Cacciapuoti Fulvio, Mauro Ciro, Crispo Salvatore, Carpinella Gerardo, Volpicelli Mario
Division of Cardiology, "A. Cardarelli" Hospital, 80131 Naples, Italy.
Department of Cardiology, Division of Arrhythmology, "Santa Maria della Pietà" Hospital, 80035 Nola, Italy.
Reports (MDPI). 2025 May 18;8(2):72. doi: 10.3390/reports8020072.
Background and Clinical Significance: Scimitar Syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return, often requiring early surgical correction. It may coexist with other congenital or acquired cardiovascular anomalies, including valvular diseases such as mitral regurgitation. When surgical correction of Scimitar Syndrome is combined with mitral valve annuloplasty, the proximity to the atrioventricular node may potentially predispose patients to late-onset conduction disturbances, although causality remains speculative. Case Presentation: We describe the case of a 53-year-old male who developed paroxysmal atrial fibrillation, atrial flutter, and intermittent second-degree AV block decades after undergoing surgical correction of Scimitar Syndrome with concomitant mitral annuloplasty. Multimodal echocardiographic evaluation revealed preserved left atrial volume, normal intra-atrial conduction time, mildly reduced strain, and maintained atrial synchrony. The patient was treated with direct oral anticoagulants and beta-blockers and underwent the implantation of a ventricular leadless pacemaker. Conclusions: This case highlights the supportive role of atrial function imaging in assessing atrial health and informing rhythm management and procedural choices in surgically corrected congenital heart disease.
弯刀综合征是一种罕见的先天性心肺异常,其特征为部分肺静脉异位引流,通常需要早期手术矫正。它可能与其他先天性或后天性心血管异常并存,包括二尖瓣反流等瓣膜疾病。当弯刀综合征的手术矫正与二尖瓣环成形术联合进行时,尽管因果关系仍存在推测,但由于靠近房室结,患者可能易发生迟发性传导障碍。病例报告:我们描述了一名53岁男性的病例,该患者在接受弯刀综合征手术矫正并同期进行二尖瓣环成形术数十年后,出现阵发性心房颤动、心房扑动和间歇性二度房室传导阻滞。多模态超声心动图评估显示左心房容积保留、房内传导时间正常、应变轻度降低且心房同步性维持。该患者接受了直接口服抗凝剂和β受体阻滞剂治疗,并植入了无导线心室起搏器。结论:本病例突出了心房功能成像在评估心房健康以及为先天性心脏病手术矫正后的节律管理和手术选择提供信息方面的支持作用。