title: Progressive changes in right ventricular geometric shortening and long-term survival in pulmonary arterial hypertension.
abstract: BACKGROUND
  Until now, many investigators have focused on describing right ventricular (RV) dysfunction in groups of patients with pulmonary arterial hypertension (PAH), but very few have addressed the deterioration of RV function over time. The aim of this study was to investigate time courses of RV geometric changes during the progression of RV failure.
  
  
  METHODS
  Forty-two patients with PAH were selected who underwent right-sided heart catheterization and cardiac MRI at baseline and after 1-year follow-up. Based on the survival after this 1-year run-in period, patients were classified into two groups: survivors (26 patients; subsequent survival of > 4 years) and nonsurvivors (16 patients; subsequent survival of < 4 years). Four-chamber cine imaging was used to quantify RV longitudinal shortening (apex-base distance change), RV transverse shortening (septum-free wall distance change), and RV fractional area change (RVFAC) between end diastole and end systole.
  
  
  RESULTS
  Longitudinal shortening, transverse shortening, and RVFAC measured at the beginning of the run-in period and 1 year later were significantly higher in subsequent survivors than in nonsurvivors (P < .05). Longitudinal shortening did not change during the run-in period in either patient group. Transverse shortening and RVFAC did not change during the run-in period in subsequent survivors but did decrease in subsequent nonsurvivors (P < .05). This decrease was caused by increased leftward septal bowing.
  
  
  CONCLUSIONS
  Progressive RV failure in PAH is associated with a parallel decline in longitudinal and transverse shortening until a floor effect is reached for longitudinal shortening. A further reduction of RV function is due to progressive leftward septal displacement. Because transverse shortening incorporates both free wall and septum movements, this parameter can be used to monitor the decline in RV function in end-stage PAH.
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Gert-Jan
authors.initials: GJ
authors.lastname: Mauritz
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Taco
authors.initials: T
authors.lastname: Kind
authors.affiliation: Department of Physics and Medical Technology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: J Tim
authors.initials: JT
authors.lastname: Marcus
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands; Department of Medicine, Division of Pulmonary and Critical Care, Virginia Commonwealth University, Richmond, VA.
authors.forename: Harm-Jan
authors.initials: HJ
authors.lastname: Bogaard
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Mariëlle
authors.initials: M
authors.lastname: van de Veerdonk
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Pieter E
authors.initials: PE
authors.lastname: Postmus
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Anco
authors.initials: A
authors.lastname: Boonstra
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands; Department of Physiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Nico
authors.initials: N
authors.lastname: Westerhof
authors.affiliation: Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands. Electronic address: a.vonk@vumc.nl.
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk-Noordegraaf
mesh_terms: D000328:Adult; D006328:Cardiac Catheterization; D003971:Diastole; D018450:Disease Progression; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006352:Heart Ventricles / Q000503:physiopathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D013599:Systole