Heart failure is not a determinant of central sleep apnea in the pediatric population.

BACKGROUND/OBJECTIVES: Adults with heart failure (HF) have high prevalence of central sleep apnea (CSA). While this has been repeatedly investigated in adults, there is a deficiency of similar research in pediatric populations. The goal of this study was to compare prevalence of CSA in children with and without HF and correlate central apneic events with heart function. METHODS: Retrospective analysis of data from children with and without HF was conducted. Eligible children were less than 18 years old with echocardiogram and polysomnogram within 6 months of each other. Children were separated into groups with and without HF based on left ventricular ejection fraction (LVEF). Defining CSA as central apnea-hypopnea index (CAHI) more than 1/hour, the cohort was also classified into children with and without CSA for comparative study. RESULTS: A total of 120 children (+HF: 19, -HF: 101) were included. The +HF group was younger, with higher prevalence of trisomy 21, muscular dystrophy, oromotor incoordination, and structural heart disease. The +HF group had lower apnea-hypopnea index (median: 3/hour vs. 8.6/hour) and lower central apnea index (CAI) (median: 0.2/hour vs. 0.55/hour). Prevalence of CSA was similar in both groups (p = .195). LogCAHI was inversely correlated to age (Pearson correlation coefficient: -0.245, p = .022). Children with CSA were younger and had higher prevalence of prematurity (40% vs. 5.3%). There was no significant difference in LVEF between children with and without CSA. After excluding children with prematurity, relationship between CAHI and age was no longer sustained. CONCLUSIONS: In contrast to adults, there is no difference in prevalence of CSA in children with and without HF. Unlike in adults, LVEF does not correlate with CAI in children. Overall, it appears that central apneic events may be more a function of age and prematurity rather than of heart function.

Analysis of factors associated with prolonged post-operative course after surgical repair of aortic coarctation.

OBJECTIVE: We sought to describe patient characteristics associated with prolonged post-operative length of stay in a contemporary cohort of infants who underwent isolated repair of aortic coarctation. METHODS: We reviewed patients less than 1 year of age who underwent isolated repair of aortic coarctation at our institution from 2009 to 2016. Prolonged post-operative length of stay was defined as length of stay within the upper tertile for the cohort. Bivariate and multi-variable analyses were performed to determine independent risk factors for prolonged length of stay. RESULTS: We reviewed 95 consecutive patients who underwent isolated repair of aortic coarctation, of whom 71 were neonates at the time of diagnosis. The median post-operative length of stay was 6.5 days. The upper tertile for post-operative length of stay was greater than 10 days; 32 patients within this tertile and 1 patient who died at 8.5 days after surgery were analysed as having prolonged post-operative length of stay. In a multi-variable analysis, pre-maturity (odds ratio: 3.5, 95% confidence interval: 1.2, 10.7), genetic anomalies (odds ratio: 4.7, 95% confidence interval: 1.2, 18), absence of pre-operative oral feeding (odds ratio: 7.4, 95% confidence interval: 2.4, 22.3), and 12-hour vasoactive-ventilation-renal score greater than 25 (odds ratio: 7.4, 95% confidence interval: 1.9, 29) were independently associated with prolonged length of stay. CONCLUSIONS: In neonates and infants who underwent isolated repair of aortic coarctation, pre-maturity, genetic anomalies, lack of pre-operative oral feedings, and 12-hour vasoactive-ventilation-renal score more than 25 were independent risk factors for prolonged post-operative length of stay. Further study on the relationship between pre-operative oral feedings and post-operative length of stay should be pursued.

Does reduced cardiopulmonary exercise testing performance predict poorer quality of life in adult patients with Fontan physiology?

BACKGROUND: Cardiopulmonary exercise testing performance has been shown to be a predictor of morbidity, mortality, and quality of life in patients with Fontan physiology; however, the role of exercise performance along with other diagnostics is not fully understood. We evaluated the hypothesis that reduced exercise performance correlates with poorer quality of life in Fontan patients as they continue to age. METHODS: Chart review was performed on patients 12 years and older with Fontan who had completed cardiopulmonary exercise testing and age-appropriate quality of life surveys. Quality of life outcomes were analysed against exercise performance and other descriptive data. RESULTS: For the younger cohort (n = 22), exercise performance predicted quality of life with different measures across domains and had a stronger correlation than echocardiographic parameters. For the older cohort (n = 34), exercise performance did not predict quality of life. CONCLUSIONS: Objective exercise performance was a useful marker for general, physical, emotional, social, and school quality of life in a younger cohort but less helpful in older adults. This is perhaps due to older patients accommodating to their conditions over time. The role of exercise performance and objective data in predicting quality of life in patients with Fontan physiology is incompletely understood and additional prospective evaluation should be undertaken.

Neonatal lupus with left bundle branch block and cardiomyopathy: a case report.

BACKGROUND: Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently reported manifestation with unknown pathophysiology and poorly defined treatment regimens. Due to the rarity of this condition, additional studies and case reports are required to better understand and manage late-onset neonatal lupus cardiomyopathies. CASE PRESENTATION: A 4-week-old female, born to a mother with known anti-SSA/Ro and anti-SSB/La autoantibodies, presents with classic cutaneous manifestations for neonatal lupus and is found to have left bundle branch block, severely dilated cardiomyopathy with an ejection fraction of 25%, and a thin echogenic dyskinetic ventricular septum. Weekly second trimester and 30-week fetal echocardiograms showed no signs of structural or conduction abnormalities. There were no histologic signs of inflammation on cardiac tissue biopsy. After a complicated hospital course, she was successfully treated with biventricular pacemaker, intravenous immunoglobulin, and plasmapheresis. CONCLUSIONS: We present a case of late-onset neonatal lupus with severe dilated cardiomyopathy, a dyskinetic ventricular septum, and left bundle branch block. To our knowledge, the dyskinetic ventricular septum has never been reported and left bundle branch block is rarely reported in NL. This case further validates the need for long term cardiac follow up for patients born with NL, even if lacking cardiac manifestations in the peripartum period. We characterize a unique presentation of a rare clinical entity, highlighting the diagnostic challenges, and describe a successful treatment course.

Risk stratification of patients with hypoplastic left heart syndrome and intact atrial septum using fetal MRI and echocardiography.

Despite prenatal diagnosis, prenatal intervention, and immediate postnatal intervention, patients with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have the highest risk for mortality. Charts for all infants diagnosed with hypoplastic left heart syndrome from 2009 to 2017 were retrospectively reviewed and compared, including pulmonary vein Doppler patterns on fetal echocardiogram and evidence of pulmonary lymphangiectasia on fetal MRI. Of the 81 newborns with hypoplastic left heart syndrome, we defined two groups. Group 1 patients had an adequate atrial septal communication (n = 69), while Group 2 met criteria for intact/restrictive septum (n = 12). No patient in Group 1 had a type C pulmonary vein Doppler pattern, while no patient in Group 2 had a type A pulmonary vein Doppler pattern. The two patients with pulmonary lymphangiectasia had type C pulmonary vein Doppler pattern and an intact atrial septum and did not survive. Survival to discharge for Group 1 was 83% compared to 58% for Group 2 (p = 0.116). Survival to stage 2 palliation was 71% for Group 1 compared to 50% for Group 2 (p = 0.186). Only 4 of the initial 12 patients from Group 2 are alive, which is an overall survival of 33%. Our experience supports previous evidence that fetal echocardiography can identify those patients with the greatest likelihood for postnatal intervention as well as those at highest risk for mortality. Fetal MRI is a novel imaging modality that may help providers separate patients at highest risk for mortality, regardless of pulmonary vein Doppler pattern.

Intermediate Outcomes of Staged Tetralogy of Fallot Repair.

BACKGROUND: The optimal surgical strategy for tetralogy of Fallot (TOF) repair in neonates and young infants requiring early surgical intervention remains controversial. Our surgical center follows the uniform strategy of a staged approach with initial systemic-to-pulmonary artery shunt the majority of time when early surgical intervention is required. We characterized a contemporary cohort of patients with TOF with pulmonary stenosis (PS) undergoing staged repair in order to determine the rate of pulmonary valve-sparing repair (PVSR), growth of the pulmonary valve annulus and pulmonary arteries, postoperative morbidity and mortality, and need for reintervention. METHODS: We retrospectively studied patients with TOF/PS who underwent staged repair from 2000 to 2017. Surgical details, postoperative course, and reinterventions were noted. Echocardiographic measurements and Z-score values of pulmonary valve annulus, main pulmonary artery (MPA), right pulmonary artery (RPA), and left pulmonary artery (LPA) diameters were evaluated. RESULTS: Of the 59 patients with staged TOF/PS, PVSR was performed in 25 (42%). There was a 5% incidence of postoperative arrhythmia. The Z-scores of MPA, RPA, and LPA were significantly higher before complete repair when compared to before palliative shunt. The 5 and 10-year survival rates were both 93%, and the probability of freedom from reoperation at 5 and 10 years was 87% and 82%, respectively. CONCLUSIONS: Staged repair of TOF in young symptomatic infants results in 42% receiving PVSR, pulmonary artery growth, low incidence of postoperative arrhythmia, and relatively low rate of reoperations.

The long-term functional outcome in Mustard patients study: Another decade of follow-up.

OBJECTIVE: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d-transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional capacity and quality of life. PARTICIPANTS: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty-five of the remaining patients agreed to participate in this current study. DESIGN: Patients underwent comparable testing to the previous studies when possible including exercise stress testing, echocardiography, MRI or CT evaluation of cardiac anatomy and function, Holter monitor, and quality of life questionnaire. RESULTS: Thirty-one percent of patients have experienced cardiac death either in the form of mortality or cardiac transplantation. The major cause of death was systemic right ventricular failure. Sixty-five percent have continuing abnormalities of rhythm. Exercise time and workload showed a statistically significant decrease from the original study (Time 1) to both 10-year (Time 2) and 20-year (Time 3) follow-up points. Right ventricular ejection fraction decreased significantly from the Time 1 to Time 2, and again to this current follow-up. Quality of life measures of energy level decreased significantly from the original study to both the Time 2 and Time 3. CONCLUSION: Cardiac mortality for Mustard patients remains high, and over time, systemic right ventricular ejection fraction, rhythm, exercise tolerance, and quality of life assessments show deterioration. There does not appear to be a single clear predictor of poor outcome.

Atretic anomalous left subclavian artery as part of an unusual vascular ring.

In this report, a unique case of a symptomatic vascular ring formed by right aortic arch, aberrant left subclavian artery, and left ligamentum arteriosus in which there is atresia of the proximal left subclavian artery is described. Imaging modalities were non-diagnostic and the patient was sent to surgery based on strong clinical suspicion. Her anatomy was delineated in the operating room and the ring was successfully repaired.

The Effects of Pulmonary Valve Replacement for Severe Pulmonary Regurgitation on Exercise Capacity and Cardiac Function.

Patients may develop hemodynamic abnormalities after right ventricular outflow tract (RVOT) repair. Re-intervention timing remains a dilemma. This study evaluates exercise capacity and RV function before and after intervention using age-related comparisons. Twenty-six patients with severe pulmonary regurgitation (PR) after initial repair scheduled for pulmonary valve replacement (PVR) were enrolled. Metabolic treadmill testing (EST) and MRI were obtained before and after surgery. EST results were compared with matched controls. Preoperative exercise time and peak oxygen consumption (VO2 max) were significantly diminished compared with controls but were not significantly different postoperatively. The patients were then split into age-related cohorts. When comparing pre-PVR and post-PVR exercise time and VO2 max among themselves, neither cohort showed significant differences. However, patients younger than 25 years had better postoperative results, an age-related difference not seen in the controls. Preoperative MRI showed significantly dilated RV, PR, and low normal function. After PVR, the right to left ventricular end-diastolic volume ratio (RVEDV:LVEDV) and pulmonary artery regurgitant fraction (RF) significantly decreased. There was no change in ventricular ejection fractions (EF). Severe PR, decreased RVEF, and RV dilation can significantly diminish exercise capacity. PVR improves RVEDV:LVEDV and RF, but not EF. Younger patients had better exercise capacity that was maintained postoperatively. This age-related difference was not seen in the controls, indicating that earlier intervention may preserve exercise capacity. Serial ESTs in patients with severe PR following RVOT repair may identify deteriorating exercise capacity as an early indicator for the need for PVR.

Why exercise capacity does not improve after pulmonary valve replacement.

Optimal timing of pulmonary valve replacement (PVR) for pulmonary regurgitation is a debated topic. It is logical that maximal aerobic capacity (VO2peak) would decline when a PVR is needed, but a diminished VO2peak is not always present before PVR, and previous studies show no improvement in VO2peak after PVR. This study aimed to evaluate changes in resting spirometry from pre- to post-PVR sternotomy, to determine the limiting factors of VO2peak before and after PVR, and to determine whether changes in resting lung function after PVR may explain the lack of improvement in VO2peak after surgery. For 26 patients (age, 19.7 ± 7.8 years) with a history of right ventricular outflow tract revision, the study prospectively evaluated echocardiograms, resting spirometry, and maximal exercise tests before PVR and then an average of 15 months after PVR. Flow volume loops were reviewed by a pulmonologist and categorized as obstructive, restrictive, both obstructive and restrictive, or normal. Exercise tests were interpreted using Eschenbacher's algorithm to determine the primary factors limiting exercise. No change in VO2peak or spirometry after PVR was observed. Before PVR, many patients had abnormal resting lung functions (85 % abnormal), which was unchanged after PVR (86 5 % abnormal). The majority of the patients had a ventilatory limitation to VO2peak before PVR (66.7 %), whereas 28.5 % had a cardiovascular limitation, and 4.8 % had no clear limitation. After PVR, 65.2 % of the patients had a ventilatory limitation, whereas 30.4 % had a cardiovascular limitation, and 4.4 % had no clear limitation to VO2peak. Pulmonary function did not change up to 15 months after surgical PVR. The frequency of pulmonary limitation to VO2peak after PVR did not increase. The effect of pulmonary function on exercise-related symptoms must be considered in this patient population. Improved cardiac hemodynamics are unlikely to improve VO2peak in a primarily pulmonary-limited patient.

Comparison between transthoracic echocardiography and cardiac magnetic resonance imaging in patients status post atrial switch procedure.

OBJECTIVES: This study compares image quality, cost, right ventricular ejection fraction analysis, and baffle visualization between transthoracic echocardiography and cardiac magnetic resonance imaging in those status post atrial switch for transposition of the great arteries. BACKGROUND: This population requires imaging for serial evaluations. Transthoracic echocardiography is often first line but has drawbacks, many of which are addressed by cardiac magnetic resonance imaging. METHODS: Twelve patients (mean age 25 years) with relatively concurrent (mean 157 days) studies were included. Three separate echocardiography and magnetic resonance imaging physicians independently analyzed baffles, image quality, and right ventricular ejection fractions. Institutional and Medicaid charges were compared. RESULTS: For right ventricular ejection fraction, echocardiography (36.1%) underestimated cardiac magnetic resonance imaging (47.8%, P = .002). Image quality for transthoracic echocardiography was significantly rated lower than cardiac magnetic resonance imaging (P = .002). Baffles were better seen in cardiac magnetic resonance imaging (transthoracic echocardiography vs. cardiac magnetic resonance imaging: superior vena cava 86% vs. 100% [P = .063]; inferior vena cava 33% vs. 97% [P = .002]; pulmonary vein 92% vs. 100% [P = .250]). Comparing hospital charges and Medicaid reimbursement, transthoracic echocardiography respectively costs 18% and 38% less than cardiac magnetic resonance imaging. CONCLUSIONS: In conclusion, transthoracic echocardiography underestimated right ventricular ejection fraction compared to cardiac magnetic resonance imaging. Cardiac magnetic resonance imaging had consistently higher image quality and better visualization of the baffles. Cost differences are minimal. We propose that cardiac magnetic resonance imaging be considered first line for imaging in certain patients' status post atrial switch procedure.

Pulmonary limitation to exercise after repair of D-transposition of the great vessels: atrial baffle versus arterial switch.

This study evaluated resting pulmonary function and its impact on exercise capacity after atrial baffle (BAFFLE) and arterial switch (SWITCH) repair of D-transposition of the great vessels (DTGV). Previously decreased exercise capacity in DTGV patients has been primarily attributed to cardiovascular limitations, whereas pulmonary limitations have largely been overlooked. Resting flow volume loops were compared for BAFFLE (n = 34) and SWITCH (n = 32) patients. Peak exercise variables were compared for BAFFLE (n = 30) and SWITCH (n = 25). Lung disease (restrictive and/or obstructive) was present in 53% of DTGV patients (BAFFLE 62% and SWITCH 44%; p = 0.14). BAFFLE patients had a normal breathing reserve, whereas that of SWITCH patients was decreased (27.3 ± 28.3 vs. 13.0 ± 19.2; p = 0.04). BAFFLE patients attained a lower percent of predicted peak oxygen pulse (82.7 ± 20.5% vs. 94.7 ± 19.3%; p = 0.04) and peak oxygen consumption (VO(2peak)) (26.6 ± 6.7 ml/kg/min vs. 37.3 ± 8.5 ml/kg/min; p < 0.01) than SWITCH patients. Patients after surgical repair for DTGV have an underappreciated occurrence of lung disease, even post-SWITCH. SWITCH patients have diminished breathing reserves, suggesting a pulmonary limitation to VO(2peak). BAFFLE patients have lower VO(2peaks), greater breathing reserves, and lower oxygen pulses than SWITCH patients, suggesting a cardiac limitation to peak aerobic capacity with probable secondary pulmonary limitations. Treating underlying lung disease in symptomatic patients after repair of DTGV may improve functional status.

Myocardial perfusion and exercise capacity 12 years after arterial switch surgery for D-transposition of the great arteries.

Peak exercise myocardial perfusion was evaluated in patients with D-transposition of the great arteries 12 years after the arterial switch operation (SWITCH) to evaluate coronary perfusion. Gas-exchange measurements were used to assess cardiac limiting factors to exercise capacity in SWITCH patients when compared to healthy gender-matched controls (CON). Peak myocardial perfusion was evaluated in 42 patients 12 years post-SWITCH, using technetium-99 m (Tetrofosmin). SWITCH exercise data was compared to 42 gender-matched controls (CON). One symptomatic and one asymptomatic SWITCH patient had abnormal exercise myocardial perfusion; both patients had variant coronary anatomy preoperatively. SWITCH patients had lower VO(2peak) (p < 0.01), peak heart rates (p = 0.01), percentages of age-predicted peak heart rates (p < 0.01), and peak oxygen pulses indexed to body surface area (p < 0.01) than CON patients. Exercise testing with myocardial perfusion imaging helped to identify the rare SWITCH patient with coronary insufficiencies. This study demonstrates that exercise testing with myocardial perfusion scans can help identify patients at risk for myocardial events. This study also demonstrated that SWITCH patients have a mildly diminished VO(2peak) when compared to CON patients.

Left ventricular noncompaction is associated with mutations in the mitochondrial genome.

Left ventricular noncompaction (LVNC) is a genetically heterogeneous condition and several nuclear loci have been associated with the defect. However, they only account for a small percentage of patients. Existing evidences suggest that pathogenic mitochondrial DNA (mtDNA) mutations and consequent mitochondrial malfunction can be an important component in the etiology of LVNC. To investigate if mtDNA mutation can serve as a primary cause for LVNC, complete nucleotide sequences of mitochondrial genomes from 20 LVNC patients were determined by Illumina parallel sequencing technology and analyzed by MitoMaster. Substitutions of a highly conserved Met31 in ND1 caused by rare mitochondrial single nucleotide polymorphisms (mtSNP) A3397G and T3398C were identified from two LVNC patients. Previously, T3398C has been reported from another LVNC patient, indicating mutations in Met31 in ND1 and resultant defects in complex I can be associated with LVNC. Additionally, three mtSNPs in protein-coding genes, seven variants in rRNA genes, and two transitions in tRNA genes were unrelated to the haplogroup and infrequent in the general population, suggesting that these mtSNPs could also be pathogenic. Our study revealed some mtSNPs could represent pathogenic mutations, lead to compromised mitochondrial function, and be associated with LVNC.

Sildenafil for the treatment of pulmonary hypertension in pediatric patients.

Sildenafil is a phosphodiesterase 5 inhibitor widely used for the treatment of pulmonary hypertension in children. Despite limited available safety and efficacy evidence, use of sildenafil continues to increase. To date, sildenafil use for pediatric pulmonary hypertension has been characterized for 193 children through 16 studies and 28 case series and reports. The primary efficacy data suggest that sildenafil is beneficial for facilitating the weaning of inhaled nitric oxide in children after cardiac surgery. Compiled safety data suggest that sildenafil is well tolerated among children with idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease. This review summarizes the available data describing the use, safety, and efficacy of sildenafil for children with pulmonary hypertension.

Is left ventricular noncompaction in children truly an isolated lesion?

Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 +/- 5.6 years, and the mean duration of follow-up was 1.9 +/- 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52%; mean 39.5% +/- 13.1%). Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein's anomaly (n = 5 [11%]). Electrocardiogram abnormalities were found in 80% of patients; most commonly they included left (n = 15 [43%]) and right ventricular hypertrophy (n = 19 [54%]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20%, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 +/- 6.1 years) was higher than nonsurvivors (0.4 +/- 0.7 years) (p < 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.

Outcome of cardiac thrombi in infants.

Use of central lines in the neonatal intensive care unit (NICU) has led to the formation of intracardiac thrombi. A paucity of data exists on the management of neonatal cardiac thrombi, with the few reported cases focusing on outcomes following thrombolytic therapy. This study was undertaken to evaluate the outcome of cardiac thrombi in neonates who do not receive thrombolytic therapy. Nineteen patients younger than 3 months of age diagnosed with cardiac thrombi were included. All 19 patients had a central line. Management consisted of a combination of antibiotics and low-molecular-weight heparin (n = 16) or surgical removal (n = 2). In one case, no treatment was instituted. One patient was lost to follow-up after partial resolution of the thrombus. Complete thrombus resolution occurred in 18 patients, 9 with negative blood cultures and 9 with positive blood cultures. It took longer for resolution of thrombi associated with positive blood cultures than for sterile thrombi. No patient had evidence of thrombus embolization. From these data we concluded that the natural history of cardiac thrombi is resolution. Infected thrombi require more prolonged therapy. Surgery is seldom required and thrombolytics are not usually necessary for clot resolution.

Long-term functional outcome of patients following the mustard procedure: the next decade of follow-up.

BACKGROUND: Nearly 10 years ago, we studied a population of patients with d-transposition of the great arteries (DTGA) who had undergone the Mustard procedure 8-26 years earlier. The present study was undertaken to determine how that cohort of patients is currently functioning. METHODS: Of the 45 original patients, 44 were located. Six (13%) had either died (n = 4) or received a cardiac transplant (n = 2). In total, 35 of the remaining 38 patients (78%) chose to undergo testing. Systemic right ventricular ejection fraction (RVEF) was estimated using radionuclide angiocardiography. Exercise stress testing, echocardiography, Holter monitoring, and a quality of life questionnaire were also performed. RESULTS: Those 6 that died or received cardiac transplantation did so between 16 and 25 years of age. The surviving participants ranged in age from 19 to 37 years. Peak oxygen consumption was significantly diminished at 27.7 +/- 6.9 mL/kg/min. Comparison of exercise duration from the original study demonstrated a significant decrease (11.2 +/- 2.2 minutes to 9.1 +/- 2.9 minutes) (P < 0.001). Comparison of RVEF data from the original study did not demonstrate a significant decrease (0.54 +/- 0.10 to 0.53 +/- 0.10) (P = 0.27). Quality of life data suggested that Mustard patients do not score as well in physical functioning, general health, and level of energy as normal adults, but are comparable with adult patients with other chronic diseases. Mustard patients are similar to normal adults and feel better than other adults with chronic illness in their interpretation of social functioning and bodily pain. CONCLUSION: Cardiac mortality of 13% occurred during the second and third decade of life in this cohort of patients with DTGA palliated by the Mustard procedure. Despite continuing deterioration in exercise performance, right ventricular function, and cardiac rhythm, many surviving patients with DTGA continue to lead normal lives into the 4th decade after Mustard procedure.