Management of systemic to pulmonary shunts and elevated pulmonary vascular resistance.

Background

Repair of systemic to pulmonary shunts is timed to prevent the development of irreversible pulmonary vascular disease, including in patients with other factors contributing to pulmonary hypertension. This study assessed outcomes of an individualised strategy for managing patients with mild-moderately elevated pulmonary vascular resistance (PVR) deemed borderline eligible for repair.

Methods

A retrospective chart review was conducted of patients with systemic to pulmonary shunts and baseline indexed PVR (PVRi) ≥3 WU·m

Results

30 patients (18 females) met criteria for inclusion. Median age at diagnosis of pulmonary arterial hypertension was 1.3 years (range 0.03-54 years) and at surgery was 4.1 years (range 0.73-56 years). Median follow-up time was 5.8 years (range 0.2-14.6 years) after repair. Most patients received at least one targeted pulmonary arterial therapy prior to repair and the majority (80%) underwent fenestrated shunt closure. There was a significant decrease in mean pulmonary arterial pressure (mPAP) (p<0.01), PVRi (p=0.0001) and PVR/systemic vascular resistance (p<0.01) between baseline and preoperative catheterisation and a decrease in PVRi (p<0.005), mPAP (p=0.0001) and pulmonary to systemic flow ratio (p<0.03) from baseline to most recent catheterisation. WHO FC improved from FC II-III at baseline to FC I post repair in most patients (p<0.003).

Conclusions

In carefully selected patients with systemic to pulmonary shunts and elevated PVR considered borderline for operability, the use of preoperative targeted therapy in conjunction with fenestrated or partial closure of intracardiac shunts is associated with improvement in WHO FC and clinical outcomes.

Informations de copyright

Copyright ©The authors 2023.

Déclaration de conflit d'intérêts

Conflict of interest: No disclosures for A.N. Linder, J. Hsia, S.V. Krishnan and E.A. Bacha. Conflict of interest: S. Crook receives salary support from the Babies Heart Fund. Conflict of interest: Columbia University Irving Medical Center has received research grant support from Janssen and United Therapeutics for studies for which U.S. Krishnan is the principal investigator. U.S. Krishnan has no financial conflicts. Conflict of interest: E.B. Rosenzweig's institution (Columbia University Irving Medical Center) has received research grant support from Actelion/Janssen Pharmaceuticals, Bayer, Insmed, SonVie and United Therapeutics, and E.B. Rosenzweig has research funding from the National Heart, Lung, and Blood Institute. Conflict of interest: U.S. Krishnan and E.B. Rosenzweig express gratitude to the Pediatric Pulmonary Hypertension Network for supporting care of patients with pulmonary hypertension.