Factors associated with the uncorrectable congenital heart disease in children with pulmonary arterial hypertension

Abstract

Factors associated with the uncorrectable congenital heart disease in children with pulmonary arterial hypertension Handoyo, Eka Gunawijaya, Ni Putu Veny Kartika Yantie Medical Journal of Indonesia Clinical Research ABSTRACT BACKGROUND Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common complication of uncorrected left-to-right shunt defects in acyanotic CHD and a frequent type of pulmonary hypertension in youth. The standards for operability in left-to-right shunts with increased pulmonary vascular resistance are not universally agreed upon. This study aimed to identify variables associated with uncorrectable lesion in children with PAH-CHD. METHODS This retrospective study used a database of all children who underwent cardiac catheterization at Sanglah Hospital, Bali, from May 2009 to April 2021. Pulmonary hypertension was defined as pulmonary artery pressure of >25 mmHg, while correctability was a fall of >20% in the pulmonary arterial resistance index (PARI) with final value of <6 WU/m2 when doing an acute vasoreactivity test using 100% oxygen. The analyses were carried out using SPSS software version 22.0 (IBM Corp., USA). RESULTS A total of 104 children were included. Cardiac catheterization showed that the uncorrectable group had a higher PARI (14.4 [8.88] WU/m2 versus 8.43 [3.85] WU/m2 ) and lower flow ratio (1.27 [0.83] versus 1.47 [0.77]) at baseline. In terms of correctability, pre-tricuspid lesions (OR = 0.05; 95% CI = 0.01–0.47; p = 0.01) and younger age group (OR = 0.32; 95% CI = 0.12–0.85; p = 0.01) were protective variables, whilst high baseline PARI (OR = 4.54; 95% CI = 1.64–12.57; p = 0.01) was unfavorable. CONCLUSIONS High baseline PARI was the most significant variable in predicting uncorrectable left-to-right shunt defects in PAH-CHD. KEYWORDS children, congenital heart disease, heart septal defects, pulmonary hypertension