Mohd Nizam Mat Bah, Norazah Zahari, Noor Adibah Abdullah, Mohd Hanafi Sapian, EmieliyuzaYusnita Alias
Ministry of Health Malaysia and Hospital Sultanah Aminah. University of Malaya.
Malaysia
Pediatric Cardiology
Pediatr Cardiol 2024;
DOI: 10.1007/s00246-024-03732-1
Abstract
Limited studies are available on the outcome of infants with trisomy-21 and pulmonary hypertension (PHT) in lower- and middle-income countries. This population-based cohort study aims to determine the outcome and survival from birth to 5 years of infants with trisomy-21 and PHT born between 2016 and 2021. The mortality rate and Kaplan-Meier survival analysis were calculated to assess survival rates at 1 and 5 years. Multivariate Cox regression analysis was used to examine mortality-related factors. A total of 488 trisomy-21 infants were identified, with 176 (36%) having PHT and 245 (50%) having congenital heart disease (CHD). Of 176 PHT, 74 (42%) had moderate to severe PHT, and 115 (65%) patients had their PHT resolved at a median age of 7 weeks (Interquartile range [IQR]: 3 to 16.8 weeks), and 48 (27%) died at a median age of 3.6 months (IQR: 0.6 to 7.1 months). The survival at 1 and 5 years was 74% and 71%, respectively. The independent factors for mortality were infants with birth weight less than 2.5 kg (adjusted Hazard Ratio [aHR] 2.1 95% confidence interval [CI] 1.1-4.2, p = .02), symptomatic infants (aHR 3.3 95% CI 1.4-7.6, p = .006), late-onset PHT (aHR 3.8 95% CI 1.6-8.9, p = .002), with CHD (aHR 2.1 95% CI 1.1-4.2, p = .03) and those with Persistent Pulmonary Hypertension of Newborn [PPHN] (aHR 2.1 95% CI 1.0-4.3, p = .047). One-third of infants with trisomy-21 experienced PHT, with seven out of ten surviving until age five. Those with low birth weight, symptomatic infants, CHD, late-onset PHT, and PPHN were associated with low survival rates.
Category
Genetic Factors Associated with Pulmonary Vascular Disease
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Class I. Persistent Pulmonary Hypertension of the Newborn
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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