Author + information
- Received May 5, 2011
- Accepted June 7, 2011
- Published online December 6, 2011.
- Mariëlle C. van de Veerdonk, MD⁎,
- Taco Kind, MD⁎,
- J. Tim Marcus, PhD†,
- Gert-Jan Mauritz, MSc⁎,
- Martijn W. Heymans, PhD‡,
- Harm-Jan Bogaard, MD, PhD⁎,§,
- Anco Boonstra, MD, PhD⁎,
- Koen M.J. Marques, MD, PhD∥,
- Nico Westerhof, PhD⁎,¶ and
- Anton Vonk-Noordegraaf, MD, PhD⁎,⁎ ()
- ↵⁎Reprint requests and correspondence:
Prof. Anton Vonk-Noordegraaf, Department of Pulmonary Diseases, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, the Netherlands
Objectives The purpose of this study was to examine the relationship between changes in pulmonary vascular resistance (PVR) and right ventricular ejection fraction (RVEF) and survival in patients with pulmonary arterial hypertension (PAH) under PAH-targeted therapies.
Background Despite the fact that medical therapies reduce PVR, the prognosis of patients with PAH is still poor. The primary cause of death is right ventricular (RV) failure. One possible explanation for this apparent paradox is the fact that a reduction in PVR is not automatically followed by an improvement in RV function.
Methods A cohort of 110 patients with incident PAH underwent baseline right heart catheterization, cardiac magnetic resonance imaging, and 6-min walk testing. These measurements were repeated in 76 patients after 12 months of therapy.
Results Two patients underwent lung transplantation, 13 patients died during the first year, and 17 patients died in the subsequent follow-up of 47 months. Baseline RVEF (hazard ratio [HR]: 0.938; p = 0.001) and PVR (HR: 1.001; p = 0.031) were predictors of mortality. During the first 12 months, changes in PVR were moderately correlated with changes in RVEF (R = 0.330; p = 0.005). Changes in RVEF (HR: 0.929; p = 0.014) were associated with survival, but changes in PVR (HR: 1.000; p = 0.820) were not. In 68% of patients, PVR decreased after medical therapy. Twenty-five percent of those patients with decreased PVR showed a deterioration of RV function and had a poor prognosis.
Conclusions After PAH-targeted therapy, RV function can deteriorate despite a reduction in PVR. Loss of RV function is associated with a poor outcome, irrespective of any changes in PVR.
- magnetic resonance imaging
- pulmonary arterial hypertension
- right ventricular function
Dr. Kind was financially supported by the Netherlands Organisation for Scientific Research, Toptalent grant, project 021.001.120. Dr. Vonk-Noordegraaf was financially supported by the NWO, Vidi grant, project 91.796.306. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received May 5, 2011.
- Accepted June 7, 2011.
- American College of Cardiology Foundation