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Clinical differences between children and adults with idiopathic and heritable pulmonary arterial hypertension

Published online by Cambridge University Press:  04 November 2022

Shinichi Takatsuki Open the ORCID record for Shinichi Takatsuki [Opens in a new window] ,
Hiroto Shimokawahara ,
Yurika Shimizu ,
Reiko Kawai ,
Hiroyuki Matsuura  and
Hiromi Matsubara
Shinichi Takatsuki*
Affiliation:
Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan
Hiroto Shimokawahara
Affiliation:
Department of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan
Yurika Shimizu
Affiliation:
Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan
Reiko Kawai
Affiliation:
Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan
Hiroyuki Matsuura
Affiliation:
Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan
Hiromi Matsubara
Affiliation:
Department of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan
*
Author for correspondence: S. Takatsuki, Department of Pediatrics, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 1438541, Japan. Tel: +81-3-3762-4151; Fax: +81-3-3298-8217. E-mail: s-taka@med.toho-u.ac.jp
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Abstract

Background:

Although previous studies have demonstrated that paediatric pulmonary arterial hypertension remains distinct from that in adults, there are limited studies evaluating a direct comparison between children and adults. The aim of this head-to-head comparison study was to compare the gender, haemodynamic parameters, and prognosis between paediatric and adult pulmonary arterial hypertension.

Methods and Results:

We retrospectively assessed the clinical differences in 40 childhood-onset (under 20 years old) patients and 40 adult-onset patients with idiopathic and heritable pulmonary arterial hypertension who were followed up at two centres. There was no female predominance among patients with childhood-onset pulmonary arterial hypertension (child female: 42.5%, adult female: 80%). The percent of New York Heart Association functional class IV in adult-onset pulmonary arterial hypertension tended to be higher than those in childhood-onset pulmonary arterial hypertension (22.5 and 10%, respectively), although children had worse haemodynamic parameters at diagnosis (mean pulmonary artery pressure (children versus adults); median 65 mmHg versus 49 mmHg, p < 0.001). There was no significant difference in the event-free survival rate between the two groups (95% vs. 85%) during the follow-up period (median, 96 months; range, 1–120 months).

Conclusions:

Although paediatric pulmonary arterial hypertension patients had worse haemodynamic parameters at diagnosis than adults, children survived as long as adults with appropriate therapeutic strategies.

Keywords

Childrenadultsprognosispulmonary arterial hypertension
Type
Original Article
Information
Cardiology in the Young , Volume 33 , Issue 10 , October 2023 , pp. 1909 - 1912
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

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