Resolution of acquired pulmonary arteriovenous malformations in a patient with total anomalous systemic venous return.
An 11-year-old male with total anomalous systemic venous return had surgical repair except for the hepatic venous return, which drained to the left atrium. He developed progressive cyanosis and fatigue and was diagnosed with large pulmonary arteriovenous malformations (PAVMs) during cardiac catheterization with the use of bubble contrast echocardiography. After surgical redirection of hepatic venous flow to the right heart and pulmonary arterial system, resolution of these PAVMs was demonstrated clinically and by contrast echocardiography. This unique case report demonstrates the development of PAVMs with exclusion of hepatic venous return through the pulmonary vascular bed while pulsatile pulmonary blood flow remains intact. It reinforces the likelihood of the absence of an as yet unidentified hepatic vasoactive substance as the source for development of PAVMs.
Johnson
T R
TR
Department of Pediatrics, Section of Pediatric Cardiology, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Riley Research 104, 702 Barnhill Drive, Indianapolis, IN 46202-5225, USA.
Schamberger
M S
MS
Brown
J W
JW
Girod
D A
DA
D001165:Arteriovenous Malformations / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D006503:Hepatic Veins / Q000601:surgery; D006801:Humans; D008102:Liver Circulation; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities*; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000002:abnormalities*; D011673:Pulsatile Flow; D012039:Regional Blood Flow