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一例成年法洛四联症修复术后因多种晚期并发症导致的心力衰竭病例。

A case of heart failure due to multiple late complications after repair of tetralogy of Fallot in adulthood.

作者信息

Goten Chiaki, Usui Soichiro, Takatori Osamu, Sakata Kenji, Murata Akira, Takemura Hirofumi, Takamura Masayuki

机构信息

Department of Cardiovascular Medicine, Kanazawa University, Kanazawa, Japan.

Department of Cardiology, Tonami General Hospital, Tonami, Japan.

出版信息

J Cardiol Cases. 2025 Apr 2;32(1):23-26. doi: 10.1016/j.jccase.2025.03.010. eCollection 2025 Jul.

Abstract

UNLABELLED

The prognosis of tetralogy of Fallot (TOF) has improved in recent years, but complications in the late postoperative period remain a serious problem. These complications, combined with specific hemodynamic and structural abnormalities, make it difficult to determine the optimal treatment plan. A man in his early 60s had been diagnosed with TOF in his early teens; he had undergone ventricular septal defect closure and right ventricular outflow tract repair in his mid-20s. Approximately 40 years after surgery, he was referred to our hospital because of worsening heart failure due to moderate aortic regurgitation with left ventricular dysfunction caused by a residual ventricular septal defect, marked continuous right ventricular dilation and dysfunction, and severe pulmonary and tricuspid regurgitation. The patient had clearly missed the optimal time for surgery and had a high surgical risk score. After the patient had been provided sufficient information regarding treatment and risks, he underwent pulmonary and aortic valve replacement, tricuspid annuloplasty, and shunt closure. No obvious perioperative complications were observed, and the heart failure had remained stable for 4 years following reoperation. We report this complicated case of TOF repaired in adulthood with marked biventricular remodeling, associated with a residual shunt and progression of valvular disease.

LEARNING OBJECTIVE

Surgical interventions for the multiple complications that occur during the long-term course after TOF repair, particularly in the context of right ventricular remodeling, may be associated with risks. In the field of adult congenital heart disease, rather than applying a single cut-off to determine the optimal timing for intervention, clinicians should consider factors such as the patients' age, sex, and other individual characteristics, paying particular attention to the hemodynamic status.

摘要

未标注

近年来,法洛四联症(TOF)的预后有所改善,但术后晚期并发症仍是一个严重问题。这些并发症,再加上特定的血流动力学和结构异常,使得确定最佳治疗方案变得困难。一名60岁出头的男性在十几岁时被诊断出患有TOF;他在25岁左右接受了室间隔缺损修补和右心室流出道修复手术。术后约40年,他因中度主动脉瓣反流伴左心室功能障碍导致心力衰竭加重而被转诊至我院,病因包括残余室间隔缺损、明显持续的右心室扩张和功能障碍以及严重的肺动脉瓣和三尖瓣反流。该患者显然错过了最佳手术时机,手术风险评分较高。在向患者提供了关于治疗和风险的充分信息后,他接受了肺动脉瓣和主动脉瓣置换、三尖瓣环成形术以及分流关闭术。围手术期未观察到明显并发症,再次手术后心力衰竭已稳定4年。我们报告了这例成年期修复的复杂TOF病例,伴有明显的双心室重塑,存在残余分流和瓣膜疾病进展。

学习目标

TOF修复术后长期过程中出现的多种并发症的外科干预,尤其是在右心室重塑的情况下,可能存在风险。在成人先天性心脏病领域,临床医生不应采用单一的临界值来确定最佳干预时机,而应考虑患者的年龄、性别和其他个体特征等因素,尤其要关注血流动力学状态。

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