Pediatric Cardiology

, Volume 40, Issue 1, pp 209–214 | Cite as

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

  • Shinichi TakatsukiEmail author
  • Shun Yanai
  • Satoshi Ikehara
  • Tomotaka Nakayama
  • Hiroyuki Matsuura
Original Article


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.


Preserved right ventricular function Prognosis Sudden death 



Brain natriuretic peptide




New York Heart Association


Pulmonary arterial hypertension


6-min walk distance


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)


  1. 1.
    Takatsuki S, Ivy DD (2013) Current challenges in pediatric pulmonary hypertension. Semin Respir Crit Care Med 34:627–644CrossRefGoogle Scholar
  2. 2.
    Barst RJ, McGoon MD, Elliot CG, Foreman AJ, Miller DP, Ivy DD (2012) Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 125:113–122CrossRefGoogle Scholar
  3. 3.
    Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC et al (2012) Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 379:537–546CrossRefGoogle Scholar
  4. 4.
    Howarth S, Lowe J (1953) The mechanism of effort syncope in primary pulmonary hypertension and cyanotic congenital heart disease. Br Heart J 15:47–54CrossRefGoogle Scholar
  5. 5.
    Mikhail GW, Gibbs JSR, Yacoub MH (2001) Pulmonary and systemic arterial pressure changes during syncope in primary pulmonary hypertension. Circulation 104:1326–1327CrossRefGoogle Scholar
  6. 6.
    James TN (1962) On the cause of syncope and sudden death in primary pulmonary hypertension. Ann Intern Med 56:252–264CrossRefGoogle Scholar
  7. 7.
    Rubin LJ, Badesch DB (2005) Evaluation and management of the patient with pulmonary arterial hypertension. Ann Intern Med 143:282–292CrossRefGoogle Scholar
  8. 8.
    Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A et al (2016) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 37:67–119CrossRefGoogle Scholar
  9. 9.
    Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK et al (2015) Pediatric pulmonary hypertension: guidelines from the american heart association and american thoracic society. Circulation 132:2037–2099CrossRefGoogle Scholar
  10. 10.
    Le RJ, Fenstad ER, Maradit-Kremers H, McCully RB, Frantz RP, McGoon MD et al (2011) Syncope in adults with pulmonary arterial hypertension. J Am Coll Cardiol 58:863–867CrossRefGoogle Scholar
  11. 11.
    Blumberg FC, Arzt M, Lange T, Schroll S, Pfeifer M, Wensel R (2013) Impact of right ventricular reserve on exercise capacity and survival in patients with pulmonary hypertension. Eur J Heart Fail 15:771–775CrossRefGoogle Scholar
  12. 12.
    Grünig E, Tiede H, Enyimayew EO, Ehlken N, Seyfarth HJ, Bossone E et al (2013) Assessment and prognostic relevance of right ventricular contractile reserve in patients with severe pulmonary hypertension. Circulation 128:2005–2015CrossRefGoogle Scholar
  13. 13.
    Rajdev A, Garan H, Biviano A (2012) Arrhythmias in pulmonary arterial hypertension. Prog Cardiovasc Dis 55:180–186CrossRefGoogle Scholar
  14. 14.
    Ivy DD, Abman SH, Barst RJ, Berger RM, Bonnet D, Fleming TR et al (2013) Pediatric pulmonary hypertension. J Am Coll Cardiol 62:D117–D126CrossRefGoogle Scholar

Copyright information

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

Authors and Affiliations

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

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