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.

Surgery of single ventricles in humanitarian practice: surgery for which patients?

OBJECTIVES: To analyse the feasibility and effectiveness in humanitarian practice of surgical management of children with single-ventricle heart condition. METHODS: Retrospective study of children with a single ventricle, managed by the association Mécénat-Chirurgie Cardiaque since 1996, with long-term follow-up after their return home. RESULTS: Of the 138 children in our cohort, 119 had one or more surgeries (180 procedures): palliative surgery alone (systemic-pulmonary anastomosis or banding), 41; partial cavo-pulmonary connection, 47; total cavo-pulmonary connection (mean age 8.5 years), 31. Operative mortality is 5.5%. After a mean follow-up of 5.6 years, 18 children (13%) were lost to follow-up. Survival at 10 years is 79% in children receiving surgery (palliative only, 72%; partial cavo-pulmonary connection, 77%; total cavo-pulmonary connection, 97%) versus 29% in children with no surgical intervention. The prognosis is better for tricuspid atresia and double-inlet left ventricle (86 and 83% survival at 10 years) than for double-outlet right ventricle or complete atrio-ventricular canal defect (64 and 68% at 5 years). CONCLUSION: The surgery of the single ventricle in humanitarian medicine allows a very satisfactory survival after one or more surgeries tending towards a total cavo-pulmonary connection as soon as possible.

Late management of truncus arteriosus: 20 years of humanitarian experience.

OBJECTIVES: Early surgical management of common arterial trunk is well established and has good prognosis. Late diagnosis is less common. We reviewed late-diagnosed common arterial trunk management and prognosis for children in developing countries. We also discuss the need for prior catheterisation. Material and methods We reviewed all common arterial trunk patients managed by our humanitarian organization since 1996. RESULTS: A total of 41 children with common arterial trunk were managed at a mean age of 3 years old. The lack of adequate facilities in developing countries explains the late management. The decision to proceed with surgery was based on clinical and radiological symptoms of persistent shunt, particularly a high cutaneous saturation level, regardless of catheterization - not carried out systematically. Eight children had to be withdrawn and 33 (80.5%) received operation - mean saturation 91%. The postoperative course was marked by pulmonary arterial hypertension requiring specific treatment in 30% of cases. The operative mortality was 1/33. The 32 children returned home without treatment after a mean post operative stay of 49 days and were followed up (mean FU 3.4 years, none lost to follow-up). At last contact, 1 child died six months after surgery, 1 child had a massive truncal valve insufficiency, 5 had a significant stenosis of the RV-PA tube, and 2 have had further surgery for tube replacement. CONCLUSIONS: Late management and surgery of common arterial trunk is possible with good long-term results without prior hemodynamic examination up to an advanced childhood when signs of left-to-right shunt persist. A high saturation level (above 88%) seems to be a good operability criterion.

Nitric oxide synthase expression by pulmonary arteries: a predictive marker of Fontan procedure outcome?

OBJECTIVES: We retrospectively analyzed lung biopsy specimens from patients who underwent the Fontan procedure to identify predictive markers of outcome. METHODS: We studied the intra-acinar pulmonary arteries present in lung biopsy specimens from 17 patients undergoing the Fontan procedure. We evaluated both their morphology and their expression of endothelial nitric oxide synthase and endothelin 1. We compared these data with those of 6 patients who died of no pulmonary cause (control group). RESULTS: Eight patients had a good surgical outcome (group 1). Their distal arteries were thin and weakly expressed endothelin 1 and endothelial nitric oxide synthase. The procedure failed in 9 patients (group 2). Their distal arteries displayed muscle extension with an increased wall thickness (P <.01 vs group 1). Their endothelin 1 expression remained low (not significant vs group 1). By contrast, endothelial nitric oxide synthase was markedly overexpressed (P <.001 vs group 1). CONCLUSION: Distal pulmonary arteries of patients in whom the Fontan procedure failed exhibited a markedly increased wall thickness and a clear endothelial nitric oxide synthase overexpression. In addition to giving clues to the pathogenesis of the procedure's failure, our study might help to define reliable predictive markers of its outcome.

Histomorphometric analysis of pulmonary vessels in single ventricle for better selection of patients for the Fontan operation.

OBJECTIVE: In cases of single-ventricle physiology, the Fontan procedure often fails even when the usual selection criteria are strictly respected. We analyzed specimens from intraoperative open lung biopsies performed on 40 patients with single-ventricle physiology who were considered to be good candidates for the Fontan procedure. Histomorphometric study was performed to determine histologic factors predictive of failure of the Fontan procedure. METHODS: Histomorphometric studies were performed on samples from 40 patients aged 6 months to 23 years with single-ventricle physiology, either tricuspid atresia (n = 14) or univentricular heart (n = 26). The preoperative pulmonary arterial pressure was 18 mm Hg or less in 35 cases and greater than 18 mm Hg in 5 cases. Eighteen patients underwent total cavopulmonary connection, 16 patients underwent partial cavopulmonary connection, and 6 underwent a palliative procedure, as determined according to clinical and hemodynamic findings. RESULTS: Lung biopsy specimens from all 5 patients with pulmonary arterial pressure greater than 18 mm Hg appeared abnormal, whereas they appeared abnormal only 51% of the time in the low pulmonary arterial pressure group. The most frequent histologic abnormality observed was extension of smooth muscle cells in the wall of distal intra-acinar pulmonary arteries. Of the 18 patients who underwent the Fontan procedure, 9 had normal distal pulmonary arteries and good surgical results (except 1 with the Fontan circulation taken down for an anatomic reason). The remaining 9 had thick-walled distal intra-acinar pulmonary arteries with poor results of the Fontan procedure, and 6 died. The mean percentage wall thickness of small intra-acinar pulmonary arteries was significantly greater among the patients with bad results than among those with good results of the procedure (P <.01). CONCLUSIONS: Lung biopsy specimens were abnormal in 51% of patients with low pulmonary arterial pressure, there was no relationship between preoperative pulmonary arterial pressure and outcome, and extension of muscle in peripheral arteries was always present in cases of failure of the Fontan procedure. Histomorphometric study is therefore a useful adjunct to the usual selection criteria for surgical decision making in cases of single-ventricle physiology.