SURGICAL REPAIR OF PERIPHERAL PULMONARY ARTERY STENOSIS

Abstract

Peripheral pulmonary artery stenosis (PPAS) is a rare form of congenital heart disease and is typically associated with Williams or Alagille syndrome. However, there are patients without either syndrome who present with PPAS. The purpose of this study was to review our surgical experience in those patients. This is a retrospective review of 30 patients without either Williams or Alagille syndrome who underwent surgical repair of PPAS at a single institution. Median age at surgery was 3.6 years (range 0.3-18.8 years). Seventeen of the 30 patients (57%) had undergone previous cardiac operations and 16 patients (53%) previously underwent peripheral pulmonary artery balloon angioplasty or stent implantation. Concomitant anatomical diagnoses were found in 20 patients (67%) and included: supravalvar aortic stenosis (n=8), tetralogy of Fallot (n=4), d-transposition of the great arteries(n=2), truncus arteriosus (n=2), hypoplastic left heart syndrome (n=2), ventricular septal defect (n=1) and patent ductus arteriosus (n=1). Additional medical diagnoses were found in 15 patients (50%) and included: elastin arteriopathy (n=9), pulmonary artery calcinosis (n=1), arterial tortuosity syndrome (n=1), DiGeorge syndrome (n=1), Noonan syndrome (n=1) and others (n=2). The mean number of peripheral pulmonary stenoses surgically addressed was 19±8. Median duration of cardiopulmonary bypass was 369 minutes (IQR 292 to 515 minutes). With surgical repair, mean right ventricle-to-aortic systolic pressure ratio decreased from 0.95±0.2 pre-operatively to 0.28±0.08 post-operatively (p < 0.0001). Similarly, mean right ventricle systolic pressure decreased from 81±22mmHg to 26±8mmHg (p < 0.0001). Survival to hospital discharge was 100% and there was no mortality at a mean follow-up of 2.3 years. Post-operatively, 4 patients underwent balloon dilation of peripheral pulmonary arteries and no patient required cardiac reoperation. Freedom from pulmonary artery catheter-based reintervention was 95% and 80% at 12 and 36 months, respectively. This cohort of patients with PPAS underwent successful surgical repair with a significant reduction in right ventricle to aortic pressure ratios, achieving absolute right ventricular systolic pressures within the normal range. Various medical and anatomic diagnoses may be associated with PPAS. These results suggest a surgical approach should be considered the first-line treatment for PPAS.View Large Image Figure ViewerDownload Hi-res image Download (PPT)