Surgical Strategy for Atrioventricular Septal Defect and Tetralogy of Fallot or Double-Outlet Right Ventricle

Autor: Vijayakumar Raju, Hartzell V. Schaff, Joseph A. Dearani, Benjamin W. Eidem, Harold M. Burkhart, Heidi M. Connolly, Natalie Rigelman Hedberg, Zhuo Li
Rok vydání: 2013
Předmět:
Male
Time Factors
Kaplan-Meier Estimate
Cohort Studies
Ventricular outflow tract
Hospital Mortality
Atrioventricular Septal Defect
Child
Tetralogy of Fallot
Academic Medical Centers
Heart septal defect
Mortality rate
Combined Modality Therapy
Double Outlet Right Ventricle
Echocardiography
Doppler
Survival Rate
Treatment Outcome
Child
Preschool
Cardiology
Female
Cardiology and Cardiovascular Medicine
Adult
Heart Defects
Congenital
Pulmonary and Respiratory Medicine
medicine.medical_specialty
Adolescent
Minnesota
Risk Assessment
Young Adult
Double outlet right ventricle
Internal medicine
medicine
Humans
Abnormalities
Multiple
Cardiac Surgical Procedures
Survival rate
Proportional Hazards Models
Retrospective Studies
business.industry
Heart Septal Defects
Infant
medicine.disease
Surgery
Logistic Models
Heart failure
Multivariate Analysis
business
Follow-Up Studies
Zdroj: The Annals of Thoracic Surgery. 95:2079-2085
ISSN: 0003-4975
Popis: Background Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. Methods From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. Results Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 ( p = 0.008) and the presence of significant common AVV regurgitation ( p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV ( p = 0.95). The presence of significant right AVV regurgitation was associated with late death ( p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV ( p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV ( p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation ( p = 0.018) and repair before 1990 ( p = 0.041) were risk factors for late reoperation in both groups. Conclusions Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.
Databáze: OpenAIRE
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