Surgical repair of peripheral pulmonary artery stenosis: A two-decade experience with 145 patients
Mac Felmly1, *Richard Mainwaring1, Tom Collins2, Kirstie Lechich2, Elisabeth Martin1, Michael Ma1, *Frank L. Hanley1
1Cardiac Surgery, Stanford University School of Medicine, Stanford, California, United States, 2Pediatric Cardiology, Stanford University, Stanford, California, United States
OBJECTIVE: Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease that is often associated with Williams syndrome, Alagille syndrome, and Elastin arteriopathy. This disease is characterized by stenoses at nearly all of the lobar and segmental ostia and results in systemic level right ventricular pressures. Interventional catheterization techniques have proven ineffective in addressing PPAS due to the peripheral location and unique position at the ostia. The current study summarizes our experience with the surgical treatment of PPAS.
METHODS: This was a retrospective review of 145 patients who underwent surgical repair at our institution from 2002 thru 2021. This includes 43 patients with Williams syndrome, 39 patients with Alagille syndrome, and 21 patients with Elastin arteriopathy. Other diagnoses include tetralogy of Fallot with PPAS (n = 21), truncus arteriosus with PPAS (n = 5), transposition with PPAS (n = 3), double outlet right ventricle with PPAS (n = 2), arterial tortuosity syndrome (n = 3), and other (n = 8).
RESULTS: The median pre-operative right ventricle to aortic peak systolic pressure ratio was 1.01 (range 0.60 to 1.60) and this was reduced to a median of 0.30 (range 0.17 to 0.60) post-operatively (Figure A). Five of the 145 patients (3.4%) had a post-operative pressure ratio > 0.50.
The median combined number of lobar and segmental ostial repairs was 17 (range 6 to 34). The median duration of cardiopulmonary bypass was 398 (range 92 to 844) minutes. Forty-eight of the 145 patients required a period of aortic cross-clamp with a median of 28 (range 17 to 202) minutes.
There were three in-hospital deaths (2.1%), one with Williams syndrome and two with Alagille syndrome. Median hospital length of stay was 12 days (range 4 to 118 days). Median duration of follow-up was 22 months (range 1 to 220 months) with four late deaths (2.8%). The Kaplan-Meier survival curve is shown in Figure B.
Sixty-eight of the 138 survivors (50%) have subsequently undergone a cardiac catheterization. At cath, the pulmonary artery to aortic pressure ratio was less than 0.50 in 63 of the 68 patients (93%). One patient underwent surgical re-operation to re-repair PPAS and four patients with elevated pulmonary artery pressure ratios underwent balloon angioplasty.
CONCLUSIONS: The surgical treatment of PPAS resulted in a 70% reduction in right ventricular pressure ratios. At three years, 94% of the patients are alive and 93% of those evaluated continue to maintain low pulmonary artery pressures. These results suggest that the surgical treatment of PPAS is highly successful in the vast majority of patients.
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