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OBJECTIVE: Double outlet right ventricle (DORV) is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair. This study aimed to describe the clinical outcomes of biventricular (BiV) repair for DORV. METHODS: Patients with DORV, who underwent BiV repair between January 2000 and December 2017 were retrospectively reviewed. Group 1 underwent primary BiV repair, whereas group 2 underwent staged BiV repair over a series of operations. The decision to pursue staged approach included complexity of intracardiac anatomy, age of the patient, and the size and function of the ventricles and the atrioventricular valves. Time-dependent surgical reintervention for LVOTO and mortality were evaluated using Kaplan-Meier survival analysis. RESULTS: A total of 238 patients with DORV underwent BiV repair at a median age of 6.2 months (range, 1.1 month-27.5 years) (158 in group 1, 80 in group 2). Twenty-two patients (7.8%) required surgical reintervention within 30 days of BiV repair. Overall survival at 5 years was 89.0%. Freedom from LVOTO reoperation at 5 years was 84%. Primary outcomes were not significantly different between groups. CAVC repair and right ventricle to pulmonary artery conduit at BiV repair were associated with higher surgical reintervention (hazard ratio, 2.9 and 1.75, respectively). CONCLUSIONS: Patients with DORV and complex anatomy may undergo staged BiV repair with acceptable outcomes. Although LVOTO is a potential complication in these patients, the rate of surgical reintervention for LVOTO does not differ significantly from patients undergoing primary BiV repair.
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OBJECTIVES: To describe technical aspects of successful fetal aortic valvuloplasty, with particular attention to balloon size. STUDY DESIGN: We retrospectively reviewed all procedural records and echocardiograms pertaining to 26 attempts at fetal aortic valve dilation performed at a single center over a period of 4 years. We assessed the effect of valvuloplasty as determined by echocardiographic appearance at the time of intervention and in follow-up. RESULTS: In 20 of 26 fetuses who had technically successful aortic valve dilation (median balloon:annulus ratio=1.1), all had improved antegrade flow and 12 had at least mild regurgitation after dilation. Use of an oversized balloon was associated with the onset of moderate or severe aortic regurgitation, seen in 5 fetuses. This aortic regurgitation was well tolerated and improved through the remainder of gestation. CONCLUSIONS: These data imply that fetal aortic valves can be dilated safely with larger balloons than are commonly used for postnatal dilation. The observation of spontaneous improvement in postdilation aortic regurgitation further suggests that fetal valve tissue behaves uniquely.