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.

Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease.

INTRODUCTION AND OBJECTIVES: Outcome in patients with congenital heart diseases and pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Two-dimensional echocardiographic parameters, such as strain imaging or RV end-systolic remodeling index (RVESRI) have emerged to quantify RV function. METHODS: We prospectively studied 30 patients aged 48±12 years with pretricuspid shunt and PAH and investigated the accuracy of multiple echocardiographic parameters of RV function (tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity, RV systolic-to-diastolic duration ratio, right atrial area, RV fractional area change, RV global longitudinal strain and RVESRI) to RV ejection fraction measured by cardiac magnetic resonance. RESULTS: RV ejection fraction <45% was observed in 13 patients (43.3%). RV global longitudinal strain (ρ [Spearman's correlation coefficient]=-0.75; P=.001; R2=0.58; P=.001), right atrium area (ρ=-0.74; P <.0001; R2=0.56; P <.0001), RVESRI (ρ=-0.64; P <.0001; R2=0.47; P <.0001), systolic-to-diastolic duration ratio (ρ=-0.62; P=.0004; R2=0.47; P <.0001) and RV fractional area change (ρ=0.48; P=.01; R2=0.37; P <.0001) were correlated with RV ejection fraction. RV global longitudinal strain, RVESRI and right atrium area predicted RV ejection fraction <45% with the greatest area under curve (0.88; 95%CI, 0.71-1.00; 0.88; 95%CI, 0.76-1.00, and 0.89; 95%CI, 0.77-1.00, respectively). RV global longitudinal strain >-16%, RVESRI ≥ 1.7 and right atrial area ≥ 22 cm2 predicted RV ejection fraction <45% with a sensitivity and specificity of 87.5% and 85.7%; 76.9% and 88.3%; 92.3% and 82.4%, respectively. CONCLUSIONS: RVESRI, right atrial area and RV global longitudinal strain are strong markers of RV dysfunction in patients with pretricuspid shunt and PAH.

Cardiac tomography-echocardiography imaging fusion: a new approach to congenital heart disease.

INTRODUCTION AND OBJECTIVES: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. METHODS: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. RESULTS: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n=4, 29%), moderate (n=4, 29%), or complex (n=6, 42%). 3D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. CONCLUSIONS: We established the feasibility and accuracy of 3D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.

Anomalous aortic origin of coronary arteries: is the unroofing procedure always appropriate?

OBJECTIVES: Anomalous aortic origin of the coronary artery (AAOCA) with an interarterial ± an intramural course is a rare anomaly that carries a high risk of ischaemic events and even sudden death. The unroofing of the intramural course has been adopted as the gold standard surgical treatment. However, some anatomical forms need alternative techniques. METHODS: We reviewed the surgical cohort with AAOCA managed at our institution between 2005 and 2019 and analysed the anatomical and clinical outcomes. RESULTS: Thirty-nine patients underwent surgical interventions. The median age was 14 years (10-26 years). Twenty-eight patients (72%) had right AAOCA, and 11 (28%) had left AAOCA. Thirty-one (80%) patients presented with symptoms. The symptoms were chest pain in 22 patients (56%), syncope in 5 patients (13%), cardiac arrest during exercise in 2 patients (5%), dyspnoea in 6 patients (15%) and dizziness in 13 patients (33%). An ischaemic test was performed in 32 patients: Only 4 patients (10%) had positive results from the ischaemic test. All patients had computed tomography angiography scans to confirm the precise anatomical features of the anomaly. Repair techniques included 30 unroofing procedures (77%) with an associated translocation of the pulmonary artery for 11 patients in our early experience. In 6 patients the unroofing procedure was not feasible because of the absence of an intramural distinct segment or was judged intraoperatively not appropriate. A reimplantation of the anomalous coronary artery was performed in 2 patients (5%); 3 patients had coronary artery bypass grafting procedures (7%); and 3 (8%) had an isolated translocation of the pulmonary artery. There were no early or late deaths. All patients were free of symptoms. Computed tomography angiography scans performed in 31 cases showed a patent, non-restrictive coronary artery ostium. Seventeen patients underwent postoperative ischaemia testing and showed no evidence of ischaemia. CONCLUSIONS: Surgical correction in AAOCA is mandatory both for symptomatic and for asymptomatic patients with evidence of myocardial ischaemia under stress or with a restricted coronary artery segment. Surgical unroofing remains the gold standard but is not appropriate for all forms: alternative techniques should be considered. Surgical results are promising.

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.

Is surgical intervention still indicated in recurrent aortic arch obstruction?

BACKGROUND: Introduction of balloon dilatation has become the standard treatment for recurrent aortic arch obstruction and has changed the therapeutic approach to patients with this disorder. OBJECTIVES: Whether all patients with recurrent aortic arch obstruction are candidates for balloon dilatation remains unanswered. In addition, only few reports have tried to compare the results between patients undergoing balloon dilatation or redo operations. METHODS: Since 1983, 97 patients underwent reintervention for recurrent aortic arch obstruction (42 dilations and 55 reoperations). Eight had immediate unsuccessful dilatation and were shifted to the surgical group (n = 63). The median age at reintervention was 21.7 months (10 days-45 years), and the median delay was 13.6 months (7 days-17 years). Anatomy of the aortic arch oriented the surgical approach to treat arch hypoplasia. It could be performed through a left thoracotomy in 52 patients, with extended end-to-end anastomosis in 34 patients, subclavian flap repair in 9 patients, conduit insertion in 6 patients, and patch enlargement in 3 patients. More recently, an anterior approach with cardiopulmonary bypass without circulatory arrest was applied to enlarge the patch in all the aortic arches. RESULTS: There was one early death in the surgical intervention group and 2 late deaths in the dilation group. Major complications and recurrence were higher in the dilated group (4 vs 0, P <.01, and 14 vs 5, P <.0004, respectively). At a mean follow-up of 11.8 +/- 4.1 years in the surgical intervention group and 7.5 +/- 2.5 years in the dilated group, systemic hypertension was normalized in all but 5 patients in the surgical intervention group and 6 patients in the dilated group. CONCLUSION: Reoperation for recurrent aortic arch obstruction can be performed safely, with low rates of mortality and morbidity. This approach should be considered versus balloon angioplasty, especially in patients older than 4 years and in the presence of aortic arch hypoplasia.

Biventricular repair for double-outlet right ventricle.

Double-outlet right ventricle (DORV) is a heart malformation that describes an anomalous ventriculoarterial connection which can be associated with considerable variant of associated lesions. When this malformation is present with two ventricles, biventricular repair is feasible in the vast majority of cases. This report describes the surgical techniques for biventricular repair in all forms of encountered DORV, as well as the surgical strategy employed at our institution. Copyright 2000 by W.B. Saunders Company