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Published on: May 11, 2018
Surgical results in patients with double outlet right ventricle: a 20-year experience
J W Brown1, M Ruzmetov, Y Okada
1Section of Cardiothoracic Surgery, James W. Riley Hospital for Children, and Indiana University School of Medicine, Indianapolis 46202-5123, USA. jobrown@iupui.edu
Insights
This study reviewed surgical strategies for double outlet right ventricle in 124 children, finding high survival rates with various repair methods. Careful preoperative assessment is key to successful surgical outcomes and reduced reoperation rates.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Anatomy
Background:
- Double outlet right ventricle (DORV) is a complex congenital heart defect.
- Surgical strategies for DORV require careful consideration of patient anatomy.
- Risk factors for mortality and reoperation in DORV patients need thorough evaluation.
Purpose of the Study:
- To review surgical strategies for double outlet right ventricle (DORV) in pediatric patients.
- To identify risk factors associated with early and late mortality after DORV repair.
- To assess the need for reoperation in children undergoing DORV repair.
Main Methods:
- Retrospective review of 124 pediatric patients with DORV undergoing repair between 1980 and 2000.
- Patients were categorized into three groups based on anatomical complexity: noncomplex, Taussig-Bing, and complex anomalies.
- Surgical outcomes, including mortality, reoperation rates, and functional status, were analyzed.
Main Results:
- Four primary repair types were performed: intraventricular tunnel, conduit repair, arterial switch operation, and Fontan procedures.
- Overall 15-year survival rates were high, ranging from 89.5% to 95.8% across patient groups.
- Risk factors for adverse outcomes included VSD characteristics, outflow tract issues, prior palliation, and operative approach.
Conclusions:
- High survival rates are achievable for DORV patients with various surgical techniques, including intraventricular tunnel repair, arterial switch, conduit repair, and modified Fontan procedures.
- Individualized surgical planning based on detailed preoperative anatomical assessment is crucial for optimizing patient outcomes.
- Long-term follow-up indicates good functional status in the majority of survivors, with a notable reoperation rate in certain complex cases.
Background:
The objective of this study was to review our surgical strategy in children with double outlet right ventricle and to assess risk factors for early and late mortality and reoperation.
Methods:
Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ventricle. The patients were divided into three groups. Group 1 (n = 47) had noncomplex patients with atrioventricular concordance, a single ventricular septal defect, balanced ventricles, no straddling atrioventricular valves, and no major pulmonary artery anomalies. Group 2 (n = 39) included patients with double outlet right ventricle and a subpulmonary ventricular septal defect (Taussig-Bing). Group 3 (n = 38) had patients with complex anomalies including straddling atrioventricular valves, atrioventricular septal defects or a hypoplastic valve or ventricle, or a combination of atrioventricular septal defects and hypoplastic valve or ventricle.
Results:
Four types of definitive repairs were performed: (1) intraventricular tunnel repair with a baffle from the left ventricle to the aorta (n = 53); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33). Two patients with late postoperative cardiomyopathy had heart transplantation. Potential risk factors included location of the largest ventricular septal defect, presence of additional ventricular septal defects, ventricular outflow obstruction or hypoplasia, or both ventricular outflow obstruction and hypoplasia, previous palliation, and type of definitive operation. There were six early deaths (4.8%) and four late deaths (3.2%), and two heart transplants (1.6%). Overall 15-year survival was 95.8%, 89.7%, and 89.5% for groups 1, 2, and 3, respectively (p = 0.08). Thirteen patients (11.4%) have required 15 reoperations. Mean follow-up for survivors was 76.6 +/- 52.8 months. Up-to-date follow-ups are available on 114 surviving patients. Ninety-five of these patients (83.3%) were in New York Heart Association class I, and the remaining 19 patients (16.7%) were in New York Heart Association class II. Freedom from reoperation was 87%, 72%, and 100% at 15 years for groups 1, 2, and 3, respectively (p = 0.11).
Conclusions:
Survival was high for all patients with double outlet right ventricle undergoing intraventricular tunnel repair, arterial switch operation, and repair with a conduit or a modified Fontan procedure. Careful attention to preoperative anatomy dictates the best surgical approach and will enhance outcomes.
