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Perioperative management of pulmonary arterial hypertension in children undergoing congenital heart surgery: a systematic review and meta-analysis

Ikwuanusi E, Rajah T, Scandrett K, Asamane EA, Volpi S

Journal of cardiothoracic surgery, 2026

520 participants

Abstract

BACKGROUND: Children with pre-existing pulmonary arterial hypertension (PAH) undergoing surgery for congenital heart disease (CHD) are at an increased risk of morbidity and mortality, primarily due to complications associated with increased pulmonary arterial pressures. Despite the clinical significance of this risk, no comprehensive review of perioperative strategies to manage pre-existing PAH in paediatric CHD patients undergoing cardiac surgery exists. This systematic review aims to address this gap. METHODS: A comprehensive systematic literature search was conducted on Scopus, Medline, Embase, PubMed, Cochrane Library and grey literature; studies were screened using eligibility criteria. Data was extracted using a pre-tested standard form and the methodological quality appraised using the RoB 2 tool. A meta-analysis was used to analyse, summarise, and interpret the extracted data. RESULTS: Ten randomised controlled studies were included in this review, comprising 520 patients, ages ranged from 0.21 to 13.8 years across 6 countries. All studies reported a greater decrease in postoperative mean pulmonary arterial pressure (mPAP) in the intervention groups compared to the control groups. Although perioperative management did not significantly reduce postoperative PAP (pooled mean difference, -0.44; 95% CI: -4.62-3.73; I2 = 88.73%), their use resulted in statistically significant decreases in mean ICU stays (pooled mean difference, -1.08; 95% CI: -1.90-0.25, p-value = 0.01;…

How strong is the evidence?

Evidence Hub reads this as a meta-analysis, done in people. Its evidence level is 1 of 5 (Highest · pooled evidence). Pooled evidence from several studies sits at the top of the evidence pyramid. Reported size: 520 participants or records.

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