Tailored approach to trans-catheter palliation of critically reduced pulmonary blood supply. Data on long term follow up

Abstract

The most appropriate treatment of duct dependent pulmonary circulation is still a matter of debate. In particular data on long term results of trans-catheter palliation are still inconclusive. We present the long term outcome of trans-catheter palliation in this group of patients according to the anatomy and to a prespecified algorithm, with particular focus on development of pulmonary tree.We retrospectively analyzed data on patients who had undergone trans-catheter palliation of duct dependant congenital heart disease between 2005 and 2017 in our institution. Arterial duct or right ventricular outflow tract (RVOT) percutaneous transluminal angioplasty (PTA)/stenting were performed according a pre-specified algorithm.47 patients underwent either arterial duct stenting (n = 42) or RVOT PTA/Stenting (n = 5). Median age at catheterization was 13 days (IQR 3–686). Median weight was 3 kg (1.5–4.3). Median follow up was 1127 (6–4396) days. 11 patients out of 47 died (23%). One year, five year and ten year survival were respectively: 82% (95% CI 68–90), 75% (95% CI 60–85) and 60% (95% CI 27–81). At Cox regression analysis, weight at the initial Trans-catheter palliation and univentricular physiology remained independently associated with long term mortality, respectively Hazard ratio 0.3 (95% CI 0.12–0.88, p = 0.03) and Hazard ratio 5 (95% CI 1.3–20.2, p = 0.02). In the overall study cohort 36 patients (76%) needed further intervention, either surgical or trans-catheter, before surgical repair or definitive palliation. Overall, median time to re-intervention was 107 days (2–750). Central pulmonary arteries grew in all patients. Tailored trans-catheter management of congenital heart disease associated with critically reduced pulmonary flow resulted in proper maturation of pulmonary tree allowing surgical repair or definite palliation despite the need for repeated procedures.