Right ventricular and pulmonary vascular reserve in asymptomatic BMPR2 mutation carriers

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• 作者：Guido Claessen ; MD ; PhD^a ; ^b ; ¹ ; ^{guido.claessen@uzleuven.be} ; Andre La Gerche ; MD ; PhD^a ; ^c ; ¹ ; Thibault Petit ; MD^a ; ^b ; Hilde Gillijns ; BSc^a ; Jan Bogaert ; MD ; PhD^b ; ^d ; Mathias Claeys ; MD^a ; ^b ; Steven Dymarkowski ; MD ; PhD^b ; ^d ; Piet Claus ; MSc ; PhD^a ; Marion Delcroix ; MD ; PhD^b ; ^e ; ² ; Hein Heidbuchel ; MD ; PhD^f ; ²
• 关键词：BMPR2 ; cardiac magnetic resonance imaging ; exercise physiology ; hypoxia ; pulmonary vascular function ; right ventricular function
• 刊名：The Journal of Heart and Lung Transplantation
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：36
• 期：2
• 页码：148-156
• 全文大小：3404 K
• 卷排序：36

文摘

Non-invasive estimates have suggested that asymptomatic BMPR2 mutation carriers may have an abnormal pulmonary vascular response to exercise and hypoxia. However, this has not been assessed with “gold standard” invasive measures.MethodsEight 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 mm Hg/liter/min.ResultsDuring 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 mm 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.ConclusionsThe 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.