Survival of left-to-right shunt repair in children with pulmonary arterial hypertension at a tertiary hospital in a low-to-middle-income country

Arafuri, Nadya and Murni, Indah K. and Idris, Nikmah S. and Uiterwaal, Cuno S.P.M. and Savitri, Ary I. and Nugroho, Sasmito and Noormanto, Noormanto (2021) Survival of left-to-right shunt repair in children with pulmonary arterial hypertension at a tertiary hospital in a low-to-middle-income country. Global Heart, 16 (1). ISSN 22118160

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Abstract

Background: In low-to-middle-income countries, repair of the left-to-right shunts congenital heart disease (CHD) are often done with existing pulmonary arterial hypertension (PAH). Long-term outcomes data of this condition in either low-to-middle or high-income countries are limited. We conducted a study to evaluate the outcomes of children with PAH related to left-to-right shunt CHD who underwent surgical or transcatheter repair. Methods: All cases of PAH related to left-to-right shunt CHD repairs from 2015–2018 were retrospectively reviewed with additional new patients who underwent repair within our study period (2019–2020). Cases with complex congenital heart disease and incomplete hemodynamic echocardiography or catheterization measurements were excluded. Kaplan-Meier curves, log-rank test, Cox regression with Firth’s correction and restricted mean survival time were used for survival analysis. Results: Of the 118 patients, 103 patients were enrolled and 15 patients were excluded due to complex congenital heart disease and missing hemodynamic measurements prior to repair. Overall, median age at intervention was 44 months, mPAP mean was 43.17 ± 16.05 mmHg and Pulmonary Vascular Resistance index (PVRi) mean was 2.84 ± 2.09 (WU.m2). Nine patients died after repair. The survival rate for patients with PAH-CHD at 1 day, 30 days and 1400 days (4 years) was 96.1, 92.1, and 91.0 respectively. Patients with persisting PAH after correction had –476.1 days (95 confidence interval CI: –714.4, –237,8) shorter survival over 4 years of follow up compared to patients with reversed PAH. PVRi was found to be the influencing covariate of the difference of restricted mean survival time between these groups. Conclusion: In low-to-middle income settings, with accurate PAH reversibility assessment prior to intervention, repair of left-to-right shunt CHD with existing PAH in children has a favourable outcome. Inferior survival is found in patients with persistence of PAH. PVRi at baseline predicts between-group survival difference. © 2021 The Author(s).

Item Type: Article
Additional Information: Cited by: 4; All Open Access, Gold Open Access, Green Open Access
Uncontrolled Keywords: Child; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Retrospective Studies; Tertiary Care Centers; hemoglobin; sildenafil; altitude; Article; cause of death; child; controlled study; Down syndrome; echocardiography; female; follow up; heart catheterization; heart left right shunt; heart right ventricle failure; heart surgery; human; Indonesia; lung vascular resistance; major clinical study; male; malnutrition; mean survival time; medical history; pediatrics; pericardial effusion; priority journal; pulmonary hypertension; retrospective study; survival; survival analysis; survival rate; survival time; tertiary care center; time of death; pulmonary hypertension
Subjects: R Medicine > RL Dermatology
Divisions: Faculty of Medicine, Public Health and Nursing > Public Health and Nutrition
Depositing User: Sri JUNANDI
Date Deposited: 18 Sep 2024 01:47
Last Modified: 18 Sep 2024 01:47
URI: https://ir.lib.ugm.ac.id/id/eprint/4850

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