Borghol William, Alfaksh Faisal, Karaja Saja, Barakat Lyne, Takkem Saleh
Faculty of Medicine, Hama University, Hama, Syria.
Department of Cardiology, Hama National Hospital, Hama, Syria.
J Med Case Rep. 2025 May 17;19(1):229. doi: 10.1186/s13256-025-05300-4.
Double-inlet right ventricle is an extremely rare and complex cardiac malformation characterized by the opening of both atria into the right ventricle and is usually associated with other major cardiac malformations. In this case report, we have discussed an isolated case of double-inlet right ventricle without other malformations and conducted a literature review to compare our case, which is the third in the medical literature, with previously published papers on double-inlet right ventricle.
A 2-month-old Syrian male infant was admitted to the cardiology department due to symptoms of central cyanosis and failure to thrive. Echocardiography revealed double-inlet right ventricle with a hypoplastic left ventricle from which the hypoplastic aorta emerged, while the pulmonary trunk emerged from the dilated right ventricle. The child was sent abroad for the Norwood procedure.
Although double-inlet right ventricle is a very rare malformation, it can be encountered in clinical practice and should be taken into consideration by practitioners. Clinical researchers should document and publish cases of double-inlet right ventricle diagnosed to draw attention to this malformation and study it in greater depth.
双入口右心室是一种极其罕见且复杂的心脏畸形，其特征为两个心房均开口于右心室，并且通常与其他主要心脏畸形相关。在本病例报告中，我们讨论了一例无其他畸形的孤立性双入口右心室病例，并进行了文献综述，以将我们的病例（医学文献中的第三例）与先前发表的关于双入口右心室的论文进行比较。
一名2个月大的叙利亚男婴因中心性发绀和发育不良症状入住心内科。超声心动图显示双入口右心室，左心室发育不全，发育不全的主动脉由此发出，而肺动脉干则从扩张的右心室发出。该患儿被送往国外进行诺伍德手术。
尽管双入口右心室是一种非常罕见的畸形，但在临床实践中仍可能遇到，从业者应予以考虑。临床研究人员应记录并发表诊断为双入口右心室的病例，以引起对这种畸形的关注并进行更深入的研究。
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