Development of intrapulmonary arteriovenous shunting in postoperative biliary atresia: evaluation by contrast-enhanced echocardiography.
OBJECTIVE
This report presents biliary atresia (BA) patients with intrapulmonary arteriovenous shunting (IPS), which was evaluated suitably by contrast-enhanced echocardiography (CEC).


METHODS
Of 88 BA patients seen in the last 20 years, 8 (9.1%) had IPS at 8 months to 16 years of age. Two were associated with polysplenia syndrome, 1 had persistent jaundice after hepatic portoenterostomy, and 2 underwent splenorenal shunt. According to the comparison between microbubbles in left atrium (LA) and in right atrium (RA) detected by CEC, IPS was classified as grade I, mild (LA << RA); grade II, moderate (LA < RA); grade II, severe (LA = RA).


RESULTS
Grade I consisted of 4 patients whereas grade II and III held 2 patients each. Clinical symptoms such as cyanosis, exertional dyspnea, or clubbing were present in 50% of grade I and all of grade II and IlI. Mean PaO2 in grade I, II, and III was 70.5, 50.4, and 35.3 mm Hg, respectively. In 1 patient with grade I, IPS spontaneously disappeared, but pulmonary hypertension developed later. One patient in grade II died of pulmonary complications, and the other is considered a candidate for livertransplantation (LTx). One patient in grade III died of liver failure, whereas the other is free of IPS after LTx.


CONCLUSIONS
IPS can lead to a life-threatening complication in postoperative BA patients, and CEC may be a convenient and useful method to evaluate the degree of IPS and determine therapeutic strategy.
Sasaki
T
T
Department of Pediatric Surgery, Osaka University Medical School, Suita City, Japan.
Hasegawa
T
T
Kimura
T
T
Okada
A
A
Mushiake
S
S
Matsushita
T
T
D000293:Adolescent; D001164:Arteriovenous Fistula / Q000000981:diagnostic imaging* / Q000209:etiology / Q000401:mortality; D001656:Biliary Atresia / Q000175:diagnosis / Q000601:surgery*; D001784:Blood Gas Analysis; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D004452:Echocardiography / Q000379:methods; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000000981:diagnostic imaging / Q000209:etiology / Q000401:mortality; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000000981:diagnostic imaging; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000000981:diagnostic imaging* / Q000503:physiopathology; D011667:Pulmonary Veins / Q000000981:diagnostic imaging* / Q000503:physiopathology; D011856:Radiographic Image Enhancement / Q000379:methods; D012680:Sensitivity and Specificity; D015996:Survival Rate
D003287:Contrast Media