Perinatal outcome after prenatal diagnosis of single-ventricle cardiac defects.

OBJECTIVES: To investigate the perinatal outcome of cases with a prenatal diagnosis of single-ventricle cardiac defects, single ventricle being defined as a dominant right ventricle (RV) or left ventricle (LV), in which biventricular circulation was not possible. METHODS: We reviewed patients with a prenatal diagnosis of single-ventricle cardiac defects, made at one institution between 1995 and 2008. Cases diagnosed with double-inlet LV, tricuspid atresia, pulmonary atresia with intact ventricular septum and severe RV hypoplasia and those with hypoplastic left heart syndrome (HLHS) were included in the study population. Patients with HLHS were identified prenatally as being standard risk or high risk (HLHS with highly restrictive or intact atrial septum, mitral stenosis with aortic atresia and/or LV coronary artery sinusoids). Patients with an address over 200 miles from the hospital, diagnosed with heterotaxy syndrome or referred for fetal intervention, were excluded. RESULTS: We identified 312 cases of single-ventricle cardiac defect (208 dominant RV; 104 dominant LV) that were diagnosed prenatally. Most (96%) patients with a dominant RV had HLHS. Among the total 312 cases there were 98 (31%) elective terminations of pregnancy (TOP), 12 (4%) cases of spontaneous fetal demise, 12 (4%) cases lost to prenatal follow-up and 190 (61%) live births. Among the 199 patients that underwent fetal echocardiography before 24 weeks' gestation, there were 97 (49%) cases of elective TOP. There was no difference in prenatal outcome between those with a dominant RV and those with a dominant LV (P = 0.98). Of the 190 live births, five received comfort care. With an average of 7 years' follow-up (to obtain data on the Fontan procedure), transplantation-free survival was lower in those with a dominant RV than in those with a dominant LV (standard-risk HLHS odds ratio (OR), 3.0 (P = 0.01); high-risk HLHS OR, 8.8 (P < 0.001)). CONCLUSIONS: The prenatal outcome of cases with single-ventricle cardiac defects was similar between those with a dominant RV and those with a dominant LV, however postnatal intermediate-term survival favored those with a dominant LV. High-risk HLHS identified prenatally was associated with the lowest transplantation-free survival.

A risk-prediction model for in-hospital mortality after heart transplantation in US children.

We sought to develop and validate a quantitative risk-prediction model for predicting the risk of posttransplant in-hospital mortality in pediatric heart transplantation (HT). Children <18 years of age who underwent primary HT in the United States during 1999-2008 (n = 2707) were identified using Organ Procurement and Transplant Network data. A risk-prediction model was developed using two-thirds of the cohort (random sample), internally validated in the remaining one-third, and independently validated in a cohort of 338 children transplanted during 2009-2010. The best predictive model had four categorical variables: hemodynamic support (ECMO, ventilator support, VAD support vs. medical therapy), cardiac diagnosis (repaired congenital heart disease [CHD], unrepaired CHD vs. cardiomyopathy), renal dysfunction (severe, mild-moderate vs. normal) and total bilirubin (≥ 2.0, 0.6 to <2.0 vs. <0.6 mg/dL). The C-statistic (0.78) and the Hosmer-Lemeshow goodness-of-fit (p = 0.89) in the model-development cohort were replicated in the internal validation and independent validation cohorts (C-statistic 0.75, 0.81 and the Hosmer-Lemeshow goodness-of-fit p = 0.49, 0.53, respectively) suggesting acceptable prediction for posttransplant in-hospital mortality. We conclude that this risk-prediction model using four factors at the time of transplant has good prediction characteristics for posttransplant in-hospital mortality in children and may be useful to guide decision-making around patient listing for transplant and timing of mechanical support.

Trends in wait-list mortality in children listed for heart transplantation in the United States: era effect across racial/ethnic groups.

We sought to evaluate trends in overall and race-specific pediatric heart transplant (HT) wait-list mortality in the United States (US) during the last 20 years. We identified all children <18 years old listed for primary HT in the US during 1989-2009 (N = 8096, 62% White, 19% Black, 13% Hispanic and 6% Other) using the Organ Procurement and Transplant Network database. Wait-list mortality was assessed in four successive eras (1989-1994, 1995-1999, 2000-2004 and 2005-2009). Overall wait-list mortality declined in successive eras (26%, 23%, 18% and 13%, respectively). The decline across eras remained significant in adjusted analysis (hazard ratio [HR] 0.70 in successive eras, 95% confidence interval [CI], 0.67-0.74) and was 67% lower for children listed during 2005-2009 versus those listed during 1989-1994 (HR 0.33; CI, 0.28-0.39). In models stratified by race, wait-list mortality decreased in all racial groups in successive eras. In models stratified by era, minority children were not at higher risk of wait-list mortality in the most recent era. We conclude that the risk of wait-list mortality among US children listed for HT has decreased by two-thirds during the last 20 years. Racial gaps in wait-list mortality present variably in the past are not present in the current era.

Changes in echocardiographic measures of systolic and diastolic function in children 1 year after hematopoietic SCT.

Hematopoietic SCT (HSCT) is a life-saving therapy in children, but has been associated with heart failure. Little is known about subclinical changes in cardiac function. We examined changes in systolic and diastolic function from pre- to 1-year post HSCT by echocardiography. All patients (n=74, 61% men, median age 9.1 years, mean left-ventricular (LV) ejection fraction 61.3±4.9%) who underwent HSCT at Children's Hospital Boston between 2005 and 2008, were <21 years at time of HSCT, and had routine pre- and 1-year post echocardiograms were included. Systolic function parameters, including LV ejection fraction, rate-corrected velocity of fiber shortening (Vcfc) and stress-velocity index and diastolic parameters, including tissue Doppler imaging (TDI)-derived velocities, and left-ventricular flow propagation, were compared before and after transplant. At 1-year post HSCT, systolic function, as measured by Vcfc (1.10±0.15 vs 1.04±0.12 circ/s; P=0.03) and stress-velocity index (z-score 0.40±1.4 vs -0.20±1.1; P=0.02), had worsened; diastolic function parameters, including mitral E' velocity (16.6±3.9 vs 15.0±3.4 cm/s; P=0.01) and tricuspid E' velocity (14.3±3.6 vs 12.4±2.8 cm/s; P=0.002) had also decreased. At 1-year post HSCT, children have subclinical declines in systolic and diastolic function. These small changes might become clinically important over time. Serial non-invasive assessment of cardiac function should be considered in all children following HSCT.

Improved survival in pediatric heart transplant recipients: have white, black and Hispanic children benefited equally?

We assessed whether the improvement in posttransplant survival in pediatric heart transplant (HT) recipients during the last two decades has benefited the major racial groups in the United States equally. We analyzed all children <18 years of age who underwent their first HT in the US during 1987-2008. We compared trends in graft loss (death or retransplant) in white, black and Hispanic children in five successive cohorts (1987-1992, 1993-1996, 1997-2000, 2001-2004, 2005-2008). The primary endpoint was early graft loss within 6 months posttransplant. Longer-term survival was assessed in recipients who survived the first 6 months. The improvement in early posttransplant survival was similar (hazard ratio [HR] for successive eras 0.80, 95% confidence interval [CI] 0.7, 0.9, p = 0.24 for black-era interaction, p = 0.22 for Hispanic-era interaction) in adjusted analysis. Longer-term survival was worse in black children (HR 2.2, CI 1.9, 2.5) and did not improve in any group with time (HR 1.0 for successive eras, CI 0.9, 1.1, p = 0.57; p = 0.19 for black-era interaction, p = 0.21 for Hispanic-era interaction). Thus, the improvement in early post-HT survival during the last two decades has benefited white, black and Hispanic children equally. Disparities in longer-term survival have not narrowed with time; the survival remains worse in black recipients.

Racial and ethnic differences in mortality in children awaiting heart transplant in the United States.

Racial differences in outcomes are well known in children after heart transplant (HT) but not in children awaiting HT. We assessed racial and ethnic differences in wait-list mortality in children <18 years old listed for primary HT in the United States during 1999-2006 using multivariable Cox models. Of 3299 listed children, 58% were listed as white, 20% as black, 16% as Hispanic, 3% as Asian and 3% were defined as 'Other'. Mortality on the wait-list was 14%, 19%, 21%, 17% and 27% for white, black, Hispanic, Asian and Other children, respectively. Black (hazard ratio [HR] 1.6, 95% confidence interval [CI] 1.3, 1.9), Hispanic (HR 1.5, CI 1.2, 1.9), Asian (HR, 2.0, CI 1.3, 3.3) and Other children (HR 2.3, CI 1.5, 3.4) were all at higher risk of wait-list death compared to white children after controlling for age, listing status, cardiac diagnosis, hemodyamic support, renal function and blood group. After adjusting additionally for medical insurance and area household income, the risk remained higher for all minorities. We conclude that minority children listed for HT have significantly higher wait-list mortality compared to white children. Socioeconomic variables appear to explain a small fraction of this increased risk.

Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair.

BACKGROUND: Factors associated with impaired clinical status in a cross-sectional study of patients with repaired tetralogy of Fallot (TOF) have been reported previously. OBJECTIVES: To determine independent predictors of major adverse clinical outcomes late after TOF repair in the same cohort during follow-up evaluated by cardiac magnetic resonance (CMR). METHODS: Clinical status at latest follow-up was ascertained in 88 patients (median time from TOF repair to baseline evaluation 20.7 years; median follow-up from baseline evaluation to most recent follow-up 4.2 years). Major adverse outcomes included (a) death; (b) sustained ventricular tachycardia; and (c) increase in NYHA class to grade III or IV. RESULTS: 22 major adverse outcomes occurred in 18 patients (20.5%): death in 4, sustained ventricular tachycardia in 8, and increase in NYHA class in 10. Multivariate analysis identified right ventricular (RV) end-diastolic volume Z >or=7 (odds ratio (OR) = 4.55, 95% confidence interval (CI) 1.10 to 18.8, p = 0.037) and left ventricular (LV) ejection fraction <55% (OR = 8.05, 95% CI 2.14 to 30.2, p = 0.002) as independent predictors of outcome with an area under the receiver operator characteristic curve of 0.850. LV ejection fraction could be replaced by RV ejection fraction <45% in the multivariate model. QRS duration >or=180 ms also predicted major adverse events but correlated with RV size. CONCLUSIONS: In this cohort, severe RV dilatation and either LV or RV dysfunction assessed by CMR predicted major adverse clinical events. This information may guide risk stratification and therapeutic interventions.

Prospective single-arm protocol of carvedilol in children with ventricular dysfunction.

The objective of this study was to evaluate the safety and efficacy of carvedilol in pediatric patients with stable moderate heart failure. We performed a single-arm prospective drug trial at three academic medical centers and the results were compared to historical controls. Patients were 3 months to 17 years old with an ejection fraction <40% in the systemic ventricle for at least 3 months on maximal medical therapy including ACE inhibitors. Treated patients were started on 0.1 mg/kg/day and uptitrated to 0.8 mg/kg/day or the maximal tolerated dose. Echocardiographic parameters of function were prospectively measured at entry and at 6 months. Two composite endpoints were recorded: severe decline in status and significant clinical change. Adverse events were reviewed by a safety committee. Data were also collected from untreated controls with dilated cardiomyopathy meeting entry criteria, assessed over a similar time frame. Twenty patients [12 dilated cardiomyopathy (DCM) and 8 congenital] with a median age of 8.4 years (range, 8 months to 17.8 years) were treated with carvedilol. Three patients discontinued the drug during the study. At entry, there was no statistical difference in age, weight, or ejection fraction between the treated group and controls. The ejection fraction of the treated DCM group improved significantly from entry to 6 months (median, 31 to 40%, p = 0.04), with no significant change in ejection fraction in the control group [median, 29 to 27%, p = not significant (NS)]. The median increase in ejection fraction was larger for the treated DCM group than for the untreated DCM controls (7 vs 0%, p = 0.05). By Kaplan-Meier analysis, time to death or transplant tended to be longer in treated patients (p = 0.07). The difference in the proportion of patients with severe decline in status or significant clinical change in the treated group was not significant compared to the controls (5 vs 12%, p = NS). We conclude that in this prospective protocol of pediatric patients, the use of adjunct carvedilol in the DCM group improved ejection fraction compared to untreated controls and trended toward delaying time to transplant or death.

Racial and ethnic disparities in mortality following congenital heart surgery.

Our objective was to assess risk-adjusted racial and ethnic disparities in mortality following congenital heart surgery. We studied 8483 congenital heart surgical cases from the Kids' Inpatient Database 2000. Black sub-analysis was performed using predetermined regional categories. For our Hispanic sub-analyses, we categorized Hispanics into state groups according to a state's predominant Hispanic group: West (Mexican-American), Southeast (Cuban-American), Northeast (Puerto Rican), and Mixed/Heterogeneous. Risk adjustment was performed using the Risk Adjustment for Congenital Heart Surgery method. Multivariate analyses assessed the effect of race/ethnicity and Hispanic state group on mortality and explored the effects of gender, income, insurance type, and region. Black children had a higher risk for death than Whites odds ratio (OR), [1.65; p = 0.003]. Hispanics and the Cuban-American state group showed a trend toward a higher death risk (Hispanic: OR, 1.24; p = 0.16; Southeast Cuban-American: OR 1.55; p = 0.08). Disparities were not influenced by insurance. Among Blacks, disparities were greatest in the Northeast region (OR, 2.25; p = 0.007). After adjusting for gender, income, and region, Blacks (OR, 1.76; p = 0.002) and Hispanics (OR, 1.34; p = 0.05) had a higher death risk. Racial and ethnic disparities in risk-adjusted mortality following congenital heart disease exist for Blacks and Hispanics. These disparities are not due to insurance but are partially explained by gender and region.

Health-related quality of life in children attending a cardiology clinic.

Our purpose was to evaluate the impact of suspicion or confirmation of heart disease on the physical and psychosocial health of children. We utilized the Child Health Questionnaire (CHQ PF-50). Children ages 5 to 18 years attending a general cardiology clinic were eligible. Those with primary noncardiac diagnoses unrelated to heart disease were excluded. Children with similar conditions were grouped together for analysis. Group and subgroup means were compared to a U.S. population normative sample using the two-sample t test. The CHQ was administered to 321 patients (median age, 10.6 years). Overall, parents reported mean Physical and Psychosocial Summary Scores comparable to those for the normative sample (mean, 51.5 vs 53.0, p = 0.04; mean, 52.3 vs 51.2, p = 0.10). There was a trend toward worse physical health in most subgroups, especially those with cardiomyopathy (CM) (46.5; p = 0.01), and a comparable trend toward better psychosocial health except in those requiring major interventions. In subscale analyses, most subgroups reported worse Physical Functioning than the normative sample, especially CM (85.1 vs 96.1; p = 0.02). Parents of children with CM (53.2 vs 73.0; p = 0.002) and the intervention subgroups (except minor) reported worse General Health Perceptions. Parents experienced increased Parental Impact-Emotional, especially parents of children undergoing evaluations for chest pain (62.5 vs 80.3; p = 0.007). Most parents reported comparable or better health for the Family Cohesion and Bodily Pain subscales. Generally, parents of children attending a cardiology clinic report physical and psychosocial health comparable to that for the general U.S. population. However, diagnosis or confirmation of heart disease resulted in worse physical functioning and health perceptions and a significant negative emotional impact on parents.

Regional racial and ethnic differences in mortality for congenital heart surgery in children may reflect unequal access to care.

The objective of this study was to explore racial differences in mortality for congenital heart surgery. We performed a population-based retrospective cohort study using hospital discharge abstract data from four states in 1996. The outcome measure was risk-adjusted in-hospital mortality. Cases of pediatric congenital heart surgery were classified into six risk categories using the Risk Adjustment in Congenital Heart Surgery method. Differences in risk-adjusted in-hospital mortality among racial groups were explored. Analyses stratified by state were used to identify regional differences. Of 5791 cases, 4822 (83%) were assigned to a risk group for analysis. Surgical mortality differed for whites compared to non-whites (3.7 vs 5.1%, p = 0.02). Among non-white groups, unadjusted mortality rates varied: Asian, 5.3%; black, 4.1%; Hispanic 4.9%; other, 7.3%; and missing, 7.6% (p = 0.008). Adjusted mortality also differed by race but was inconsistent across regions, making explanatory factors based solely on biology implausible. For example, compared to whites, blacks had a higher risk of dying in Massachusetts [odds ratio (OR) = 6.39, p = 0.08] but lower in Pennsylvania (OR = 0.41, p = 0.009). Adding insurance type to models did not eliminate racial differences. In risk-adjusted analyses, non-white groups had a higher risk of dying after congenital heart surgery than whites. Inconsistent effects among regions suggest that differential mortality is due to unequal access to care rather than biology.

Risk of death for Medicaid recipients undergoing congenital heart surgery.

The objective of this study was to explore the effect of insurance type on mortality for congenital heart surgery. We performed a population-based retrospective cohort study using hospital discharge abstract data from five states in 1992 and 1996. The outcome measure was risk-adjusted in-hospital mortality. Cases of pediatric congenital heart surgery were identified and placed into six risk categories using the Risk Adjustment in Congenital Heart Surgery method. Multivariate analyses were used to determine the effect of insurance type on risk-adjusted mortality; regional effects were explored. Using standardized mortality ratios, institutions were grouped by outcome; within and between group differences were examined. Of 11,636 cases, 9656 (83%) were placed in a risk group for analysis. In 1996, children with Medicaid had a higher risk of death than those with commercial or managed care in both unadjusted (p = 0.002) and adjusted (p < 0.001) analyses. Overall mortality rates decreased between 1992 and 1996 (p = 0.001). However, improvement was not consistent among insurance groups. Differences were present within and between low, average, and high-mortality hospitals, suggesting that the adverse effect of Medicaid may be due to both differential referral and other differences in care among patients treated at similar institutions. Children with Medicaid insurance have a higher risk of dying after congenital heart surgery than those with commercial and some managed care insurance. Barriers to access go beyond differences in referral patterns.

Healing response to the Clamshell device for closure of intracardiac defects in humans.

The healing response to intracardiac devices in humans is largely unknown. During regulatory trials using the Clamshell device in over 800 patients, attempts were made to perform histopathological evaluation of all explanted devices. We reviewed all those with complete histopathological examination (n = 12) from Fontan baffles (n = 4), ventricular septal defects (n = 2), and atrial septal defects (ASD; n = 6), explanted at 2.7 months to 3.6 years (median, 1.6 years), at autopsy (n = 1) or surgery (n = 11), performed for residual defects (n = 5), atrial masses (n = 3), or Fontan revision (n = 3). All but one were nearly (n = 3) or completely (n = 8) covered by pseudointima, composed of fibroelastic tissue, predominantly collagen, with focal foreign body reaction in contact with fabric, without acute inflammation or infection. Atrial masses of granulation tissue were present in three cases (ASD), opposite to protruding fractured arms. No associations were identified between coverage and closure status, position, arm fractures, or implant period. In conclusion, the healing response to transcatheter Clamshell implantation in humans is characterized by a relatively rapid development of a nonthrombotic pseudointima composed of fibroelastic tissue with minimal foreign body reaction. Cathet Cardiovasc Intervent 2001;54:101-111.

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

BACKGROUND: Decisions regarding surgical strategy in patients with multiple left heart obstructive or hypoplastic lesions often must be made in the newborn period and are seldom reversible. Predictors of outcome of biventricular repair have not been well defined in this heterogeneous group of patients, and risk factors described for critical aortic valve stenosis have been shown to be inapplicable to patients with other left heart obstructive lesions. The goal of this study was to identify echocardiographic predictors of outcome of biventricular repair for infants with multiple left heart obstructive lesions. METHODS AND RESULTS: Patients with >/=2 areas of left heart obstruction or hypoplasia, diagnosed at

Balloon dilation angioplasty of peripheral pulmonary stenosis associated with Williams syndrome.

BACKGROUND: Experience of balloon dilation of peripheral pulmonary stenosis (PPS) in Williams syndrome (WS) is limited. METHODS AND RESULTS: Catheterizations in all patients with WS undergoing therapy for PPS from 1984 to 1999 were reviewed. Criteria for successful dilation included an increase >50% in predilation diameter and a decrease >20% in ratio of right ventricular (RV) to aortic (Ao) systolic pressure. Median age and weight were 1.5 years and 9.5 kg. There were 134 dilations during 39 procedures in 25 patients. The success rate for initial dilations was 51%. In multivariate analysis, successful dilation was more likely (1) in distal than in central pulmonary arteries (P=0.02), (2) if the balloon waist resolved with inflation (P=0.001), and (3) with larger balloon/stenosis ratio (P<0.001). RV pressure was unchanged after dilation (96+/-30 versus 97+/-31 mm Hg), primarily because of failure to enlarge central pulmonary arteries. The Ao pressure increased (102+/-14 versus 109+/-19 mm Hg, P=0.03), and the RV/Ao pressure ratio decreased (0.97+/-0.34 versus 0.91+/-0.30, P=0.05). Aneurysms developed after 24 dilations (18%) and were not related to balloon/stenosis ratio. Balloon rupture in 12 dilations produced an aneurysm in all 7 cases when rupture was in a hypoplastic segment. Three patients died, none from pulmonary artery trauma, and all before 1994. CONCLUSIONS: Mortality occurred early in our experience. Despite successful dilation of distal pulmonary arteries, there was modest initial hemodynamic improvement, mainly because of persistent central pulmonary artery obstruction. A serial approach of distal dilations followed by surgical repair of proximal obstruction may be a rational and successful therapy.

Heart size on chest x-ray as a predictor of cardiac enlargement by echocardiography in children.

To determine the usefulness of heart size on chest radiograph (CXR) in predicting cardiac enlargement (CE) in children, we prospectively evaluated 95 consecutive outpatients, who had both a CXR and echocardiography performed. Their median age was 5.0 years (2 days to 19.9 years). All patients underwent CXR assessment by a pediatric radiologist, with classification of cardiac silhouette as normal, borderline, or enlarged. Echocardiographic assessment of CE was performed by a pediatric echocardiographer. Sensitivity, specificity, and predictive values of the pediatric radiologist's interpretation of heart size on CXR were estimated. The presence of CE by echocardiography was used as the gold standard. Seventy-nine patients (83.2%) had no CE on CXR, and 16 patients (16.8%) had CE. Sensitivity of the CXR to identify CE was 58.8%, 95% confidence interval (CI) [32.9, 81.6], with a positive predictive value of 62.5% [35.4, 84.8]. Specificity was 92.3% [84.0, 97.1], with a negative predictive value of 91.1% [82.6, 96.4]. These data suggest that the assessment of CE on CXR to predict CE by echocardiography has a relatively high specificity and negative predictive value, but a low sensitivity and positive predictive value. The limitations of CXR as a diagnostic test should be understood by clinicians using the test when screening children for cardiac disease.

Clinical outcome of fenestrated Fontan patients after closure: the first 10 years.

BACKGROUND: The late clinical status of Fontan patients after fenestration closure is unknown. Data are now available on all patients who underwent closure from 1989 to 1999. METHODS AND RESULTS: All patients who underwent catheter closure of a Fontan fenestration were enrolled in either the Clamshell (1989 to 1994) or CardioSEAL (1996 to 1999) regulatory trials. Physiological values obtained at catheterization helped assess the hemodynamic effects of fenestration occlusion. In addition to survival, outcomes assessed included O(2) saturations, medication use, significant clinical findings (eg, heart failure, protein-losing enteropathy, or new arrhythmias), and somatic growth. Of 181 patients who underwent closure, 27 had additional significant leaks. The remaining 154 patients constituted the study group. Median time from closure to latest follow-up was 3.4 years (range 0.4 to 10.3 years). Fenestration closure increased O(2) saturation 9.4% on average (P:<0. 001). The numbers of patients receiving digoxin or diuretics decreased at the most recent follow-up compared with baseline (P:<0. 001), but use of antiarrhythmic agents increased marginally (P:=0. 05). Height and weight percentiles rose (medians of 2 and 4, respectively; P:<0.001). Clinical decompensation during follow-up of 154 patients was rare (4.5%), with 2 deaths, 3 Fontan revisions, and 1 patient each with protein-losing enteropathy and ascites. No other patient developed chronic congestive symptoms; 21 patients developed new arrhythmias, and 2 had a stroke or transient ischemic attack. CONCLUSIONS: Fenestration closure in Fontan patients was followed by improved oxygenation, reduced need for anticongestive medication, and improved somatic growth at latest follow-up. Death (1.3%) or chronic decompensation (3.2%) was rare.

Twenty-five-year experience with rastelli repair for transposition of the great arteries.

OBJECTIVE: Our purpose was to describe the outcome of the Rastelli repair in D -transposition of the great arteries and to determine the risk factors associated with unfavorable events. METHODS: From March 1973 to April 1998, 101 patients with D -transposition of the great arteries and ventricular septal defect underwent a Rastelli type of repair. Median age and weight were 3.1 years (10th to 90th percentiles 0.3-9.9 years) and 12.8 kg (5.9-28.2). Pulmonary stenosis was present in 73 patients and pulmonary atresia in 18; 10 patients had no left ventricular outflow tract obstruction. RESULTS: There were 7 early deaths (7%) and no operative deaths in the last 7 years of the study. Risk factors for early death, by univariable analysis, included straddling tricuspid valve (P =.04) and longer aortic crossclamping times (P =.04). At a median follow-up of 8.5 years, there were 17 late deaths and 1 patient had undergone heart transplantation. Forty-four patients had reoperations for conduit stenosis, 11 for left ventricular outflow tract obstruction, and 28 had interventional catheterization to relieve conduit stenosis. Nine patients had late arrhythmias, and there were 5 sudden deaths. Overall freedom from death or transplantation (Kaplan-Meier) was 82%, 80%, 68%, and 52% at 5, 10, 15, and 20 years, respectively. Freedom from death or reintervention (catheterization or surgical treatment) was 53%, 24%, and 21% at 5, 10, and 15 years of follow-up, respectively. CONCLUSIONS: The Rastelli repair can be performed with low early mortality. However, substantial late morbidity and mortality are associated with conduit obstruction, left ventricular outflow tract obstruction, and arrhythmia.