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Clinical Effects of Syncope on Disease Severity and Adverse Outcomes in Children with Idiopathic and Heritable Pulmonary Arterial Hypertension

  • Shinichi Takatsuki
  • Shun Yanai
  • Satoshi Ikehara
  • Tomotaka Nakayama
  • Horoyuki Matsuura
Original Article

Abstract

Syncope is more common in children with idiopathic pulmonary arterial hypertension (PAH) than in adults with PAH. Although syncope is associated with a poor prognosis in adult PAH, the clinical effects of syncopal events on disease severity and outcome in children have not been carefully investigated. This study assessed the prevalence of syncope in pediatric PAH and examined its clinical, hemodynamic, and prognostic importance. This retrospective study assessed clinical data, including syncope status, from 78 children (37 girls) with idiopathic and heritable PAH (median age at diagnosis, 11 years). Patients were classified as syncopal or non-syncopal, and clinical data from the two groups were compared. The primary outcome was a composite of lung transplantation and cardiac mortality. Overall, 31 (38%) children had a history of syncope at presentation. Median age at diagnosis, sex ratio, brain natriuretic peptide level, and 6-min walk distance at diagnosis did not differ between groups. The hemodynamic parameters of initial right heart catheterization were similar between the syncope and non-syncope group (mean pulmonary artery pressure, 67 versus 71 mm Hg; cardiac index, 2.9 versus 2.9 l/min/m2, respectively). There was not significantly difference in event-free survival rate between two groups. Although syncopal events are common in children with PAH, our findings suggest that syncope may not be correlated with disease severity or outcome in pediatric PAH.

Keywords

Preserved right ventricular function Prognosis Sudden death 

Abbreviations

BNP

Brain natriuretic peptide

ECG

Electrocardiogram

NYHA

New York Heart Association

PAH

Pulmonary arterial hypertension

6MWD

6-min walk distance

Notes

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Ethical Approval

All procedures performed in studies of human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all study participants.

Supplementary material

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Supplementary material 1 (DOCX 148 KB)
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Supplementary material 2 (DOCX 363 KB)
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Supplementary material 3 (DOCX 16 KB)
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Supplementary material 4 (DOCX 16 KB)

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Shinichi Takatsuki
    • 1
  • Shun Yanai
    • 1
  • Satoshi Ikehara
    • 1
  • Tomotaka Nakayama
    • 1
  • Horoyuki Matsuura
    • 1
  1. 1.Department of PediatricsToho University Omori Medical CenterTokyoJapan

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