title: Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy.
abstract: OBJECTIVE
  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.
authors: Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, the Netherlands.
authors: Mariëlle C
authors: MC
authors: van de Veerdonk
authors: Taco
authors: T
authors: Kind
authors: J Tim
authors: JT
authors: Marcus
authors: Gert-Jan
authors: GJ
authors: Mauritz
authors: Martijn W
authors: MW
authors: Heymans
authors: Harm-Jan
authors: HJ
authors: Bogaard
authors: Anco
authors: A
authors: Boonstra
authors: Koen M J
authors: KM
authors: Marques
authors: Nico
authors: N
authors: Westerhof
authors: Anton
authors: A
authors: Vonk-Noordegraaf
mesh_terms: D006328:Cardiac Catheterization; D018450:Disease Progression; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013318:Stroke Volume; D016019:Survival Analysis; D014655:Vascular Resistance; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology*; D016278:Ventricular Function, Right