Multicentre study on late outcomes of biventricular repair of double outlet right ventricle.

OBJECTIVES: The goal of this retrospective multicentre study was to present late surgical outcomes of the treatment of children with double outlet right ventricle (DORV) coming from emerging countries. METHODS: The Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated on in 9 hospitals that specialize in paediatric cardiac surgery. Data are collected from the Mécénat Chirurgie Cardiaque comprehensive database, with a strict postoperative follow-up. The patients included only those who had biventricular repair of DORV with 2 viable ventricles. According to the classification of the Eleventh Revision of the International Classification of Diseases, DORV was defined as a congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle. RESULTS: From January 1996 to January 2022, a total of 81 consecutive DORV biventricular repair operations were performed. There were 6 subtypes of DORV divided into 2 groups: DORV-committed ventricular septal defect (VSD): DORV-VSD (n = 25), DORV-Fallot (n = 34), DORV-transposition of the great arteries (n = 5); and DORV-non-committed (nc) VSD: DORV-ncVSD-no pulmonary stenosis (PS) (n = 7), DORV-ncVSD-PS (n = 5) and DORV-atrioventricular septal defect (AVSD)-PS (n = 5). Four Fontan patients were excluded. Three patients were lost to follow-up (3.4%). The overall perioperative mortality was 7.4% ± 2.6%, 6/81 (95% confidence interval: 2.8%-15.4%) ranging from 0% in DORV-AVSD-PS to 14% for DORV-ncVSD-no PS. The overall 10-year survival was 86%. The early mortality of DORV-ncVSD at 5.9% ± 2.4% (1/17) was similar to that of DORV-committed VSD at 7.8% ± 2.7% (5/64) (P = 0.79). There was a trend towards an optimal outcome for the arterial switch operation and the DORV-AVSD-PS repair. VSD enlargement was significantly more frequent in DORV-ncVSD at 42% (5/12) (P = 0.001). There were low numbers in the complex groups. The number of Fontan cases was noticeably low. The aorta located entirely on the right ventricle represents the fundamental anomaly and the surgical challenge of DORV. CONCLUSIONS: Overall survival at 10 years was 86%. This study shows a trend towards satisfactory early and late outcomes in BVR of simple DORV with committed VSD, compared to complex DORV with ncVSD.

Surgery for severe congenital heart diseases in children from developing nations.

BACKGROUND: Children with severe congenital heart disease (CHD) are rarely treated in developing countries and have very little to no chance to survive in their local environment. Mécénat Chirurgie Cardiaque (MCC) flies to France children with CHD from developing countries. This report focuses on the early, mid, and late outcomes of 531 children with severe CHD sent to MCC for surgery from 1996 to 2019. METHODS: The inclusion criteria were based on diagnosis and not on procedure. MCC is present in 66 countries and has developed a robust staff, including 12 permanent employees and 700 volunteers, with 350 host families based in France, 120 local correspondents, and 100 local physicians. Since 1996, MCC has organized a basic training of local pediatric cardiologists yearly, offering a free 1-month training course. Over time, MCC could count on a pool of doctors trained in basic pediatric cardiology. Flights were secured by the Aviation Sans Frontieres Foundation. Nine French centers performed the surgeries. A robust follow-up was conducted in all the nations where MCC operates. RESULTS: The most frequent pathologies were single ventricle (n = 126), double-outlet right ventricle (n = 116), pulmonary atresia with ventricular septal defect (n = 68), transposition of the great arteries with ventricular septal defect and transposition of the great arteries with intact ventricular septum (n = 61), arterial trunk (n = 39), transposition of the great arteries with ventricular septal defect and left ventricle outflow tract obstruction (n = 35), complete atrioventricular septal defect (n = 18), congenitally corrected transposition of the great arteries (n = 16), and so on. The median age was 5.4 years (range, 1 month-26 years). The mean perioperative mortality was 5.5% (29 out of 531) (95% confidence limit, 3.5%-7.4%). The follow-up was 91.3%, with a mean follow-up of 5.1 years. The global actuarial survival at 5, 10, and 15 years was, respectively, 85%, 83%, and 74%. There was a significant higher late mortality for patients surviving only with a Blalock-Taussig shunt (P = .001). CONCLUSIONS: Operating on 531 children with severe CHD from developing nations was achieved with satisfactory early and long-term results. Children with severe CHD are rarely operated on in developing nations. Programs like MCC's offer a viable option to save these children born with severe CHD.

A rare cause of wheezing in a child with pulmonary atresia.

The determination of the exact cause for symptomatic airway obstruction in pediatric patients not responding to medication can be a clinical dilemma. Very rarely external vascular compressions can produce airway obstruction symptoms unresponsive to usual bronchodilator medications. The successful management of a child with pulmonary atresia and an innominate artery compression syndrome with respiratory compromise due to tracheal compression is described.

How Could Aortic Atresia With Interrupted Aortic Arch Survive? About a Neonatal Repair on Two Ventricles.

Aortic atresia with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery. Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an aorto-pulmonary window (AP window), an aberrant right subclavian artery or a bilateral patent ductus arteriosus (PDA). We report a case of aortic atresia with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.

Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era.

BACKGROUND: The optimal management and prognostic factors of postsurgical pulmonary vein stenosis remain controversial. We sought to determine current postsurgical pulmonary vein stenosis outcomes and prognostic factors in a multicentric study in the current era. METHODS: Seventy-five patients with postsurgical pulmonary vein stenosis who underwent 103 procedures in 14 European/North American centers (2000-2012) were included retrospectively. A specific pulmonary vein stenosis severity score was developed on the basis of the assessment of each pulmonary vein. End points were death, pulmonary vein reintervention, and restenosis. A univariate and multivariate risk analysis was performed. RESULTS: Some 76% of postsurgical pulmonary vein stenosis occurred after repair of a total anomalous pulmonary venous return. Sutureless repair was used in 42 of 103 procedures (41%), patch veinoplasty was used in 28 procedures (27%), and endarterectomy was used in 16 procedures (16%). Overall pulmonary vein restenosis, reintervention, and mortality occurred in 56% (n = 58/103), 49% (n = 50/103), and 27% (n = 20/75), respectively. Sutureless repair was associated with less restenosis (40% vs 67%; P = .007) and less reintervention (31% vs 61%; P = .003). Mortality after sutureless repair (20%; 7/35) tends to be lower than after nonsutureless repair (33%; 13/40) (P = .22). A high postoperative residual pulmonary vein stenosis score at the time of hospital discharge was an independent risk factor for restenosis (hazard ratio [HR], 1.55; P < 10-4), reintervention (HR, 1.33; P < 10-4), and mortality (HR, 1.37; P < 10-4). The sutureless technique was an independent protective factor against restenosis (HR, 0.27; P = .006). CONCLUSIONS: Postsurgical pulmonary vein stenosis still has a guarded prognosis in the current era. The sutureless technique is an independent protective factor against restenosis. The severity of the residual disease evaluated by a new severity score is an independent risk factor for poor outcomes regardless of surgical technique.

Pulmonary Hemorrhage After Arterial Switch Operation.

Major aortopulmonary collateral arteries can influence the postoperative course of arterial switch operation, with heart failure being the common clinical presentation. A relatively rare presentation of an aortopulmonary collateral artery in the form of persistent postoperative pulmonary hemorrhage was encountered after an uneventful arterial switch operation in a neonate with transposition of the great arteries, intact interventricular septum, and situs inversus totalis. The aortopulmonary collateral artery was coil embolized with a successful outcome.

Initial 2-year results of CardioCel® patch implantation in children.

OBJECTIVES: We present the initial 2-year results of CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) implantation in paediatric patients with congenital heart diseases. METHODS: This was a single-centre retrospective study with prospectively collected data of all patients aged 18 years and under operated for congenital heart disease. The patch was introduced in 2014, with clinical practice committee approval and a special consent in case of an Ozaki procedure. Standard follow-up was performed with systematic clinical exams and echocardiograms. In case of reoperation or graft failure, the patch was removed and sent for a histological examination. RESULTS: Between March 2014 and April 2016, 101 patients had surgical repair using a CardioCel patch. The mean age was 22 (±36.3) months, and the mean weight was 9.7 (±10.3) kg. No infections and no intraoperative implantation difficulties were associated with the patch. The median follow-up period was 212 (range 4-726) days. The overall 30-day postoperative mortality was 3.8% (n = 4), none of which were related to graft failure. Five children were reoperated because of graft failure, 4 of whom had the patch implanted for aortic and were aged less than 10 days. The indications for patch implantation in the aortic position were aortopulmonary window, truncus arteriosus, coarctation and aortic arch hypoplasia repair. The median time between the first and the second operation for graft failure was 245 (range 5-480) days. CONCLUSIONS: Our experience shows that the patch is well tolerated in the septal, valvar and pulmonary artery positions. However, we experienced graft failures in infants in the aortic position.

Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study.

BACKGROUND: Primary pulmonary vein stenosis (PPVS) still carries a poor prognosis, and prognostic factors remain controversial. The aim of this study was to determine outcomes and prognostic factors after PPVS repair in the current era. METHODS: Thirty patients with PPVS and a normal pulmonary vein (PV) connection operated on in 10 European/North American centers (2000-2012) were included retrospectively. A specific PVS severity score was developed based on the assessment of each PV. Studied end points were death, PV reoperation, and restenosis. A univariate and multivariate risk analysis was performed. RESULTS: The mean number of affected PVs per patient was 2.7 ± 1.1. Sutureless repair was used in 21 patients (70%), endovenectomy was used in 5 patients, and patch venoplasty was used in 4 patients. Overall PV restenosis, reoperation, and mortality occurred in 50%, 40%, and 30% of patients respectively. Freedom from mortality, reoperation, and restenosis at 8 years of follow-up was 70% ± 8%, 62% ± 8%, and 47% ± 9%, respectively. Restenosis and mortality rates after sutureless repair versus nonsutureless repair were 57% (n = 12 of 21) versus 33% (n = 3 of 9) (p = 0.42) for restenosis and 38% (n = 8 of 21) versus 11% (n = 1 of 9) (p = 0.21) for mortality. Patients selected for a sutureless technique were younger and smaller and had more severe disease before operation. A postoperative high PVS score and pulmonary hypertension 1 month after the operation were independent risk factors for restenosis (hazard ratio [HR], 1.34; p = 0.002 and HR, 6.81; p = 0.02, respectively), reoperation (HR, 1.24; p = 0.01 and HR, 7.60; p = 0.02), and mortality (HR, 1.39; p = 0.01 and HR, 39.5; p = 0.008). CONCLUSIONS: Primary PVS still has a guarded prognosis in the current era despite adoption of the sutureless technique. Postoperative pulmonary hypertension and severity of disease evaluated by a new severity score are independent prognostic factors regardless of surgical technique.

Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair.

OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage. METHODS: Three patients presenting with situs solitus and extreme leftward displacement of the septum primum were included. All of the pulmonary veins drained anomalously into the anatomical right atrium, and the distance between the mitral valve and the abnormal septum primum was greatly reduced, compromising the size of the left atrial chamber, but with normal left ventricle diameters. Preoperative 3D echocardiographic findings are reported. We achieved a biventricular surgical repair in all cases. The atrial septation was accomplished using an autologous pericardial patch after removing the abnormal septal membrane. RESULTS: The postoperative course was free from any cardiovascular complications. Echocardiographic scans showed a harmonious reconstruction without pulmonary venous obstructions or stenosis. CONCLUSIONS: This article reports the severe leftward displacement of the septum primum presented as an isolated cardiac malformation; 3D transthoracic echocardiography allowed an accurate diagnosis of this malformation and helped in choosing the best surgical strategy.

The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2017 Update on Outcomes and Quality.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%).

Transient Electromechanical Dissociation Caused by a Transesophageal Echocardiography Probe.

Intraoperative transesophageal echocardiography is an established modality for confirming the preoperative diagnosis, assess adequacy of surgical repair, identify residual problems, and help improve the outcome after operation. Despite the multitude of advantages, intraoperative transesophageal echocardiography can affect the hemodynamic parameters in small neonates and children. A severe transient electromechanical dissociation caused by the anteflexion of a transesophageal echocardiography probe during a total anomalous pulmonary venous connection repair in an infant is reported.

The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2016 Update on Outcomes and Quality.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2011 to December 2014 and documenting overall discharge mortality (interquartile range among programs with more than 9 cases): off-bypass coarctation, 1.0% (0.0% to 0.9%); ventricular septal defect repair, 0.7% (0.0% to 1.1%); tetralogy of Fallot repair, 1.0% (0.0% to 1.7%); complete atrioventricular canal repair, 3.2% (0.0% to 6.5%); arterial switch operation, 2.7% (0.0% to 5.6%); arterial switch operation plus ventricular septal defect, 5.3% (0.0% to 6.7%); Glenn/hemiFontan, 2.1% (0.0% to 3.8%); Fontan operation, 1.4% (0.0% to 2.4%); truncus arteriosus repair, 9.6% (0.0 % to 11.8%); and Norwood procedure, 15.6% (10.0% to 21.4%).