Left main coronary artery compression by a dilated main pulmonary artery and left coronary sinus of Valsalva aneurysm in a patient with heritable pulmonary arterial hypertension and FLNA mutation.
Left main coronary artery compression by a dilated main pulmonary artery and left coronary sinus of Valsalva aneurysm in a patient with heritable pulmonary arterial hypertension and FLNA mutation.
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DOI：
10.1177/2045893217716107
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发表时间：
2017-07
影响因子：
2.6
通讯作者：
Kondo T
中科院分区：
文献类型：
--
作者：
Hirashiki A;Adachi S;Nakano Y;Kamimura Y;Ogo T;Nakanishi N;Morisaki T;Morisaki H;Shimizu A;Toba K;Murohara T;Kondo T
左冠状动脉主干病变是一种罕见的临床疾病，其原因是由于扩张的主肺动脉（MPA）的外部压迫。在这里，我们描述了一个52岁的女性肺动脉高压（PAH）和前间隔陈旧性心肌梗死（OMI）。OMI的原因是扩张的MPA和左冠状窦动脉瘤对LMCA的外部压迫。患者的姐姐（56岁）也被诊断为PAH，两名女性在细丝蛋白A（FLNA）基因中均存在新型杂合剪接突变IVS 2 -2A > G（NM_001456中c.374-2A > G）。据我们所知，这是第一次报告的高肺动脉高压，这可能是由于FLNA突变和压缩的LMCA之间的扩张MPA和动脉瘤的左冠状窦的主动脉。
Left main coronary artery (LMCA) disease due to external compression by a dilated main pulmonary artery (MPA) is an uncommon clinical entity. Here, we describe a 52-year-old woman with pulmonary arterial hypertension (PAH) and anteroseptal old myocardial infarction (OMI). The cause of the OMI was external compression of the LMCA by the dilated MPA and aneurysm of the left coronary sinus of Valsalva. The patient’s sister (aged 56 years) had also been diagnosed with PAH and both women had a novel heterozygous splicing mutation, IVS2-2A > G (c.374-2A > G in NM_001456), in the filamin A (FLNA) gene. To our knowledge, this is the first report of HPAH which is likely to be due to FLNA mutation and compression of the LMCA between a dilated MPA and aneurysm of the left coronary sinus of Valsalva.
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影响因子：
9.8
作者：
Zenker, M;Rauch, A;Reis, A
通讯作者：
Reis, A
影响因子：
0.6
作者：
Faillace, Bruno L. R.;Galon, Micheli Z.;Lemos, Pedro A.
通讯作者：
Lemos, Pedro A.
影响因子：
16.2
作者：
Fox, JW;Lamperti, ED;Walsh, CA
通讯作者：
Walsh, CA
影响因子：
2.3
作者：
Chaikriangkrai, Kongkiat;Polsani, Venkateshwar;Chang, Su Min
通讯作者：
Chang, Su Min
影响因子：
2.6
作者：
Jaijee, Shareen K.;Ariff, Ben;Gibbs, J. Simon R.
通讯作者：
Gibbs, J. Simon R.