Signs of right ventricular deterioration in clinically stable patients with pulmonary arterial hypertension.
BACKGROUND
Even after years of stable response to therapy, patients with idiopathic pulmonary arterial hypertension (IPAH) may show an unexpected clinical deterioration due to progressive right ventricular (RV) failure. Therefore, the aim of this study was to assess in 5-year clinically stable patients with IPAH whether initial differences or subsequent changes in RV volumes precede late clinical progression.


METHODS
Included were 22 clinically stable patients with IPAH as reflected by stable or improving New York Heart Association functional class II-III and exercise capacity during 5 years of follow-up. Twelve patients subsequently remained stable during a total follow-up of 10 years, whereas 10 other patients showed late progression leading to death or lung transplantation after a follow-up of 8 years. All patients underwent right-sided heart catheterization and cardiac MRI at baseline and at 1½, 3½, 6½, and, if still alive, 10 years follow-up.


RESULTS
Baseline hemodynamics were comparable in both groups and remained unchanged during the entire follow-up period. Baseline RV end-systolic volume (RVESV) was higher and RV ejection fraction (RVEF) was lower in late-progressive patients. Late-progressive patients demonstrated a gradually increased RV end-diastolic volume and RVESV and a decline in RVEF, whereas long-term stable patients did not show any RV changes.


CONCLUSIONS
In patients with stable IPAH for 5 years, subsequent late disease progression is preceded by changes in RV volumes. The results indicate that monitoring RV volumes anticipates clinical worsening, even at a time of apparent clinical stability.
Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
Mariëlle C
MC
van de Veerdonk
Department of Physics and Medical Technology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
J Tim
JT
Marcus
Department of Physiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
Nico
N
Westerhof
Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
Frances S
FS
de Man
Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
Anco
A
Boonstra
Department of Biostatistics and Epidemiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
Martijn W
MW
Heymans
Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands.
Harm-Jan
HJ
Bogaard
Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands. Electronic address: a.vonk@vumc.nl.
Anton
A
Vonk Noordegraaf
D000328:Adult; D006328:Cardiac Catheterization; D018450:Disease Progression; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013318:Stroke Volume; D013997:Time Factors; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology*; D016278:Ventricular Function, Right / Q000502:physiology*