Neighborhood Socioeconomic Status and Readmission in Acute Type A Aortic Dissection Repair.

INTRODUCTION: We examined the association of socioeconomic status as defined by median household income quartile (MHIQ) with mortality and readmission patterns following open repair of acute type A aortic dissection (ATAAD) in a nationally representative registry. METHODS: Adults who underwent open repair of ATAAD were selected using the US Nationwide Readmissions Database and stratified by MHIQ. Patients were selected based on diagnostic and procedural codes. The primary endpoint was 30-d readmission. RESULTS: Between 2016 and 2019, 10,288 individuals (65% male) underwent open repair for ATAAD. Individuals in the lowest income quartile were younger (median: 60 versus 64, P < 0.05) but had greater Elixhauser comorbidity burden (5.9 versus 5.7, P < 0.05). Across all groups, in-hospital mortality was approximately 15% (P = 0.35). On multivariable analysis adjusting for baseline comorbidity burden, low socioeconomic status was associated with increased readmission at 90 d, but not at 30 d. Concomitant renal disease (odds ratio [OR], 1.68; P < 0.001), pulmonary disease (OR, 1.26; P < 0.001), liver failure (OR 1.2, P = 0.04), and heart failure (OR, 1.17; P < 0.001) were all associated with readmission at 90 d. The primary indication for readmission was most commonly cardiac (33%), infectious (16.5%), and respiratory (9%). CONCLUSIONS: In patients who undergo surgery for ATAAD, lower MHIQ was associated with higher odds of readmission following open repair. While early readmission for individuals living in the lowest income communities is likely attributable to greater baseline comorbidity burden, we observed that 90-d readmission rates are associated with lower MHIQ regardless of comorbidity burden. Further investigation is required to determine which patient-level and system-level interventions are needed to reduce readmissions in the immediate postoperative period for resource poor areas.

Contemporary Applications and Outcomes of Pulmonary Artery Banding: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: Pulmonary artery banding (PAB) in isolation or combined with a congenital cardiac surgical procedure is common and has important mortality. We aimed to determine patient characteristics, clinical outcomes, variation in clinical outcomes by diagnoses, and center variation in PAB use. METHODS: Using The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD), this study evaluated outcomes of patients undergoing PAB across diagnoses, participating centers, and additional procedures. Patients were identified by procedure and diagnosis codes from 2016 to 2019. We separated patients into groups of main and bilateral PAB and described their outcomes, focusing on patients with main PAB. RESULTS: This study identified 3367 PAB procedures from 2016 to 2019 (3% of all STS CHSD cardiovascular cases during this period): 2677 main PAB, 690 bilateral PAB. Operative mortality was 8% after main PAB and 26% after bilateral PAB. There was significant variation in use of main PAB by center, with 115 centers performing at least 1 main PAB procedure (range, 1-134; Q1-Q3, 8-33). For patients with main PAB, there were substantial differences in mortality, depending on timing of main PAB relative to other procedures. The highest operative mortality (25%; P < .0001) was in patients who underwent main PAB after another separate procedure during their admission, with extracorporeal membrane oxygenation being the most frequent preceding procedure. CONCLUSIONS: PAB is a frequently used congenital cardiac procedure with high mortality and variation in use across centers. Outcomes vary widely by banding type and patient diagnosis. Main PAB after cardiac surgical procedures, especially extracorporeal membrane oxygenation, is associated with very high operative mortality.

The Congenital Heart Surgeons' Society Kirklin/Ashburn Fellowship: The Fellows' Perspective.

Since its establishment in 2001, the Congenital Heart Surgeons' Society John W. Kirklin/David Ashburn Fellowship has contributed substantially to the field of congenital heart surgery research while simultaneously training the next generation of surgeon- scientists. To date, ten fellows (and counting) have successfully completed this rigorous training, producing over 40 published articles focused on longitudinal outcomes from the various Congenital Heart Surgeons' Society cohorts. As the Kirklin/Ashburn Fellowship expands and additional fellows matriculate, its legacy, the network of support, and the contribution to congenital heart surgery research will undoubtedly hold strong.

24-Year Results of Nonfenestrated Extracardiac Fontan Including Fontan Conversions.

BACKGROUND: There is active debate regarding the optimal method of Fontan palliation. In light of this, we reviewed our experience with the nonfenestrated extracardiac Fontan including Fontan conversion. METHODS: We performed a retrospective review of all nonfenestrated extracardiac Fontan and Fontan conversion operations at our institution from December 1, 1994 to December 31, 2018. Standard demographic data were collected, including underlying anatomy, preoperative ventricular and valvular function, operative details, perioperative data, and clinical outcomes. Statistical analysis included comparison between initial extracardiac Fontan patients and Fontan conversions, as well as analysis of risk factors for adverse outcomes. RESULTS: There were 341 patients with an overall operative mortality of 4 patients (1.2%). Of these, 193 were extracardiac nonfenestrated Fontan completion operations (57%) and 148 were Fontan conversions (43%). Length of stay was 11 days (SD, 6 days) with ventilator duration of 28 hours (SD, 26 hours). Six of the completion Fontan patients (3%) required Fontan takedown at a median time of 2.5 days. Upon multivariable analysis, risk factors associated with adverse events (mortality, Fontan takedown, and/or transplant) included increased cardiopulmonary bypass time, preoperative decreased dominant ventricular function, and length of stay. Kaplan-Meier curves demonstrated that mild or greater preoperative ventricular dysfunction decreased survival as well as freedom from adverse events for both initial extracardiac Fontan and Fontan conversion patients. CONCLUSIONS: Over the past 24 years, our strategy of nonfenestrated extracardiac Fontan has achieved low operative mortality for both initial Fontan and Fontan conversion. There is a steady attrition of Fontan patients to cardiac transplantation; the key risk factor is preoperative ventricular dysfunction.

Outcomes after anomalous aortic origin of a coronary artery repair: A Congenital Heart Surgeons' Society Study.

OBJECTIVES: It remains unclear when sudden cardiac event risk outweighs surgical risk for patients with anomalous aortic origin of a coronary artery. The Congenital Heart Surgeons' Society sought to characterize the surgical risks by determining the techniques, complications, and outcomes of repair. METHODS: Between January 2000 and September 2018, 682 patients with anomalous aortic origin of a coronary artery aged 30 years or less were enrolled. Demographic, morphologic, operative, imaging, and ischemia-related data were analyzed. RESULTS: There were 395 of 682 (57%) surgical patients (45 centers, median follow-up 2.8 years). In addition to primary repair (87% unroofing, 26% commissural manipulation), 13 patients had 15 coronary-related reoperations. Of 358 patients with pre/postoperative aortic insufficiency assessment, 27 (8%) developed new mild or greater aortic insufficiency postoperatively, and 7 (2%) developed new moderate or greater aortic insufficiency. Freedom from mild aortic insufficiency differed in those with versus without commissural manipulation (85%/91% at 6 months, 83%/90% at 1 year, and 77%/88% at 3 years, respectively) (P = .05). Of 347 patients with preoperative/postoperative ejection fraction, 6 (2%) developed new abnormal ejection fraction (<50%) within 30 days of surgery which persisted. Although 64 of 395 patients (16%) had preoperative ischemia, after surgery 51 of 64 patients (80%) no longer had ischemia (13 = new postoperative ischemia, P < .0001). Four patients died postoperatively (preoperatively 2 asymptomatic, 1 symptomatic, 1 in extremis). Composite surgical adverse event rates were 7% to 13% in the entire cohort (increasing/decreasing by presentation/anatomy/repair strategy). CONCLUSIONS: Anomalous aortic origin of a coronary artery surgery may relieve ischemia with low mortality; however, it can result in a variety of important morbidities, varying by the group evaluated. Strategies avoiding commissural manipulation may decrease the risk of developing aortic insufficiency. Understanding these risks should inform surgical decision-making and support the need for standardized assessment and management.

Longitudinal functional health status in young adults with repaired dextro-transposition of the great arteries: A Congenital Heart Surgeons' Society study.

OBJECTIVES: Improved survival has led to interest in functional health status (FHS) as patients with dextro-transposition of the great arteries (d-TGA) transition to adulthood. Our primary objectives were (1) evaluation of The Medical Outcomes Study Short Form-36 Health Survey (SF-36) results; (2) comparison with results of patients who completed the Child Health Questionnaire-Child Form 87 (CHQ-CF87) previously, or the PedsQL Generic Core Scales (PedsQL) survey subsequently; and (3) determination of factors associated with SF-36 domains. METHODS: Survivors from the d-TGA Congenital Heart Surgeons' Society cohort (1985-1989) completed the SF-36 (2010) as a measure of FHS (n = 210; age 21-26 years). Patient characteristics, medical history, psychosocial factors, and previous adolescent CHQ-CF87 FHS assessment (2000) were explored for association with SF-36 domains, along with comparison with recent PedsQL data (2017). RESULTS: Patients scored themselves the same/higher than published normative data in 10 of 10 SF-36 summary scores/domains and similar in 5 of 6 PedsQL summary scores/domains. Factors commonly associated with lower summary scores/domains of the SF-36 were presence of cardiac symptoms, heart condition impacting physical activity/overall health/quality of life, unemployment, and lack of postsecondary education. Less commonly associated factors were lower birth weight, greater total medication number, female sex, shorter procedure-free interval, poor health knowledge, lower family income, younger age at SF-36, living with parents, and being married. These factors accounted for 17% to 47% of the variation in FHS summary scores/domains. FHS was minimally related to d-TGA morphology and repair type. CONCLUSIONS: Patients with d-TGA surviving into adulthood, regardless of morphology or repair type, can primarily expect normal FHS. Addressing the challenges of patients with d-TGA entering adulthood requires consideration of psychosocial factors and clinical management.

Pulmonary artery banding in complete atrioventricular septal defect.

OBJECTIVES: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. METHODS: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. RESULTS: At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). CONCLUSIONS: PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.

Normalization of hemodynamics is delayed in patients with a single ventricle after pediatric heart transplantation.

OBJECTIVES: The time course for hemodynamic normalization after pediatric heart transplantation has not been well characterized. We hypothesized that patients with a single ventricle would normalize later than those with dilated cardiomyopathy. Establishing the expected course based on the underlying pathophysiology will allow identification of patients who are outliers, requiring further investigation. METHODS: We performed a retrospective review of patients with dilated cardiomyopathy, Glenn, and Fontan who underwent heart transplantation from January 2007 to December 31, 2017, and had 6-month and 1-year catheterization data. Hemodynamic data were examined for sustained normalization of pressures. Myocardial biopsies were reviewed for clinically significant rejection within the first year. RESULTS: Ninety-four patients comprised the cohort (47 dilated cardiomyopathy, 18 Glenn, 29 Fontan) with a median age of 6.8 (12) years. Patients with dilated cardiomyopathy were more likely to normalize hemodynamics by 6 months (85% vs 28% Fontan, 44% Glenn, P < .05), and 96% of patients with dilated cardiomyopathy had normalized hemodynamics by 1 year (vs 62% Fontan, 78% Glenn, P < .001). The pulmonary capillary wedge pressure at 6 months was higher in patients who underwent the Fontan and Glenn (median 12.8 [8.8] mm Hg and 11.2 [5.9] mm Hg, respectively) compared with patients with dilated cardiomyopathy (7.0 [3.3] mm Hg, P < .001). Patients with dilated cardiomyopathy demonstrated normalized hemodynamics earlier (121 ± 72 days) than patients who underwent the Fontan (329 ± 62 days) and Glenn (233 ± 11 days, P < .001). Eighteen patients (19%) experienced significant rejection, which was not increased in patients with delayed hemodynamic normalization. The 6-month pulmonary capillary wedge pressure was associated with delayed normalization (hazard ratio, 1.36; 95% confidence interval, 1.16-1.60; P < .001). CONCLUSIONS: Patients with a single ventricle demonstrated delayed hemodynamic normalization compared with dilated cardiomyopathy heart transplant recipients, without affecting survival or need for retransplantation.

Management and outcome of intracranial hemorrhage in patients with left ventricular assist devices.

OBJECTIVE: As the use of left ventricular assist devices (LVADs) has expanded, cerebrovascular complications have become an increasing source of morbidity and mortality in this population. Intracranial hemorrhage (ICH) in particular remains a devastating complication in patients who undergo LVAD placement with no defined management guidelines. The authors therefore reviewed surgical and anticoagulation management and outcomes of patients with LVADs who presented to their institution with ICH. METHODS: This retrospective cohort study assessed outcomes of patients who underwent LVAD placement at a single institution between 2007 and 2016 and in whom imaging demonstrated ICH. RESULTS: During the study period, 281 patients had a HeartMate II or HeartWare LVAD placed. There were 37 episodes of ICH (recurrent in 3 cases). ICHs were categorized as intraparenchymal hemorrhage (IPH; n = 22, 59%), subdural hemorrhage (SDH; n = 6, 16%), and subarachnoid hemorrhage (SAH; n = 9, 24%). Neurosurgical intervention was deemed necessary in 27.3%, 66.7%, and 0% of patients with IPH, SDH, and SAH, respectively; overall survival > 30 days for each type of hemorrhage was 41%, 83%, and 89%, respectively. No patients had LVAD thrombus as a result of reversal of anticoagulation. Combined with prior reports, good outcomes are seen more often following surgery for SDH than for IPH (57% vs 7%, p = 0.004) in patients who underwent VAD placement. CONCLUSIONS: Patients with IPH who undergo LVAD placement have poor outcomes regardless of anticoagulation reversal or neurosurgical intervention, whereas those with SDH may have good outcomes with medical and surgical intervention, and those with SAH appear to do well without anticoagulation reversal or surgery. When needed, anticoagulation reversal was not associated with an increase in LVAD thrombosis in this series.

Late Survival and Patient-Perceived Health Status of the Congenital Heart Surgeons' Society dextro-Transposition of the Great Arteries Cohort.

BACKGROUND: Improved survival for patients with dextro-transposition of the great arteries (d-TGA) has led to an increased focus on functional health status (FHS). We assessed late survival and patient-perceived FHS for repaired TGA patients. METHODS: From 1985-1990, 830 neonates admitted to 24 Congenital Heart Surgeons' Society (CHSS) institutions with d-TGA underwent repair, including 516 arterial switch, 110 Mustard, 175 Senning, and 29 Rastelli operations. Median follow-up was 24.0 years (range, 0-32.7 years). We performed multiphase parametric hazard analysis for death after repair. Patients completed Pediatric Quality of Life Inventory (PedsQL) Core Scales and Cardiac Module Adult Forms. Patient and operative factors and CHSS General Questionnaire responses were analyzed for association with FHS using multiple linear regression. RESULTS: Survival at 30 years after repair was arterial switch, 80% ± 2%; Mustard, 81% ± 5%; Senning, 70% ± 4%; and Rastelli, 86% ± 8%. The arterial switch had the lowest hazard for late death. TGA patients reported FHS similar to a healthy population in all domains except physical health (lower scores). Symptoms, including chest pain and fainting, and having a pacemaker were associated with lower, and being employed with higher, self-reported physical health. Arterial switch patients reported higher FHS than the atrial switch patients in all domains. CONCLUSIONS: Arterial switch patients have a lower risk of premature death and better FHS than those with an atrial switch. Increased surveillance in atrial switch patients is warranted because of their increased risk of late death. Presence of symptoms, pacemaker, and lack of employment are associated with reduced FHS.

Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: A Congenital Heart Surgeons' Society study.

OBJECTIVES: We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery. METHODS: We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports. RESULTS: There were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P < .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features. CONCLUSIONS: Anatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.

Intervention for arch obstruction after the Norwood procedure: Prevalence, associated factors, and practice variability.

OBJECTIVE: Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality. METHODS: From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed. RESULTS: Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg). CONCLUSIONS: Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes.

The Congenital Heart Surgeon's Society Complete Atrioventricular Septal Defect Cohort: Baseline, Preintervention Echocardiographic Characteristics.

Quantifying unbalance, the threshold for single ventricle palliation vs biventricular repair in patients with unbalanced complete atrioventricular septal defect (AVSD), is challenging. Using a core laboratory review of baseline echocardiograms, we sought to assess the correlations among commonly used measures of unbalance and common atrioventricular valve (AVV) and ventricular sizes. A single reviewer evaluated baseline echocardiograms from an inception cohort of babies age < 1 year with complete AVSD admitted to 1 of 25 Congenital Heart Surgeon's Society institutions. A standardized echo review protocol of 111 quantitative and qualitative measures was used. Descriptive statistics were computed and Pearson correlation coefficients were calculated to assess correlation among unbalance indices with valvar and ventricular dimensions. Two-hundred fifty-seven baseline echocardiograms of infants with complete AVSD were included. Median age at baseline echocardiogram was 11 days (interquartile range 1-79) and mean atrioventricular valve index was 0.45 ± 0.1. Mean right ventricle/left ventricle inflow angle was 90.2 ± 15.6° and median left ventricular inflow index was 0.46 (interquartile range 0.4-0.5). There are weak or moderate correlations between the measures of unbalance. Correlations between the measures of unbalance with common AVV leaflet or ventricular sizes are also weak to moderate, when statistically significant. Measures of unbalance in common clinical use correlate poorly, or not at all, with one another, common AVV, and ventricular dimensions. The concept of "unbalance" is difficult to define using baseline echocardiographic indices. These findings suggest that the indices may describe different morphologic and functional characteristics. Further analysis is necessary to quantify the contributions of unbalance indices to patient outcome.

Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.

AIMS: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions. METHODS AND RESULTS: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Only 17% of patients with AS/MS had an apex-forming left ventricle, compared with 0% of those with AA/MA and AA/MS (P < 0.0001). Aortic arch hypoplasia and coarctation were common across all four groups, while those with AA/MA and AA/MS had the smallest ascending aorta diameters. Flow in the ascending aorta was retrograde in 43% and 10% of the patients with AS/MS and iAS, respectively. The right ventricle was apex forming in 100% of patients with AA/MA and AA/MS, 96% with AS/MS and 70% with iAS (P < 0.0001). Moderate to severe tricuspid regurgitation was present in 13% of all patients. CONCLUSION: This large multi-institutional study generates insight into the distribution of the functional and morphologic spectrum in patients with critical left-sided heart disease and identifies differences in these functional and morphologic characteristics between the main anatomic subtypes of critical left heart obstruction.

Repair of Partial Atrioventricular Septal Defect: Age and Outcomes.

BACKGROUND: We evaluated the outcomes of patients undergoing surgical repair of partial atrioventricular septal defect (AVSD) and analyzed the effect of age on outcome. METHODS: In this single-center retrospective study, we included all children who underwent repair of partial AVSD between 1990 and 2014. We divided the patients into 4 age quartiles (first quartile: 0-0.75 years, n = 22; second quartile: 0.75-1.5 years, n = 21; third quartile: 1.5-3.75 years, n = 22; and fourth quartile: >3.75 years, n = 21). These quartiles were evaluated for their association with the time-to-event outcomes of survival, freedom from left atrioventricular valve regurgitation (LAVVR), and freedom from reoperation using log-rank analysis. RESULTS: During the study period, 86 patients underwent partial AVSD repair at a median age of 1.5 years. There were no operative deaths and 2 late deaths (unknown cause and trauma). There were 13 reoperations. The most common cause of reoperation was left ventricular outflow tract obstruction (LVOTO) (5 patients [first quartile, 2 cases; second quartile, 1 case; third quartile, 2 cases, and fourth quartile, 0 cases]). LAVV reoperation for insufficiency or stenosis was performed in 4 patients (first quartile, 1 case; second quartile, 1 case; third quartile, 1 case; and fourth quartile, 1 case). Two patients underwent pacemaker placement (second quartile, 1 case and fourth quartile, 1 case). There were no statistically significant differences in the most common complications-LVOTO, LAVVR, and AV heart block-between the 4 age quartiles. Median follow-up was 7.1 years (interquartile range [IQR], 0.8-11.4 years). On echocardiography, 72 patients (84%) had less than or equal to mild LAVVR, 8 (9%) patients had mild to moderate LAVVR, 5 (6%) patients had moderate LAVVR, and 1 (1%) patient had severe LAVVR. Age at repair had no significant association with degree of late AV valve insufficiency. CONCLUSIONS: Results of partial AVSD repair at a median age of 1.5 years are excellent. Operating at this age is not associated with increased mortality, reoperation, or LAVVR.

Doubly committed and juxtaarterial ventricular septal defect: outcomes of the aortic and pulmonary valves.

BACKGROUND: The morphology of ventricular septal defects (VSDs) that are doubly committed and juxtaarterial places the patient at risk for aortic valvar prolapse and aortic valvar insufficiency (AI). Surgical repair of this type of defect often involves placing sutures through the base of one or more of the leaflets of the pulmonary valve, raising concern for late pulmonary valvar insufficiency (PI). The purpose of this review was to analyze the postoperative follow-up relating to potential late complications with the aortic and pulmonary valves. METHODS: Between 1980 and 2012, 106 patients with doubly committed juxtaarterial VSD underwent intracardiac repair. Median age at repair was 1.1 years. Preoperative evaluation showed 69 patients (65%) had aortic valvar prolapse and 51 (48%) had AI. Operative approach was through the pulmonary trunk in 88 (83%) of the patients. In 81 patients (76%), sutures securing the VSD patch had been placed through the base of the pulmonary valvar leaflets. RESULTS: Operative survival was 100%. Follow-up ranges from 6 months to 17 years, with a mean of 4.9 years. No patient had heart block or residual shunting. Of the 70 patients with long-term contemporary echocardiographic follow-up, 66 (94%) had trivial or no AI and 4 (6%) had mild AI. Of these patients, 49 (70%) had trivial or no PI, and 21 (30%) had mild PI. In 1 patient having aortic valvoplasty at the time of VSD closure, the aortic valve was replaced 7 months later. No other patient had worrisome progression of their AI or PI. CONCLUSIONS: The incidence of aortic valvar prolapse and AI in the setting of doubly committed juxtaarterial VSD is quite high. The optimal surgical approach is through the pulmonary trunk. Sutures placed through the base of the pulmonary valvar leaflets do not predispose to clinically significant late pulmonary valvar insufficiency. Timely surgical closure of this type of defect prevents progression of AI.