Case report
A 9-year-old boy with a history of cough and pneumonia and no previous history of abdominal pain was admitted to the cardiovascular center of Beijing Children’s Hospital with a dilated left ventricle, even after taking oral coenzyme Q10 fructose sodium diphosphate and other cardiac nutritional drugs for five years. The patient’s medical history included a normal electrocardiogram, myocardial enzyme tests, and normal liver enzymes. The patient was jaundiced at birth. Dandy-Walker syndrome with hydrocephalus was revealed by computed tomography (CT) following an episode of head trauma.
Several imaging modalities were used. Echocardiography revealed that the left ventricle was moderately enlarged, and the wall motion amplitude was enhanced. Color Doppler revealed an abundant blood flow signal from systemic circulation to pulmonary circulation on suprasternal long-axis imaging (Figure 1A, 1B ). CT with contrast enhancement revealed MAPCAs; the size of the main portal artery was normal, but the right portal artery was significantly slimmer than the left portal artery. Most right portal artery flow into the inferior vena cava bypassed the liver, and the remaining blood drained into the liver (Figure 1C) . There were increased tortuous bronchial arteries and several vascular shadows in the mediastinum, most of which were in the mediastinum and hilum of the lung; some extended into the lung and returned to the pulmonary vein of the corresponding region. On this basis, a pulmonary arteriovenous malformation was diagnosed.
Selective angiography revealed that the thoracic aorta and the upper abdominal aorta-pulmonary artery collateral vessels (which were thickened and tortuous) supplied the left and right lungs, respectively. Laboratory tests showed serum glutamic pyruvic transaminase levels, glutamic oxalacetic transaminase, total bilirubin, indirect bilirubin, and direct bilirubin were elevated; levels of serum total protein and albumin were decreased (Table 1) . These findings suggested a diagnosis of Abernethy malformation Type II.
Laparoscopy and portal shunt ligation, and intraoperative portal vein angiography were performed. The left branch of the portal vein merged into the inferior vena cava through the venous catheter, and the development of the intrahepatic portal vein was poor. After venous catheter ligation, the development of the intrahepatic portal vein was more significant. The body of the pulmonary artery collateral circulation was closed with wave divisions of 6-mm and 3-mm diamond spring coils, respectively, to block the main body pulmonary collateral circulation blood vessels (Figures 1D-F) .
The patient remained well one month after surgery. Echocardiography revealed no apparent abnormal blood flow sign in section of the superior sternal fossa. The inner diameters of the cardiac chambers were smaller than before. The liver function also improved (Table 1 ). Postoperative CT revealed that the left hepatic portal vein and inferior vena cava were truncated and not filled. The patient remains in follow-up.