Single Stage Repair For Pulmonary And Aortic Obstruction In William's Syndrome - Surgical Tips And Pitfalls Mrinal Patel, Manan Desai, Syed Bukhari, Aybala Tongut, Yves d'Udekem. Children's National Hospital, Washington, DC, USA.
IntroductionThis video demonstrates a single-stage surgical approach for reconstruction of the pulmonary arterial tree and repair of supravalvar pulmonary and aortic stenosis in a patient with Williams syndrome.Case DescriptionA 4-year-old boy presented with bilateral branch pulmonary artery stenosis, supra-systemic right ventricular pressures, and a 40 mmHg supravalvar aortic gradient. He was asymptomatic but had a positive family history. A single-stage repair was performed via median sternotomy.Key technical steps included: (1) Transection of main pulmonary artery, division of the right and left pulmonary arteries for optimal exposure; (2) “V”-plasty to address ostial stenoses: (3) homograft patch augmentation for long segment stenoses; (4) careful reconstruction of the main, right, and left pulmonary artery confluence to avoid distortion; (5) sinus augmentation to relieve supravalvar pulmonary and aortic stenosis; (6) meticulous inspection of coronary ostia with excision of obstructive tissue; and (7) extension of the patch through the non-coronary sinus onto the greater curvature of the ascending aorta(8) separate patch for arch reconstruction may be used (not used in this case).The patient was weaned off cardiopulmonary bypass with half systemic right ventricular pressures and was discharged home after 4 weeks.ConclusionSingle-stage repair of multilevel pulmonary artery and supravalvar aortic stenosis in Williams syndrome is feasible, reproducible, and effective.
