TY - JOUR
T1 - Signs of Right Ventricular Deterioration in Clinically Stable Patients With Pulmonary Arterial Hypertension
AU - van de Veerdonk, Marielle C.
AU - Marcus, J. Tim
AU - Westerhof, Nico
AU - de Man, Frances S.
AU - Boonstra, Anco
AU - Heymans, Martijn W.
AU - Bogaard, Harm-Jan
AU - Vonk Noordegraaf, Anton
N1 - Funding Information: Financial/nonfinancial disclosures: The authors have reported toCHESTthe following conflicts of interest: Dr Marcus has a consultancy agreement with Actelion Pharmaceuticals Ltd. Dr Boonstra has or had financial relationships with companies including, but not limited to, Actelion Pharmaceuticals Ltd; Pfizer, Inc; Teva Pharmaceutical Industries Ltd; Sun Pharmaceuticals Industries Ltd; Bayer HealthCare AG; United Therapeutics Corporation; THERABEL Group; Ferrer Internacional; Boehringer Ingelheim GmbH; GlaxoSmithKline plc; mondoBIOTECH Holding AG; and Chiesi Pharmaceuticals BV. In addition to being an investigator in trials involving these companies, relationships include financial reimbursement for consultancy service, membership on scientific advisory boards, and speaker fees. All payments from 2012 larger than ɛ500 can be found on www.transparantieregister.nl under number 59020144101. Dr Bogaard has received research support from Boehringer Ingelheim GmbH; speaker fees from Bayer HealthCare AG and Pfizer, Inc; and consulting fees from Bayer HealthCare AG, United Therapeutics Corporation, and Actelion Pharmaceuticals Ltd. Dr Vonk Noordegraaf has received lecture fees from Actelion Pharmaceuticals Ltd; Bayer HealthCare AG; GlaxoSmithKline plc; Eli Lilly and Company; and Pfizer, Inc, and served on industry advisory boards for Actelion Pharmaceuticals Ltd and Bayer HealthCare AG and on steering committees for Actelion Pharmaceuticals Ltd; Bayer HealthCare AG; and Pfizer, Inc. Drs van de Veerdonk, Westerhof, de Man, and Heymans have reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article. Funding Information: Funding/Support: Drs de Man and Vonk Noordegraaf were financially supported by Vidi [91.796.306] and Veni [016.146.099] grants from the Dutch Foundation for Scientific Research (NWO). Drs de Man, Bogaard, and Vonk-Noordegraaf were further supported by CardioVasculair Onderzoek Nederland [CVON 2012-08 ] . Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details. Publisher Copyright: © 2015 American College of Chest Physicians.
PY - 2015
Y1 - 2015
N2 - BACKGROUND: Even aft er 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 aft er a follow-up of 8 years. All patients underwent right-sided heart catheterization and cardiac MRI at baseline and at 11/2, 31/2, 61/2, 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
AB - BACKGROUND: Even aft er 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 aft er a follow-up of 8 years. All patients underwent right-sided heart catheterization and cardiac MRI at baseline and at 11/2, 31/2, 61/2, 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
UR - http://www.scopus.com/inward/record.url?scp=84927135587&partnerID=8YFLogxK
U2 - https://doi.org/10.1378/chest.14-0701
DO - https://doi.org/10.1378/chest.14-0701
M3 - Article
C2 - 25376008
SN - 0012-3692
VL - 147
SP - 1063
EP - 1071
JO - Chest
JF - Chest
IS - 4
ER -