Ahmed Rizwan, Al Hashmi Haitham, Mora Bassem
Consultant Adult Congenital Heart Disease, Department of Cardiology, Sheikh Shakhbout Medical City-Mayo Clinic partnership, Abu Dhabi, United Arab Emirates.
Consultant Adult Congenital Heart Disease, Department of Cardiology, Rashid Hospital, Dubai, United Arab Emirates.
Eur Heart J Case Rep. 2023 Jan 13;7(1):ytad021. doi: 10.1093/ehjcr/ytad021. eCollection 2023 Jan.
Children with congenital heart disease remain at risk of being lost to follow-up. They may present at a later stage with complications related to underlying condition that usually require in-depth phenotyping for optimal management. We describe an unusual case of a double outlet right ventricle (DORV) who presented three decades after initial intervention.
A 32-year-old female sought attention for worsening breathlessness. Available data suggested that she had double outlet right ventricle (DORV) and underwent pulmonary artery (PA) banding in infancy. On examination, she was frail, clubbed and cyanosed with oxygen saturation reduced to 75% at rest. Blood tests demonstrated elevated haemoglobin (208 g/L) and thrombocytopenia (70 *10/L). Echocardiography was limited but demonstrated DORV with increased gradient across both outflow tracts. Cardiac magnetic resonance (CMR) imaging clearly demonstrated DORV with doubly committed interventricular communication, subvalvular aortic valve stenosis and supravalvular pulmonary stenosis due to a previous PA band. CMR was also remarkable for demonstrating severely impaired left ventricle and difficulty achieving optimal contrast between myocardium and blood pool on late enhancement images. She underwent relief of subaortic stenosis with immediate improvement in left ventricular (LV) function and PA plasty with application of a new PA band.
Lost to follow-up congenital patients present a particular challenge due to complications of chronic unusual physiology. This requires careful assessment by a multidisciplinary team to define optimal management strategy.
先天性心脏病患儿仍有失访风险。他们可能在后期出现与基础疾病相关的并发症，通常需要进行深入的表型分析以实现最佳管理。我们描述了一例罕见的双出口右心室（DORV）病例，该患者在初次干预后三十年才出现症状。
一名32岁女性因呼吸急促加重前来就诊。现有资料显示她患有双出口右心室（DORV），婴儿期接受过肺动脉（PA）环扎术。检查发现，她身体虚弱，有杵状指，面色发绀，静息时氧饱和度降至75%。血液检查显示血红蛋白升高（208 g/L）和血小板减少（70×10/L）。超声心动图检查结果有限，但显示为DORV，两个流出道的压力阶差增加。心脏磁共振（CMR）成像清楚地显示为DORV，伴有双心室连接、瓣下主动脉瓣狭窄和由于先前PA环扎导致的瓣上肺动脉狭窄。CMR还显示左心室严重受损，在延迟强化图像上难以实现心肌与血池之间的最佳对比。她接受了主动脉瓣下狭窄解除术，左心室（LV）功能立即改善，并进行了肺动脉成形术，同时应用了新的PA环扎带。
失访的先天性心脏病患者由于慢性特殊生理并发症而面临特殊挑战。这需要多学科团队进行仔细评估，以确定最佳管理策略。
