Pedun Bernadette, Pitua Ivaan, Bongomin Felix, Bugeza Samuel, Luzinda Rita Nassanga
Uganda Cancer Institute Kampala Uganda.
Department of Radiology and Radiotherapy, School of Medicine, College of Health Sciences Makerere University Kampala Uganda.
Clin Case Rep. 2025 Jan 6;13(1):e70076. doi: 10.1002/ccr3.70076. eCollection 2025 Jan.
Double outlet right ventricle (DORV) is a rare congenital heart defect where both the aorta and pulmonary artery originate from the right ventricle, often accompanied by additional cardiac anomalies to mitigate circulatory imbalance, though such compensations usually fail. We report a 15-month-old infant with recurrent respiratory infections and poor weight gain, referred for computed tomography angiography. Physical examination showed a small, non-syndromic infant with pallor, tachypnea, irritability, and finger clubbing. Initial imaging revealed cardiomegaly and lung infiltrates; echocardiography and computed tomography angiography confirmed additional intracardiac defects of double superior vena cavae, a hypoplastic aortic arch, hypertrophic right ventricular wall, and a patent ductus arteriosus. Corrective surgery was delayed due to respiratory complications. This case emphasizes the critical need to consider cardiac pathology in pediatric patients with recurrent respiratory symptoms, as untreated DORV can lead to high mortality.
右心室双出口（DORV）是一种罕见的先天性心脏缺陷，其中主动脉和肺动脉均起源于右心室，常伴有其他心脏异常以减轻循环失衡，尽管这种代偿通常是失败的。我们报告了一名15个月大的婴儿，有反复呼吸道感染且体重增加不佳，转诊接受计算机断层血管造影检查。体格检查显示该婴儿身材矮小，无综合征表现，面色苍白、呼吸急促、易激惹且有杵状指。初始影像学检查显示心脏扩大和肺部浸润；超声心动图和计算机断层血管造影证实存在额外的心内缺陷，包括双上腔静脉、主动脉弓发育不全、右心室壁肥厚以及动脉导管未闭。由于呼吸并发症，矫正手术被推迟。该病例强调了对于有反复呼吸道症状的儿科患者考虑心脏病理的迫切必要性，因为未经治疗的右心室双出口可导致高死亡率。
