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Original Articles: Pediatric Cardiac

Midterm Outcomes in Supravalvular Aortic Stenosis Demonstrate the Superiority of Multisinus Aortoplasty

^a Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois
^b Division of Cardiology, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois
^c Cleveland Clinic Foundation, Case Western Reserve University, Cleveland, Ohio

Accepted for publication February 5, 2010.

^_* Address correspondence to Dr Kaushal, Children's Memorial Hospital, Division of Cardiovascular-Thoracic Surgery 2300 Children's Plaza, mc 22, Chicago, IL 60614 (Email: skaushal{at}childrensmemorial.org).

Presented at the Fifty-sixth Annual Meeting of the Southern Thoracic Surgical Association, Marco Island, FL, Nov 4–7, 2009.

Background: Surgical techniques for repair of supravalvular aortic stenosis (SVAS) include McGoon's one-patch, Doty's two-patch, and Brom's three-patch method. In this review we evaluated mid-term clinical outcomes of these techniques at our institution.

Methods: Our cardiac surgery database identified patients with SVAS repair from 1990 to 2008. Follow-up records, reintervention and reoperation data, and most recent echocardiograms were obtained.

Results: From 1990 to 2008, 20 patients (70% male) underwent surgery for SVAS. Mean age was 3.6 ± 5.6 years. In chronological sequence, 8 patients had single-patch aortoplasty, 4 had the Doty procedure, and 8 received Brom's symmetric three-patch aortoplasty. Of the Brom patients, 6 had Williams syndrome. Aortic cross-clamp times were 40.1 ± 13.6 minutes (one-patch), 60.3 ± 38.8 minutes (Doty), and 104 ± 20.5 minutes (Brom). Perioperative mortality was 5.0% (1 patient in one-patch group). Mean postoperative length of stay was 10 ± 10.6 days. Follow-up data were available for all survivors (mean follow-up, 6.3 ± 6.0 years; range, 6 months to 16 years). There were no late deaths. Follow-up echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 33 ± 18.0 mm Hg (one-patch), 10 ± 1 mm Hg (Doty), and 18 ± 12 mm Hg (Brom). All patients in the Doty and Brom groups had less than moderate aortic insufficiency. Reoperations were required in 5 of 8 one-patch patients (62%) for residual aortic stenosis (n = 3), aortic insufficiency (n = 1), and subvalvar stenosis (n = 1). No Doty or Brom patient has required aortic reoperations, which was nearly statistically associated with freedom from reoperation (p = 0.06). Subvalvar stenosis was the only risk factor associated with reoperation (p = 0.0028).

Conclusions: Despite a longer cross-clamp time, SVAS repair by Doty or Brom aortoplasty restores normal hemodynamics and reduces the need for reoperation when compared with the classic one-patch technique. Our current preference for SVAS repair is the Brom three-patch symmetric aortoplasty.

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