Rangwala Zarin S, Ramachandran Kaushik, Sagar Sunil, Sukesan Subin, Dharan Baiju S
Department of CVTS, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Department of CTVA, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Ann Pediatr Cardiol. 2024 Jul-Aug;17(4):285-287. doi: 10.4103/apc.apc_90_24. Epub 2024 Nov 15.
Association of Ebstein's anomaly of tricuspid valve (TV) with tetralogy of Fallot (TOF) is extremely rare. Their coexistence is unique as they modify each other's physiology. We report a case of a girl child having this rare combination along with Diamond-Blackfan syndrome awaiting a bone marrow transplant. She underwent intracardiac repair for TOF along with cone reconstruction of the TV, as tricuspid regurgitation of Ebstein's anomaly would worsen with TOF correction alone. Through this surgery, we aimed to achieve a competent pulmonary valve using a transannular patch with a monocusp and adequate relief of the right ventricular tract obstruction, thus preserving the right ventricular function, as both can affect the repaired TV function in the long term. The child had an uneventful postoperative course and is asymptomatic on follow-up.
三尖瓣埃布斯坦畸形(TV)与法洛四联症(TOF)的关联极为罕见。它们的共存很独特,因为会相互改变对方的生理状况。我们报告一例患有这种罕见组合并伴有戴蒙德-布莱克范综合征且正在等待骨髓移植的女童病例。她接受了法洛四联症的心内修复以及三尖瓣的圆锥重建,因为仅矫正法洛四联症会使埃布斯坦畸形的三尖瓣反流恶化。通过此次手术,我们旨在使用带单瓣叶的跨环补片实现肺动脉瓣功能正常,并充分缓解右心室流出道梗阻,从而保留右心室功能,因为这两者从长远来看都会影响修复后的三尖瓣功能。该患儿术后过程顺利,随访时无症状。