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Please use this identifier to cite or link to this item: http://hdl.handle.net/1942/23754

Title: Right ventricular and pulmonary vascular reserve in asymptomatic BMPR2 mutation carriers
Authors: Claessen, Guido
La Gerche, Andre
Petit, Thibault
Gillijns, Hilde
Bogaert, Jan
Claeys, Mathias
Dymarkowski, Steven
Claus, Piet
Delcroix, Marion
Heidbuchel, Hein
Issue Date: 2017
Publisher: ELSEVIER SCIENCE INC
Citation: JOURNAL OF HEART AND LUNG TRANSPLANTATION, 36(2), p. 148-156
Abstract: BACKGROUND: Non-invasive estimates have suggested that asymptomatic BMPR2 mutation cat-tiers may have an abnormal pulmonary vascular response to exercise and hypoxia. However, this has not been assessed with "gold standard" invasive measures. METHODS: Eight controls and 8 asymptomatic BMPR2 mutation carriers underwent cardiac magnetic resonance imaging with simultaneous invasive pressure recording during bicycle exercise in normoxia, hypoxia and after sildenafil administration. Abnormal pulmonary vascular reserve was defined as an increase in mean pulmonary artery pressure relative to cardiac output (P/Q slope) >3 nun Hg/liter/min. RESULTS: During normoxic exercise, BMPR2 mutation carriers had a similar P/Q slope when compared with healthy subjects. Only 1 of 8 BMPR2 mutation carriers had a P/Q slope >3 mm Hg/liter/min. During exercise in hypoxia, 3 of 8 BMPR2 mutation carriers had P/Q slopes >3 nun Hg/liter/min compared with none of the controls. Sildenafil decreased the P/Q slope both in controls and BMPR2 mutation carriers. The exercise-induced increase in right ventricular ejection fraction was similar between groups. None of the BMPR2 mutation carriers developed pulmonary arterial hypertension within 2 (range 1.3 to 2.8) years. CONCLUSIONS: The presence of a BMPR2 mutation, per se, is not associated with an abnormal pulmonary vascular and right ventricular functional response to exercise in asymptomatic individuals. Longer follow-up will be required to determine whether a P/Q slope of >3 mm Hg/liter/min during exercise in normoxia or hypoxia is a sign of pre-clinical disease expression. (C) 2016 International Society for Heart and Lung Transplantation. All rights reserved.
Notes: [Claessen, Guido; La Gerche, Andre; Petit, Thibault; Gillijns, Hilde; Claeys, Mathias; Claus, Piet] Katholieke Univ Leuven, Dept Cardiovasc Sci, Leuven, Belgium. [Claessen, Guido; Petit, Thibault; Bogaert, Jan; Claeys, Mathias; Dymarkowski, Steven; Delcroix, Marion] Univ Hosp Leuven, Leuven, Belgium. [La Gerche, Andre] Baker IDI Heart & Diabet Inst, Melbourne, Vic, Australia. [Bogaert, Jan; Dymarkowski, Steven] Katholieke Univ Leuven, Dept Imaging & Pathol, Leuven, Belgium. [Delcroix, Marion] Katholieke Univ Leuven, Dept Clin & Expt Med, Leuven, Belgium. [Heidbuchel, Hein] Univ Hasselt, Hasselt, Belgium. [Heidbuchel, Hein] Jessa Hosp, Ctr Heart, Hasselt, Belgium.
URI: http://hdl.handle.net/1942/23754
DOI: 10.1016/j.healun.2016.06.018
ISI #: 000393532500006
ISSN: 1053-2498
Category: A1
Type: Journal Contribution
Appears in Collections: Research publications

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