Shadman Shahrad, Asadi Mohammad Sadegh, Nomigolzar Soroush, Sarfarazi Mohammad
Internal Medicine, University of Maryland Capital Region Health, Cheverly, USA.
Cardiovascular Disease, Howard University Hospital, Washington, D.C., USA.
Cureus. 2021 Jan 29;13(1):e12986. doi: 10.7759/cureus.12986.
Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly that commonly becomes symptomatic in the fifth or sixth decade of life and can present as an isolated finding or in association with other congenital cardiovascular abnormalities. Previously, QAV was mainly a postmortem or intraoperative diagnosis and data was very limited on its natural history, associated complications, and long-term outcomes. In recent decades, however, there has been an increase in the reported cases of QAV, considering the advances in the diagnostic modalities such as transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance (CMR). In this article, we present a case of a congenital QAV associated with aortic regurgitation as well as briefly review the QAV classification systems, treatment options, and long-term outcomes. A 48-year-old woman with a significant past medical history (PMH) of hypertension and coronary artery disease presented with shortness of breath, chest pain, and orthopnea for two to three weeks. The patient's TTE showed severe aortic regurgitation with significant compromise in cardiac output that could not be otherwise explained. Subsequently, her TEE revealed QAV as the underlying source of these findings. Although the diagnosis of QAV could be very challenging, it is crucial to be considered when evaluating a patient with inscrutable progressive aortic regurgitation. Today, as a result of technological advancement, QAV is being diagnosed more accurately and promptly. Since there are no universal guidelines defined for this cardiac anomaly, regular follow-up with these patients is imperative to monitor for early signs of valvular compromise and to treat accordingly through medical and surgical interventions.
四叶式主动脉瓣（QAV）是一种罕见的先天性心脏异常，通常在生命的第五或第六个十年出现症状，可单独出现或与其他先天性心血管异常相关。以前，QAV主要是尸检或术中诊断，关于其自然病史、相关并发症和长期预后的数据非常有限。然而，近几十年来，考虑到经胸超声心动图（TTE）、经食管超声心动图（TEE）和心脏磁共振（CMR）等诊断方式的进步，QAV的报告病例有所增加。在本文中，我们介绍一例与主动脉瓣反流相关的先天性QAV病例，并简要回顾QAV的分类系统、治疗选择和长期预后。一名48岁有高血压和冠状动脉疾病重要既往病史（PMH）的女性，出现呼吸急促、胸痛和端坐呼吸两到三周。患者的TTE显示严重主动脉瓣反流，心输出量显著受损，无法用其他原因解释。随后，她的TEE显示QAV是这些发现的潜在原因。虽然QAV的诊断可能非常具有挑战性，但在评估患有难以解释的进行性主动脉瓣反流的患者时，必须考虑到这一点。如今，由于技术进步，QAV能够更准确、迅速地被诊断出来。由于针对这种心脏异常没有普遍适用的指南，对这些患者进行定期随访以监测瓣膜损害的早期迹象并通过药物和手术干预进行相应治疗至关重要。
