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

• Published in: Journal of the American College of Cardiology Vol. 58; no. 24; pp. 2511 - 2519
• Main Authors: van de Veerdonk, Mariëlle C., MD, Kind, Taco, MD, Marcus, J. Tim, PhD, Mauritz, Gert-Jan, MSc, Heymans, Martijn W., PhD, Bogaard, Harm-Jan, MD, PhD, Boonstra, Anco, MD, PhD, Marques, Koen M.J., MD, PhD, Westerhof, Nico, PhD, Vonk-Noordegraaf, Anton, MD, PhD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 06.12.2011; Elsevier; Elsevier Limited
• Subjects: Biological and medical sciences; Cardiac Catheterization; Cardiology; Cardiology. Vascular system; Cardiovascular; Confidence intervals; Disease Progression; Exercise Test; Female; Heart; Heart failure, cardiogenic pulmonary edema, cardiac enlargement; Heart rate; hemodynamics; HIV; Human immunodeficiency virus; Humans; Hypertension, Pulmonary - complications; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - mortality; Internal Medicine; Intubation; Magnetic Resonance Imaging; Male; Medical prognosis; Medical sciences; Middle Aged; Mortality; Pneumology; pulmonary arterial hypertension; Pulmonary arteries; Pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; right ventricular function; Stroke Volume; survival; Survival Analysis; Variables; Vascular Resistance; Ventricular Dysfunction, Right - diagnosis; Ventricular Dysfunction, Right - etiology; Ventricular Dysfunction, Right - physiopathology; Ventricular Function, Right; Netherlands; ESVI; magnetic resonance imaging; RVEF; mean pulmonary artery pressure; RV; RHC; PVR; right heart catheterization; right ventricular function; cardiac output; 6-min walk test; pulmonary arterial hypertension; end-systolic volume index; right ventricular/ventricle; survival; CMR; PCWP; pulmonary capillary wedge pressure; CO; 6MWT; hemodynamics; EDVI; right ventricular ejection fraction; PAH; end-diastolic volume index; pulmonary vascular resistance; cardiac magnetic resonance; PAP; Human; Respiratory disease; Cardiovascular disease; Patient; Heart ventricle; Right ventricular failure; Artery; Pulmonary hypertension; Progressive; Treatment; Heart disease; Circulatory system; Cardiology
• ISSN: 0735-1097; 1558-3597
• DOI: 10.1016/j.jacc.2011.06.068

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.