Primary Biventricular Repair Research Articles

Impact of left ventricular rehabilitation on surgical outcomes in patients with borderline left heart hypoplasia

ObjectiveClinical significance of left ventricular (LV) rehabilitation for borderline left ventricular (LV) hypoplasia is controversial. This study aimed to review the surgical results of patients with borderline LV hypoplasia, and to evaluate the impact of the LV rehabilitation on outcomes. MethodsPatients diagnosed with borderline LV hypoplasia and surgically treated from 2018 to 2022 were included. Overall surgical outcomes were evaluated and the changes in LV volumes calculated using angiography and zlog-NT-proBNP levels were analyzed in patients who underwent LV rehabilitation. ResultsThirty-three patients were included. Sixteen patients underwent primary biventricular repair, three patients underwent primary single ventricle palliation, and the remaining 14 patients underwent LV rehabilitation, including nine bilateral pulmonary artery banding (PAB) and ductal stenting, four central PAB, and one ductal stenting. Among fourteen patients who received LV rehabilitation, one died, one underwent single ventricle palliation, one was waiting for further procedure, and 11 underwent biventricular repair. After biventricular repair, 2 patients died, and 1 patient developed hemodynamic failure. As a result, only 8 patients were alive and in good condition. In patients who underwent LV rehabilitation, LV end-diastolic/end-systolic volume index, and LV stroke volume index increased over time after LV rehabilitation (p=0.001, p=0.007, and p=0.009, respectively). The zlog-NT-proBNP levels were stable until biventricular repair, but significantly higher in patients who presented with hemodynamic failure following biventricular repair compared to patients who did not exhibit hemodynamic failure. ConclusionsIn patients with borderline left heart hypoplasia, LV rehabilitation procedure promoted an increase in LV volume and contributed to establishing a biventricular circulation. The short-term results of this strategy are satisfactory, but further studies are essential to determine the long-term outcomes.

Primary vs Staged Biventricular Repair for Neonatal IAA with VSD and LVOTO

BackgroundThis study sought to determine the safety of primary and staged biventricular repair in neonates with interrupted aortic arch (IAA), ventricular septal defect (VSD), and severe left ventricular outflow tract obstruction (LVOTO). MethodsPatients with a fundamental diagnosis of IAA and VSD between 2015 and 2020 were extracted from The Society of Thoracic Surgeons National Database by using a Participant User File. The objective was to compare outcomes for neonates undergoing primary and staged Yasui and Ross operations. Primary end points were operative morbidity and mortality. ResultsDuring the study period, 11.4% (123 of 1079) of neonates with a fundamental diagnosis of IAA and VSD underwent operations indicative of severe LVOTO. Of these patients, 42 (34%) underwent primary biventricular repair (Yasui or Ross/Ross-Konno), and 81 underwent a potential staging procedure (Norwood or hybrid stage I). No differences were observed in preoperative patient characteristics between groups. Neonates undergoing staged repair experienced fewer major complications (0 vs 1; P = .04) and total complications (2 vs 4; P = .02), but similar operative mortality (5% vs 12%; P = .27) as neonates undergoing primary repair. A total of 58 patients undergoing Rastelli, biventricular repair, Yasui, or Ross/Ross-Konno operations with a diagnosis of IAA and VSD and history of neonatal Norwood or hybrid stage I procedures were also identified. Operative mortality for second-stage biventricular conversion operations was 2% (1 of 58). Only 4 centers performed 1 or more complex biventricular repairs for IAA and VSD with LVOTO per year. ConclusionsPrimary and staged biventricular repairs for IAA and VSD with LVOTO are associated with low operative mortality in the modern era and may be favorable to long-term single-ventricle palliation.

Staged repair of borderline hypoplastic heart disease with early biventricular conversion

ObjectiveIn select patients with borderline ventricular hypoplasia, we adopted a strategy of initial single-ventricle palliation followed by staged or direct biventricular conversion by 2 years of age. MethodsBetween 2018 and 2023, 14 newborns with borderline hypoplastic heart disease deemed high risk for primary biventricular repair underwent palliative procedures as a neonate/infant, followed by staged or direct biventricular conversion. ResultsOf the 14 patients, 6 had borderline left ventricles and 8 had borderline right ventricles. Index neonatal operations were performed in 12 patients and included the Norwood operation (n = 5), pulmonary artery band (n = 3), ductal stent (n = 3), and hybrid Norwood (n = 1). Five patients underwent direct biventricular conversion, and the remaining 9 patients underwent staged ventricular recruitment operations at a mean age of 6 months (range, 3-11 months). Ventricular recruitment operations included atrial septation with or without ventricular rehabilitation, atrioventricular valve repair, or outflow tract operations. At a mean duration of 8 months (range, 4-10 months) after ventricular recruitment, there was a significant increase in chamber volume, aortic valve, and mitral valve size in patients with borderline left ventricles, and a normalization of the right ventricle:left ventricle end-diastolic volume ratio in patients with borderline right ventricles. To date, 13 of 14 patients have undergone successful biventricular conversion at a mean age of 16 months (range, 4-31 months). ConclusionsIn select newborns with borderline hypoplastic heart disease, single-ventricle palliation followed by staged or direct biventricular conversion may increase infant survival while allowing for early attainment of a biventricular circulation.

Cardiac magnetic resonance predictors for successful primary biventricular repair of unbalanced complete common atrioventricular canal.

Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal. We conducted a retrospective cohort study of children with common atrioventricular canal who underwent MRI prior to repair. Associations between MRI and echocardiographic measures and surgical outcome were tested using logistic regression, and models were compared using area under the receiver operator characteristic curve. We included 28 patients (median age at MRI: 5.2 months). The optimal MRI model included the novel end-diastolic volume index (using the ratio of left ventricular end-diastolic volume to total end-diastolic volume) and the left ventricle-right ventricle angle in diastole (area under the curve 0.83, p = 0.041). End-diastolic volume index ≤ 0.18 and left ventricle-right ventricle angle in diastole ≤ 72° yield a sensitivity of 83% and specificity of 81% for successful biventricular repair. The optimal multimodality model included the end-diastolic volume index and the echocardiographic atrioventricular valve index with an area under the curve of 0.87 (p = 0.026). Cardiac MRI can successfully predict successful biventricular repair in patients with unbalanced common atrioventricular canal utilising the end-diastolic volume index alone or in combination with the MRI left ventricle-right ventricle angle in diastole or the echocardiographic atrioventricular valve index. A prospective cardiac MRI study is warranted to better define the multimodality characteristic predictive of successful biventricular surgery.

Current outcomes of live-born children with double outlet right ventricle in Norway.

This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). All children born with DORV between 2003 and 2017 were identified in the Oslo University Hospital registry. Patients' characteristics, interventions, complications and deaths were recorded. Echocardiographic data were reviewed for classification according to current standards. We investigated time-dependent surgical reintervention and mortality using Kaplan-Meier analyses and determinants of treatment complications, reintervention and death using regression analyses. Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10000 births in Norway. Six children received palliative care. With an intention to treat, a surgical route with the primary biventricular repair was followed for 62 children, staged biventricular repair for 15 and univentricular repair for 10 children. Major complications occurred in 1.0% and 6.2% of children following catheter or surgical intervention, respectively. No significant determinants of the complications were identified. Overall survival following treatment was 91.9%, 90.8%, 89.5% and 89.5% and corresponding freedom from surgical reintervention was 88.0%, 79.0%, 74.9% and 69.4% at 1, 2, 5 and 10 years, respectively. The presence of atrioventricular septal defect predicted an increased risk of mortality (hazard ratio: 7.16) but did not increase the risk of surgical reintervention. In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death.

Primary Versus Staged Repair in Neonates With Pulmonary Atresia and Ventricular Septal Defect

The 2 surgical strategies for neonates with ductal-dependent pulmonary atresia and ventricular septal defect are primary biventricular repair (BVR) or initial palliation with a modified Blalock-Taussig shunt (BTS) followed by second stage repair. In this study, we report the combined outcomes from 2 hospitals using different strategies. Between 2004 and 2017, 66 neonates underwent surgery with palliative shunts (BTS group: n= 30, 45.5%) or primary biventricular repair (pBVR group: n= 36, 54.5%). The 2 groups were similar in age, body weight, and Nakata index scores. The overall mean follow-up duration was 7.51 ± 4.35 years, and early and late results were compared between the groups. The 10-year overall survival was 84.8% (94.4% for pBVR vs 75.7% for BTS, P= .032). The BTS group had 2 early and 6 interstage mortalities, and the pBVR group had no early and 2 late mortalities. In the BTS group, the Nakata index score significantly increased during the interstage period (P < .001). In univariable analysis, genetic or extracardiac anomalies were a risk factor for mortality (hazard ratio, 5.56; P= .038). After achieving BVR, the pBVR group underwent significantly more frequent right ventricle outflow tract reinterventions (P < .001) at a much earlier period (P= .017) compared with the BTS group. In neonates with ductal-dependent pulmonaryatresia and ventricular septal defect, the primary BVR approach provides an excellent survival rate, but the burden of right ventricle outflow tract reintervention is heavy. The staged approach with BTSpromotes pulmonary artery growth, but hospital and interstage mortality are significant. Genetic and extracardiac anomalies are significant risk factors for mortality.

Biventricular Conversion in the Borderline Hypoplastic Heart.

The development of biventricular repair and conversion pathways for patients with borderline hypoplastic heart disease represents an area of recent inquiry and innovation. This review summarizes emerging techniques and novel treatment algorithms for borderline hypoplastic heart disease with a focus on surgical advances within the last 10years. Many patients with borderline hypoplastic heart disease are amenable to primary biventricular repair, or biventricular conversion following single-ventricle palliation coupled with ventricular rehabilitation strategies. New insights into the potential for growth and recovery of borderline ventricles have been uncovered. However, questions remain regarding optimal patient selection and the long-term outcomes of select patient groups treated with single-ventricle palliation versus biventricular repair/conversion or transplantation. Efforts to direct a greater proportion of borderline hypoplastic heart patients towards a biventricular circulation are accelerating and represent important avenues for progress and future research in the field of congenital heart disease.

Pulmonary artery banding in complete atrioventricular septal defect

ObjectivesTo 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. MethodsPAB 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. ResultsAt 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). ConclusionsPAB 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.

Preoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect.

In complete atrioventricular canal defect (CAVC), there are limited data on preoperative clinical and echocardiographic predictors of operative timing and postoperative outcomes. A retrospective, single-center analysis of all patients who underwent primary biventricular repair of CAVC between 2006 and 2015 was performed. Associated cardiac anomalies (tetralogy of Fallot, double outlet right ventricle) and arch operation were excluded. Echocardiographic findings on first postnatal echocardiogram were correlated with surgical timing and postoperative outcomes using bivariate descriptive statistics and multivariable logistic regression. 153 subjects (40% male, 84% Down syndrome) underwent primary CAVC repair at a median age of 3.3 (IQR 2.5-4.2) months. Median postoperative length of stay (LOS) was 7 (IQR 5-15) days. Eight patients (5%) died postoperatively and 24 (16%) required reoperation within 1year. On multivariable analysis, small aortic isthmus (z score < -2) was associated with early primary repair at < 3months (OR 2.75, 95% CI 1.283-5.91) and need for early reoperation (OR 3.79, 95% CI 1.27-11.34). Preoperative ventricular dysfunction was associated with higher postoperative mortality (OR 7.71, 95% CI 1.76-33.69). Other factors associated with mortality and longer postoperative LOS were prematurity (OR 5.30, 95% CI 1.24-22.47 and OR 5.50, 95% CI 2.07-14.59, respectively) and lower weight at surgery (OR 0.17, 95% CI 0.04-0.75 and OR 0.55, 95% CI 0.35-0.85, respectively). Notably, preoperative atrioventricular valve regurgitation and Down syndrome were not associated with surgical timing, postoperative outcomes or reoperation, and there were no echocardiographic characteristics associated with late reoperation beyond 1 year after repair. Key preoperative echocardiographic parameters helped predict operative timing and postoperative outcomes in infants undergoing primary CAVC repair. Aortic isthmus z score < -2 was associated with early surgical repair and need for reoperation, while preoperative ventricular dysfunction was associated with increased mortality. These echocardiographic findings may help risk-stratified patients undergoing CAVC repair and improve preoperative counseling and surgical planning.

Retrospective study of complete atrioventricular canal defects: Anesthetic and perioperative challenges.

Objective:The objective of this study was to highlight anesthetic and perioperative management and the outcomes of infants with complete atrioventricular (AV) canal defects.Design:This retrospective descriptive study included children who underwent staged and primary biventricular repair for complete AV canal defects from 1999 to 2013.Setting:A single-center study at a university affiliated heart center.Participants:One hundred and fifty-seven patients with a mean age at surgery of 125 ± 56.9 days were included in the study. About 63.6% of them were diagnosed as Down syndrome. Mean body weight at surgery was 5.6 ± 6.3 kg.Methods:Primary and staged biventricular repair of complete AV canal defects. Measurements and main results: A predefined protocol including timing of surgery, management of induction and maintenance of anesthesia, cardiopulmonary bypass, and perioperative intensive care treatment was used throughout the study. Demographic data as well as intraoperative and perioperative Intensive Care Unit (ICU) data, such as length of stay in ICU, total duration of ventilation including reintubations, and total length of stay in hospital and in hospital mortality, were collected from the clinical information system. Pulmonary hypertension was noted in 60% of patients from which 30% needed nitric oxide therapy. Nearly 2.5% of patients needed permanent pacemaker implantation. Thorax was closed secondarily in 7% of patients. In 3.8% of patients, reoperations due to residual defects were undertaken. Duration of hospital stay was 14.5 ± 4.7 days. The in-hospital mortality was 0%.Conclusion:Protocolized perioperative management leads to excellent outcome in AV canal defect repair surgery.

新生児 Ebstein 奇形に対する一期的両心室修復として cone 手術を行った1例

新生児Ebstein奇形に対する一期的両心室修復としてのcone手術の報告はほとんどない．症例は日齢14の女児，胎児エコーで重症Ebstein奇形と診断され帝王切開で出生した．プロスタグランジンが開始され当院に搬送となった．CTRは74%，三尖弁逆流はsevere，肺動脈弁は機能的閉鎖であった．プロスタグランジンを離脱しても動脈管は縮小せず，むしろ肺血管抵抗低下に伴い心不全が悪化したため新生児期での手術介入となった．三尖弁は前尖も高度にplasteringしており，弁の形態的にはCarpentier type Cに相当するものであった．しかしcone手術により三尖弁逆流は制御され，右心機能は改善し，肺血流は右室の順行性拍出のみで維持できた．術後6日目に抜管し，術後8日目に集中治療室を退室した．CTRは58%と縮小し，三尖弁逆流はmoderate以下であった．右室機能は良好であり，心房間交通は右左シャントではあるがSpO2 は90%以上であった．Cone手術は三尖弁前尖が低形成で，両心室修復の可否がborder lineであるような新生児Ebstein奇形に対しても有効であった．

Results of Primary Repair Versus Shunt Palliation in Ductal Dependent Infants With Pulmonary Atresia and Ventricular Septal Defect

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. 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. 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). 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.

Should Repair of Partial Atrioventricular Septal Defect Be Delayed Until Later in Childhood?

Surgical repair of partial atrioventricular septal defects (AVSD) has been successful for more than 60years. However, recent data from the Pediatric Heart Network show that 31% of patients have moderate or severe left atrioventricular valve regurgitation (LAVVR) at follow-up. Previously, our institution found that only 9% of patients had more than moderate LAVVR at the last follow-up. Our objective was to determine the long-term outcomes after repair of partial AVSD in the current era. We reviewed all patients with partial AVSD who had primary biventricular repair from January 1995 to June 2011 at our institution. The Kaplan-Meier method was used to estimate the survival free of an event, and factors were evaluated for an association with each outcome using the log-rank test. All 105 patients with partial AVSD who had surgery during this period were evaluated. The median age at surgery was 7.9years. The overall survival rate at 1 year was 97%. Median follow-up was 5.3years (interquartile range 1.7 to 11.1). At 3years, the survival rate free from reoperation was 89%. Thirteen patients required reoperations with the most common reason being LAVVR. A total of 10 patients developed more than moderate LAVVR with a cumulative incidence of 8% by 2years. The discrepancy with the Pediatric Heart Network data may be due to the later age of operation for patients in our cohort suggesting that elective repair of partial AVSD should be deferred until children are somewhat older (ages 5 to 8years). Neither patient age (p=0.11) nor severity of preoperative LAVVR (p= 0.16) were identified as statistically significant risk factors. In conclusion, there is less morbidity and mortality after surgical repair for partial AVSD.

Repair of Ebstein's anomaly in neonates and small infants: impact of right ventricular exclusion and its indications†

In cases of severe Ebstein's anomaly, it is essential to determine whether biventricular repair (BVR) or single-ventricle palliation is feasible. Since 1999, in our institution, we have used the novel technique comprising tricuspid valve (TV) closure and right ventricular and right atrial (RV/RA) exclusion to reduce the deleterious effects of an enlarged RV in patients with severe Ebstein's anomaly. However, in cases with good RV function, primary BVR is performed. In the present study, we describe our surgical strategy in the treatment of severely symptomatic neonates with Ebstein's anomaly. From June 1999 to October 2011, 12 neonates with a severely symptomatic Ebstein's anomaly underwent surgical repair. The mean age at the first operation was 29 ± 25 (range, 5-92) days; and the mean body weight was 2.8 ± 0.5 (range, 2.0-4.1) kg. The associated anomalies included pulmonary atresia with an intact ventricular septum in 7, critical pulmonary stenosis in 1, ventricular septal defect in 3 and coarctation of the aorta in 1 patient. The mean cardio-thoracic ratio (CTR) was 80 ± 14% (range, 57-98%). Preoperatively, 9 patients had grade IV tricuspid regurgitation (TR), as detected by echocardiography, and 6 required ventilator support. Five patients underwent primary BVR. Seven patients underwent staged palliation using a modified Blalock-Taussig shunt (BT shunt) with/without RV/RA exclusion. There was 1 case each of hospital death and late death. The median follow-up duration in the present study was 6.5 years. Among the 8 patients who underwent TV repair, postoperative TR was trivial or mild in 6 patients, moderate in 1 and absent in 1. After surgery, the mean CTR and serum B-type natriuretic peptide levels decreased to 59 ± 14% (range, 45-70%) and 46 ± 28 (range, 12-83) pg/dl, respectively. Critically ill neonates with Ebstein's anomaly can be successfully treated using RV/RA exclusion combined with a modified BT shunt in cases where RV function is poor. However, in cases of good RV function, we recommend the use of primary BVR.

Primary Biventricular Repair of Atrioventricular Septal Defects: An Analysis of Reoperations

The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair. Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle. There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients. Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality.

Intermediate-term clinical outcomes of primary biventricular repair for left ventricular outflow tract obstruction and ventricular septal defect

Primary biventricular repair for left ventricular outflow tract obstruction and ventricular septal defect remains challenging. The intermediate-term outcomes and risk factors for mortality remain undefined. All patients undergoing primary biventricular repair of left ventricular outflow tract obstruction and ventricular septal defect from 1995 to 2008 at the C. S. Mott Children's Hospital, University of Michigan Health Systems were analyzed. Thirty-one patients (mean age, 18 days; 20 male) with a median follow-up of 6.7 years (range, 0.3-13.5 years) were identified. The ventricular septal defect was enlarged in 15 patients, and a limited atrial septal defect was constructed in 16 patients. There were 6 hospital and 2 late deaths. Ten-year patient survival was 72.3%. Lower body weight (P = .040), complete atrial septal defect closure (P = .026), and longer cardiopulmonary bypass time (P = .026) were risk factors of hospital mortality. An atrial septal defect was patent in 16 patients at discharge, 2 of whom required later surgical closure. Relief of recurrent left ventricular outflow tract obstruction was performed in 1 patient. No patient required pacemaker implantation. Five-year freedom from right ventricle-to-pulmonary artery conduit replacement was 39.3%. Smaller-sized conduit (P = .020) and use of aortic allograft (P = .048) were risk factors for early failure. Primary biventricular repair for patients with left ventricular outflow tract obstruction and ventricular septal defect provides good early and intermediate-term outcomes. Maintaining a small atrial septal defect may improve hospital mortality. Selective ventricular septal defect enlargement and careful construction of the intraventricular pathway result in a low incidence of recurrent left ventricular outflow tract obstruction, as well as avoidance of heart block. Maximizing valve diameter and avoiding aortic allografts may lengthen conduit longevity.

Surgical management of congenital heart defects associated with heterotaxy syndrome☆

Heterotaxy syndrome (HS) is generally associated with complex congenital cardiac defects and has a high morbidity and mortality despite recent surgical progress. Only few reports deal with an overall surgical population. Between 1989 and 2008, 139 patients with HS entered a programme of surgical repair. Fifty-one patients were suitable for univentricular heart repair (UVR) and 88 for biventricular repair (BVR). Among those tracked for UVR, two were switched to BVR and 11 from BVR to UVR. Median age at first surgery was 4.4 months (range: 3 days to 43 years] of whom 34 were neonates. The mean number of surgical procedure per patient was 1.99. Primary BVR was performed in 37 patients. Re-operation was required in 22 patients, 15 after BVR and seven after UVR (p>0.05). The overall mortality was 20.8%. It was 7.2% after the first surgery, 6.6% after the second and 11.5% after the third. The overall mortality in patients with univentricular physiology was 25.5% and 18.2% in the biventricular group (p<0.05). According to the surgical track, in the UVR group, mortality was 18% and 15.6% in the BVR group (p=NS). This rate was 40% in patients with long-lasting palliation (p<0.05 vs both other groups). Median follow-up was 127 months (range: 1 month to 19 years). The overall survival rate at 15 years was 70.6%. When considering ventricular anatomy, survival rates at 15 years were 69% for the univentricular group and 74.2% for the biventricular group (p>0.05). According to the type of surgical approach, at 15 years they were 85.1% for UVR and 77% for BVR (p=NS). For the palliation group, it was 15% only at 15 years (p<0.05 vs both other groups). Risk factors for overall mortality were neonatal surgery, long-standing palliation, total anomalous pulmonary vein return (TAPVR) and right ventricular outflow tract obstruction (RVOTO). At the last visit, all survivors were in the New York Heart Association (NYHA) class I to II and only two presented with supraventricular arrhythmias. HS remains a difficult situation with high morbidity and mortality. An aggressive approach to repair TAPVR when present should be considered. Early decision to track the patient in either uni- or biventricular repair programme should avoid long-lasting deleterious palliation.

Partial and Transitional Atrioventricular Septal Defect Outcomes

Surgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort. We studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change. Median age at surgery was 1.8 years; median weight z-score was -0.88. Median days for ventilation were 1, intensive care 2, and hospitalization 5, all independent of age, with 1 in-hospital death. At 1 month, 27% (16 of 73) had ejection fraction less than 55%; 20% (17 of 87) had significant LAVVR; 2 had residual shunts; 1 each had subaortic stenosis and LAVV stenosis. At 6 months (n = 60), there were no interim deaths, reinterventions, or new development of subaortic or LAVV stenosis. Weight z-score improved by a median 0.4 units (p < 0.001), especially for underweight children less than 18 months old. Left atrioventricular valve regurgitation occurred in 31% (change from baseline, p = 0.13), occurring more frequently in patients repaired at 4 to 7 years (p = 0.01). Three patients had ejection fraction less than 55%, and 1 had a residual atrial shunt. Surgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age.

Aortic Atresia or Severe Left Ventricular Outflow Tract Obstruction with Ventricular Septal Defect: Results of Primary Biventricular Repair in Neonates

Aortic Atresia or Severe Left Ventricular Outflow Tract Obstruction with Ventricular Septal Defect: Results of Primary Biventricular Repair in Neonates

Aortic Atresia or Severe Left Ventricular Outflow Tract Obstruction with Ventricular Septal Defect: Results of Primary Biventricular Repair in Neonates

Aortic atresia or severe aortic stenosis and left ventricular outflow tract obstruction is a frequent component of complex congenital heart disease. Aortic atresia or severe aortic stenosis and left ventricular outflow tract obstruction with two adequate ventricles is sometimes treated by Norwood palliation followed by late biventricular repair. We reviewed our experience with primary biventricular repair in this group of neonates. Retrospective review identified 17 neonates (10 males) with aortic atresia or severe left ventricular outflow tract obstruction with ventricular septal defect and an adequate left ventricle undergoing primary biventricular repair between 1986 and 2002. Mean age was 7.7 +/- 2.9 days, weight 3.3 +/- 0.7 kg, and body surface area 0.21 +/- 0.04 kg/m2. Associated anomalies included arch hypoplasia, 7 (41%); aortic atresia, 7 (41%); and coarctation, 5 (29%). Results are reported as mean +/- standard deviation. Median follow-up was 6 years (range, 1 to 17.7 years). Three of the 17 (18%) died within 30 days. There were no deaths in this series since 1992. Nine patients (38.9%) required one reoperation, 7 of which were for conduit stenosis, 1 for left ventricular outflow tract obstruction, and 1 for residual ventricular septal defect with left ventricle-to-right atrium shunt. Freedom from death at 10 years was 82% by Kaplan-Meier estimate. Excellent long-term survival can be achieved by primary biventricular repair as corroborated by our survival rate of 82%. Primary biventricular repair is an effective operation for aortic atresia and severe left ventricular outflow tract obstruction with adequate sized left ventricle that avoids interstage attrition associated with Norwood palliation and is our procedure of choice.