative care. In conclusion, the suffering of these children at the end of their young lives is pronounced and many parents feel prepared neither for medical problems nor for the child's death. An effective and mutually trusting partnership between pediatric cardiology and pediatric palliative care would appear to be a prerequisite for the timely involvement of palliative care in further supporting these children and their families.

Neurocognitive functioning in young adults with congenital heart disease: insights from a case-control study.

BACKGROUND: While there is evidence that cognitive impairment of children with congenital heart disease (CHD) may persist into adolescence, little is known about the spectrum of neurocognitive functioning of young adults with this disorder. The aim of this study was to assess neurocognitive functioning in a population of young adults with different types of CHD. METHODS: Cross-sectional cohort study in young adults with CHD and a group-matched healthy control group. We assessed neurocognitive and general intellectual functioning with a comprehensive battery of standardised neuropsychological tests. In addition to task-based assessments, questionnaire data of executive dysfunctions in everyday life were measured with the Behaviour Rating Inventory of Executive Function - Adult Version. RESULTS: A total of 67 patients (55% men) with CHD and 55 healthy controls (51% men) were included for analysis. Mean age at assessment was 26.9 (3.68) and 26.0 (3.32) years, respectively. The CHD group performed poorer in the domains of Executive Functions, Memory, Attention & Speed, and general intellectual functioning. Patients with a CHD of severe complexity were more affected than patients with simple or moderate complexity. Behaviour Rating Inventory of Executive Function - Adult Version scores indicated that patients' self-rated deficits in behaviour regulation in everyday life was higher compared with healthy controls. CONCLUSION: Our findings indicate lower neurocognitive functioning in young adults with a CHD, particularly in those with severe defect complexity. In view of the potentially enhanced risk for cerebrovascular and neurodegenerative disease in this patient group as reported in the literature, systematic longitudinal monitoring of cognitive functioning is recommended.

Altered white matter microstructure is related to cognition in adults with congenital heart disease.

Adults with congenital heart disease are at risk for persisting executive function deficits, which are known to affect academic achievement and quality of life. Alterations in white -matter microstructure are associated with cognitive impairments in adolescents with congenital heart disease. This study aimed to identify microstructural alterations potentially associated with executive function deficits in adults with congenital heart disease. Diffusion tensor imaging and tract-based spatial statistics were conducted in 45 patients (18 females) and 54 healthy controls (26 females) aged 18-32 years. Fractional anisotropy of white matter diffusion was compared between groups and correlated with an executive function score, derived from an extensive neuropsychological test battery. Patients showed widespread bilateral reduction in fractional anisotropy (P < 0.05, multiple comparison corrected) compared to controls. Lower fractional anisotropy was driven by patients with moderate and severe defect complexity (compared to controls: P < 0.001). Executive function scores were lower in patients (P < 0.05) and associated with lower fractional anisotropy in the left superior corona radiata and the corticospinal tract (corrected P < 0.05). Our findings confirm alterations of white matter microstructure in adults with congenital heart disease, mainly in those patients of moderate to severe complexity. These alterations are associated with impairments in executive functioning. A better understanding of the neurocognitive deficits may help counselling and care of patients with congenital heart disease across their lifespan and have the potential to improve their outcome and quality of life.

Brain volumes in adults with congenital heart disease correlate with executive function abilities.

Congenital heart disease is the most common birth defect, and patients are at risk for neurodevelopmental impairment and brain abnormalities. Yet, little is known about the link between brain volumes and cognitive function in adults with congenital heart disease. Forty-four patients and 53 controls between 18 and 32 years underwent brain magnetic resonance imaging and cognitive testing, assessed with an intelligence quotient and executive function global score. Associations between brain volumes and cognitive function were calculated using linear models. Cognitive function in patients was within the normal range (intelligence quotient: 97.74 (10.76)). Total brain volume was significantly smaller in patients compared to controls (1067.26 (113.53) vs 1113.04 (97.88) cm3, P < 0.01), irrespective of cardiac factors (heart defect complexity, cyanosis, cardiopulmonary bypass: all P > 0.4). After adjusting for total brain volume, only corpus callosum volume remained significantly smaller (P = 0.03). Smaller total brain volume was associated with poorer overall executive functioning (P = 0.02) and inhibition (P < 0.01), in both patients and controls. The association between total brain volume and overall executive functioning was moderated by parental socioeconomic status (lower socioeconomic status was associated with a stronger association between brain volume and EF; interaction P = 0.03). In adults with congenital heart disease, despite normal intelligence quotient, brain volume alterations persist into adulthood and are related to executive functioning, in particular inhibitory control. Adults coming from low socioeconomic background and with altered brain volumes are especially vulnerable and should thus be followed-up during adulthood to ensure optimal social and educational support.

Right ventricular dilatation in patients with pulmonary regurgitation after repair of tetralogy of Fallot: How fast does it progress?

OBJECTIVE: Pulmonary valve regurgitation (PR) and right ventricular (RV) dilatation are important residual findings after surgical repair of tetralogy of Fallot (TOF). We sought to describe the natural course of RV dilatation over time in patients with severe PR after TOF repair and to determine risk factors for quick progression of RV dilatation and dysfunction. METHODS: Data of 85 consecutive TOF patients with PR and RV dilatation, undergoing serial cardiovascular magnetic resonance (CMR) scans between July 2002 and December 2016 in two institutions, were retrospectively reviewed. The dataset was analyzed regarding right and left ventricular (LV) volume and function and potential risk factors of progressive RV dilatation. RESULTS: There was no significant increase in RV end-diastolic volumes (RVEDVi) indexed body surface area (BSA) (median 150 [81-249] vs. 150 [82-260] mL/m2) and end-systolic volumes indexed for BSA (RVESVi) (75 [20-186] vs. 76 [39-189] mL/m2) between the first and last CMR in the overall group. Similarly, there were no significant changes in LV volumes indexed for BSA (LVEDVi 78 [56-137] vs. 81 [57-128] mL/m2 and LV end-systolic volume index 34 [23-68] vs. 35 [18-61] mL/m2). Global function remained also unchanged for both ventricles. RVEDVi increased statistically significantly (≥20 mL/m2) in twenty patients (24%) from 154 mL/m2 (87-237) to 184 mL/m2 (128-260, P < 0.001). LV dimensions showed a similar trend with LVEDVi increase from 80 ml/m2 (57-98) to 85 ml/m2 (72-105, P = 0.002). Shorter time interval between repair and first CMR was the only risk factor predictive for progressive RV dilatation. CONCLUSION: In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years. RV dilatation seems to progress early after surgery and subsequently stabilize. RV dilatation significantly progresses in a subgroup of 24% of patients, with a shorter time interval since surgical repair.

Structural brain abnormalities in adults with congenital heart disease: Prevalence and association with estimated intelligence quotient.

BACKGROUND: Little is known about the prevalence of structural brain abnormalities and cognitive functioning in the growing population of patients with adult congenital heart disease (ACHD). Thus, our aim was to assess structural abnormalities on brain magnetic resonance imaging (MRI) and their association with intelligence quotient (IQ) in ACHD patients. METHODS: Cross-sectional study in ACHD patients and healthy controls as comparison group. Brain MRI was performed on a 3 T MR scanner, and inspection of structural abnormalities was performed blinded to ACHD or control status. IQ was estimated using the vocabulary and matrix reasoning subtests from the Wechsler Adult Intelligence Scale, Fourth Edition. RESULTS: A total number of 67 (55% males) ACHD patients and 55 (51% males) controls were included (mean age 26.9 and 26.0 years respectively). Abnormalities on brain MRI were detected in 29 of 46 (63%) ACHD patients and in none of the controls. Abnormalities consisted of focal infarction or atrophy, white matter lesions, microhemorrhages, and global atrophy. Mean estimated IQ was significantly lower in ACHD patients than in controls (98.51 versus 104.38; 95% CI: -10.09 to -1.66; P value = 0.007). Comparison between patients with and without cerebral abnormalities revealed no significant difference in estimated IQ. CONCLUSION: Our findings indicate a high prevalence and wide spectrum of structural brain abnormalities in ACHD patients. Furthermore, this population is at a higher risk of impaired intellectual functioning than healthy controls. However, the present study could not establish a statistically significant association between MRI findings and estimated IQ. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov ID: NCT04041557; URL: https://clinicaltrials.gov/ct2/show/NCT04041557?term=NCT04041557&rank=1.

Right and Left Ventricular Strain Patterns After the Atrial Switch Operation for D-Transposition of the Great Arteries-A Magnetic Resonance Feature Tracking Study.

Introduction: Adult survivors of the atrial switch operation for transposition of the great arteries present with a systemic morphologic right ventricle and a subpulmonary morphologic left ventricle. This physiology can be considered a model for the effects of long-term right ventricular pressure overload and of decreased left ventricular afterload. We aimed to determine the impact of these chronically altered loading conditions on myocardial deformation of the ventricles. Materials and methods: Two-dimensional steady state free precession cine images of 29 patients after atrial repair (age 29 ± 7 years) and 19 controls (24 ± 10 years; n.s.) were post-processed with feature tracking software (TomTec 2D CPA). Volumes, ejection fractions, global and free wall longitudinal and circumferential strains of both ventricles were compared between both groups. Results: Systemic right ventricular global longitudinal strain was decreased in patients compared to controls (-12.9 ± 3.3% vs. -18.9 ± 4.6%, p < 0.001), while right ventricular circumferential strain was unchanged (-15.8 ± 3.4% vs. -15.1 ± 5%; n.s.). Left ventricular longitudinal strain was similar in both groups (-17 ± 5.6% vs. -17.5 ± 4.6%; n.s.), but global left ventricular circumferential strain was lower in patients (-20.7 ± 4.1% vs. -27.3 ± 4.5%, p < 0.001). The systemic right ventricle, compared to the systemic left ventricle, showed decreased global longitudinal (p < 0.001) and circumferential strain (p < 0.001). The subpulmonary left ventricle, compared to the subpulmonary right ventricle, demonstrated similar longitudinal (p = 0.223) but higher circumferential strain (p < 0.001). Conclusions: In patients after atrial switch repair for transposition of the great arteries, the systemic right ventricle shows poor longitudinal strain, but maintains normal right ventricular circumferential strain. The left ventricle shows higher circumferential strain than the right ventricle, in both systemic and subpulmonary positions.

Predictors of quality of life in young adults with congenital heart disease.

AIMS: The aim of this study was to identify medical and psychosocial risk factors for impaired health-related quality of life (HRQoL) and poor psychological adjustment (PA) in young adults with congenital heart disease (CHD). METHODS AND RESULTS: A group of 188 patients (43% females, ages 18-30 years) with various types of CHD and 139 age-matched healthy controls (57% females) completed questionnaires assessing HRQoL, PA, social support, significant life events in the past year, education level, civil status, and employment status. Medical variables were retrieved from the patients' hospital records. Patients reported worse physical HRQoL than controls but similar mental HRQoL and PA. Female CHD patients showed worse physical and mental HRQoL and poorer PA than males. In CHD patients, a lower educational level and lower physical exercise capacity predicted lower physical HRQoL, but complexity of CHD was not related to HRQoL or PA. Less social support was associated with lower mental HRQoL and poorer PA in CHD patients. CONCLUSION: Young adults with CHD have impaired physical HRQoL but normal mental HRQoL and PA. Lower physical exercise capacity, female sex, less social support, and lower educational level predict an unfavourable quality of life and PA. This subgroup of patients should be monitored more closely and provided with special psychosocial care to improve long-term outcome.

Swiss Adult Congenital HEart disease Registry (SACHER) - rationale, design and first results.

BACKGROUND: In 2013, a prospective registry for adults with congenital heart disease (CHD) was established in Switzerland, providing detailed data on disease characteristics and outcomes: Swiss Adult Congenital HEart disease Registry (SACHER). Its aim is to improve the knowledge base of outcomes in adults with CHD. The registry design and baseline patient characteristics are reported. METHODS: All patients with structural congenital heart defects or hereditary aortopathies, followed-up at dedicated adult CHD clinics, are asked to participate in SACHER. Data of participants are pseudonymised and collected in an electronic, web-based, database (secuTrial®). Collected data include detailed diagnosis, type of repair procedures, previous complications and adverse outcomes during follow-up. RESULTS: From May 2014 to December 2016, 2836 patients (54% male, mean age 34 ± 14 years), with a wide variety of congenital heart lesions, have been enrolled into SACHER. Most prevalent were valve lesions (25%), followed by shunt lesions (22%), cyanotic and other complex congenital heart disease (16%), diseases affecting the right heart, i.e., tetralogy of Fallot or Ebstein anomaly (15%), and diseases of the left ventricular outflow tract (13%); 337 patients (12%) had concomitant congenital syndromes. The majority had undergone previous repair procedures (71%), 47% of those had one or more reinterventions. CONCLUSION: SACHER collects multicentre data on adults with CHD. Its structure enables prospective data analysis to assess detailed, lesion-specific outcomes with the aim to finally improve long-term outcomes.

Impact of growing cohorts of adults with con-genital heart disease on clinical workload: a 20-year experience at a tertiary care centre.

BACKGROUND: Population based studies show a steady increase in adult patients with congenital heart defects. The aim of this study was to assess the evolution of such a patient cohort and its burden on clinical care at a dedicated tertiary care centre. METHODS: All patients with congenital heart disease followed up by a dedicated multidisciplinary team at our institution were identified (n = 1725). Disease characteristics, the increase in patient numbers and interventions and the increase in selected complications were analysed and compared between the first (1996-2005) and second (2006-2015) decades of the study period. RESULTS: Between the two decades of the study period, the number of patients in follow-up increased by 109%, the number of patients who died or underwent transplantation more than doubled and the number of outpatient visits increased by 195%. One fourth of all patients underwent at least one surgical procedure and 14% had at least one percutaneous intervention. The increase in surgical procedures between the two decades was 27% and the increase in percutaneous interventions 159%. Between the two decades the number of patients requiring direct current cardioversion increased from 32 to 95 (+197%), the number of patients requiring admission for infective endocarditis increased from 7 to 29 (+314%) and the number of women followed up during pregnancy increased from 18 to 115 (+539%). CONCLUSION: As a result of the increasing number and complexity of adult survivors with congenital heart disease more resources will be needed to cope with the demands of this novel cohort of complex patients in adult cardiology.

Burden and impact of congenital syndromes and comorbidities among adults with congenital heart disease.

BACKGROUND: Our aim was to assess the overall burden of congenital syndromes and non-cardiac comorbidities among adults with congenital heart disease and to assess their impact on circumstances of living and outcomes. METHODS: Within a cohort of 1725 adults with congenital heart defects (65% defects of moderate or great complexity) followed at a single tertiary care center, congenital syndromes and comorbidities were identified by chart review. Their association with arrhythmias, circumstances of living and survival was analyzed. RESULTS: Within the study cohort, 232 patients (13%) had a genetic syndrome, 51% at least one comorbidity and 23% ≥2 comorbidities. Most prevalent comorbidities were systemic arterial hypertension (11%), thyroid dysfunction (9%), psychiatric disorders (9%), neurologic disorders (7%), chronic lung disease (7%), and previous stroke (6%). In contrast to higher congenital heart defect complexity, the presence of comorbidities had no impact on living circumstances but patients with comorbidities were less likely to work full-time. Atrial arrhythmias were more common among patients with moderate/great disease complexity and those with comorbidities but were less common among patients with congenital syndromes (p<0.01 for all comparisons). Patients with ≥2 comorbidities had lower survival estimates compared to those with ≤1 comorbidity (p=0.013). CONCLUSION: Congenital syndromes and comorbidities are highly prevalent in adults with congenital heart disease followed at specialist centers and add to the overall complexity of care. The presence of these additional factors has an impact on living circumstances, is associated with arrhythmias and needs to be further explored as prognostic markers.

High prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries.

AIMS: To determine the prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries, as these may predispose to paradoxical embolic events, particularly in patients with transvenous pacemaker or defibrillator leads. METHODS AND RESULTS: We routinely perform contrast echocardiography with agitated saline in all patients after atrial switch operations. For this study, we analysed patients who had saline contrast echocardiography between 2010 and 2012. The presence of baffle leaks and the severity of right-to-left shunting were assessed. We compared baseline characteristics and oxygen saturation at rest and during exercise between patients with and without baffle leaks. A total of 65 patients (56 Senning and 9 Mustard repair) without previously known baffle leaks were included (mean age 32 ± 8 years, 77% males). Right-to-left shunting was identified in 42 patients (65%) and occurred without provocation manoeuvres in 88%. There were no differences in baseline characteristics, echocardiographic findings, or exercise capacity between patients with and without baffle leaks, except for lower oxygen saturation at peak exercise in those with baffle leaks (29% had oxygen saturations below 90% at peak exercise compared to none without baffle leaks, P = 0.011). Four patients with baffle leaks had previous implantation of transvenous pacemaker leads; one of them had suffered a stroke. Two other patients with baffle leaks had a history of potential embolic stroke. CONCLUSIONS: Because of the high prevalence of baffle leaks in adults after atrial switch operations, we propose routine screening with agitated saline contrast, particularly prior to implantation of transvenous pacemaker or defibrillator leads.

Atrial Function after the Atrial Switch Operation for Transposition of the Great Arteries: Comparison with Arterial Switch and Normals by Cardiovascular Magnetic Resonance.

OBJECTIVES: The atria serve as reservoir, conduit, and active pump for ventricular filling. The performance of the atrial baffles after atrial switch repair for transposition of the great arteries may be abnormal and impact the function of the systemic right ventricle. We sought to assess atrial function in patients after atrial repair in comparison to patients after arterial switch repair (ASO) and to controls. METHODS: Using magnetic resonance imaging, atrial volumes and functional parameters were measured in 17 patients after atrial switch repair, 9 patients after ASO and 10 healthy subjects. RESULTS: After the atrial switch operation, the maximum volume of the pulmonary venous atrium was significantly enlarged, but not of the systemic venous atrium. In both patients groups, independently from the surgical technique used, the minimum atrial volumes were elevated, which resulted in a decreased total empting fraction compared with controls (P < .01). The passive empting volume was diminished for right atrium, but elevated for left atrium after atrial switch and normal for left atrium after ASO; however, the passive empting fraction was diminished for both right atrium and left atrium after both operations (P < .01). The active empting volume was the most affected parameter in both atria and both groups and active empting fractions were highly significantly reduced compared with controls. CONCLUSION: Atrial function is abnormal in all patients, after atrial switch and ASO repair. The cyclic volume changes, that is, atrial filling and empting, are reduced when compared with normal subjects. Thus, the atria have lost part of their capacity to convert continuous venous flow into a pulsatile ventricular filling. The function of the pulmonary venous atrium, acting as preload for the systemic right ventricle, after atrial switch is altered the most.

Medical treatment of aortic aneurysms in Marfan syndrome and other heritable conditions.

Thoracic aortic aneurysms can be triggered by genetic disorders such as Marfan syndrome (MFS) and related aortic diseases as well as by inflammatory disorders such as giant cell arteritis or atherosclerosis. In all these conditions, cardiovascular risk factors, such as systemic arterial hypertension, may contribute to faster rate of aneurysm progression. Optimal medical management to prevent progressive aortic dilatation and aortic dissection is unknown. ß-blockers have been the mainstay of medical treatment for many years despite limited evidence of beneficial effects. Recently, losartan, an angiotensin II type I receptor antagonist (ARB), has shown promising results in a mouse model of MFS and subsequently in humans with MFS and hence is increasingly used. Several ongoing trials comparing losartan to ß-blockers and/or placebo will better define the role of ARBs in the near future. In addition, other medications, such as statins and tetracyclines have demonstrated potential benefit in experimental aortic aneurysm studies. Given the advances in our understanding of molecular mechanisms triggering aortic dilatation and dissection, individualized management tailored to the underlying genetic defect may be on the horizon of individualized medicine. We anticipate that ongoing research will address the question whether such genotype/pathogenesis-driven treatments can replace current phenotype/syndrome-driven strategies and whether other forms of aortopathies should be treated similarly. In this work, we review currently used and promising medical treatment options for patients with heritable aortic aneurysmal disorders.

Surgical outcome of discrete subaortic stenosis in adults: a multicenter study.

BACKGROUND: Discrete subaortic stenosis is notable for its unpredictable hemodynamic progression in childhood and high reoperation rate; however, data about adulthood are scarce. METHODS AND RESULTS: Adult patients who previously underwent surgery for discrete subaortic stenosis were included in this retrospective multicenter cohort study. Mixed-effects and joint models were used to assess the postoperative progression of discrete subaortic stenosis and aortic regurgitation, as well as reoperation. A total of 313 patients at 4 centers were included (age at baseline, 20.2 years [25th-75th percentile, 18.4-31.0 years]; 52% male). Median follow-up duration was 12.9 years (25th-75th percentile, 6.2-20.1 years), yielding 5617 patient-years. The peak instantaneous left ventricular outflow tract gradient decreased from 75.7±28.0 mm Hg preoperatively to 15.1±14.1 mm Hg postoperatively (P<0.001) and thereafter increased over time at a rate of 1.31±0.16 mm Hg/y (P=0.001). Mild aortic regurgitation was present in 68% but generally did not progress over time (P=0.76). A preoperative left ventricular outflow tract gradient ≥80 mm Hg was a predictor for progression to moderate aortic regurgitation postoperatively. Eighty patients required at least 1 reoperation (1.8% per patient-year). Predictors for reoperation included female sex (hazard ratio, 1.53; 95% confidence interval, 1.02-2.30) and left ventricular outflow tract gradient progression (hazard ratio, 1.45; 95% confidence interval, 1.31-1.62). Additional myectomy did not reduce the risk for reoperation (P=0.92) but significantly increased the risk of a complete heart block requiring pacemaker implantation (8.1% versus 1.7%; P=0.005). CONCLUSIONS: Survival is excellent after surgery for discrete subaortic stenosis; however, reoperation for recurrent discrete subaortic stenosis is not uncommon. Over time, the left ventricular outflow tract gradient slowly increases and mild aortic regurgitation is common, although generally nonprogressive over time. Myectomy does not show additional advantages, and because it is associated with an increased risk of complete heart block, it should not be performed routinely.

Management and outcome of Ebstein's anomaly in children.

OBJECTIVES: To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. BACKGROUND: Data on long-term outcome of children with Ebstein's anomaly are scarce. METHODS: Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. RESULTS: A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%. CONCLUSION: In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Congenital valvular aortic stenosis in young adults: predictors for rate of progression of stenosis and aortic dilatation.

BACKGROUND: Congenital aortic stenosis (AS) is the most common obstructive left-sided cardiac lesion in young adults, however little is known about the progression in adults. Therefore, we aimed to evaluate the progression rate of AS and aortic dilatation in a large multicenter retrospective cohort of asymptomatic young adults with congenital valvular AS. METHODS: Data were obtained from chart abstraction. Linear mixed-effects models were used to evaluate the progression of AS and aortic dilatation over time. A joint model combining longitudinal echocardiographic and survival data was used for survival analysis. RESULTS: A total of 414 patients (age 29 ± 10 years, 68% male) were included. Median follow-up duration was 4.1 (2.5-5.1) years (1587 patient-years). Peak aortic velocity was 3.4 ± 0.7 m/s at baseline and did not change over time in the total patient population (-0.01 ± 0.03 m/s/year). Increased left ventricular mass was significantly associated with faster AS progression (p<0.001). Aortic dilatation was present in 34% at baseline and 48% at follow-up (p<0.001). The aortic diameter linearly increased over time with a rate of 0.7 ± 0.2mm/year. Rate of aortic dissection was 0.06% per patient-year. Seventy patients required an aortic valve intervention (4.4% per patient-year), with AS progression rate as most powerful predictor (HR 5.11 (95% CI 3.47-7.53)). CONCLUSIONS: In the majority of patients with mild-to-moderate congenital AS, AS severity does not progress over time. However patients with left ventricular hypertrophy are at risk for faster progression and should be monitored carefully. Although aortic dissections rarely occur, aortic dilatation is common and steadily progresses over time, warranting serial aortic imaging.

Outcome of biventricular repair in infants with multiple left heart obstructive lesions.

The decision to perform biventricular repair for infants with multiple obstructive or hypoplastic left heart lesions (LHL) and borderline left ventricle (LV) may be controversial. This study sought to assess the mortality and morbidity of patients with LHL after biventricular repair and to determine the growth of the left-sided cardiac structures. Retrospective analysis of 39 consecutive infants with LHL who underwent biventricular repair was performed. The median age at surgery was 7 days (range 1-225 days), and the median follow-up period was 34 months (range 1-177 months). Between diagnosis and the end of the follow-up period, the size of the aortic annulus (z-score -4.1 ± 2.8 vs. -0.1 ± 2.7) and the LV (LV end-diastolic diameter z-score -1.7 ± 2.8 vs. 0.21 ± 1.7) normalized. During the follow-up period, 23 patients required 39 reinterventions (62%) consisting of redo surgery for 21 patients (57%) and catheter-guided reinterventions for 8 patients (22%). At the end of the follow-up period, 25 of 34 patients were doing subjectively well; 10 children (29%) received cardiac medication; 12 (35%) presented with failure to thrive (weight ≤ P3) and 5 (15%) with pulmonary hypertension. The overall mortality rate was 13%. Biventricular repair for patients with multiple LHL results in sufficient growth of the left-sided cardiac structures. Nevertheless, residual or newly developing obstructive lesions and pulmonary hypertension are frequent, causing significant morbidity that requires reintervention.

Do predictors exist for a successful withdrawal of preoperative prostaglandin E(1) from neonates with d-transposition of the great arteries and intact ventricular septum?

Prostaglandin E(1) (PGE(1)) is given to neonates with d-transposition of the great arteries (d-TGA) to reduce cyanosis by reopening and maintaining the patency of the ductus arteriosus. To avoid side effects, this medication can be stopped for hemodynamically stable patients after balloon atrial septostomy (BAS). A consecutive series of neonates with d-TGA and an intact ventricular septum (IVS) presenting from 2000 through 2005 was analyzed retrospectively to search for side effects of PGE(1) and to identify predictors for a safe preoperative withdrawal. The medication was stopped for hemodynamically stable patients with transcutaneous oxygen saturations higher than 80% after BAS and reinitiated for patients with an oxygen saturation lower than 65%. Patients successfully weaned were compared with those who had failed weaning in terms of atrial septal defect (ASD) size, ductus arteriosus size, and the transcutaneous oxygen saturation. Prostaglandin E(1) was initiated for all 43 neonates with d-TGA. The median maintenance dose of PGE(1) was 0.00625 µg/kg/min (range, 0.00313-0.050 µg/kg/min) for a median duration of 6 days (range, 1-12 days). For 16 patients, PGE(1) was preoperatively withdrawn but then had to be reinitiated for 7 of the 16 patients. No predictors for a successful weaning of PGE(1) were found based on ASD size, ductus arteriosus size, or oxygen saturation. The adverse effects of PGE(1) were apnea in 10 patients and fever in 19 patients. Neither seizures nor necrotizing enterocolitis was documented. Prostaglandin E(1) was successfully withdrawn for a minority of hemodynamically stable patients with d-TGA. No predictors for a successful weaning could be identified. Because apnea and fever are common side effects, withdrawal of PGE(1) after BAS may improve patient safety and comfort. In this patient group, if PGE(1) withdrawal was not well tolerated, it could be safely reinitiated. There were no serious side effects of PGE(1.).