Right ventricular and pulmonary vascular reserve in asymptomatic BMPR2 mutation carriers
Right ventricular and pulmonary vascular reserve in asymptomatic BMPR2 mutation carriers
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DOI：
10.1016/j.healun.2016.06.018
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发表时间：
2017-02-01
影响因子：
8.9
通讯作者：
Heidbuchel, Hein
中科院分区：
文献类型：
--
作者：
Claessen, Guido;La Gerche, Andre;Heidbuchel, Hein
背景：无创性估计表明，无症状的BMPR2突变猫可能对运动和低氧有异常的肺血管反应。方法：8名对照组和8名无症状的BMPR2突变携带者在常氧、低氧和给予西地那非后的踏车运动中进行心脏磁共振成像和同步有创压力记录。肺血管储备异常定义为平均肺动脉压相对于心输出量(P/Q斜率)的增加(P/Q斜率)。结果：在常氧运动中，BMPR2突变携带者的P/Q斜率与正常对照组相似。8名BMPR2突变携带者中只有1人的P/Q斜率为3毫米汞/升/分。在低氧运动中，8名BMPR2突变携带者中有3名的P/Q斜率为3nnHg/L/min，与无对照组相比。西地那非降低了对照组和BMPR2突变携带者的P/Q斜率。运动引起的右室射血分数的增加在两组之间是相似的。BMPR2突变携带者中无一人在2年内(1.3~2.8)内发生肺动脉高压。结论：BMPR2突变的存在本身与无症状个体的肺血管和右室功能异常反应无关。在常氧或低氧条件下锻炼时，P/Q斜率达到3毫米汞柱/升/分钟，是否是临床前疾病表现的标志，还需要更长时间的跟踪调查。(C)2016年国际心肺移植学会。版权所有。
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.