Raghuram Krishnan A, Bijulal Sasidharan, Krishnamoorthy Kavasseri M, Tharakan Jaganmohan A
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Medical College P O, Thiruvananthapuram, 695011, India,
Pediatr Cardiol. 2013;34(8):1882-5. doi: 10.1007/s00246-012-0428-z. Epub 2012 Jul 28.
A 1-year-old boy who had left isomerism and corrected transposition of the great arteries (c-TGA) with moderate-sized ventricular septal defect, severe pulmonary artery hypertension (PAH), and pulmonary vascular disease with significant right-to-left shunting received a diagnosis of type 2 Abernethy malformation, which was partly responsible for disproportionate PAH in the child. The malformation was treated by plugging of the portosystemic shunt. Follow-up cardiac catheterization on sildenafil demonstrated significant left-to-right shunting (2.16:1) and a fall in pulmonary vascular resistance, making surgical correction possible. This case highlights the importance of searching for additional rare causes of PAH in patients with congenital heart diseases when the degree of pulmonary hypertension is disproportional to the defect size.
一名1岁男孩，患有左位异构和矫正型大动脉转位（c-TGA），伴有中度室间隔缺损、重度肺动脉高压（PAH）以及具有明显右向左分流的肺血管疾病，被诊断为2型阿伯内西畸形，这在一定程度上导致了该患儿肺动脉高压不成比例。通过封堵体循环-门静脉分流对该畸形进行了治疗。服用西地那非后的随访心导管检查显示有明显的左向右分流（2.16:1）且肺血管阻力下降，使得手术矫正成为可能。该病例凸显了在先天性心脏病患者中，当肺动脉高压程度与缺损大小不成比例时，寻找肺动脉高压其他罕见病因的重要性。
