Dhillon Mukesh, Sharma Aditi
Department of Medicine, Command Hospital, Panchkula, India.
Department of Pediatrics, Command Hospital, Panchkula, India.
Egypt Heart J. 2024 Oct 29;76(1):145. doi: 10.1186/s43044-024-00572-5.
Developmental abnormalities of aortic valve cusps are relatively common with the bicuspid valve being the most frequently encountered congenital heart disease. However, the quadricuspid aortic valve (QAV) is an exceedingly rare abnormality.
We report a case involving a young, otherwise healthy male who presented with non-exertional syncope and was subsequently diagnosed with complete heart block (CHB). Further evaluation revealed the coexistence of a rare quadricuspid aortic valve and CHB. This combination, in the absence of surgery or infective endocarditis, has only been reported once before in the literature.The patient underwent successful permanent pacemaker implantation and continues to be monitored for aortic regurgitation.
The coexistence of a QAV with CHB, in the absence of infective endocarditis or aortic valve surgery, is extremely rare and necessitates careful evaluation and follow-up.
主动脉瓣叶发育异常相对常见，其中二叶式主动脉瓣是最常遇到的先天性心脏病。然而，四叶式主动脉瓣（QAV）是一种极其罕见的异常情况。
我们报告一例病例，患者为一名年轻的、其他方面健康的男性，因非劳力性晕厥就诊，随后被诊断为完全性心脏传导阻滞（CHB）。进一步评估发现罕见的四叶式主动脉瓣与CHB并存。在没有手术或感染性心内膜炎的情况下，这种组合在文献中仅被报道过一次。该患者成功植入了永久性起搏器，并继续接受主动脉瓣反流监测。
在没有感染性心内膜炎或主动脉瓣手术的情况下，QAV与CHB并存极为罕见，需要仔细评估和随访。
