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European Heart Journal Advance Access originally published online on January 22, 2007
European Heart Journal 2007 28(10):1250-1257; doi:10.1093/eurheartj/ehl477
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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension

Serge A. van Wolferen1, Johannes T. Marcus2, Anco Boonstra1, Koen M.J. Marques3, Jean G.F. Bronzwaer3, Marieke D. Spreeuwenberg4, Pieter E. Postmus1 and Anton Vonk-Noordegraaf1,*

1 Department of Pulmonary Diseases, Institute for Cardiovascular Research ICaR-VU, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands
2 Department of Physics and Medical Technology, Institute for Cardiovascular Research ICaR-VU, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands
3 Department of Cardiology, Institute for Cardiovascular Research ICaR-VU, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands
4 Clinical Epidemiology and Biostatistics, Institute for Cardiovascular Research ICaR-VU, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands

Received 19 June 2006; revised 11 December 2006; accepted 21 December 2006; online publish-ahead-of-print 22 January 2007.

* Corresponding author. Tel: ±31 20 4444728; fax: ±31 20 4444382. E-mail address: a.vonk{at}vumc.nl

See page 1187 for the editorial comment on this article (doi:10.1093/eurheartj/ehm074)

Aims: This study investigated the relationship between right ventricular (RV) structure and function and survival in idiopathic pulmonary arterial hypertension (IPAH).

Methods and results: In 64 patients, cardiac magnetic resonance, right heart catheterization, and the six-minute walk test (6MWT) were performed at baseline and after 1-year follow-up. RV structure and function were analysed as predictors of mortality. During a mean follow-up of 32 months, 19 patients died. A low stroke volume (SV), RV dilatation, and impaired left ventricular (LV) filling independently predicted mortality. In addition, a further decrease in SV, progressive RV dilatation, and further decrease in LV end-diastolic volume (LVEDV) at 1-year follow-up were the strongest predictors of mortality. According to Kaplan–Meier survival curves, survival was lower in patients with an inframedian SV index ≤ 25 mL/m2, a supramedian RV end-diastolic volume index ≥ 84 mL/m2, and an inframedian LVEDV≤40 mL/m2.

Conclusions: The RV contains prognostic information in IPAH. A large RV volume, low SV, and a reduced LV volume are strong independent predictors of mortality and treatment failure.

Key Words: Pulmonary hypertension • Prognosis • Magnetic resonance imaging • Right ventricle


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