Document Detail

Biventricular repair in patients with heterotaxy syndrome.
MedLine Citation:
PMID:  19185155     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Complex intracardiac and extracardiac anatomy is often confronted during biventricular repair in patients with heterotaxy syndrome. We examined factors affecting surgical outcomes in these patients. METHODS: Between January 1990 and July 2007, 371 patients received a diagnosis of heterotaxy syndrome; 91 (91/371, 24.5%) underwent biventricular repair. Left atrial isomerism was present in 73% (66/91) and right atrial isomerism in 10% (9/91), with indeterminate atrial anatomy in 17% (16/91). Median age at biventricular repair was 6.8 months (5 days to 22.3 years). Systemic venous anomalies were present in 75 patients, pulmonary venous anomalies in 26, and endocardial cushion defects in 36. Transposition complexes were present in 15 patients with atrioventricular discordance in 10; 8 underwent double switch, 2 received a physiologic repair, 2 underwent arterial switch, and 3 underwent the Rastelli operation. Other conotruncal anomalies included double-outlet right ventricle in 10 patients, tetralogy of Fallot in 3, and hemitruncus in 2. Separation of systemic from pulmonary venous return included intra-atrial baffling in 48 patients and extracardiac grafting in 2. Combined lesions were common, occurring in 99% (90/91). Statistical analysis with Kaplan-Meier and Cox proportional hazards models were performed. RESULTS: Average follow-up was 44.9 +/- 57.5 months (3 days to 189.3 months). Kaplan-Meier estimated survival was 93.4% at 10 years; unbalanced complete atrioventricular canal was the only risk factor for mortality (P = .006). Subsequent procedures were common with a 10-year freedom from reoperation or reintervention of 38% +/- 7.5%. Arrhythmias occurred in 36 (39.6%) patients; bradyarrhythmia in 27 (29.7%) and tachyarrhythmia in 15 (16.5%). Freedom from any arrhythmia was 53.9% +/- 6.7% at 10 years. CONCLUSIONS: Excellent survival for patients with heterotaxy undergoing biventricular repair can be expected, even for multiple, complex lesions. Reintervention is common, and arrhythmia is a long-term concern. This experience shows that patients with heterotaxy syndrome and complex cardiac anatomy can be considered for biventricular repair. Patients with unbalanced complete atrioventricular canal are a high-risk group for which selection criteria are particularly important.
H G Lim; E A Bacha; G R Marx; A Marshall; F Fynn-Thompson; J E Mayer; P Del Nido; F A Pigula
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  137     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-02-02     Completed Date:  2009-02-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  371-379.e3     Citation Subset:  AIM; IM    
Department of Cardiac Surgery, Children's Hospital of Boston, Harvard Medical School, Boston, Mass.
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MeSH Terms
Abnormalities, Multiple / mortality,  surgery*
Arrhythmias, Cardiac / epidemiology
Cardiac Surgical Procedures
Child, Preschool
Double Outlet Right Ventricle / surgery
Endocardial Cushion Defects / surgery
Heart Defects, Congenital / mortality,  surgery*
Heart Ventricles / surgery
Infant, Newborn
Kaplan-Meiers Estimate
Postoperative Complications / epidemiology
Pulmonary Veins / abnormalities,  pathology
Risk Factors
Tetralogy of Fallot / surgery
Transposition of Great Vessels / surgery
Young Adult

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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