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We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages.First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53%), shunt in 173 (33%), and band in 73 (14%). Outcomes were compared between those with GS/EC anomalies (121 [23%]) and without GS/EC anomalies (409 [77%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation).GS/EC anomalies varied among SV defects (range, 3% for double-inlet left ventricle to 100% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13% vs 11%, p = 0.552) and unplanned reoperation (16% vs 11%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24% vs 12%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60% vs 77%, p = 0.002) and lower 10-year survival (56% vs 76%, p0.001). After adjustment for other risk factors, GS/EC anomalies significantly affected survival in almost all subgroups of patients.The presence of GS/EC anomalies varies among SV anomalies and is associated with additional risk factors such as prematurity and low weight. After adjusting for other risk factors, GS/EC anomalies are associated with prolonged recovery after first-stage palliation and increased hospital and interstage death, with subsequently fewer patients progressing toward Glenn shunt. The increased death risk in those patients is highest in the first 6 months and persists for 2 to 3 years after first-stage palliation, suggesting the need for more vigilant monitoring and outpatient care in these high-risk patients.

Aortic arch hypoplasia is commonly present in neonates born with ductal-dependent coarctation of the aorta. The ideal surgical repair of neonates with proximal arch hypoplasia continues to be debated. Controversy exists about the fate of the hypoplastic proximal aortic arch following surgical repair and whether that will eventually grow to normal size upon relief of the distal obstruction or will persist as a residual lesion that can affect the long-term outlook of those patients. There is new evidence that residual proximal arch hypoplasia and the shape of the reconstructed arch both have an important impact on vascular remodeling and on the subsequent development of hypertension. Those concerns about late outcomes despite what was originally deemed a successful repair in infancy, coupled with improved cardiopulmonary bypass and cerebral perfusion techniques that allow surgeons to address proximal arch hypoplasia with low morbidity, have rekindled the debate on how to address proximal arch hypoplasia, with the aim to offer a neonatal surgery that would last for a lifetime and provide both optimal early recovery and late freedom from hypertension and related complications.

Objectives Primary pulmonary vein stenosis (PPVS) is increasingly diagnosed in children with no prior pulmonary vein intervention history, and management is challenging. We describe characteristics of patients who underwent surgical repair of PPVS at our center, and examine factors associated with treatment failures. Methods A retrospective review of all patients who underwent surgical intervention for PPVS (2002-2016) was completed. Patients who had undergone prior cardiac surgery involving the pulmonary veins or atrial switch were excluded. Regression analyses were performed to examine characteristics, PPVS features, including severity score, and surgical details associated with treatment failures. Results Thirty-four children underwent initial surgical intervention for PPVS. Median age was 8.9 months (interquartile range, 5.9-18.4 months). Most patients (n = 31; 91%) had unilateral pulmonary vein involvement and the median PPVS severity score was 3.5 (interquartile range, 3-5). On competing risk analysis, 1 year following surgical repair, 9% of patients had died, 14% had undergone reintervention, and 77% were alive without reintervention; at 5 years the numbers were 9%, 30%, and 61%, respectively. Factors associated with mortality included bilateral disease and PPVS severity score >6. Bilateral disease and PPVS severity score >5 were associated with reintervention risk. Conclusions Multidisciplinary management strategy is required for PPVS. Despite satisfactory early repair, patients continue to be at risk for recurrence and subsequent mortality, especially those with extensive primary involvement. The disappointing results underscore the need for multi-institutional collaborations to better understand this complex disease, establish management and follow-up protocols, and explore investigational treatment modalities that could modify the unfavorable outcome of this uncommon and challenging disease.

The Adult Congenital Heart Association (ACHA) is dedicated to supporting patients with congenital heart disease. To guide patients to qualified providers and programs, it maintains a publicly accessible directory of dedicated adult congenital cardiac programs. We analyzed the directory in 2006 and 2015, aiming to evaluate the growth of the directory as a whole and to evaluate the growth of individual programs within the directory. We also hope this raises awareness of the growing opportunities that exist in adult congenital cardiology and cardiac surgery.Data in the directory are self-reported. Only data from US programs were collected and analyzed.By the end of 2015, compared to 2006, there were more programs reporting to the directory in more states (107 programs across 42 states vs 57 programs across 33 states), with higher overall clinical volume (591 vs 164 half-day clinics per week, 96,611 vs 34,446 patient visits). On average, each program was busier (5 vs 2 half-day clinics per week per program). Over the time period, the number of reported annual operations performed nearly doubled (4,346 operations by 210 surgeons vs 2,461 operations by 125 surgeons). Access to ancillary services including specific clinical diagnostic and therapeutic services also expanded.Between 2006 and 2015, the clinical directory and the individual programs have grown. Current directory data may provide benchmarks for staffing and services for newly emerging and existing programs. Verifying the accuracy of the information and inclusion of all programs will be important in the future.

BackgroundAlthough much is known about morbidity and mortality, there are limited data focussing on the financial aspect of the Norwood operation. Our objective is to characterise the hospitalisation and detail the hospital costs.MethodsWe retrospectively studied 86 newborns with hypoplastic left heart syndrome who underwent Norwood palliation between 2008 and 2012. Clinical and financial data were collected. Financial data have been reported for 2011–2012.ResultsAt surgery, median age and weight of the patients were 4 days (range 1–13) and 3 kg (range 2–4.8), respectively. The median time from admission to surgery was 4 days (range 1–10), with the postoperative ICU stay and total length of stay at the hospital being 10 days (range 4–135) and 16 days (range 5–136), respectively. Discharge mortality was 14/86 (16%) patients.For patients operated on between 2011 and 2012 (n=40), median hospital costs, charges, and collections per patient were $117,021, $433,054, and $198,453, respectively, and mean hospital costs, charges, and collections per patient were $322,765, $1,109,500, and $511,271, respectively.A breakdown of total hospital costs (direct and indirect) by department showed that the top four areas of resource utilisation (excluding physician fees) were as follows: the cardiac ICU (35%), laboratory (12%), pharmacy (12%), and operating room (7%). Interestingly, point-of-care laboratory evaluations accounted for almost half of the laboratory total (5%). Extracorporeal membrane oxygenation, although only utilised in eight patients between 2011 and 2012, accounted for 7% of utilisation.General radiology only accounted for 2%, despite numerous radiographs.ConclusionsLimited data are available that detail the hospitalisation and costs associated with the Norwood operation. We hope that this analysis will identify areas for quality and value improvement from both system and patient perspectives.

Background: We have previously identified risk factors for readmission following congenital heart surgery – Hispanic ethnicity, failure to thrive, and original hospital stay more than 10 days. As part of a quality initiative, changes were made to the discharge process in hopes of reducing the impact. All discharges were carried out with an interpreter, medications were delivered to the hospital before discharge, and phone calls were made to families within 72 hours following discharge. We hypothesised that these changes would decrease readmissions.Methods: The current cohort of 635 patients underwent surgery in 2012. Demographic, preoperative, operative, and postoperative variables were evaluated. Univariate and multivariate risk factor analyses were performed. Comparisons were made between the initial (2009) and the current (2012) cohorts.Results: There were 86 readmissions of 77 patients during 2012. Multivariate risk factors for readmission were risk adjustment for congenital heart surgery score and initial hospital stay >10 days. In comparing 2009 with 2012, the overall readmission rate was similar (10 versus 12%, p=0.27). Although there were slight decreases in the 2012 readmissions for those patients with Hispanic ethnicity (18 versus 16%, p=0.79), failure to thrive (23 versus 17%, p=0.49), and initial hospital stay >10 days (22 versus 20%, p=0.63), they were not statistically significant.Conclusions: Potential risk factors for readmission following paediatric cardiothoracic surgery have been identified. Although targeted modifications in discharge processes can be made, they may not reduce readmissions. Efforts should continue to identify modifiable factors that can reduce the negative impact of hospital readmissions.

Surgical correction of total anomalous pulmonary venous connection is associated with an important risk of postrepair pulmonary vein obstruction. Primary sutureless anastomosis at the time of initial surgical repair might mitigate that risk. A simplified primary sutureless technique is described in this report.

Taussig-Bing is a form of double outlet right ventricle that is characterized by the presence of subpulmonary ventricular septal defect, double conus and side by side great arteries. Frequently, associated anomalies such as aortic coarctation, arch hypoplasia, subaortic obstruction, and atypical coronary artery anatomy are present; all increasing the complexity of the repair. Advances in perioperative care, perfusion and surgical techniques have allowed the performance of complete repair of Taussig-Bing anomaly with excellent results. Consequently, single stage repair has become the preferred treatment method in most pediatric cardiac centers. Reoperation for right ventricular outflow tract obstruction remains the most common late complication however aggressive resection of the right ventricular muscle bundles creating subaortic obstruction at time of initial repair and reconstruction of the neo pulmonary artery with a generous patch have mitigated that reoperation risk.

ABSTRACT: Several aortic valve (AV) pathologies might necessitate intervention. Percutaneous or surgical AV repair is generally recommended as the initial management strategy in children with AV disease, offering the advantage of stabilization of the heart dimensions and improvement of patients‘ symptoms. When AV repair is not possible or fails, AV replacement is necessary and is associated with several challenges in children. This review will focus on treatment strategy, AV repair techniques, AV replacement choices and outcomes of AV disease management in children.

Multi-stage palliation is the current management strategy for the treatment of children with various single ventricle (SV) cardiac malformations. The success of this strategy depends on the presence of favorable anatomic and hemodynamic criteria. Several SV anomalies have the potential of developing systemic ventricular outflow tract obstruction (SVOTO) that might be evident early on or progress later after palliative surgeries. SVOTO could result in ventricular hypertrophy, impaired diastolic function and subendocardial ischemia with subsequent deleterious effects on the SV and disturbance of some of those criteria for a successful multi-stage palliation strategy. Careful identification of SV patients at risk of developing SVOTO and proper planning of the optimal palliation sequence beginning at the 1st stage procedure are vital factors that would affect long-term outcomes in those patients. In the current review, we describe the morphology of SV patients with potential SVOTO risk, surgical procedures that address potential or present SVOTO, and optimal timing of those procedures within the multi-stage palliation chain. We attempt to provide a treatment algorithm for various patients taking into consideration their unique anatomic and physiologic characteristics.

Aortic root replacement (ARR) is advocated for irreparable autograft failure after the Ross procedure to avoid late aneurysm formation. However, redo ARR is complex and associated with bleeding and coronary injury risks. We examine results of autograft valve replacement (AuVR) with stented prostheses (SP) without ARR with special focus on the fate of the remaining root and need for reintervention.Between 1994 and 2011, 50 of 510 Ross patients underwent AuVR with SP. Serial postoperative echocardiograms (n = 342) were analyzed and regression models adjusted for repeated measures were used to model longitudinal change of the remaining root and ascending aorta dimensions after AuVR.Fifty patients, median age 21 years (range 11 to 50 years) underwent AuVR with SP: mechanical (n = 38) or tissue (n = 12). Thirty patients (60%) had concomitant procedures; most commonly mitral valve surgery (n = 20) or conduit change (n = 12). There were no operative deaths and 10-year survival was 95%. Freedom from prosthesis, root, and all-cause reoperations was 97%, 98%, and 90% at 10 years, respectively. Serial echocardiography data showed that there was little but, nevertheless, progressive increase of the remaining root (EST: +0.0190 [0.0041] cm/year, p0.001) and ascending aorta diameters (EST: +0.0191 [0.0037] cm/year, p0.001). While there was small steady non-statistically significant increase in mean prosthesis gradient (estimate [EST]: +0.16 [0.09] mm Hg/year, p = 0.08); ejection fraction remained stable with time (EST: -0.12 [0.14] %/year, p = 0.41).Our results indicate that AuVR with SP without ARR for failing autografts is justified as it is associated with low mortality and reoperation risk. Preemptive complex ARR should be reserved for those with significant root dilatation at time of AuVR. Although root reinterventions are rare, patients should be followed for progressive root dilatation. Faster growth is seen in those who fail with regurgitation and dilated annulus.

OBJECTIVES: Survival of children having cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) is very poor. We sought to examine current era outcomes of extracorporeal CPR (ECPR) support for refractory arrest following surgical correction of congenital heart disease. METHODS: Demographic, anatomical, clinical, surgical and support details of children requiring postoperative ECPR (2007–12) were included in multivariable logistic regression models to determine the factors associated with survival. RESULTS: Thirty-nine children, median age 44 days (4 days–10 years), required postoperative ECPR at a median interval of 1 day (up to 15 days) after surgery. Thirteen (33%) children had single-ventricle pathology; Risk Adjustment in Congenital Heart Surgery (RACHS)-1 categories were 2, 3, 4 and 6 in 6, 15, 13 and 5 patients, respectively. Median CPR duration was 34 (8–125) min, while median support duration was 4 (1–17) days. Seven (18%) patients underwent cardiac re-operation, 28 (72%) survived >24 h after support discontinuation and 16 (41%) survived. Survival rates in neonates, infants and older children were 53, 39 and 17% (P=0.13). Survival rates for single- vs two-ventricle pathology patients were 54 and 35%, (P=0.25) and 50, 47, 23 and 60% in RACHS-1 2, 3, 4 and 6 patients, respectively (P=0.37). Survivors had shorter CPR duration (25 vs 34 min, P=0.05), lower pre-arrest lactate (2.6 vs 4.6 mmol/l, P=0.05) and postextracorporeal membrane oxygenation (ECMO) peak lactate (15.4 vs 20.0 mmol/l, P

Objectives To assess left ventricular myocardial mechanics in a patient with acute severe aortic regurgitation. Methods Left ventricular strain was derived from two-dimensional echocardiographic data. Results There is marked decrease in longitudinal and radial strain at the left ventricular base with mild reduction at the apex. Circumferential strain and rotation were mildly reduced basally and preserved apically with normal torsion. Conclusions Our findings reflect elevated wall stress, mostly at the left ventricular base, and at the longitudinal, predominantly subendocardial fibers. The apex and the circumferential midwall fibers where wall stress is lower are less affected. To our knowledge, this is the first description of left ventricular mechanics by strain imaging in acute aortic regurgitation.

BackgroundPatients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.MethodsWe reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.ResultsA total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).ConclusionThe surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

The Emory Adult Congenital Heart (Emory University, Atlanta, GA) program was founded in 2001. In 2004, the surgical component transitioned from a pediatric facility to an adult facility. The aim of this article is characterize the program as a whole, outline changes in the program, and discuss the challenges of the transition process. Between 2001 and 2015, changes in program structure and personnel were evaluated. There has been significant growth of the program between 2001 and 2015. There are currently 19 half-day clinics per week, with 2,700 clinic visits per year. There are six cardiologists, three congenital cardiac surgeons, two sonographers, one advanced practice provider, and one social worker dedicated to the program. There are Accreditation Council for Graduate Medical Education-accredited adult congenital cardiology and congenital cardiac surgery fellowships. One thousand forty-four operations were performed between 2001 and 2015. There were 828 open-heart operations, of which 581 (70%) were re-operations. Over the study period, the number of yearly operations increased from 30 to 119, and the mean age at surgery increased from 22 to 35 years. Over time, more of the operations were performed at the adult hospital: increasing from 3% in 2001 to 82% in 2015, and more of the operations were performed by congenital cardiac surgeons: 87% (114 of 131) before the 2004 transition to 97% (881 of 913) afterward. The Emory Adult Congenital Heart program has undergone significant growth and change, including transition of the surgical component from the pediatric to the adult facility. While numerous obstacles have been overcome and great progress has been made, additional challenges remain.

Background Differences in right ventricle and tricuspid valve structure compared with left ventricle and mitral valve structure make them less equipped to support the systemic circulation long term, with subsequent systemic right ventricle failure. We examined the effect of dominant ventricle morphology on single ventricle palliation outcomes. Methods We grouped 530 neonates who underwent first-stage palliation into two groups based on dominant ventricle morphology: right dominant ventricle (RV group; n = 302, 57%) and left dominant ventricle or functional single ventricle with two well-formed ventricles (LV group; n = 228, 43%). Comparisons of hospital outcomes, interstage mortality, progression to subsequent palliation stages, and late survival was performed, and factors affecting outcomes were examined. Results After first-stage palliation, the RV group and LV group, respectively, had comparable extracorporeal membrane oxygenation requirements (12% versus 11%, p = 0.648), unplanned reoperation (12% versus 13%, p = 0.586), and hospital death (16% versus 13%, p = 0.437). Among hospital survivors, interstage mortality (11% versus 9%, p = 0.509) and progression to Glenn operation (89% versus 84%, p = 0.182) were comparable; however, death after Glenn was higher in the RV group (10%, versus LV group 4%, p = 0.020) with a trend for lower 8-year survival (66% versus 73%, p = 0.081). On multivariable analysis, dominant RV was not associated with mortality (hazard ratio 0.75, 95% confidence interval: 0.6 to 1.0, p = 0.081), whereas factors such as genetic syndromes, weight 2.5 kg or less, underlying cardiac anomaly, and first-stage palliation type affected survival. Conclusions At midterm follow-up, underlying cardiac anomaly and patient characteristics affect single ventricle palliation outcomes more than dominant ventricular morphology. As right ventricle and associated tricuspid valve failure might occur at late stages, the impact of dominant ventricular morphology on long-term outcomes requires further assessment.

BackgroundPost-cardiotomy shock (PCS) has a complex etiology. Although treatment with inotrops and intra-aortic balloon pump (IABP) support improves cardiac performance, end-organ injuries are common and lead to prolonged ICU stay, extended hospitalization and increased mortality. Early consideration of mechanical circulatory support may prevent such complications and improve outcome.MethodsBetween January 1997 and January 2002, 321 patients required IABP and inotropic support for PCS following coronary artery bypass grafting (CABG) at our institution. Perioperative variables including age, mixed venous saturation (MVO2), inotropic requirements and LV function were analyzed using multivariate statistical methods. All explanatory variables with a univariate p value 0.5μg/kg/min (OR=3.2), age>75 (OR=2.7), adrenaline>0.1μg/kg/min (OR=1.5). A 15-point risk score was developed based on the regression model. Hospital mortality in patients with a score >6 was 46% (n=13/28), 3–6 was 31% (n=9/29) and

Objectives The Glenn bidirectional cavopulmonary connection (BCPC) is an established procedure in multistage palliation of various single ventricle (SV) anomalies. We aimed to report the current outcomes following BCPC and to examine risk factors affecting survival and progression to the next palliation stage. Methods Two hundred and twenty-seven consecutive children with variable SV pathologies underwent BCPC from 2002 to 2007. Competing risk analyses were performed to model events after BCPC (death and transition to Fontan) and subsequently after Fontan (death and cardiac reoperation) and to examine the associated risk factors for poor outcomes. Results There were 139 males (61%) with a median age of 7.6 months [interquartile range (IQR) 6.0-10.8] and median weight of 6.2 kg (IQR 5.2-7.4). Forty-three patients (19%) had primary BCPC and 184 (81%) had prior palliation: aortopulmonary shunt (APS) (n=83), Norwood (n=55), pulmonary artery (PA) band (n=48), atrial septectomy (n=25), PA reconstruction (n=14), anomalous pulmonary venous connection repair (n=7) and other (n=8). Predominant ventricle was left morphology (n=122, 54%), right morphology (n=95, 42%) and two equally developed ventricles (n=10, 4%). Twenty-six patients (12%) had bilateral superior vena cava. Concomitant surgery included atrioventricular valve repair (n=18), PA augmentation (n=80), percutaneous Fontan preparation (n=34) and other (n=24). Competing risk analysis showed that 5 years following BCPC, ∼17% have died, 76% have undergone Fontan and 7% were alive awaiting or not qualifying for Fontan. On multivariable analysis, risk factors for death prior to Fontan were pulmonary vascular resistance (PVR) index of >3 WU/M2 [hazard ratio (HR) 3.9, P=0.001], dominant right ventricle (HR 2.1, P=0.03) and prior palliation other than APS (HR 0.4, P=0.03). Competing risk analysis showed that 3 years following 172 Fontan operations, ∼10% have died, 6% have undergone further cardiac surgery and 84% were alive and free from reoperation. Overall, 8-year survival following BCPC was only 74%. Conclusions Despite established selection criteria and improved surgical technique and medical management, there is a continuous failure and attrition risk following BCPC. Outcomes are influenced by underlying cardiac anomaly; patients with dominant left ventricle (i.e. tricuspid atresia, double inlet left ventricle) having the best survival while those with dominant right ventricle (i.e. hypoplastic left heart syndrome, double outlet right ventricle with heterotaxy) having the worst survival. Increased PVR remains a significant factor affecting mortality.

Background: Ross procedure is the aortic valve replacement of choice in children. Nonetheless, late autograft reoperation for dilatation and/or regurgitation is concerning. We examine whether Ross procedure is suitable in children with rheumatic fever. Methods: Medical records of 104 children with rheumatic fever who underwent Ross procedure were reviewed (1991-2004). Competing risks methodology determined time-related prevalence and associated factors for two mutually exclusive end states: autograft reoperation and death prior to subsequent reoperation. Results: Mean age was 13.8 ± 2.7, 83 (80%) were males. Hemodynamic dysfunction was primarily regurgitation (n = 92, 88%) and stenosis/mixed (n = 12, 12%). Competing risks analysis showed that in ten years after the Ross procedure, 1% of patients died, 32% underwent autograft reoperation, and 67% were alive and free from reoperation. Ten-year freedom from aortic regurgitation greater than or equal to moderate was 63%. Ten-year freedom from autograft reoperation was 65% for regurgitation versus 90% for stenosis/mixed disease. Risk factors for autograft reoperation were earlier surgery year (PE: 0.26 ± 0.06 per year; P < .001), additional surgery (PE: 0.82 ± 0.39, P = .04), no annular stabilization (PE: 1.21 ± 0.61, P = .05). Ten-year freedom from homograft replacement was 83%. Risk factors were fresh homografts (PE: 1.36 ± 0.71; P = .06) and aortic homografts (PE: 1.15 ± 0.59; P = .05). Ten-year freedom from any cardiac reoperation was 53%. Concomitant cardiac surgery was risk factor (PE: 1.37 ± 0.47; P = .004). Conclusions: Ross procedure in children with rheumatic fever is associated with excellent survival but results are plagued by aortic regurgitation and frequent autograft reoperation. Risk factors include preoperative regurgitation, concomitant surgery, dilated annulus, and earlier surgery era. Better patient selection in later era has mitigated autograft reoperation risk. Continued, improved candidate selection, along with modifications in autograft implantation and root/sinotubular stabilization techniques, may further decrease late autograft failure.

Atrioventricular block, but not tachyarrhythmia, is associated with increased mortality after the Norwood procedure.

Background We report early results of surgical preparation and subsequent percutaneous Fontan completion strategy for the treatment of single-ventricle defects. Methods Two hundred twenty-seven patients underwent bidirectional cavopulmonary connection (BDCPC) between 2002 and 2007. Thirty-four patients had lateral tunnel created at time of BDCPC, fenestrated with 10 to 14 mm openings with the cardiac superior vena cava end patched to maintain BDCPC physiology. At second stage, Fontan circulation was established by superior vena cava patch perforation, tunnel dilatation, and stenting plus fenestration device closure. Results Thirty-four patients underwent Fontan preparation with BDCPC. Median age was 7.7 months (5 to 51) and 29 patients (85%) had previous palliation. Mean bypass and ischemic times were 141 and 72 minutes, respectively. Median ventilation, intensive care, and hospital stay durations were 1, 5, and 10 days, respectively. There was one early death and two take-downs. Twenty-eight patients underwent Fontan procedure: surgical (n = 3), percutaneous (n = 25). None of the patients who underwent percutaneous Fontan completion required inotropes, chest tube insertion, or mechanical ventilation. Median intensive care and hospital stay durations were 1 and 6 days, respectively. There were no early mortalities after percutaneous Fontan but one late death and one surgical revision. Overall survival after BDCPC with Fontan preparation was 77%. Conclusions Despite longer bypass and ischemic times, Fontan preparation at time of BDCPC is feasible and associated with encouraging early outcomes. Percutaneous Fontan completion is associated with short recovery, low morbidity and excellent early dynamics, and echocardiographic and clinical outcomes. Further follow-up is needed to confirm those favorable results.

Objectives: The Ross procedure is the aortic valve-replacement procedure of choice in children. Nonetheless, late autograft re-operation for dilatation and/or valve regurgitation is of concern. We examined whether preoperative haemodynamic manifestation (e.g., stenosis, regurgitation and mixed aortic valve disease) affected late re-operation risk. Methods: Medical records of 227 children who underwent the Ross procedure (1991—2004) were reviewed. Competing-risks methodology determined time-related prevalence and associated factors for two mutually exclusive end-states after the Ross procedure: (1) death prior to subsequent autograft re-operation and (2) autograft re-operation, with the remainder of patients being alive and free from subsequent autograft re-operation.Results:There were 162 male patients (71%) in this study. Median age at surgery was 12.1 years (range: 1 week—18 years). The haemodynamic aortic valve dysfunction was primarily stenosis (n = 40, 18%), primarily regurgitation (n = 109, 48%) and mixed disease (n = 78, 35%). Underlying pathology was rheumatic fever (n = 104, 46%), congenital heart disease (n = 113, 50%) and endocarditis (n = 8, 3%). Competing-risks analysis showed that, at 10 years following the Ross procedure, 5% of patients had died, 16% had undergone autograft re-operation with aortic valve replacement and 79% were alive and free from autograft reoperation. Ten-year freedom from autograft re-operation for patients with preoperative stenosis, regurgitation and mixed disease was 97%, 69% and 93%, respectively, (p < 0.001 for regurgitation vs others). Risk factors for increased risk of autograft re-operation were rheumatic fever (parameter estimates (PEs): 2.09 0.75, p = 0.006), and earlier year of surgery (PE: 0.20 0.06, p = 0.001). Ten-year freedom from homograft replacement was 81% and was not dependent on haemodynamic manifestation (PE: 0.16 0.38, p = 0.68). Significant factors for homograft replacement included fresh homografts (PE: 2.2 0.63, p = 0.01) and annular enlargement (PE: 1.11 0.3, p = 0.01). Ten-year freedom from cardiac re-operation other than auto-/homograft was 85%, higher in patients with preoperative aortic regurgitation (PE: 1.01 0.42, p = 0.02). Concomitant cardiac surgery was a significant factor for late cardiac re-operation other than auto-/homograft replacement (PE: 1.79 0.39, p < 0.001). Conclusions: The Ross procedure in children is associated with excellent survival. Late autograft re-operation may be required; however, it is more common in children with preoperative aortic regurgitation, especially those with rheumatic fever. Better patient selection in later era has mitigated the risk of autograft re-operation. Continued improved candidate selection, along with modifications in autograft implantation and root/sinotubular stabilisation techniques, may further decrease late autograft failure. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objectives: Although the majority of infants with severe left ventricular outflow tract obstruction (LVOTO) can be managed with balloon or surgical aortic valvotomy, a more complex biventricular repair may be required in a subset of infants with multi-level obstruction, failure of or complication to prior intervention. In the presence of normal left ventricle size and inflow, the Ross procedure is applied in patients with/without ventricular septal defect (VSD), while the Yasui procedure is applied only in those with VSD. We report mid-term outcomes in a single institution. Methods: Thirty-five consecutive infants with severe LVOTO underwent complex biventricular repair using the Ross (n = 21) or Yasui (n = 14) procedure. Outcomes were studied using univariate and multivariable parametric models. Results:The Ross procedure was done at a median age of 88 days (8—353days), in 8/21(38%) neonates.As manyas 12/21 (57%) hadprior catheterand/orsurgicalintervention. Concomitant procedures included arch reconstruction (n = 4/21, 19%) and mitral valve repair (n = 6/21, 29%). In addition, 14/21 (67%) had annular enlargement (modified Ross—Konno). Haemodynamic manifestation was isolated obstruction (n = 10/21, 48%) or mixed obstruction/regurgitation (n = 11/21, 52%). Survival was 81% at 1 month, 70% at 1 year and 63% at 5 years. In multivariable regression models, factors associated with increased risk of mortality included neonatal surgery (p = 0.007), mitral valve repair (p = 0.02), longer cross-clamp time (p = 0.003), and postoperative extracorporeal membrane oxygenator (ECMO) (p = 0.004). Freedom from any cardiac re-operation was 86% at 5 years. The Yasui procedure was done at a median age of 36 days (7—207 days), 6/14 (43%) in neonates. As many as 13/14 procedures (93%) were primary and one procedure followed a prior single-stage Norwood palliation. A total of 10/14 (71%) had critical aortic stenosis and 4/14 (29%) had atresia. All patients had VSD, and 11/14 (79%) required concomitant arch reconstruction. Survival was 79% at 1 month and 5 years while 5-year freedom from re-operation was 57%. Conclusions: Complex biventricular repair can be performed in neonates and infants with severe LVOTO with mid-term survival that is equivalent to that published following balloon or surgical aortic valvotomy. Associated lesions are significant factors that influence outcome and proper patient selection may further improve survival. In neonates with concomitant arch obstruction and VSD, the Yasui operation may be associatedwithlowerearlymortalityrisk.Neonateswithconcomitantmitralvalvepathologymaybebetterservedwithsingleventriclepalliation strategy.

Objective:Theidealvalvesubstitutein childrendoesnot exist.Biologicandbioprosthetic valvesdo not requireanticoagulation, howevertheir use is complicated by accelerated degeneration and requirement for reoperation. We examine results following mitral (MVR) or aortic (AVR) replacement with biologic and bioprosthetic valves at our institution. Methods:Medical records of children who underwentAVRor MVR from 1986 to 2006 were reviewed. Median follow-up duration was 10.5 years. Competing-risks methodology determined time-related prevalence and associated factors for three mutually exclusive end states: death, valve reoperation, and survival without subsequent reoperation. Results: One hundred and ten children (age 15.6 2.6 years, 80% females) underwent 123 valve replacements with biologic and bioprosthetic substitutes including 87 MVR and 36 AVR (13 had both). Underlying pathology was mainly rheumatic fever (91%). Thirty-nine patients (35%) had undergone a previous cardiac surgery. Most common mitral substitute was Hancock (73%) and homograft (8%); most common aortic substitute was homograft (41%) and Carpentier—Edwards (39%). Competing-risks analysis showed that 15 years after valve replacement, 16% of patients had died without subsequent reoperation, 66% underwent valve reoperations, and only 18% remained alive without further reoperation. Factors associated with increased reoperation risk included younger age at surgery (p = 0.005), AVR (p = 0.005), male gender (p = 0.02) and homograft use (p = 0.007) especially in the mitral position (p = 0.002). Fifteen-year freedom from endocarditis was 97% while freedom from bleeding and thrombo-embolic complications was 100%. Majority of patients (95%) were in NYHA functional classes I/II at last follow-up. Conclusion: While valve reoperation is inevitable following AVR and MVR with biologic and bioprosthetic substitutes; favorable results such as low valve-related morbidity rate, good long-term survival and functional status encourage their consideration as valid replacement alternatives in selected children especially females. Valve durability is higher in the mitral position and longevity of bioprosthetic valves is greater than that of homografts especially in the mitral position.

Background The Rastelli procedure is the standard surgical treatment of d-transposition of great arteries (d-TGA), ventricular septal defect (VSD), and pulmonary stenosis. Late morbidity is significant due to recurrent left ventricular outflow obstruction (LVOTO), early conduit obstruction, and arrhythmias, with troublesome late mortality. To avoid recurrent LVOTO, we routinely enlarge the VSD and resect the infundibular septum before LV baffling to the aorta. We examined the efficacy of this approach in mitigating recurrent LVOTO risk. Methods Late echocardiographic and time-related clinical results of patients undergoing the Rastelli procedure were examined. Demographics and operative variables affecting outcomes were analyzed. Results The Rastelli cohort comprised 36 patients with d-TGA, VSD, and pulmonary stenosis. Median age at operation was 2.4 years (range, 0.3 to 8.3 years). Pulmonary stenosis was present in 31 and atresia in 5. Twenty-two patients had undergone a previous aortopulmonary shunt, and 6 had an atrial septectomy. No operative or late deaths occurred. Time-related freedom from permanent pacemaker implantation, recurrent LVOTO on echocardiogram, and conduit replacement at 10 years was 82%, 100%, and 49%, respectively. Systolic function was normal in all but 3 patients and 92% were in New York Heart Association functional class I and II. None of the patients had late arrhythmias or required heart transplantation. Conclusions Early and midterm survival after the Rastelli procedure is satisfactory. Aggressive resection of the infundibular septum to enlarge the VSD has mitigated the risk of LVOTO recurrence. Late conduit obstruction remains an important source of morbidity and frequently requires reintervention.

Objective: Application of extra-corporeal life support (ECLS) following pediatriccardiac surgery varies betweendifferent institutions based on manpower availability and philosophy towards ECLS utilization. We examined a large single institution experience with postoperative ECLS in children aiming to identify outcomepredictors.Methods:Hospital records of all children who required postoperative ECLS at our institution were reviewed. Patients’ demographics, cardiac anatomy, surgical and ECLS support details were entered into a multivariable regression analysis to determine factors associated with survival. Results: Between 1990 and 2007, 180 consecutive children, median age 109 days (range: 1 day—16.9 years), required postoperative ECLS. Sixty-nine children (38%) had undergone palliative treatment for single ventricle pathology. ECLS support was required for failure to separate from cardiopulmonary bypass (n = 83) or for postoperative low cardiac output state (n = 97). Forty-eight patients (27%) received rescue extra-corporeal membrane oxygenation (ECMO) support during active chest compression for refractory cardiac arrest. Under ECLS support, 37 patients required surgical revision and 20 received orthotopic heart transplantation. One hundred and nine patients (61%)survived >24 h following ECLSdiscontinuation and68 (38%)were dischargedalive.Hospitalsurvivors requiredshorter ECLSsupport duration compared to non-survivors (median 3 vs 5 days, respectively, p = 0.05) however survival occurred after up to 16 days of ECLS support. ECLS indication (OR: 0.85 for failure to separate from bypass vs postoperative low cardiac output 95% CI (0.47—1.56), p = 0.62) and rescue ECMO (OR: 0.63 for rescue ECMO vs not 95%CI (0.32—1.24), p = 0.18) were not associated with risk of mortality. In a multivariable logistic regression model, neurological complications (p = 0.0007), prolonged ECLS duration (p = 0.003), repeat ECLS requirement (p = 0.02), renal dysfunction (p = 0.04) and not performing heart transplantation (p = 0.04) were significant factors for hospital death. Conclusion: ECLS plays a valuable role in children with low cardiac output state following cardiac surgery. More than one third of those patients, including young neonates, older children, patients with single ventricle, or those requiring rescue ECMO can be salvaged. Although prognosis worsens with prolonged ECLS duration, survival can be noted up to 16 days of support. Heart transplantation is often an important ECLS exit strategy and should be considered early in selected children. Patients’ survival could improve if renal and neurological complications are avoided.

Optimal repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) relies on the creation of a dual-coronary system. If the anomalous coronary arises at a long distance from the aorta, we use various coronary extension techniques to facilitate tension-free implantation.Thirty patients underwent ALCAPA operations using direct coronary transfer (n = 11) or coronary extension techniques (n = 19). Surgical outcomes were analyzed.Median age and weight were 5.7 months (range, 46 days to 5.45 years) and 5.35 kg (range, 3.3 to 15.9 kg). Five patients had concomitant mitral annuloplasty. Mean cardiopulmonary bypass and ischemic times were 108 +/- 38 and 57 +/- 25 minutes. Two patients required intraoperative revision of the implantation. There were three hospital deaths (10%) and no late deaths. Follow-up echocardiograms demonstrated significant improvement postoperatively vs preoperatively in shortening fraction (35% +/- 2% vs 16% +/- 2%, p0.00001), ejection fraction (64% +/- 3% vs 32% +/- 4%, p0.00001), and mitral regurgitation (11% moderate vs 70% moderate or severe, p = 0.0002). Left ventricular end-diastolic dimension Z-score decreased from 9.1 +/- 0.9 to 1.2 +/- 0.5 (p0.00001). Both techniques were equally effective. Two patients underwent reoperation 1 and 12 years postoperatively (coronary artery bypass grafting, 1; mitral repair with coronary angioplasty, 1). Surviving patients remain asymptomatic (p0.00001).Dual-coronary system can be established in patients with ALCAPA. Coronary extension implantation techniques have acceptable operative mortality and excellent cardiac recovery and late survival. Although the rate of late coronary occlusion is low, continual ventricular or mitral dysfunction should trigger evaluation of persistent coronary compromise.

Objective: The arterial-switch operation (ASO) for management of Taussig—Bing anomaly is associated with important morbidity, mainly related to multiple associated cardiac anomalies. Our surgical management has evolved to suggest a single-stage total repair strategy tailored to address all abnormalities on an individual basis. We examine the efficacy of this treatment approach. Methods: Thirty-three children, (infants n = 29), with Taussig—Bing underwent ASO (1979—2005). In our earlier experience (group 1, n = 17), initial palliation was performed as needed, including pulmonary—artery banding (n = 9), coarctation repair (n = 6), and atrial septostomy (n = 3) followed by ASO at a later age. In our later experience (group 2, n = 16), single-stage total repair was performed; ASO with ventricular septal defect closure and baffling of left ventricle to neo-aorta performed in neonates with arch obstruction (n = 8), or at age 6 weeks in those with no arch obstruction (n = 8). Concomitant relief of right-ventricle outflow-tract obstruction (RVOTO) was performed in 14 patients. Demographics and operative variables affecting outcomes were analyzed. Results: Mean age at operation for group 1 and 2 patients was 312 477 and 42 31 days (p < 0.0001). Aortic arch obstruction (52%), sub-aorticRVOTO(61%)andunusualcoronarypatterns(52%)weresimilarforbothgroups.One-yearsurvivalforgroup1and2patientswas47 5% and 100% (p = 0.001). Associated anomalies such as great vessels position, arch obstruction, and unusual coronaries were not significant risk factors for mortalityon multivariable analysis.Ten-yearfreedomfrom RVOTand arch re-operationwas 55 5% and 96 4%. Five-yearevent-free survival for groups 1 and 2 was 35 6% and 87 1% (p = 0.0016). Significant factors affecting event-free survival were group 1 (HR 108, p = 0.0005), and larger weight at surgery (HR 1.3, p = 0.02). Conclusions: The Taussig—Bing anomaly is complex and often associated with other cardiac anomalies (arch obstruction, RVOTO, unusual coronary pattern). Advances in perioperative care have significantly mitigated mortality. In our experience with single-stage total repair, event-free survival, especially freedom from RVOT re-operation, has significantly improved. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Background We reviewed surgical results after treatment of aortic coarctation (CoA) associated with ventricular septal defect (VSD) in neonates. We examined morbidity associated with the two different therapeutic strategies of combined repair versus initial coarctation repair alone and attempted to identify preoperative predictors to guide optimal surgical management. Methods Between 1990 and 2006, 141 neonates with CoA and VSDs underwent operation using two management strategies. In group A (n = 89), initial simple CoA repair was done through posterolateral thoracotomy, plus concomitant pulmonary artery banding (n = 54), followed by VSD closure. In group B (n = 52), both defects were repaired simultaneously through a sternotomy. Results Overall 10-year survival was 90.8%, with no difference between groups. The 5-year freedom from arch reoperation was 93.5%, with no difference between groups. The 10-year freedom from reoperation for subaortic obstruction was 95% for group A and 75% for group B (p = 0.016). In group A, 41 patients required secondary VSD closure at a median interval of 48 days after CoA repair. Freedom from reoperation at 1 month and 5 years was 78.5% and 45.8% in group A versus 97.8% for both in group B. Preoperative predictors for requirement for later VSD closure in group A were VSD type other than muscular (p = 0.0009) and larger VSD identified by higher VSD diameter/aortic valve annulus ratio (p Conclusions Results of both treatment strategies are good. Neonates with larger VSDs, especially outlet, malalignment, and perimembranous types, are likely to require VSD closure. Although midline sternotomy and combined treatment strategy may be necessary in neonates with proximal arch hypoplasia, initial coarctation repair alone is valid option at the possible expense of additional operation.

Despite being one of the most common congenital cardiac abnormalities, the treatment of critical left ventricular outflow tract obstruction (LVOTO) in the neonate and infant remains a significant challenge. Critical LVOTO in the neonate and infant includes continuous and diverse spectrum of anatomic diagnoses ranging from hypoplastic left heart complex to isolated aortic valvular stenosis with otherwise normally formed left heart structures. Depending on related anatomic features, there are multiple single ventricle and biventricular management strategies available for patients with critical LVOTO; all of which carry significant risk of death and requirement for future interventions. Because of the wide array of morphologic lesions and physiologic manifestations, application of the currently available published data to guide decision making in the individual patients can be challenging. Therefore, the aim of this review is to describe the different therapeutic strategies that can be applied to neonates presenting with critical LVOTO taking into consideration each patient's unique physiologic and anatomic findings.

Significant noncardiac and genetic abnormalities (NC and GA) are common in neonates with congenital heart defects. We sought to examine current-era effect of those abnormalities on early and late outcomes following cardiac surgery. The method from 2002-2012, 1538 neonates underwent repair (n = 860, 56%) or palliation (n = 678, 44%) of congenital heart defects. Regression models examined the effect of NC and GA on operative results, resource utilization, and late outcomes. Neonates with NC and GA (n = 312, 20%) had higher incidence of prematurity (21% vs 13%; P0.001) and weight ≤2.5kg (24% vs 12%; P0.001) than neonates without NC and GA (n = 1226, 80%). Although the incidence of single ventricle was comparable (34% vs 31%; P = 0.37), neonates with NC and GA underwent more palliation (52% vs 42%; P = 0.001) and subsequently had higher percentage of STAT mortality categories (Society of Thoracic Surgeons (STS) and the European Association for Cardio-thoracic Surgery (EACTS) Congenital Heart Surgery Mortality Categories) 4 and 5 procedures (78% vs 66%; P0.001). Adjusted logistic regression models that included disparate patient and operative variables showed that the presence of NC and GA was associated with increased unplanned reoperation (odds ratio = 1.7; 95% CI: 1.1-2.7; P = 0.03) and hospital mortality (odds ratio = 2.2; 95% CI: 1.3-3.6; P = 0.002). Adjusted linear regression models showed significant association between NC and GA and increased postoperative mechanical ventilation duration, intensive care unit, and hospital stays (P0.001 each). Adjusted hazard analysis showed that the presence of NC and GA was associated with diminished late survival (hazard ratio = 2.4; 95% CI: 1.9-3.1; P0.001) and that was evident in all subgroups of patients (P0.001 each). Conclusion is neonates with NC and GA commonly have associated risk factors for morbidity and mortality such as prematurity and low weight. After adjusting for those factors, the presence of NC and GA continues to have significant association with increased unplanned reoperation, hospital mortality, and resource utilization after palliative and corrective cardiac surgery. Importantly, the hazard of death in those patients continues beyond the perioperative period for at least 1 year. Our findings show that the presence of NC and GA should be emphasized during parent counseling and decision making; and underscore the need to explore strategies to improve outcomes for this high-risk population that must address perioperative care, outpatient surveillance, and management.

Background Management of infants with heterotaxy syndrome and functional single ventricle is complicated due to associated cardiac and extracardiac anomalies. We report current era palliation results. Methods Between 2002 and 2012, 67 infants with heterotaxy syndrome underwent multistage palliation. Competing risks analyses modeled events after surgery (death vs Glenn procedure) and examined factors associated with survival. In addition, early and late outcomes following first-stage palliation surgery were compared with a matched contemporaneous control group of patients with nonheterotaxy single ventricle anomalies. Results Fifty-eight patients (87%) required neonatal palliation, including a modified Blalock-Taussig shunt (n = 34; 51%), Norwood operation (n = 12; 18%) or pulmonary artery band (n = 12; 18%), whereas 9 patients (13%) underwent a primary Glenn procedure. Competing risks analysis showed that at 1 year after first-stage palliation surgery, 29% of the patients had died or undergone transplantation and 63% had undergone a Glenn procedure. By 5 years after the Glenn procedure, 64% of patients had undergone a Fontan procedure. The overall 8-year survival rate was 66%. On multivariable analysis, factors associated with mortality were unplanned reoperation (hazard ratio [HR], 2.9; 95% confidence interval [CI], 1.1-7.3; P = .005) and total anomalous pulmonary venous connection repair (HR, 2.3; 95% CI, 1.0-5.6; P = .056). Comparison with the contemporaneous matched patients with nonheterotaxy single ventricle anomalies showed that first-stage palliation in the patients with heterotaxy was associated with a higher rate of in-hospital death (27% vs 10%; P = .022), and significantly longer durations of ventilation and intensive care unit stay. Interstage mortality, survival after the Glenn procedure, and progression to the Fontan procedure were comparable in the 2 groups. Conclusions The management of infants with heterotaxy and a functional single ventricle remains challenging. First-stage palliation is associated with high operative mortality and increased resource utilization owing to surgical morbidity. Nonetheless, outcomes beyond hospital discharge are comparable to those for patients with other single ventricle anomalies. Efforts to improve survival in those patients should focus on perioperative care.

Management of a patent systemic-pulmonary (SP) shunt and the resulting runoff during extracorporeal membrane oxygenation (ECMO) varies among institutions. We have used a strategy of increased flow without surgical reduction of the shunt diameter, and here report our results with this strategy.In this database review of 169 successive veno-arterial ECMO runs performed between 2002 and 2013 in infants and neonates, ECMO flow, time to achieve lactate clearance, normal pH, and negative fluid balance were compared in patients with shunts and those without shunts.Fifty-one of 169 infants (30.2%) had a shunt in situ when ECMO was initiated. Significantly higher ECMO flows were maintained in the shunt group compared with the nonshunt group (161 ± 43 mL/kg/minute vs 134 ± 41 mL/kg/minute; P .001). Infants with shunts had significantly higher pre-ECMO and peak lactate levels (12.4 ± 5.6 mmol/L vs 10.0 ± 6.3 mmol/L; P .05 and 13.7 ± 4.9 mmol/L vs 11.6 ± 5.5 mmol/L; P .02, respectively) and required a longer period of support for clearance (median, 28.8 hours [16.1-63.3 hours] vs 17.5 hours [10.8-34.5 hours]; P .001). Although the absolute rate of lactate clearance was not significantly different between the 2 groups (median, 0.46 mmol/L/hour [0.12-0.72 mmol/L/hour] vs 0.48 mmol/L/hour [0.22-0.86 mmol/L/hour]; P = .139) the presence of a shunt, neonatal age, peak lactate, extracorporeal cardiopulmonary resuscitation, and the use of hemofiltration on ECMO significantly predicted the rate of clearance. Survival to hospital discharge was similar in the shunt and nonshunt groups (49.0% vs 48.3%; P = .932).A strategy of increased ECMO flow without surgically restricting shunt diameter appears to be successful in providing circulatory support in the majority of patients with an SP shunt. Equivalent survival suggests that routine surgical reduction of shunt diameter is not indicated.

Background The 2 management strategies of neonates born with ductal-dependent pulmonary atresia and ventricular septal defect (PAVSD) include single stage primary biventricular repair (BVR) or staged palliation with modified Blalock-Taussig shunt (BTS) followed by second stage repair. Each approach is associated with specific benefits and drawbacks. We report outcomes of those 2 different strategies. Methods Between 2002 and 2012, 86 neonates with ductal-dependent PAVSD underwent surgery using primary repair (BVR group: n = 28, 33%) or shunt palliation (BTS group: n = 58, 67%). Early and late results were compared between the 2 groups. Results Median age was 6 days (interquartile range [IQR] 3–17) and median weight was 2.8 kg (IQR 2.5–3.3) with 27 patients (31%) 2.5 kg or less. Associated risk factors such as prematurity 36 weeks or less and genetic or extra-cardiac malformations were present in 30% and 40% of patients, respectively. Hospital mortality occurred in 5 (5.8%) patients (1 [3.6%] for BVR versus 4 [6.9%] for BTS, p = 1.00). Overall 8-year survival was 76.5% (85.5% for BVR versus 72.2% for BTS, p = 0.189). On multivariable analysis, risk factors for mortality were genetic or extra-cardiac malformations (hazard ratio [HR], 2.8 (95% confidence interval [CI] 1.7% to 16.0%), p = 0.036) and postoperative extracorporeal membrane oxygenation (ECMO) (HR, 4.0 [95% CI, 1.1% to 14.4), p = 0.039). Freedom from right ventricular outflow tract reoperation after achievement of repair was 63.2% at 8 years (52.4% for BVR versus 70.2% for BTS, p = 0.170). On multivariable analysis, risk factors for reoperation were the use of conduit (HR, 8.7 [95% CI, 1.1% to 65.8%], p = 0.037) and prematurity (HR, 2.8 [95% CI, 1.1% to 7.2%], p = 0.028). Conclusions Primary BVR of neonates with ductal-dependent PAVSD is associated with a trend for improved survival due to hospital and interstage mortality with the staged approach. Genetic or extra-cardiac malformations are common and are associated with worse survival.

Tricuspid atresia (TA) is a heterogeneous single-ventricle anomaly in which initial presentation and, consequently, timing and mode of palliation vary based on morphology and degree of pulmonary or systemic outflow obstruction. We report current era palliation outcomes and examine whether morphologic and, subsequently, surgical factors influence survival.From 2002 to 2012, 105 infants with TA underwent surgical palliation. Competing risks analyses modeled events after first-stage surgery (Glenn versus death) and after Glenn (Fontan versus death) and examined risk factors affecting outcomes.Seventy-eight patients (74%) required neonatal first-stage palliation, including modified Blalock-Taussig shunt (n = 46, 44%), Norwood (n = 18, 17%), and pulmonary artery band (n = 14, 13%), whereas 27 (26%) received primary Glenn as their initial surgery. Hospital mortality was 5 patients (4.8%). Competing risks models showed that by 1 year after first-stage surgery, 15% of patients had died and 83% had undergone Glenn. By 5 years after Glenn, 2% of patients had died and 80% had undergone Fontan. Overall 8-year survival was 84%. On multivariable analysis, risk factors for mortality were genetic/extracardiac anomalies (hazard ratio 7.0, 95% confidence interval: 2.4 to 20.6, p0.001) and pulmonary atresia (hazard ratio 4.4, 95% confidence interval: 1.6 to 12.2, p = 0.004). Survival was not affected by initial palliation type (p = 0.36), ventriculoarterial discordance (p = 0.25), systemic outflow obstruction (p = 0.84), or arch obstruction (p = 0.62).Despite morphologic and physiologic variations necessitating different palliative sequences, multistage palliation outcomes of various TA subtypes are comparable and generally good, with the exception of patients with associated genetic/extracardiac anomalies. The bulk of mortality is interstage, indicating continued opportunity for improvement in monitoring and managing patients during this critical period.

We evaluated our experience with aortic valve cusp extension techniques to identify predictors of successful intraoperative repair and subsequent durability.Twenty-two children (ages 5-18 years) underwent aortic cusp extension with autologous pericardium between 1999 and 2005. Sixteen children had previous surgical or percutaneous intervention. Ten children had bicuspid aortic valves. Cusp extensions were performed on 1 cusp in 3 patients, 2 cusps in 3, and 3 cusps in 16. Serial echocardiographic measures (n = 81) were obtained during a 5-year period and underwent blinded review. Longitudinal trajectories of ventricular and aortic valve function were modeled using mixed linear regression analysis.There was no hospital or late mortality. Five-year freedom from valve replacement was 75%. Comparison of preoperative and post-repair echocardiograms demonstrated reductions in aortic insufficiency (decreased in jet-width/aortic valve diameter ratio from 0.39 +/- 0.12 to 0.22 +/- 0.11; p0.0001), aortic stenosis (decreased in peak aortic valve gradient from 41 +/- 25 mm Hg to 29 +/- 15 mm Hg; p = 0.04), and left ventricular end-diastolic dimensions Z-score (decreased from 1.39 +/- 0.38 to 1.16 +/- 0.34; p0.001). During the follow-up period, post-repair jet-width and aortic valve diameter increased nonlinearly (p0.001). Patients with postoperative peak aortic gradients greater than 30 mm Hg had progression of aortic stenosis, whereas those with lesser postoperative peak gradients tended to regress during follow-up (p0.001). The decrement in Z-score of the left ventricular end-diastolic dimensions remained stable during the follow-up period.Aortic valve cusp extension can result in acceptable hemodynamic results with stabilization of left ventricular geometry. However, residual lesions are common and progression and regression of these lesions can be predicted based on echocardiographic data.

Background: Hospital survival for infants who require extracorporeal membrane oxygenation (ECMO) following the Norwood operation is 30% to 60%. However, little is known about late outcomes of hospital survivors and their ability to progress through subsequent palliative stages. Methods: Between 2002 and 2012, 38 (13.4%) of the 284 neonates with hypoplastic left heart syndrome or other single ventricle variants received ECMO support following Norwood. We examined factors affecting hospital death and compared postdischarge events between hospital survivors who received postoperative ECMO (n = 16 of 38) and a control of hospital survivors who did not receive ECMO (220 of 246). Results: Unplanned cardiac reoperation was the only predictor of postoperative ECMO requirement. Overall, 22 (58%) of the 38 patients were weaned from ECMO support and 16 (42%) of the 38 survived to hospital discharge. The ECMO duration was a significant factor for hospital mortality (odds ratio = 1.52 per 1-day increase [1.03-2.24], P = .035). Following discharge, 15 (94%) of the 16 underwent Glenn and 1 (6%) of the 16 had interstage mortality. In the control group, 194 (88%) of the 220 underwent Glenn and 26 (12%) of the 220 had interstage mortality or received transplantation ( P = .499). Following Glenn, 3 (20%) of the 15 patients had interstage mortality or received transplantation and 12 (80%) of the 15 proceeded to Fontan or were alive awaiting Fontan. In the control group, 23 (12%) of the 194 had interstage mortality or received transplantation and 171 (88%) proceeded to Fontan or were alive awaiting Fontan ( P = .357). Overall, 81% of hospital survivors were alive 5 years following discharge in both ECMO and non-ECMO groups. Conclusions: ECMO support following Norwood is associated with high probability of hospital death. Nonetheless, interstage mortality, progression to subsequent palliative stages, intermediate survival, and freedom from heart transplantation are comparable to those in patients who did not require postoperative ECMO support.

Extracorporeal life support has assumed a very effective role in the support of patients with refractory heart failure after repair of congenital heart disease, with hospital survival between 37% and 42%. We reviewed our results of different applications of extracorporeal life support in the last 2 years.Between January 2001 and October 2003, 671 patients underwent surgery for congenital heart disease at our institution. We retrospectively reviewed the hospital and clinic charts of the patients who required extracorporeal life support postoperatively, and studied the factors associated with survival.Thirty-six patients (5.36%) received extracorporeal life support after surgery, between 1 day and 8 years of age (age30 days, n = 34). We divided the patients into four groups. Group 1 consisted of 13 patients who were electively placed on ventricular support without an oxygenator (univentricular assist device) after repair of single-ventricle disease. Group 2 consisted of 16 patients who required extracorporeal membrane oxygenation after surgery for failed hemodynamics. Group 3 consisted of 2 patients who required left ventricle support (left ventricular assist device) after surgery for two-ventricle disease but who did not require biventricular (extracorporeal membrane oxygenation) support. Group 4 consisted of 5 patients who required conversion from ventricular assist device to extracorporeal membrane oxygenation. Overall, 28 patients were weaned successfully (78%), and 24 survived to discharge (67%). Hospital survival in groups 1, 2, 3, and 4 was 100%, 50%, 100%, and 20%, respectively. Univariate factors associated with survival were age, weight, ventricular assist device type, duration, single-ventricle disease, reexploration, number of complications, and specific complications such as sepsis, renal failure, and pulmonary failure.Extracorporeal life support utilization was expanded to include different applications with different outcomes. The extracorporeal life support registry should be altered to reflect those changes.

Background: Congenital heart disease continues to be an important indication for pediatric heart transplantation (HT) and is often complicated by systemic venous anomalies. The need for reconstruction, surgical technique used, as well as the outcomes of these have limited documentation. Methods: Descriptive, retrospective study of patients transplanted at Emory University between 2006 and 2017. We reviewed surgical data, follow-up, and interventions for patients necessitating venous reconstruction during transplantation. Results: A total of 179 transplants were performed during the time period of which 74 (41%) required systemic venous reconstruction. Mean age at transplant was 6.3 (±6.16) years, and 74.3% of these patients carried a diagnosis of single ventricle; 51 (68.9%) of 74 patients required pulmonary artery reconstruction at the time of HT. Forty patients required superior vena caval reconstruction, while 22 patients required inferior vena caval reconstruction due to prior palliation or anomaly. Venous anomalies along with other anatomic features necessitated biatrial transplantation in four patients. Posttransplant evaluation revealed systemic venous stenosis in 14 (18.9%) of 74 patients. Eight (10.8%) patients required 12 interventions for the systemic veins. Patients with bilateral Glenn anastomosis prior to transplant were at high risk for the development of stenosis and needing interventions. Systemic venous complications were uncommon in those with native systemic veins without Glenn or Fontan procedure. Conclusion: Systemic venous reconstruction needs are high in pediatric HT. Posttransplant stenosis and the need for interventions are relatively common. Current techniques for systemic venous reconstruction for complex congenital heart disease patients may deserve further review to optimize these outcomes.