Management of pulmonary vascular disease associated with congenital left-to-right shunts: A single-center experience

Hythem Nawaytou; Ramya Lakkaraju; Leah Stevens; Vadiyala Mohan Reddy; Naveen Swami; Roberta L Keller; David F Teitel; Jeffrey R Fineman

doi:10.1016/j.jtcvs.2024.05.007

Management of pulmonary vascular disease associated with congenital left-to-right shunts: A single-center experience

J Thorac Cardiovasc Surg. 2024 May 18:S0022-5223(24)00438-0. doi: 10.1016/j.jtcvs.2024.05.007. Online ahead of print.

Authors

Hythem Nawaytou¹, Ramya Lakkaraju², Leah Stevens³, Vadiyala Mohan Reddy⁴, Naveen Swami⁴, Roberta L Keller⁵, David F Teitel⁶, Jeffrey R Fineman⁷

Affiliations

¹ Department of Pediatrics, Cardiology, University of California, San Francisco, Calif. Electronic address: Hythem.nawaytou@ucsf.edu.
² Department of Pediatrics, Cardiology, University of California, San Francisco, Calif; University of Massachusetts Chan Medical School, Worcester, Mass.
³ Department of Pediatrics, Intensive Care, University of California, San Francisco, Calif.
⁴ Department of Surgery, Cardiothoracic Surgery, University of California, San Francisco, Calif.
⁵ Department of Pediatrics, Neonatology, University of California, San Francisco, Calif.
⁶ Department of Pediatrics, Cardiology, University of California, San Francisco, Calif.
⁷ Department of Pediatrics, Intensive Care, University of California, San Francisco, Calif; Cardiovascular Research Institute, University of California, San Francisco, Calif.

PMID: 38763305
DOI: 10.1016/j.jtcvs.2024.05.007

Abstract

Objective: The study objective was to describe the course and outcomes of children under 18 years of age, with left-to-right shunts and pulmonary arterial hypertension undergoing 1 of 2 management approaches: pulmonary arterial hypertension treatment before left-to-right shunt repair (Treat First) and left-to-right shunt repair first with or without subsequent pulmonary arterial hypertension treatment (Repair First).

Methods: We performed a retrospective single-center study, conducted from September 2015 to September 2021, of children with left-to-right shunts and pulmonary arterial hypertension (defined as indexed pulmonary vascular resistance ≥ 4 Wood units [WU]∗m²) but without Eisenmenger physiology. Patient characteristics, longitudinal hemodynamics data, pulmonary arterial hypertension management, left-to-right shunt repair, and outcomes were reviewed.

Results: Of 768 patients evaluated for left-to-right shunt closure, 51 (6.8%) had left-to-right shunts associated with pulmonary arterial hypertension (median age 1.1 [0.37-5] years, median indexed pulmonary vascular resistance 6 [5.2-8.7] WU∗m²). In the "Treat First" group (n = 33, 65%), 27 patients (82%) underwent left-to-right shunt closure and 6 patients (18%) did not respond to pulmonary arterial hypertension therapy and did not undergo left-to-right shunt closure. In the "Repair First" group (n = 18, 35%), 12 patients (67%) received pulmonary arterial hypertension therapy and 6 patients (33%) did not. Mortality rates were 6% in the "Treat First" group and 11% in "Repair First" group with follow-ups of 3.4 and 2.5 years, respectively. After left-to-right shunt closure, there was no significant change in indexed pulmonary vascular resistance over a median follow-up of 2 years after surgery (P = .77).

Conclusions: In children with left-to-right shunts and associated pulmonary arterial hypertension, treatment with pulmonary arterial hypertension-targeted therapy before defect repair does not appear to endanger the subjects and may have some benefit. The response to pulmonary arterial hypertension-targeted therapy before shunt closure persists 2 to 3 years postclosure, providing valuable insights into the long-term management of these patients.

Keywords: congenital heart disease; pulmonary hypertension; pulmonary vascular disease.