Clinical Research
Pulmonary Hypertension
Progressive Right Ventricular Dysfunction in Patients With Pulmonary Arterial Hypertension Responding to Therapy

https://doi.org/10.1016/j.jacc.2011.06.068Get rights and content
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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.

Key Words

hemodynamics
magnetic resonance imaging
pulmonary arterial hypertension
right ventricular function
survival

Abbreviation and Acronyms

CMR
cardiac magnetic resonance
CO
cardiac output
EDVI
end-diastolic volume index
ESVI
end-systolic volume index
PAP
mean pulmonary artery pressure
PAH
pulmonary arterial hypertension
PCWP
pulmonary capillary wedge pressure
PVR
pulmonary vascular resistance
RHC
right heart catheterization
RV
right ventricular/ventricle
RVEF
right ventricular ejection fraction
6MWT
6-min walk test

Cited by (0)

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.