METHODS: Adults attending the National Centre for AS in England were studied. All patients underwent biochemical, 12- lead electrocardiography, echocardiography, and cardiovascular magnetic resonance imaging.
RESULTS: 47 adults with AS (64% male; mean age 33 years; 66% white British) were studied. Seven (15%) survived infantile cardiomyopathy and 23 (49%) developed adult-onset cardiomyopathy. Conventional risk factors for cardiovascular disease were present in 39 (83%). Abnormalities were present on biomarkers in 16 (34%), ECG 30 (64%), echocardiography 19 (40%) and CMR 31 (66%). Coronary artery imaging was performed in six (13%), with abnormalities in two. Cardiac, renal, and liver markers were more often impaired in older patients, with impaired left ventricular ejection fraction, reduced global longitudinal strain and late enhancement. 6 (13%) had severe pulmonary hypertension (mean pulmonary artery pressure 46 mmHg) due to left heart disease on invasive testing.
CONCLUSIONS: Cardiomyopathy is common in adults with AS, complicated in a significant proportion by atherosclerotic coronary artery disease and restrictive cardiomyopathy, confirmed on CMR and invasive testing. With advancing age, cardiovascular complications are compounded by contemporaneous renal and liver disease.
方法:研究了在英格兰国家AS中心就诊的成年人。所有患者均接受生化检查,12导联心电图,超声心动图,和心血管磁共振成像。
结果:研究了47名患有AS的成年人(64%为男性;平均年龄33岁;66%为英国白人)。7例(15%)在婴儿心肌病中幸存下来,23例(49%)发展为成人发作性心肌病。39例(83%)存在心血管疾病的常规危险因素。16位(34%)的生物标志物存在异常,心电图30(64%),超声心动图19(40%)和CMR31(66%)。6例(13%)进行了冠状动脉成像,有两个异常。心脏,肾,在老年患者中,肝脏标志物更容易受损,左心室射血分数受损,降低整体纵向应变和后期增强。6(13%)在侵入性测试中由于左心疾病而出现严重的肺动脉高压(平均肺动脉压46mmHg)。
结论:心肌病常见于成人AS,动脉粥样硬化性冠状动脉疾病和限制性心肌病占很大比例,在CMR和侵入性测试中确认。随着年龄的增长,同时发生的肾脏和肝脏疾病会加重心血管并发症。