Babi Amal, Shebli Baraa, Ghabally Mike, Alkanj Hussein
Department of Echocardiography, Aleppo University Hospital, University of Aleppo, Aleppo, Syria.
Cardiology Division, Internal Medicine Department, Aleppo University Hospital, University of Aleppo, Aleppo, Syria.
Oxf Med Case Reports. 2024 Nov 20;2024(11):omae131. doi: 10.1093/omcr/omae131. eCollection 2024 Nov.
Intravascular leiomyoma (IVL) with intracardiac extension (ICE) represents an exceedingly rare diagnosis of a cardiac mass. We present the case of a 42-year-old woman with recurrent syncopal episodes. Cardiac investigations revealed an extensive, mobile mass stretching from the inferior vena cava (IVC) through the right heart to the bifurcation of the pulmonary artery. Emergent surgery was conducted to excise the mass. Post-operative assessment indicated a potential malignancy in the adnexa. A subsequent surgery to resect the uterus with the adnexa, the primary origin of the mass, confirmed the diagnosis of IVL with ICE. The initial diagnostic ambiguity and the urgent pulmonary artery involvement necessitated a two-step surgical approach. Despite the propensity for recurrence, a 5-year follow-up remained unremarkable. This case underscores the importance of considering IVL with ICE in the differential diagnosis, which can expedite both diagnosis and treatment.
血管内平滑肌瘤(IVL)伴心内延伸(ICE)是一种极为罕见的心脏肿块诊断。我们报告一例42岁复发性晕厥发作的女性病例。心脏检查发现一个广泛的、可移动的肿块,从下腔静脉(IVC)延伸穿过右心至肺动脉分叉处。紧急进行手术切除肿块。术后评估显示附件有潜在恶性病变。随后进行手术切除肿块的原发部位子宫及附件,确诊为IVL伴ICE。最初的诊断不明确以及肺动脉的紧急受累需要采取两步手术方法。尽管有复发倾向,但5年随访结果无异常。该病例强调了在鉴别诊断中考虑IVL伴ICE的重要性,这可以加快诊断和治疗。