法洛四联症手术修复后的第七个十年:严重肺动脉反流的晚期表现

Kyle Varkoly, Akarsh Parekh, Melissa Ianitelli, Mostafa Hamada, Alexandra Lucas, Thomas Forbes
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摘要

经手术修复的法洛氏四联症(TOF)是一种先天性心脏病,在单队列纵向研究中,其第 4 个十年的累积存活率为 72%。一旦发生肺动脉反流,成人法洛氏四联症患者的保守治疗与手术治疗之间存在争议。一名 73 岁的男性 TOF 患者经手术矫正后出现心衰症状。他在 2 岁时接受了经典的右侧 Blalock-Taussig 分流术进行 TOF 修复,并在 18 岁时接受了经环修补术。超声心动图显示右心室收缩压升高、右心室严重扩张和肺动脉反流。我们对患者新发的右侧心力衰竭进行了药物治疗和利尿。在后来的一次住院计划中,他通过经皮途径接受了新的肺动脉瓣,症状得到缓解,三尖瓣反流也得到改善。一旦出现中度以上的 PR,I 级建议对其进行肺动脉瓣介入治疗;但应首先进行医疗优化。在充分优化 RV 负荷后,我们的患者成功接受了经导管肺动脉瓣植入术,症状得到缓解,并停用了利尿剂。
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Surgically repaired tetralogy of Fallot in the 7th decade: a late presentation of severe pulmonic regurgitation
Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical management in adults with TOF once pulmonary regurgitation occurs. A 73-year-old male with surgically corrected TOF presented with heart failure symptoms. He underwent ToF repair with a classic right Blalock–Taussig shunt at 2 years of age with transannular patching at 18 years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular dilatation, and pulmonary regurgitation. Our patient’s new-onset right-sided heart failure was managed medically with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization with resolution of symptoms and improved tricuspid regurgitation. It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs; however, medical optimization should take place first. Following adequate RV load optimization, our patient underwent successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.
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