A retrospective study: Long term prognosis in adults with PA-VSD-MAPCAs.

BACKGROUND: Pulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment. METHODS: A prospectively established database of 169 PA-VSD-MAPCAs patients treated and followed up at University Hospitals Leuven was accessed. Patients were divided into three groups according to the treatment strategy. Kaplan-Meier survival curves were plotted, and Log Rank tests were used for comparison. RESULTS: The overall mean survival for patients with PA-VSD-MAPCAs was 38.5 years (95%-CI: 33.1-43.9). Patients with complete intracardiac repair had the longest mean survival of 43.8 years (95%-CI: 38.1-49.6) versus the other groups (p < 0.001). A longer mean event-free survival time was found in patients with normal, well-developed NPAs (p = 0.047). Finally, patients with poorly developed or absent NPAs had worse survival rates when a surgical approach was followed. Systemic-pulmonary shunt placement or unifocalisation had limited effect on prognosis in the absence of total repair (p = 0.167). CONCLUSIONS: Patients with PA-VSD-MAPCAs who underwent complete intracardiac repair and/or with well-developed native pulmonary arteries had the best prognosis. Our analyzed data suggest that incomplete surgical repair resulted in survival rates comparable to those seen with a non-surgical approach.

Preoperative Malnutrition Increases Risk of In-Hospital Mortality, Major Infection, and Longer Intensive Care Unit Stay After Ventricular Septal Defect Closure.

BACKGROUND: High energy requirements and poor feeding can lead to growth failure in patients with ventricular septal defect (VSD), but effects of preoperative malnutrition on surgical outcomes are poorly understood, especially in low-resource settings. METHODS AND RESULTS: We analyzed a cohort of children <5 years of age undergoing VSD closure at 60 global centers participating in the International Quality Improvement Collaborative for Congenital Heart Disease, 2015 to 2020. We calculated adjusted odds ratios (ORs) for in-hospital death and major infection and adjusted coefficients for duration of intensive care unit stay for 4 measures of malnutrition: severe wasting (weight-for-height Z score, <-3), moderate wasting (-3

Association between pregnancy and long-term cardiac outcomes in individuals with congenital heart disease.

BACKGROUND: As early life interventions for congenital heart disease improve, more patients are living to adulthood and are considering pregnancy. Scoring and classification systems predict the maternal cardiovascular risk of pregnancy in the context of congenital heart disease, but these scoring systems do not assess the potential subsequent risks following pregnancy. Data on the long-term cardiac outcomes after pregnancy are unknown for most lesion types. This limits the ability of healthcare practitioners to thoroughly counsel patients who are considering pregnancy in the setting of congenital heart disease. OBJECTIVE: We aimed to evaluate the association between pregnancy and the subsequent long-term cardiovascular health of individuals with congenital heart disease. STUDY DESIGN: This was a retrospective longitudinal cohort study of individuals identifying as female who were receiving care in two adult congenital heart disease centers from 2014 to 2019. Patient data were abstracted longitudinally from a patient age of 15 years (or from the time of entry into the healthcare system) to the conclusion of the study, death, or exit from the healthcare system. The primary endpoint, a composite adverse cardiac outcome (death, stroke, heart failure, unanticipated cardiac surgery, or a requirement for a catheterized procedure), was compared between parous (at least one pregnancy >20 weeks' gestation) and nulliparous individuals. By accounting for differences in the follow-up, the effect of pregnancy was estimated based on the time to the composite adverse outcome in a proportional hazards regression model adjusted for the World Health Organization class, baseline cardiac medications, and number of previous sternotomies. Participants were also categorized according to their lesion type, including septal defects (ventricular septal defects, atrial septal defects, atrioventricular septal defects, or atrioventricular canal defects), right-sided valvular lesions, left-sided valvular lesions, complex cardiac anomalies, and aortopathies, to evaluate if there is a differential effect of pregnancy on the primary outcome when adjusting for lesion type in a sensitivity analysis. RESULTS: Overall, 711 individuals were eligible for inclusion; 209 were parous and 502 nulliparous. People were classified according to the World Health Organization classification system with 86 (12.3%) being classified as class I, 76 (10.9%) being classified as class II, 272 (38.9%) being classified as class II to III, 155 (22.1%) being classified as class III, and 26 (3.7%) being classified as class IV. Aortic stenosis, bicuspid aortic valve, dilated ascending aorta or aortic root, aortic regurgitation, and pulmonary insufficiency were more common in parous individuals, whereas dextro-transposition of the great arteries, Turner syndrome, hypoplastic right heart, left superior vena cava, and other cardiac diagnoses were more common in nulliparous individuals. In multivariable modeling, pregnancy was associated with the composite adverse cardiac outcome (36.4%% vs 26.1%%; hazard ratio, 1.83; 95% confidence interval, 1.25-2.66). Parous individuals were more likely to have unanticipated cardiac surgery (28.2% vs 18.1%; P=.003). No other individual components of the primary outcome were statistically different between parous and nulliparous individuals in cross-sectional comparisons. The association between pregnancy and the primary outcome was similar in a sensitivity analysis that adjusted for cardiac lesion type (hazard ratio, 1.61; 95% confidence interval, 1.10-2.36). CONCLUSION: Among individuals with congenital heart disease, pregnancy was associated with an increase in subsequent long-term adverse cardiac outcomes. These data may inform counseling of individuals with congenital heart disease who are considering pregnancy.

Predictors of Mortality in Primary Surgical Repair of Postinfarction Ventricular Septal Defects: The Leipzig Experience.

BACKGROUND: A postinfarction ventricular septal defect (pVSD) as a complication of acute myocardial infarction (AMI) is associated with high mortality. This retrospective, single-center study aimed to identify predictors of early and long-term outcomes in patients undergoing primary surgical repair of pVSD managed by various surgical techniques. METHODS: We reviewed data from 77 consecutive patients who underwent primary surgical repair after pVSD in our institution. Prospectively collected demographic and perioperative data were analyzed retrospectively. Predictors of 30-day mortality and long-term outcome were assessed. RESULTS: pVSD was anterior in 45 patients (58.4%) and posterior in 32 (41.6%). Buttressed mattress suture (n = 9, 11.7%), simple single septal patch (n = 34, 44.2%), simple double septal patch (n = 2, 2.6%), sandwich double patch (n = 1, 1.3%), and the infarct exclusion technique (n = 31, 40.3%) were performed for surgical closure. Fifty-three patients (68.8%) had preoperative cardiogenic shock. The 30-day mortality was 42.8% (33 patients). Independent risk factors of 30-day mortality were duration between AMI and surgery <7 days (odds ratio [OR] 5.229, P = .011), preoperative absence of diuretics (OR 6.913, P = .005), and preoperative cardiogenic shock (OR 3.558, P = .011). Cumulative survival rates at 1, 5, and 10 years were 57.1%, 57.1%, and 31.2%, respectively. CONCLUSION: In pVSD, the 30-day mortality remains high, and preoperative cardiogenic shock significantly influenced mortality in our study. None of the surgical techniques or materials used in our investigation influenced the outcome.

Survival Outcomes of Two-Stage Intracardiac Repair in Large Ventricular Septal Defect and Trisomy 18.

BACKGROUND: Surgical management has not been encouraged in patients with trisomy 18 (T18) and congenital heart diseases due to poor survival. This study aimed to investigate (1) the appropriateness of palliative surgeries followed by intracardiac repair (ICR) (i.e., two-stage ICR) for patients with a large ventricular septal defect (VSD) and T18, and (2) its impact on their long-term outcomes. METHODS: Medical charts of patients with VSD and T18 who underwent two-stage ICR at the Japanese Red Cross Medical Center between January 2005 and December 2019 were retrospectively reviewed. Demographic data, timing, and types of palliative surgeries, information related to ICR, peri- and postoperative clinical information, postoperative survival, and cause of death were collected. The long-term prognosis of patients treated with two-stage ICR was compared with that of patients treated with primary ICR and palliative surgery without ICR. RESULTS: Overall, 18 (2 male, 16 female) patients underwent two-stage ICR. Pulmonary artery banding was the initial palliative surgery in all patients after a median duration of 19.5 (range 6-194) days of life. The median age and the mean body weight at the time of ICR were 18.2 (7.6-50.7) months and 6.0 ± 1.0 kg, respectively. The mean pulmonary artery pressure and pulmonary vascular resistance index before ICR were 19.1 ± 7.3 mmHg and 3.4 ± 2.0 U m2, respectively. Overall, 17/18 (94%) patients were discharged after ICR. Fourteen (78%) patients were alive during data collection. None of the patients died of cardiac insufficiency, and the median duration of survival was 46.3 (14.3-186.4) months since birth. Most patients required cardiac medications rather than pulmonary vasodilators at the last follow-up. During the study period, three patients underwent primary ICR, and 46 underwent palliative surgery without ICR. Of those who underwent primary ICR, two died in the hospital on the first and 48th day following ICR, and the third died 179 days after the ICR. The Log-rank test revealed a significantly longer survival for the patients treated with two-stage ICR compared with those treated with palliative surgery without ICR (P = 0.003). CONCLUSION: Two-stage ICR improves the long-term survival of patients with VSDs and T18. This safe surgical strategy can also prevent pulmonary hypertension in such patients.

Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis: Modified REV versus Rastelli.

The objective of this study was to evaluate and compare the results of the modified réparation à l'ètage ventriculaire (REV) and the Rastelli operation for the treatment of transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS). Records of 38 patients who underwent the modified REV (n = 16) or the Rastelli operation (n = 22) for the treatment of TGA, VSD, and PS between 2010 and 2019 were reviewed. The median age was 2.2 years (range 0.6-8.0 years) and the median weight was 11.3 kg (range 6.4-22.0 kg). No in-hospital death occurred and there were 4 early reoperations (two in each group). Overall survival at 10 years was 97.4% (100% in Modified REV group and 95.5% in Rastelli group, P = 0.39). Freedom from left ventricular outflow tract (LVOT) reoperation was 100% in both groups. Freedom from right ventricular outflow tract (RVOT) reoperation was 100% in Modified REV group and 75.4% in Rastelli group (P = 0.073). Event-free survival was 100% in Modified REV group and 72.0% in Rastelli group (P = 0.048). The most recent echocardiography showed that LVOT peak gradient was less than 10 mmHg in all patients. In Modified REV group, 30.8% of patients (4/13) had either RVOT obstruction (RVOT peak gradient more than 40 mmHg) or moderate or severe pulmonary insufficiency, while conduit stenosis (peak gradient more than 40 mmHg) was found in 25.0% of patients (3/12) in Rastelli group. The modified REV and the Rastelli operation provide satisfactory early results, as well as long-term survival and LVOT performance. However, the modified REV has better RVOT performance.

Mechanical circulatory support for early surgical repair of postinfarction ventricular septal defect with cardiogenic shock.

Postinfarction ventricular septal defect (pVSD) due to acute myocardial infarction complicated by cardiogenic shock (CS) is associated with high mortality. The aim of this study was to determine the outcome of primary surgical repair of pVSD in patients with CS and examine whether it is influenced by the use of mechanical circulatory support (MCS) devices. Between October 1994 and April 2016, primary surgical repair of pVSD complicated by CS was performed in 53 patients. Thirty-six (68%) were implanted pre-operatively with an intra-aortic balloon pump (IABP), 4 (8%) with extracorporeal life support (ECLS), and 13 (24%) received no MCS device. Prospectively collected demographic and perioperative data were analyzed retrospectively. All-cause, 30-day mortality rates were analyzed and multivariate analysis was performed to differentiate independent risk factors. No pre-operatively implanted MCS device was able to improve 30-day survival, whereas pre-operatively implanted ECLS tended to have a positive effect (P = .106). The post-operative need for a MCS device or escalation of MCS invasiveness (IABP upgrade to ECLS) was associated with a higher 30-day mortality (P = .001) compared with patients without any MCS device or those with pre-operatively implanted MCS devices. An independent risk factor for 30-day mortality was the interval between acute myocardial infarction and surgery <7 days (OR 5.895, CI 1.615-21.515; P = .007). Pre-operative implantation of ECLS for CS tends to improve the outcome of early primary surgical pVSD repair. The need for a post-operative MCS device is associated with a worse 30-day survival after early primary surgical pVSD repair.

Short- and Long-term Outcome After Interventional VSD Closure: A Single-Center Experience in Pediatric and Adult Patients.

Interventional closure of congenital ventricular septal defects (VSD) is recording a continuous rise in acceptance. Complete atrioventricular block (cAVB) and residual shunting are major concerns during follow-up, but long-term data for both are still limited. We retrospectively evaluated the outcome of patients with interventional VSD closure and focused on long-term results (> 1 year follow-up). Transcatheter VSD closures were performed between 1993 and 2015, in 149 patients requiring 155 procedures (104 perimembranous, 29 muscular, 19 residual post-surgical VSDs, and 3 with multiple defects). The following devices were used: 65 × Amplatzer™ Membranous VSD Occluder, 33 × Duct Occluder II, 27 × Muscular VSD Occluder, 3 × Duct Occluder I, 24 × PFM-Nit-Occlud®, and 3 × Rashkind-Occluder. The median age at time of implantation was 6.2 (0.01-66.1) years, median height 117 (49-188) cm, and median weight 20.9 (3.2-117) kg. Median follow-up time was 6.2 (1.1-21.3) years and closure rate was 86.2% at last follow-up. Complications resulting in device explantation include one case of cAVB with a Membranous VSD occluder 7 days after implantation and four cases due to residual shunt/malposition. Six (4%) deaths occurred during follow-up with only one procedural related death from a hybrid VSD closure. Overall, our reported results of interventional VSD closure show favorable outcomes with only one (0.7%) episode of cAVB. Interventional closure offers a good alternative to surgical closure and shows improved performance by using softer devices. However, prospective long-term data in the current era with different devices are still mandatory to assess the effectiveness and safety of this procedure.

Valve patch technique for repair of ventricular septal defect: long-term results.

OBJECTIVE: This study aimed to show the long-term results in patients who underwent unidirectional valve patch repair of ventricular septal defect with pulmonary artery hypertension. METHODS: Thirty-five acyanotic patients aged 2 to 26 years (mean 9.3 years) with a large ventricular septal defect and elevated pulmonary vascular resistance (mean 9.5 Wood units) underwent surgery in Madani Heart Hospital. The medical records and clinical outcomes were reviewed from March 1998 to March 2017. RESULTS: Five patients died in the first postoperative week. In the long-term follow-up (mean 11 years), two patients were lost to follow-up. Pulmonary artery hypertension gradually decreased in 17 patients within 6-12 months with significant improvement in right ventricular end-diastolic diameter, New York Heart Association functional class, and tricuspid regurgitation. Eleven patients with persistent pulmonary artery hypertension were divided into tolerable and non-tolerable groups. Six patients in the tolerable group had satisfactory conditions compared to before the operation, and gained weight with improved functional class despite echocardiographic findings of persistent elevated pulmonary artery pressure. One had a full-term delivery by caesarean section in the fifth postoperative year. Five patients in the non-tolerable group gradually developed right heart failure and complications such as extremity edema, ascites, pleural effusions, and died after 10-30 months. CONCLUSION: Although relatively high mortality occurred during long-term follow-up, surviving patients were in a better condition and functional class despite persistent pulmonary artery hypertension. Therefore, fear of persistent pulmonary artery hypertension should not prohibit surgery in this group of patients.

Single-stage repair for coarctation with ventricular septal defect: results of 100 cases at a single centre.

OBJECTIVES: The outcomes of the single-stage surgical repair of aortic arch hypoplasia (AAH) and/or coarctation of the aorta (CoA) associated with ventricular septal defect (VSD) remain controversial, especially in a lower middle-income country. This study reports the results of a single-stage repair protocol at our institution for AAH/CoA with VSD using selective cerebral perfusion. METHODS: This retrospective study included 100 consecutive patients who underwent single-stage repair via median sternotomy using selective cerebral perfusion for AAH/CoA with VSD from July 2010 to March 2017. RESULTS: The patients consisted of 65 males and 35 females. The median age of the patients was 67 days (range 4-2266 days); the median weight was 3.8 kg (range 2.1-15 kg). The average cardiopulmonary bypass time was 132 ± 28 min, the aortic cross-clamp time was 92 ± 23 min and the selective cerebral perfusion time was 33 ± 10 min. The survival rate of all patients was 94.7 ± 2.3%, with an in-hospital mortality of 5% and no late mortality at a median follow-up of 37 months (range 4-96 months). Four patients required reoperation due to recoarctation. The overall event-free survival rate following surgery was 87.1%. The median pressure gradient across the anastomosis at the last follow-up was 8.3 ± 2.8 mmHg. Multivariate logistic regression analysis revealed proximal aortic arch obstruction as a predictor of mortality (odds ratio = 3.8). The aortic isthmus diameter was identified as a predictor for reintervention by Cox regression (hazard ratio = 6.7). CONCLUSIONS: Single-stage repair for AAH/CoA with VSD is safe and feasible in a developing country.

Outcome of modified réparation à l'ètage ventriculaire (REV) based on anatomical characteristics for the anomalous ventriculoarterial connection with ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: The present study evaluated the results of the modified réparation à l'étage ventriculaire (REV) based on the individual anatomical and pathological findings of the patients with an anomalous ventriculo-arterial connection with ventricular septal defect (VSD) and left ventricular outflow tract obstruction. METHODS: We reviewed a series of 24 patients who underwent modified REV between 2005 and 2019. Surgical indications included ventricles and atrioventricular valves suitable for biventricular repair, severe left ventricular outflow tract obstruction (peak gradient >30 mmHg), unrestrictive subaortic VSD and coronary arteries not suitable for reimplantation. RESULTS: The mean follow-up time was 7.0 ± 4.2 years (range 0.5-14.1 years). Kaplan-Meier analyses showed that overall survival was 100% and freedom from any reoperation was 93.3% ± 6.4%. Longitudinal analyses of the available postoperative echocardiographic data showed that the left ventricular outflow tract peak gradient was less than 10 mmHg in all patients (15/15) and the left ventricular ejection fraction was more than 50% in 93.3% of patients (14/15). The right ventricular outflow tract peak gradient was less than 40 mmHg in 73.3% of patients (11/15). CONCLUSIONS: The REV remains an option for selected patients despite the increasing use in recent years of the Nikaidoh procedure and its modifications. The surgical strategy needs to be determined by the specific anatomical and pathological findings of the patient. The modified REV had excellent long-term survival and freedom from reoperation for the treatment of anomalous ventriculo-arterial connection with VSD and left ventricular outflow tract obstruction. The long-term performance of the reconstructed left ventricular outflow tract and right ventricular outflow tract is satisfactory.

Neonatal management of aortic coarctation with ventricular septal defect: a systematic review and meta-analysis.

INTRODUCTION: Neonatal management of aortic coarctation with ventricular septal defect is still under debate between the one-stage full repair by sternotomy versus the staged repair of the coarctation first by thoracotomy (with or without banding the pulmonary artery) followed later by subsequent closure of the ventricular septal defect. EVIDENCE ACQUISITION: The aim of this review was to synthesize the evidence in literature since 1980 for the neonatal population. A meta-analysis compared mortality between the two strategies. EVIDENCE SYNTHESIS: The analysis did not find a superiority of a strategy over the other regardless of the surgical era studied. Recoarctation rates of both strategies are presented and a management algorithm is suggested. CONCLUSIONS: Instead of comparing between the two strategies, a case-adapted management considering the anatomy of the ventricular septal defect and of the aortic arch is discussed to address this association of lesions though presenting with a wide range of settings.

Outcomes of infants and children undergoing surgical repair of ventricular septal defect: a review of the literature and implications for research with an emphasis on pulmonary artery hypertension.

BACKGROUND: Pulmonary vascular disease resulting from CHDs may be the most preventable cause of pulmonary artery hypertension worldwide. Many children in developing countries still do not have access to early closure of clinically significant defects, and the long-term outcomes after corrective surgery remain unclear. Focused on long-term results after isolated ventricular septal defect repair, our review sought to determine the most effective medical therapy for the pre-operative management of elevated left-to-right shunts in patients with an isolated ventricular septal defect. METHODS: We identified articles specific to the surgical repair of isolated ventricular septal defects. Specific parameters included the pathophysiology and pre-operative medical management of pulmonary over-circulation and outcomes. RESULTS: Studies most commonly focused on histologic changes to the pulmonary vasculature and levels of thromboxanes, prostaglandins, nitric oxide, endothelin, and matrix metalloproteinases. Only 2/44 studies mentioned targeted pharmacologic management to any of these systems related to ventricular septal defect repair; no study offered evidence-based guidelines to manage pulmonary over-circulation with ventricular septal defects. Most studies with long-term data indicated a measurable frequency of pulmonary artery hypertension or diminished exercise capacity late after ventricular septal defect repair. CONCLUSION: Long-term pulmonary vascular and respiratory changes can occur in children after ventricular septal defect repair. Research should be directed at providing an evidenced-based approach to the medical management of infants and children with ventricular septal defects (and naturally all CHDs) to minimise consequences of pulmonary artery hypertension, particularly as defect repair may occur late in underprivileged societies.

Repair of ischemic ventricular septal defect with and without coronary artery bypass grafting.

BACKGROUND AND AIM OF THE STUDY: Ventricular septal defect (VSD) following myocardial infarction (MI) is a relatively infrequent complication with high mortality. We sought to investigate the effect of concomitant coronary artery bypass graft (CABG) on outcomes following post-MI VSD repair. METHODS: Electronic search was performed to identify all relevant studies published from 2000 to 2018. Sixty-seven studies were selected for the analysis comprising 2174 patients with post-MI VSD. Demographic information, perioperative variables, and outcomes including survival data were extracted and pooled for systematic review and meta-analysis. RESULTS: Single-vessel disease was most common (47%, 95% confidence interval [CI], 42-52), left anterior descending coronary artery was the most commonly involved vessel (55%, 95% CI, 46-63), and anterior wall was the most commonly affected territory (57%, 95% CI, 51-63). Concomitant CABG was performed in 52% (95% CI, 46-57) of patients. Of these, infarcted territory was re-vascularized in 54% (95% CI, 23-82). A residual/recurrent shunt was present in 29% (95% CI, 24-34) of patients. Of these, surgical repair was performed in 35% (95% CI, 28-41) and transcatheter repair in 11% (95% CI, 6-21). Thirty-day mortality was 30% (95% CI, 26-35) in patients who had preoperative coronary angiogram, and 58% (95% CI, 43-71) in those who did not (P < .01). No significant survival difference observed between those who had concomitant CABG vs those without CABG. CONCLUSIONS: Concomitant CABG did not have a significant effect on survival following VSD repair. Revascularization should be weighed against the risks associated with prolonged cardiopulmonary bypass.

Prenatally Diagnosed Congenital Ventricular Outpouchings: An Institutional Experience and Review of the Literature.

Congenital ventricular outpouchings (CVOs) are rare congenital heart defects with limited data regarding prognosis and outcomes. We aimed to describe the characteristics, outcomes and factors associated with morbidity and mortality of prenatally diagnosed CVOs using our institutional experience and a review of published cases. A total of 86 cases of prenatally diagnosed CVOs were identified, including 3 from our institution and 83 cases identified from a review of the literature. Fetal and postnatal outcomes were analyzed for each case. Pericardial effusions (44%) and ventricular dysfunction (17%) were the most common associated findings. Excluding cases that resulted in pregnancy termination, mortality was 17%, with the majority (11/13) occurring in the prenatal period. Factors associated with mortality included an outpouching located on the left ventricle, a diagnosis of hydrops fetalis, the presence of a pericardial effusion, and an earlier gestational age at diagnosis. Of those that survived to delivery, 57% remained asymptomatic without the need for intervention, and the outpouching regressed or resolved in an additional 15%. Prenatally diagnosed congenital ventricular outpouchings are a dynamic form of congenital heart disease with a high fetal mortality rate. The outcomes associated with the outpouchings appear to be the most variable in the prenatal period and the first year after birth. Serial prenatal and postnatal evaluations should be performed to evaluate for a change in the characteristics of the outpouching.

Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect using intraventricular conduit.

OBJECTIVE: Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect is preferred, but previously developed surgical procedures are complicated and associated with high mortality and morbidity. We developed a technique using an intraventricular conduit to connect the ventricular septal defect and the aorta in this anomaly in patients aged more than 2 years. METHODS: Thirty-one patients (age 2-23 years; median, 5.4) with double-outlet right ventricle with noncommitted ventricular septal defect underwent biventricular repair with intraventricular conduit. A 16-mm or 19-mm polytetrafluoroethylene (Gore-Tex; WL Gore & Associates, Flagstaff, Ariz) vascular prosthesis was used to construct the intraventricular conduit rerouting the ventricular septal defect to the aorta, with enlargement of the ventricular septal defect and resecting the hypertrophic muscular bands in the bilateral conus when necessary. Follow-up was made in all patients with a median duration of 93 months (range, 8-140 months). RESULTS: One patient died during hospitalization and 1 patient died at 8 months after operation, making the mortality 6.5%. The peak pressure gradient across the left ventricular outflow tract was less than 30 mm Hg in all patients but 1 (3.3%). In the last patient, it increased from 16 mm Hg early after operation to 50 mm Hg at 7 years follow-up. The peak pressure gradient across the right ventricular outflow tract ranged from 6 to 30 mm Hg in all patients. One patient had moderate mitral regurgitation with New York Heart Association class II. One patient had preoperative severe pulmonary arterial hypertension (mean pressure, 50 mm Hg) and was treated with bosentan. Other patients were in New York Heart Association class I. CONCLUSIONS: Biventricular repair with intraventricular conduit is a relatively simple and safe procedure for patients aged more than 2 years with double-outlet right ventricle with noncommitted ventricular septal defect, with excellent early and midterm outcomes.

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections.

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

Biventricular repair versus Fontan completion for patients with d- or l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: D-transposition of the great arteries and l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction are complex biventricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biventricular procedures in these patients. METHODS: We analyzed 129 patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biventricular repair) undertaken between 1990 and 2015. RESULTS: Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biventricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. Biventricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biventricular groups. CONCLUSIONS: Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction and patients with l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction. Both Fontan and biventricular pathways are associated with excellent mortality and functional outcomes. Biventricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biventricular repair.

Pulmonary artery banding and venous bidirectional cava-pulmonary shunt for two-stage arterial switch procedure in late referral of patients with transposition of the great arteries and intact ventricular septum: midterm results.

OBJECTIVE: Two-stage arterial switch operation and left ventricle retraining are necessary for the patients with left ventricle dysfunction and transposition of great vessels with intact ventricular septum (TGA-IVS) who are referred late. MATERIAL AND METHODS: Forty-seven patients with the diagnosis of TGA-IVS and left ventricle dysfunction who underwent arterial switch operation in our centre between July 2013 and August 2017 were analysed retrospectively. The inclusion criteria for left ventricle retraining were patients older than 2 months of age at presentation, having an echocardiographic left ventricle mass index of less than 35 g/m², and having an echocardiographic "banana-shaped" left ventricle geometric appearance. The patients were divided into two groups: pulmonary artery banding and Blalock Taussig shunt were performed as the initial surgical procedure for later arterial switch operation in Group I (n = 19) and pulmonary artery banding and bidirectional cava-pulmonary shunt in Group 2 (n = 28). RESULTS: The average age was found to be 122.3 ± 45.6 days in Group I and 145.9 ± 37.2 days in Group II. There was no statistically significant difference (p = 0.232 versus p = 0.373) between the average left ventricle mass index of the two groups neither before the first stage nor the second stage (26.6 ± 4.8 g/m² versus 25.0 ± 4.9 g/m² and 70.5 ± 12 g/m² versus 673.8 ± 12.0 g/m², respectively). The average time interval for the left ventricle to retrain was 97.7 ± 42.9 days for Group I and 117.3 ± 40.3 days for Group II, significantly lower in Group I (p = 0.027). The time spent in ICU, length of the period during which inotropic support was required, and the duration of hospital stay were significantly higher in Group I (p<0.001, p < 0.001, and p < 0.00, respectively). CONCLUSION: Pulmonary artery banding and bidirectional cava-pulmonary shunt can be performed as a safe and effective alternative to pulmonary artery banding and arterial Blalock Taussig shunt for patients with TGA-IVS in whom arterial switch operation is needed beyond the neonatal period. This approach involves a shorter hospital stay and fewer post-operative complications.