Kar Tanushree, Arora Yatin, Velayoudam Devagourou
Datta Meghe Institute of Higher Education and Research, Sawangi, Wardha Maharashtra 420001 India.
Department of CTVS, Yashoda Hospital and Research Centre, Ghaziabad, Uttar Pradesh 201001 India.
Indian J Thorac Cardiovasc Surg. 2024 Mar;40(2):227-230. doi: 10.1007/s12055-023-01622-4. Epub 2023 Nov 28.
A combination of a ruptured sinus of Valsalva (RSOV) with 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.
The online version contains supplementary material available at 10.1007/s12055-023-01622-4.
瓦氏窦破裂（RSOV）合并异常是一种罕见且在临床上具有挑战性的情况。我们描述了一例29岁女性病例，该患者表现为进行性呼吸困难和上腹部不适，其症状和临床发现与任何单一明确诊断均不相符。在初次超声心动图评估中，她被诊断为埃布斯坦畸形。基于一些无法解释的临床特征以及心脏病学团队的常规复查超声心动图，发现她患有罕见的埃布斯坦畸形合并RSOV。进行了心导管检查以研究这两种病变血流动力学的相互作用，这在之前的文献中未曾描述过，同时也解释了与任何单一病变均不相符的临床特征。我们强调在这种罕见关联中，详细的病史采集和完善的临床评估以及随后通过诊断方法进行确证对于精确诊断和及时规划干预的重要性。
在线版本包含可在10.1007/s12055-023-01622-4获取的补充材料。
