Right ventricular (RV) dysfunction is a major determinant of long-term survival in congenital heart diseases. Early echo detection of RV failure is mandatory, but recent indices need to be validated. Objectives were to: (1) validate standard and strain echo indices for evaluation of RV systolic function, compared to hemodynamic parameters; (2) assess the accuracy of these indices for early detection of RV failure. Combined RV overload as observed in repaired tetralogy of Fallot was surgically reproduced in 2-month-old piglets (n=6). Age-matched piglets were used as controls (n=4). RV function was evaluated at baseline and 4 months of follow-up by standard and strain echo indices, compared to conductance catheter. Sarcomere shortening and calcium transients were recorded in RV isolated myocytes (IonOptix). Contractile reserve was assessed by in-vivo (dobutamine 5µg/kg) and ex-vivo (isoprenaline 100nM) β adrenergic stimulation. The integrity of T-tubules was controlled after Di-4-Anepps labeling. 4 months after surgery, hemodynamic RV ejection fraction (FEVD) was significantly decreased (29.7% [26.2-34] vs 42.9% [40.7-48.6], p<0.01), and inotropic responses to dobutamine were blunted (contractile reserve ΔEmax=51% vs 193%, p<0.05). On echocardiography FAC, TAPSE, S’ peak and RV free wall longitudinal strain rate were significantly reduced and correlated with FEVD. Peaks strain rate and S’ were correlated with ΔEmax (r=0.75 and 0.78, p<0.05). Isolated RV myocytes from operated animals showed hypertrophy, decreased sarcomere shortening peak in response to isporenaline (ΔL=7.8±2.8% vs 10.7±2.9%, p<0.05), and increased spontaneous calcium waves suggesting perturbations of calcium homeostasis. In this model, both standard and strain echo indices allowed the detection of early impairments of RV function and cardiac reserve, which are associated with cardiac excitation-contraction coupling alterations.

Background: Morphology of the left ventricular outflow tract (LVOT) in atrioventricular septal defects (AVSDs) has been reported to be at risk for development of obstruction. The purpose of the present study was to identify the incidence, the risk factors, and the surgical outcomes of subaortic stenosis in repaired AVSDs. Methods: Records of 427 consecutive patients who underwent anatomical repair for all types of AVSDs from January 2000 to December 2012 were reviewed. Outcomes, independent risk factors, reoperation, and death were analyzed. Results: In a median follow-up of five years (range: 17 months11.8 years), eight patients required nine reoperations for subaortic stenosis. Study group (n = 11) included three additional patients for whom repair was performed in a different institution. Median delay for reoperation was 11.9 years (range: 1.319.4 years). Surgical relief of subaortic stenosis was obtained by means of the enlargement of the LVOT: resection of fibrous structures in all reoperated patients and associated with septal myectomy in four. Two patients required a modified Konno procedure. Five reoperations were associated with left atrioventricular valve repair or replacement. Statistical analysis didn't reveal any morphologic or demographic risk factors. No early or late death occurred. Conclusion: The development of subaortic stenosis after repair of AVSD remains a rare complication in midterm follow-up (incidence: 1.9%). The performance of preventive gesture during repair seemed to be unjustified. The surgical management of this reoperation consists of a simple and safe procedure.

Background. - Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) or duct-dependent pulmonary valve stenosis (DD-PVS) has become a reasonable alternative to surgical right ventricle decompression. Aim. - To investigate mid-term outcomes following pulmonary valvuloplasty. Methods. - Sixty-five neonates with PA-IVS (n = 29) or DD-PVS (n = 36) (median age 4 days; mean weight 3.0 kg) undergoing pulmonary valvuloplasty were reviewed retrospectively. Procedural data and clinical outcomes were assessed. Results. - Pulmonary valvuloplasty was successful in 59 patients (90.8%). Preterm birth, larger tricuspid valve annulus diameter and PA-IVS correlated with procedural failure. Eleven patients (18.6%) required a Blalock-Taussig shunt during early follow-up, despite valvuloplasty. These neonates had smaller tricuspid and pulmonary valve annulus Z-scores (-1.9 vs. 0.8 [p=0.04] and -2.5 vs. -0.9 [P= 0.005], respectively) and a higher incidence of "bipartite" right ventricle (P= 0.02). Mean follow-up was 5.4 +/- 3.3 years. Mortality after successful valvuloplasty was 8.5% (n= 5). Among the 54 survivors, biventricular repair was achieved in 52 patients (96.3%), including nine with a previous Blalock-Taussig shunt. The cumulative rate of subsequent surgery (excluding Blalock-Taussig shunt) was 13.7% (95% confidence interval 6.8-26.7%) and 16.4% (95% confidence interval 8.5-30.4%) at 2 and 4 years, respectively. Secondary surgery was significantly more frequent in PA-IVS compared with DD-PVS, and in neonates with a Blalock-Taussig shunt (P= 0.003 and 0.01, respectively). Conclusions. - Selected neonates with DD-PVS or PA-IVS managed by transcatheter pulmonary valvuloplasty had a good mid-term outcome. In neonates with a borderline small right ventricle, a hybrid strategy with a supplementary source of pulmonary blood flow can be efficient to achieve biventricular repair. (C) 2019 Elsevier Masson SAS. All rights reserved.

BACKGROUND:The surgical reconstruction of right ventricle outflow tract (RVOT) often requires the implantation of a valved conduit. Homografts are lacking availability and are associated with limited durability in children. Our experience with the Hancock porcine-valved Dacron (DuPont, Wilmington, DE) conduit (Medtronic, Minneapolis, MN) was retrospectively assessed. METHODS:Follow-up was studied in 214 survivors who underwent 247 conduit implants between January 1990 and January 2007. Pulmonary atresia/ventricular septal defect was present in 86 (40.2%) and truncus arteriosus in 62 (29%). Conduit implantation was associated with anatomic repair in 136, conduit replacement in 96, and secondary pulmonary valve insertion in 15. Median age at operation was 62.5 months (range, 1 week to 50 years), including 14 neonates (6%). Median conduit size was 17.4 mm because of routine over-sizing. Pulmonary bifurcation patch augmentation was necessary in 26 patients. Periodic echocardiography studies were performed for a median follow-up of 98 months (range, 13 to 142 months). RESULTS:Three (1.4%) late deaths occurred. No conduit-related deaths or complications occurred. Conduit degeneration was associated with increase in valvular gradient. Valve regurgitation was absent or mild. Higher RVOT systolic pressure gradient at discharge did not influence conduit longevity. Conduit reoperation was delayed due to percutaneous balloon dilatation in 14 patients, associated with stenting in 7. Survival with freedom from conduit reoperation was 98% (95% confidence interval [CI], 97% to 100%) at 1 year, 81% (95% CI, 75% to 87%) at 5 years, and 32% (95% CI, 22% to 42%) at 10 years. CONCLUSIONS:The Hancock valved conduit is a safe and reliable alternative to homografts. It appears to be appropriate in patients with limited pulmonary vascular bed and high pulmonary artery pressures. Caution is required in neonates because of the rigidity of the Dacron housing. Initial results with secondary percutaneous procedures are encouraging.

Background. Repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) carries a high risk of operation, particularly in those with poor left ventricular function. In this study, we assessed the outcomes of patients who presented with severe preoperative left ventricular dysfunction (shortening fraction <15%) who underwent the repair under normothermic bypass.Methods. Since September 2002, 21 infants with severe left ventricular dysfunction underwent surgical repair using a direct coronary reimplantation technique. Mean age of patients was 5.3 +/- 3.8 months. Moderate or severe mitral regurgitation present in 5 patients was not addressed at the operation. Repair was performed under normothermic cardiopulmonary bypass and myocardial protection was achieved by intermittent antegrade normothermic blood cardioplegia.Results. Mean cardiopulmonary bypass and cross-clamp times were 153.5 +/- 51 and 50 +/- 14.5 minutes, respectively. There was one (4.7%) early death because of sudden cardiac arrest 48 hours after successful weaning from mechanical ventilation. One patient required urgent left coronary artery stenting on the 34th postoperative day because of early reimplantation failure. There was no late death. Echocardiographic evaluation revealed a significant improvement in terms of shortening fraction (preoperative, 10.3 +/- 3.2% vs follow-up, 33 +/- 7.7%, p < 0.0001).Conclusions. Our results suggest that the repair of anomalous origin of the left coronary artery from the pulmonary artery can be accomplished with acceptable mortality and morbidity rates even in patients having severely depressed left ventricular function. Normothermic cardiopulmonary bypass is safe and effective in children who have a limited tolerance to further ischemic insult. (Ann Thorac Surg 2010;90:856-61) (C) 2010 by The Society of Thoracic Surgeons

Objective: Postoperative left ventricular outflow tract obstruction (LVOTO) after arterial switch operation (ASO) is rare. In this retrospective study, we reviewed the cases of 10 patients with LVOTOs post-ASO and analyzed the options used for the LVOTOs corrections and the patients' outcomes. Methods: From December 1982 to December 2006, 1689 consecutive ASO were performed. Ten patients presented with postoperative LVOTOs (0.59% of all ASO and 7.62% of ASO for Taussig-Bing anomaly (TBA)) leading to reoperations. Before ASO they presented with significant subaortic gradients (n = 4) or predisposing anatomical features (n = 9) such as: conal septum malalignment (7/9), abnormal tricuspid cords insertions (4/9), muscular bundle hypertrophy (3/9) and mitral accessory tissue (2/9). Results: No patient was lost in the follow-up: 117 +/- 33 months. Subaortic gradients had developed between 5 months and 14 years after ASO (mean 41 +/- 35 months) leading to reoperations. Most LVOTOs post-ASO were due to fibrotic membranes or fibro-muscular hypertrophy, removed at first reoperation. After their first reoperations, seven patients (70%) had non-significant or low gradients and were not reoperated for LVOTO. Three patients underwent iterative surgical procedures for LVOTOs leading to two aortic valve replacements associated with LVOTO release or Konno procedure. Conclusion: Postoperative LVOTO after ASO is rare but happens more frequently in TBA. Most of them can benefit from resections of subaortic obstacles or septal plasties. In more complex cases iterative surgical procedures may lead to complications such as block or severe aortic valve regurgitation. (c) 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

OBJECTIVE:In presence of adequate pulmonary blood flow, patients presenting with unoperated or palliated pulmonary atresia with ventricular septal defect (PA/VSD) can reach adult age. However, they remain symptomatic with a limited life expectancy. METHODS:Since 1993, 27 patients underwent surgery for unrepaired PA/VSD. Median age was 20 (range: 15-43) years. Nineteen patients had 33 previous palliative procedures while eight were unoperated survivors. Major aortopulmonary collateral artery (MAPCA) had been observed in all but 2 and were still patent in 23. All bronchopulmonary segments were connected to the native pulmonary arteries (NPA) in 4 (type A), to both NPA and MAPCA in 18 (type B) and only to MAPCA in 5 (type C). The biventricular repair was performed in 17 patients: 3 type A, 12 type B and 2 type C. Ten patients underwent palliative procedure: eight aortopulmonary shunt, with unifocalisation in two and one right ventricle to NPA restrictive conduit. RESULTS:One (4%) hospital death occurred following the failure of a palliative procedure. No clinical improvement was observed in seven patients including one repaired and six palliated survivors. Two late cardiac death occurred 1 and 7 years after repair. At last visit, 15 of 16 repaired survivors were in NYHA class I or II. Only one patient awaits septation, while eight other with subsequent palliation were considered not repairable. CONCLUSION:The outcome was encouraging in patients who were eligible for completed biventricular repair. Although considered as unique alternative to cardiopulmonary transplantation, the justification for palliative surgery to improve pulmonary blood flow remains to be established.

Truncus Arteriosus (TA) is a rare congenital cardiac malformation, firstly described by Wilson in 1798 and reported with an incidence between 0.006 and 0.043 per 1,000 live births. It accounts for 0.7% of all congenital cardiac malformations [1] and for 1–2% of congenital heart diseases identified at autopsies. This disease occurs with equal frequency in male and female gender and it has no racial preference.