Poudel Priyanka, Pathak Sujan, Bhattarai Nishan
Kathmandu University School of Medical Sciences, Dhulikhel Hospital, Dhulikhel, 45210, Nepal.
Cardiology Unit, Department of Internal Medicine, Kathmandu University School of Medical Sciences, Dhulikhel Hospital, Dhulikhel, 45210, Nepal.
Ann Med Surg (Lond). 2022 Nov 5;84:104821. doi: 10.1016/j.amsu.2022.104821. eCollection 2022 Dec.
and importance: Ebstein's anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve associated with atrialization of the right ventricle. Most of the cases are diagnosed in childhood but asymptomatic cases may remain undiagnosed and survive until old age.
We present a rare case of Ebstein's Anomaly with pericardial effusion which was diagnosed for the first time in her mid-fifties when she developed atrial fibrillation and right heart failure with severe tricuspid regurgitation, which was managed medically.
The patient with Ebstein's anomaly can be found even at an older age with variable presentation and the association with pericardial effusion although very rare can present in such patients.
Ebstein's anomaly despite being a rare congenital condition can present clinically even beyond the age of fifty without any previous diagnosis and surgical intervention for the condition. It usually presents with features of heart failure and arrhythmia but can also have a rare association like pericardial effusion.
及重要性:埃布斯坦畸形(EA)是一种罕见的先天性心脏病,其特征为三尖瓣尖部移位并伴有右心室心房化。大多数病例在儿童期被诊断,但无症状病例可能未被诊断并存活至老年。
我们报告一例罕见的伴有心包积液的埃布斯坦畸形病例,该病例在患者五十多岁时首次被诊断,当时她出现房颤和右心衰竭,并伴有严重的三尖瓣反流,通过药物治疗进行处理。
埃布斯坦畸形患者即使在老年也可能被发现,表现各异,并且与心包积液的关联虽然非常罕见,但在这类患者中可能出现。
埃布斯坦畸形尽管是一种罕见的先天性疾病,但即使在五十岁以后也可能临床上出现,之前未被诊断且未进行手术干预。它通常表现为心力衰竭和心律失常的特征,但也可能有像心包积液这样罕见的关联情况。