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Original Research: Pulmonary Vascular Disease |

Signs of Right Ventricular Deterioration in Clinically Stable Patients With Pulmonary Arterial HypertensionHeart Failure in Pulmonary Arterial Hypertension

Mariëlle C. van de Veerdonk, MD; J. Tim Marcus, PhD; Nico Westerhof, PhD; Frances S. de Man, PhD; Anco Boonstra, MD, PhD; Martijn W. Heymans, PhD; Harm-Jan Bogaard, MD, PhD; Anton Vonk Noordegraaf, MD, PhD
Author and Funding Information

From the Department of Pulmonary Diseases (Drs van de Veerdonk, de Man, Boonstra, Bogaard, and Vonk Noordegraaf), Department of Physics and Medical Technology (Dr Marcus), Department of Physiology (Dr Westerhof), and Department of Biostatistics and Epidemiology (Dr Heymans), Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.

CORRESPONDENCE TO: Anton Vonk Noordegraaf, MD, PhD, Department of Pulmonary Diseases, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands; e-mail: a.vonk@vumc.nl


FUNDING/SUPPORT: Drs de Man and Vonk Noordegraaf were financially supported by Vidi [91.796.306] and Veni [016.146.099] grants from the Dutch Foundation for Scientific Research (NWO). Drs de Man, Bogaard, and Vonk-Noordegraaf were further supported by CardioVasculair Onderzoek Nederland [CVON 2012-08].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;147(4):1063-1071. doi:10.1378/chest.14-0701
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BACKGROUND:  Even after years of stable response to therapy, patients with idiopathic pulmonary arterial hypertension (IPAH) may show an unexpected clinical deterioration due to progressive right ventricular (RV) failure. Therefore, the aim of this study was to assess in 5-year clinically stable patients with IPAH whether initial differences or subsequent changes in RV volumes precede late clinical progression.

METHODS:  Included were 22 clinically stable patients with IPAH as reflected by stable or improving New York Heart Association functional class II-III and exercise capacity during 5 years of follow-up. Twelve patients subsequently remained stable during a total follow-up of 10 years, whereas 10 other patients showed late progression leading to death or lung transplantation after a follow-up of 8 years. All patients underwent right-sided heart catheterization and cardiac MRI at baseline and at 1½, 3½, 6½, and, if still alive, 10 years follow-up.

RESULTS:  Baseline hemodynamics were comparable in both groups and remained unchanged during the entire follow-up period. Baseline RV end-systolic volume (RVESV) was higher and RV ejection fraction (RVEF) was lower in late-progressive patients. Late-progressive patients demonstrated a gradually increased RV end-diastolic volume and RVESV and a decline in RVEF, whereas long-term stable patients did not show any RV changes.

CONCLUSIONS:  In patients with stable IPAH for 5 years, subsequent late disease progression is preceded by changes in RV volumes. The results indicate that monitoring RV volumes anticipates clinical worsening, even at a time of apparent clinical stability.

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