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Objective Right ventricular outflow tract reconstruction is a common feature for the treatment of congenital heart disease. However, the best substitute for pulmonary valvulation is still unknown, since the whole valves impair in above ten years. Pulmonary homograft appears to be the best substitute, but the lack of availability of this substitute, especially the low diameter, conducted to the development of other valves, biological or synthetic. The Contegra® valve is a bioprosthesis obtained from bovine jugular vein. Available in diameters from 12 to 22 mm, these conduits should constitute a good alternative to the homograft, especially concerning low weight children. The objective of this study was to describe the long-term evolution of Contegra® in our institution. Methods We conducted a retrospective monocentric observational study at the CHRU of Tours including all patients implanted with a Contegra® valve between January 2001 and December 2018. Results Over this 17-year period, 58 Contegra® implanted in 52 patients were included (average age 6.7 ± 5.4 years at valvulation). Survival without revision surgery for conduit failure was 87% (95 CI {79–97}) at 2 years, 67% (95 CI {54–84}) at 5 years and 60% (95 CI {43–82}) at 10 years ( Fig. 1 ). A lower age at implantation, a smaller diameter, and the occurrence of infective endocarditis was significantly associated with the revision surgery. Only 5 patients had infectious endocarditis over this period. Endocarditis-free survival was 94% (95 CI {88–100}) at 2 years, 92% (95 CI {84–100}) at 5 years and 92% (95 CI {84–100}) at 10 years. Conclusion Our study suggests that Contegra® valve could constitute an attractive substitute for right ventricular outflow tract reconstruction. A multicenter study comparing long-term evolution of the whole valves currently available for pulmonary valvulation could confirm our results.

Background Branch pulmonary artery (BPA) stenosis is frequently associated with congenital heart disease. Management of BPA stenosis is challenging for surgeons due to a high rate of recurrence. The purpose of this study was to assess the results of intraoperative pulmonary artery stenting associated with or without surgical angioplasty. Methods We included 33 children from our center between January 2008 and July 2014. Patients had pulmonary atresia with ventricular septal defect (13), tetralogy of Fallot (10), troncus arteriosus (4), double outlet right ventricle (2), and single left or right ventricle (4). A total of 44 balloon-expandable stents (mean diameter, 9.5 mm; range, 4 to 16 mm) were deployed in left or right PA under direct visualization, without the use of fluoroscopy, after branch angioplasty for 28 of them (64%). The mean age at surgery was 4.3 ± 4.3 years (range, 6 days to 15 years) and the mean weight was 14.3 ± 11.9 kg (range, 2.8 to 63 kg). Results Postoperative mortality was 9% (3 patients), but only 1 death was related to the stenting procedure. Twenty-five patients underwent angiographic control after a mean follow-up of 22 months after surgery. All stents were well positioned. The mean stented BPA Z-score increased from –2.6 ± 1.8 to –0.4 ± 1.6 ( p 0.0001). Eleven patients experienced intrastent proliferation (44%). Among them, 2 patients required a reoperation for severe intrastent stenosis, whereas the 9 others had mild intrastent neointimal proliferation, which was successfully managed by balloon expansion. Conclusions Intraoperative stenting of BPA is a safe and effective option to treat BPA stenosis and prevent recurrence.

Introduction Primary or secondary branch pulmonary artery (BPA) stenosis complicates the management of congenital heart diseases. Surgical pulmonary plasty is the gold standard treatment, but is associated with a low freedom from reintervention rate of 46% at 10 years [1] . As an alternative, percutaneous or intraoperative stents have been implanted to improve efficiency, but limited data are available concerning longterm outcome [2] , [3] , [4] , [5] . We hypothesized that prognosis of intraoperative or percutaneous stent implantation in BPA stenosis is good with further re-expansion and limited complications. Methods We conducted a retrospective cohort study at CHU de Tours. All stents implanted by surgery or catheterization in BPA with a minimum follow-up of 12 months and at least 1 catheterization control have been included. The primary endpoint was composite, combining cardiovascular mortality, surgical stent removing or percutaneous implantation of a new homolateral stent. Results Between February 2007 and December 2017, 76 stents in 51 patients were included (62 stents implanted by surgery, 14 by catheterization). At the time of implantation, patients had mean age and weight of 56.3 months (IQR 65.4) and 17.4 kgs (IQR 11.0) respectively. There was 68.4% of secondary stenosis. Mean BPA minimum size was 4.1 mm (mean Z-score of − 5.0), and mean initial stent diameter was 9.1 mm. During a mean follow-up of 5.3 years (range 0–11,2 years), freedom from primary endpoint was 86.8% (CI 79.6–94.8%) at 1 year, 78.9% (CI 70.2–88.6%) at 2 years, 71.5% (CI 61.9–82.7%) at 5 years and 69.6% (CI 59.6–81.2%) at 10 years ( Fig. 1 ). Among surviving stents, mean BPA size Z-score at last evaluation was increase of +4.69 compared to initial size (P Fig. 2 ). A lower BPA size at implantation seemed to be associated with a worse outcome of the stent (P Conclusion Our results suggest that percutaneous or intraoperative stent implantation could constitute a good alternative to BPA plasty alone.

Rhabdomyomas are the most common benign cardiac tumors. They may often be associated with tuberous sclerosis. In many cases, cardiac rhabdomyomas undergo spontaneous regression. Here, we report the unusual case of an infant with a large nonobstructive right ventricular rhabdomyoma at birth and at four months of age, which subsequently caused severe right ventricle outflow tract obstruction at six months of age, prompting surgery to remove the tumor. Close monitoring should be done in infants with large nonobstructive cardiac rhabdomyomas.

BACKGROUND Several publications have considered results of percutaneous angioplasty for aortic recoarctation, but none focused on procedures performed in children aged

Background Hepatic fibrosis increasing over time after a Fontan procedure is well documented, but the others potential risk factors of the severity of hepatopathy are still lacking. Method Since 2016, we started a systematic hepatic evaluation of patients undergoing a total cavo-pulmonary connection (TCPC) for at least 10 years, including clinical features, blood sample, abdominal ultrasound, liver elastography and liver biopsy. We look for clinical, biological and hemodynamic factors potentially associated with greater degree of fibrosis at biopsy. Results Fourteen patients had a complete evaluation with liver biopsy. Median time from TCPC to hepatic assessment was 15.9 years (range 3.0–25.9). Seventy one percent had biological cholestasis, and only 14% had cytolysis. Abdominal ultrasound revealed hepatomegaly in 4 patients and splenomegaly in 3, but no liver nodularity. METAVIR score of liver fibrosis at biopsies showed pre-cirrhotic or cirrhotic lesions (F3–4) in 9 patients out the 14 (F1 = 3; F2 = 2; F3 = 5; F4 = 4). The degree of fibrosis was significantly correlated to inferior vena cava pressure (IVCP) obtained by cardiac catheterization 1.4 years (range 0–3.9) after the Fontan procedure (r = 0.57, P = 0.05), and to IVCP*time from Fontan operation (r = 0.60, P = 0.03), and tended to be correlated with intrahepatic pressure recorded during the liver biopsy, but without statistical significance (r = 0.44, P = 0.11). There was no association between fibrosis and gender, age, age at the Fontan operation, type of systemic ventricle, functional status, liver stiffness at elastography, and blood markers of hepatic function. Conclusion In this study, two-thirds of patients undergoing TCPC experienced severe liver fibrosis, correlated with early measurement of IVCP and time from Fontan after the Fontan completion. Reduction of pulmonary pressure in this population should be considered to decrease the risk of severe fibrosis or cirrhosis.

Objective Percutaneous branch Pulmonary Artery (PA) stenting can be challenging, especially when associated with stenosis of the Right Ventricular Outflow Tract (RVOT), after previous angioplasty or with complexity of PAs branches. Surgical patch enlargement of Pas alone can be disappointing in these situations. A hybrid-approach, with PA stenting during surgery, may be an alternative to optimize surgical management in complex pulmonary artery stenosis.

Objective The main objective was to evaluate the safety and the efficacy of balloon angioplasty of post-surgical aortic recoarctation before one year of age. The secondary objective was to identify factors potentially associated with a failure of the procedure. Method Data from children who have one or more balloon angioplasty for aortic recoarctation before one year of age were retrospectively collected from 2006 to 2014. Results 14 children (17 procedures) have been included. The procedure immediately failed in 2 patients who underwent a new surgery. All the other 12 patients still remained free of recoarctation after one procedure for 10 of them, 2 procedures for another one and 3 procedures for the last one. Three complications due to the procedure occurred: one iliac artery thrombosis, one coronary embolism and one transitory stroke. These complications totally recovered before discharge. No aortic dissection or aneurysm has been reported. Comparing the 5 procedures which need a further intervention with the others, we observed that a higher peak gradient measured during the catheterization before (45mmHg (range 36-60) vs 32mmHg (range 10-47)) and after the angioplasty (36mmHg (range 30-50) vs 25mmHg (range 5-50)), and a lower ratio balloon size/transverse arch diameter (1.07 (range 0.88-1.20) vs 1.28 (range 0.94-1.82)) were associated with a failure of the procedure. Conclusion Balloon angioplasty of aortic recoarctation before one year of age is efficient. A lower ratio balloon size/transverse arch diameter was associated with a higher failure of the procedure that suggests the use of bigger balloon.

The second-generation Carpentier-Edwards bioprostheses, the supra-annular porcine valve and the PERIMOUNT pericardial valve, have been evaluated longitudinally for several years. This study compared clinical performance over 15 years. Aortic valve replacement was performed with a supra-annular porcine valve in 1,823 patients (group 1) aged 19–89 years (mean, 68.9 ± 10.9 years) and with a PERIMOUNT pericardial bioprosthesis in 1,430 patients (group 2) aged 16–90 years (mean, 69.5 ± 10.4 years). The groups were similar except for concomitant coronary artery bypass in 43% of group 1 and 18% of group 2 ( p < 0.001). Overall survival at 15 years was 29.3% ± 1.5% for group 1 and 35.2% ± 3.1% for group 2 ( p = 0.0009). The actual freedom from valve-related mortality was 88.5% ± 0.9% for group 1 and 84.9% ± 1.7% for group 2. The actual freedom from structural valve deterioration at 15 years was similar overall, and for patients aged > 60 years, between the groups, but was dissimilar (group 2 > group 1) for age ≤ 60 years. The predictors of structural valve deterioration were valve type (group 1 > group 2), sex (male > female), age, and concomitant coronary artery bypass. Both bioprostheses provided satisfactory clinical performance at 15 years after aortic valve replacement.

International audience; BACKGROUND: Morbidity and mortality are higher for cardiac reoperations than first operation due to the presence of post-operative adhesions. We retrospectively evaluated the efficacy of the bioresorbable membrane Seprafilm to prevent pericardial adhesions after cardiac surgery in a paediatric congenital heart disease population. METHODS: Seventy-one children undergoing reoperations with sternotomy redo and cardiopulmonary bypass for congenital malformations were included. Twenty-nine of these patients were reoperated after previous application of Seprafilm (treatment group). The duration of dissection, aortic cross clamping and total surgery were recorded. A tenacity score was established for each intervention from the surgeon's description in the operating report. RESULTS: In multivariate analysis, the duration of dissection and the tenacity score were lower in the treatment than control group (p\

IntroductionPatients with Congenital Heart Diseases (CHD) often suffer from severe pulmonary artery (PA) stenosis. Management of PA stenosis is challenging for surgeons. The purpose of this study was to assess the results of intraoperative pulmonary artery stenting, performed additionally to, or instead of, conventional surgical angioplasty.Methods and resultsBetween January 2008 and August 2013, 31 children with hypoplastic or stenosed PA, median age of 23 months (range 6 days to 15 years), and median weight of 11,8kg (range 2,8 to 63kg), underwent intraoperative placement of stents in PA. Patients had pulmonary atresia (15) tetralogy of Fallot (10) troncus arteriosus (4), and complex CHD (2). The aim of the concomitant surgical procedure was palliative surgery (10), complete repair (12), or improvement of right ventricular outflow tract after complete repair (9). A total of 42 balloon-expandable stents were deployed in left or right PA. Maximum balloon diameters ranged from 3 to 16mm (mean=9). Post-operative mortality was 9,6% (3 patients) and 2 patients did not have complete follow-up. Eighteen patients underwent angiographic control, at a mean follow-up of 15 months (± SD 10 months) after surgery. Mean PA diameter increased from 5,19 to 7,57mm (p

We propose a complete surgical approach by left retropectoral transaxillary implantation with no vein puncture to improve the aesthetic and psychological tolerance of the implantable cardioverter defibrillator and avoid the pneumothorax and the subclavian crush syndrome.

Aortic valve disease is the most common cardiac valve condition in developed countries, and its prevalence increases with patient age, especially due to calcified aortic stenosis [24]. Although aortic valve replacement (AVR) remains the most effective treatment in the majority of cases for significant aortic stenosis as well as aortic regurgitation, few data are available for long-term surgical outcome. Perimount pericardial valves offer excellent hemodynamic function, and the present authors’ 10 year experience proved to be satisfactory when these valves were implanted in both the aortic and mitral positions [2, 3]. However, the long-term durability and performance of the valve remained questionable. The study aim was to evaluate long-term results of valve replacement for significant aortic valve disease, and long-term behavior of the aortic Perimount pericardial bioprosthesis, which the present authors have been implanting since 1984.

Objectives The long-term patency rate of coronary artery bypass grafting for which arterial grafts are used is known to be high in the pediatric population. However, this issue remains uncertain in children under 3 years of age. Here, we report the outcome in this specific population. Methods From July 1988 to July 2007, 18 children less than 3 years of age (age at operation, 0.1–35 months; median, 4 months) underwent 20 coronary artery bypass graft operations using an arterial graft. Indications for bypass grafting were coronary artery complications related to the arterial switch operation for transposition of the great arteries in 12 patients (coronary obstruction in 8 patients, peroperative coronary anomalies precluding coronary transfer in 4 patients), congenital anomalies of the coronary arteries in 4 patients, and Kawasaki disease in 2 patients. Results After a mean follow-up of 55 months (range, 1–176 months; median, 41 months), patency of 19 bypass grafts was assessed. One was occluded and 2 have necessitated a percutaneous procedure. Two patients died suddenly (1 with an occluded graft and 1 with a patent graft and hypertrophic myocardiopathy) 3.5 and 4.6 months, respectively, after bypass grafting. Conclusions Coronary artery bypass grafting should be considered as a possible alternative for coronary revascularization in young children. Although our series shows quite a good patency rate, this procedure remains a technical challenge and requires careful follow-up.

We have developed a new technique for interrupted aortic arch repair in which the pulmonary artery anterior wall is cut off and tailored so as to re-establish aortic continuity with an autologous tube. We are describing this method herein, with an 8-year follow-up of the first patient.

Background . The first generation of pericardial valves was withdrawn from the market for a high rate of premature failure. With an original design, Carpentier-Edwards pericardial valves promised improved results. Methods . Seven hundred eighty-seven patients who underwent isolated aortic valve replacement and 182 patients who underwent isolated mitral valve replacement between July 1984 and December 1995 with Carpentier-Edwards pericardial bioprostheses in our institution were followed up. The patients' mean age was 68.3 (aortic valve replacement, AVR) and 63.9 (mitral valve replacement, MVR) years. All but five AVR patients were followed up for an average of 4.7 years after operation, with a total follow-up of 3,624 patient-years. All patients with MVR were followed up for an average of 5.3 years after operation, with a total follow-up of 969 patient-years. Results . After 12 years, actuarial survival rate is 53% for AVR and 54% for MVR. Freedom from valve-related complications for aortic versus mitral valve replacement is, respectively, 68% and 55%, freedom from valve-related death is 84% and 85%, freedom from thromboembolism 87% and 94%, and freedom from endocarditis 97% and 94%. The behavior of the aortic valve is better than that of the mitral valve: freedom from reoperation is 92% and 76%, respectively; freedom from valve failure is 94% and 78%. Age is an important factor, especially in the mitral position: freedom from valve failure is 52% in patients younger than 60 years and 100% in patients older than 60 years. Conclusions . With a low rate of valve-related events at 12 years and a low rate of structural deterioration, this prosthesis is a reliable choice for AVR and in patients over 60 years for MVR. A more durable mitral bioprosthesis is needed for patients younger than 60 years.

Objective: The first generation of pericardial valves was withdrawn from the market because of a high rate of premature failure. With an original design, Carpentier-Edwards pericardial valves promised improved results. Methods: One hundred fifty patients who underwent isolated mitral valve replacement, between July 1984 and December 1993, with Carpentier-Edwards pericardial bioprostheses in our institution were followed up. Patient mean age was 62.9 ± 11.9 years. Operative mortality was 3.3%. All but three patients were followed up for an average of 4.7 years after operation, and total follow-up was 710 patient-years. Results: At the time this article was written, over 80% of patients were in New York Heart Association class I or II. After 10 years, actuarial survival rate was 71% (confidence limit 61% to 81%). Valve-related complications included the following: six valve-related deaths (0.8% patient-year), five thromboembolic episodes (0.7% patient-year), three cases of endocarditis (0.4% patient-year), four reoperations (0.5% patient-year), and four structural valve failures with calcification and stenosis (0.5% patient-year). After 10 years, freedom from valve-related complications was 66% (confidence limit 46% to 86%), from valve-related death 94% (confidence limit 89% to 99%), from reoperation 90% (confidence limit 82% to 98%), and from valve failure 76% (confidence limit 62% to 90%). Conclusions: With a low rate of valve-related events at 10 years and a low rate of structural deterioration with no leaflet tear, this prosthesis is a reliable choice for patients over 60 years of age. (J Thorac Cardiovasc Surg 1997;113:476-91)

From July 1984 to December 1991, 420 patients underwent isolated aortic valve replacement with a Carpentier-Edwards pericardial bioprosthesis in our Institution. Of the patients 71.5% were male, the mean age was 66.9 +/- 11.9 years and 48% were in NYHA clinical stage III or IV. The operative mortality rate was 2.9% (12/420). All patients but six were followed up for an average of 3.9 years after their operation and total follow-up was 1444 patient-years. At this time of the study, over 80% of the patients are in NYHA class I or II, 6% are in atrial fibrillation and 7% receive anticoagulation treatment. There were 41 late deaths. After 8 years the actuarial survival rate is 80% +/- 6%. Nine patients died of valve-related causes (three endocarditis, three thromboembolic complication, one structural failure, and two sudden deaths). The actuarial rate of freedom from valve-related death was 97% +/- 3% at 8 years. Valve-related complications included 12 thromboembolic episodes (0.8% patient-year), 7 endocarditis (0.5% patient-year), 4 anticoagulant-related complications (0.2% patient-year), 6 reoperations (0.4% patient-year) and 2 structural valve failures (0.1% patient-year). After 8 years, freedom from thromboembolic complication was 96% +/- 3%, from endocarditis 97% +/- 2%, from reoperation 98% +/- 2% and from valve failure 99% +/- 1%. There were only two structural deteriorations (calcification and stenosis) and one explanation. No leaflet tear was observed. We conclude that these intermediate results are better than those obtained with previous pericardial bioprostheses.