Biventricular Repair in Heterotaxy Syndrome
Frank Pigula, Hong-Gook Lim, Emile Bacha, Audrey Marshall, John Mayer, Francis Fynn-Thompson, Pedro Del Nido; Children's Hospital Boston, Boston, MA
Comment on this Abstract
Objective: Complex intra- and extra-cardiac anatomy is often confronted during biventricular repair (BVR)in patients with heterotaxy syndrome. We examined factors affecting surgical outcomes in these patients.
Methods: Between Jan 1990 and July 2007, 371 patients were diagnosed with heterotaxy syndrome; 91(91/371, 24.5%)underwent (BVR).Left atrial isomerism was present in 73% (66/91), right atrial isomerism in 10% (9/91), with indeterminate atrial anatomy in 17% (16/91). Median age at BVR was 6.8 months (5 days -22.3 years). Systemic venous anomaly was present in 75 patients, pulmonary venous anomaly in 26, and endocardial cushion defect in 36. Transposition complexes were present in 15 patients with AV discordance in 10; 8 underwent double switch, 2 received a physiologic repair, 2 underwent arterial switch, and 3 underwent Rastelli. Conotruncal anomalies included DORV in 10 patients, tetralogy in 3, and hemitruncus in 2. Combined lesions were common, occuring in 99% (90/91). Separation of systemic from pulmonary venous return included intraatrial baffling 48 patients, and extracardiac graft in 2. Statistical analysis using Kaplan-Meier and Cox proportional harzard models were performed.
Results: Average follow-up was 44.9 ± 57.5 months (3 days - 189.3 months). There were 4 deaths (4/91, 4.4%); unbalanced CAVC was the only risk factor for mortality (p=0.006). Pulmonary stenosis (p=0.001), pulmonary atresia (p=0.002), and common AV valve (p=0.008) were risk factors for reintervention. Arrhythmias occurred in 36 patients (39.6%); bradyarrhythmia in 27 (29.7%), and tachyarrhythmia in 15 (16.5%). At 15 years, freedom from any arrhythmia was 41.2 ± 9.8%. Pulmonary stenosis (p=0.038) was related to bradyarrhythmia, while older age at operation (p=0.005) was associated with tachyarrhythmia.
Conclusion: Excellent survival for heterotaxy patients undergoing BVR can be expected, even for multiple, complex lesions. Reintervention is common, and arrhythmia is a longterm concern.Patients with unbalanced CAVC represent a high risk group for which single ventricle palliation can be considered.
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