Ruptured sinus of Valsalva aneurysm in a case of Ebstein anomaly: a clinical masquerade of two perfectly balanced lesions.

在 Ebstein 异常的情况下 ， Valsalva 动脉瘤的破裂窦 ： 两个完全平衡的病变的临床伪装。

• 发表时间: 2024 Mar
• 来源期刊: Indian J Thorac Cardiovasc Surg
• DOI: 10.1007/s12055-023-01622-4
• PMID: 38389768
• 文章类型: Case Reports
• 作者列表: Kar T,Arora Y,Velayoudam D

关键词: Cath; Clinical evaluation; Diagnosis

Abstract

A combination of a ruptured sinus of Valsalva (RSOV) with Ebstein anomaly is a rare and clinically challenging entity. We describe a case of a 29-year-old female who presented with progressive dyspnoea and epigastric discomfort with symptoms and clinical findings not consistent with any single definitive diagnosis. In the primary echocardiography assessment, she was diagnosed with Ebstein anomaly. On the grounds of several unexplained clinical features and routine review echocardiography by the cardiology team, she was found to have a rare combination of Ebstein anomaly associated with RSOV. A cath study was performed to study the interplay of the hemodynamics of the two lesions, not described before in the literature, and also explain the clinical features not consistent with any one entity. We emphasize the significance of meticulous history-taking and sound clinical evaluation followed by corroboration with the diagnostic modalities for precise diagnosis and timely planned intervention in such rare associations.
UNASSIGNED: The online version contains supplementary material available at 10.1007/s12055-023-01622-4.

摘要

Valsalva破裂窦（RSOV）与Ebstein异常的组合是一种罕见且具有临床挑战性的实体。我们描述了一例29岁的女性，该女性患有进行性呼吸困难和上腹部不适，症状和临床表现与任何单一的明确诊断都不一致。在初次超声心动图评估中，她被诊断为Ebstein异常.根据几个原因不明的临床特征和心脏病学小组的常规超声心动图检查，她被发现患有与RSOV相关的Ebstein异常的罕见组合。进行了一项导管研究，以研究两个病变的血流动力学的相互作用，文献中没有描述过，并解释与任何一个实体不一致的临床特征。我们强调细致的病史记录和合理的临床评估的重要性，然后通过诊断方式进行确证，以精确诊断并及时计划干预这种罕见的关联。
■在线版本包含补充材料，可在10.1007/s12055-023-01622-4获得。