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Development of Unilateral Pulmonary Arteriovenous Malformations due to Unequal Distribution of Hepatic Venous Flow

2001; Lippincott Williams & Wilkins; Volume: 103; Issue: 8 Linguagem: Inglês

10.1161/01.cir.103.8.e39

1524-4539

Henri Justino, Lee Benson, Robert M. Freedom,

Vascular anomalies and interventions

HomeCirculationVol. 103, No. 8Development of Unilateral Pulmonary Arteriovenous Malformations due to Unequal Distribution of Hepatic Venous Flow Free AccessOtherPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessOtherPDF/EPUBDevelopment of Unilateral Pulmonary Arteriovenous Malformations due to Unequal Distribution of Hepatic Venous Flow Henri Justino, Lee N. Benson and Robert M. Freedom Henri JustinoHenri Justino From the Department of Pediatrics, Division of Cardiology, and the Variety Club Cardiac Catheterization Laboratories, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada. , Lee N. BensonLee N. Benson From the Department of Pediatrics, Division of Cardiology, and the Variety Club Cardiac Catheterization Laboratories, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada. and Robert M. FreedomRobert M. Freedom From the Department of Pediatrics, Division of Cardiology, and the Variety Club Cardiac Catheterization Laboratories, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada. Originally published27 Feb 2001https://doi.org/10.1161/01.CIR.103.8.e39Circulation. 2001;103:e39–e40A female child with visceral heterotaxy (presumed left isomerism), interrupted inferior vena cava with azygos continuation to the superior vena cava, and double-outlet right ventricle underwent a total cavopulmonary anastomosis (end-to-side anastomosis of the superior vena cava to the right pulmonary artery) in infancy. Completion of Fontan circulation was undertaken at 3 years of age with construction of a tunnel from the hepatic veins to the pulmonary artery confluence.By 6 years of age, the child had become increasingly cyanotic, and cardiac catheterization was performed to define the mechanism of the cyanosis. Figure 1 is a pulmonary artery angiogram that reveals moderate hypoplasia of the pulmonary artery confluence. Figure 2 is a selective angiogram performed in the hepatic vein tunnel, with hepatic venous flow directed predominantly toward the left lung. The left pulmonary artery angiogram was normal. A right pulmonary artery angiogram (Figure 3) was markedly abnormal, showing a diffusely granular appearance of the distal vasculature and early appearance of contrast in the right pulmonary veins, characteristic of the presence of pulmonary arteriovenous malformations. The development of arteriovenous malformations in the right lung allowed for intrapulmonary right-to-left shunting, accounting for the patient's progressive cyanosis. Figure 4 is an azygos vein angiogram, performed after the implantation of an intravascular stent into the hypoplastic pulmonary artery confluence. Note the presence of a duplicated inferior vena cava (the left inferior vena cava received the left renal vein).This case illustrates the importance of hepatic venous return circulating through the pulmonary vasculature in inhibiting pulmonary angiogenesis. The "antiangiogenic" properties of hepatic venous blood are attributed to an as-yet uncharacterized hepatic factor. The left lung, which received hepatic venous return through the surgically created hepatic vein tunnel, was normal. However, the right lung, which was underperfused with hepatic venous effluent both by virtue of the geometry of the hepatic vein to pulmonary artery tunnel and the narrowing of the pulmonary artery confluence, developed arteriovenous malformations. Reprint requests to Dr Robert M. Freedom, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8. The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke's Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.Download figureDownload PowerPoint Figure 1. Pulmonary artery angiogram, anteroposterior view. Catheter course is from azygos vein to superior vena cava to right pulmonary artery (RPA) via cavopulmonary anastomosis. White arrowhead denotes hypoplastic segment between right pulmonary artery and left pulmonary artery (LPA).Download figureDownload PowerPoint Figure 2. Selective angiogram in hepatic vein tunnel (HVT) outlining hepatic veins (HV). Note distribution of hepatic venous flow toward left pulmonary artery (LPA). No evidence of pulmonary arteriovenous malformations was found in the left lung.Download figureDownload PowerPoint Figure 3. Selective angiogram in right pulmonary artery (RPA). Note early appearance of contrast in right pulmonary veins (RPV) draining into pulmonary venous atrium (PVA) and granular appearance of distal pulmonary vasculature (compare with normal appearance of left pulmonary vasculature in Figure 2).Download figureDownload PowerPoint Figure 4. Angiogram in azygos vein (Az) depicting interruption of inferior vena cava with azygos continuation to superior vena cava. Large black arrow denotes a duplicated (left) inferior vena cava. Double small black arrows depict the intravascular stent implanted into hypoplastic segment between right (RPA) and left (LPA) pulmonary arteries. Previous Back to top Next FiguresReferencesRelatedDetailsCited By Loomba R, Morales D and Redington A (2019) Heterotaxy Critical Heart Disease in Infants and Children, 10.1016/B978-1-4557-0760-7.00067-X, (796-803.e3), . Heo K, Berk B and Abe J (2016) Disturbed Flow-Induced Endothelial Proatherogenic Signaling Via Regulating Post-Translational Modifications and Epigenetic Events , Antioxidants & Redox Signaling, 10.1089/ars.2015.6556, 25:7, (435-450), Online publication date: 1-Sep-2016. 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February 27, 2001Vol 103, Issue 8 Advertisement Article InformationMetrics Copyright © 2001 by American Heart Associationhttps://doi.org/10.1161/01.CIR.103.8.e39 Originally publishedFebruary 27, 2001 PDF download Advertisement