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.