Annals of Pediatric Cardiology
About us | Current Issue | Archives | Ahead of Print | Instructions | Submission | Subscribe | Advertise | Contact | Login 


    Advanced search

    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

    Article Figures

 Article Access Statistics
    PDF Downloaded313    
    Comments [Add]    

Recommend this journal


IMAGES Table of Contents   
Year : 2009  |  Volume : 2  |  Issue : 2  |  Page : 177-178
Pulmonary arteriovenous malformations after cavopulmonary anastomosis

Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Click here for correspondence address and email

Date of Web Publication12-Dec-2009


Pulmonary arteriovenous malformation (PAVM) is common after cavopulmonary anastomosis. PAVMs appear on chest X-ray film as diffuse opacity in one or both lungs. Angiographically, it appears as spidery diffuse vascularity with near simultaneous opacification of pulmonary arteries and veins.

Keywords: Angiogram, cavopulmonary anastomosis, pulmonary arteriovenous malformation

How to cite this article:
Singhi AK, Kumar R K. Pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Pediatr Card 2009;2:177-8

How to cite this URL:
Singhi AK, Kumar R K. Pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Pediatr Card [serial online] 2009 [cited 2022 Oct 1];2:177-8. Available from:

We present images of pulmonary arteriovenous malformation (PAVM) in a 6-year-old child after cavopulmonary anastomosis. The right pulmonary angiogram shows extensive and diffuse spidery PAVMs [Figure 1]a in the right lower lobe. The pulmonary artery and vein are seen to opacify almost simultaneously. The left pulmonary angiogram shows normal pulmonary arborization with no PAVMs [Figure 1]b. A chest skiagram shows diffuse opacification in the right lower lobe [Figure 2].

PAVMs are a cause of progressive cyanosis after cavopulmonary anastomosis in children with single ventricle physiology who are on the pathway to a Fontan procedure. Clinically significant PAVMs occur in as many as 25% of patients who are followed for several years after cavopulmonary anastomosis. [1] The exact cause of PAVMs after cavopulmonary anastomosis remains unknown. The absence of pulsatile flow, hepatic factor, and hypoxia are the various reasons cited for the development of PAVMs after cavopulmonary anastomosis. Recent studies suggest a key role of the liver in the development of these lesions. [2] PAVMs develop whenever hepatic venous effluent does not perfuse the pulmonary arteries directly either on a congenital basis or postoperatively. [3],[4],[5] The liver is known to produce precursors of angiogenesis inhibitors. Collagen XVIII and plasminogen are produced in large quantities by the liver and secreted into hepatic venous effluent where subsequent action by proteolytic enzymes cleaves these precursors into the potent angiogenesis inhibitors endostatin and angiostatin, respectively. Exclusion of these substances from the pulmonary arterial circulation after cavopulmonary anastomosis may result in vascular proliferation that would normally be held in check. [6],[7]

Chest X-ray shows spidery pulmonary blood vessels distributed evenly throughout both lungs. The diagnosis is confirmed by pulmonary angiography; the central and the middle sized arteries are dilated and untapered, filling a myriad of spidery small branches that extend far into the periphery of the lung with evidence of right-to-left shunting demonstrated by the rapid appearance of contrast in the pulmonary veins after pulmonary artery injection. Contrast echocardiography is considerably more sensitive than angiography for detecting this condition. It shows the early appearance of micro bubbles in the pulmonary venous atrium after peripheral venous injection of agitated saline. When used as a screening tool, contrast echocardiography detects intrapulmonary right-to-left shunting in a large percentage of children after cavopulmonary anastomosis, even in the absence of clinically evident PAVMs. [7],[8]

   References Top

1.Mathur M, Glenn WW. Long-term evaluation of cavopulmonary artery anastomosis. Surgery 1973;74:899-916.  Back to cited text no. 1      
2.Duncan BW, Desai S. Pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Thorac Surg 2003;76:1759-66.  Back to cited text no. 2      
3.Lamberti JJ, Spicer RL, Waldman JD, Grehl TM, Thomson D, George L, et al. The bidirectional cavopulmonary shunt. J Thorac Cardiovasc Surg 1990;100:22-9.  Back to cited text no. 3      
4.Srivastava D, Preminger T, Lock JE, Mandell V, Keane JF, Mayer JE Jr, et al. Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 1995;92:1217-22.  Back to cited text no. 4      
5.Moore JW, Kirby WC, Madden WA, Gaither NS. Development of pulmonary arteriovenous malformations after modified Fontan operations. J Thorac Cardiovasc Surg 1989;98:1045-50.  Back to cited text no. 5      
6.Clement B, Musso O, Lietard J, Theret N. Homeostatic control of angiogenesis: A newly identified function of the liver? Hepatology 1999;29:621-3.  Back to cited text no. 6      
7.Larsson ES, Solymar L, Eriksson BO, de Wahl Granelli A, Mellander M. Bubble contrast echocardiography in detecting pulmonary arteriovenous malformations after modified Fontan operations. Cardiol Young 2001;11:505-11.  Back to cited text no. 7      
8.Feinstein JA, Moore P, Rosenthal DN, Puchalski M, Brook MM. Comparison of contrast echocardiography versus cardiac catheterization for detection of pulmonary arteriovenous malformations. Am J Cardiol 2002;89:281-5.  Back to cited text no. 8      

Correspondence Address:
R Krishna Kumar
Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Kochi - 682 026, Kerala
Login to access the Email id

Source of Support: None, Conflict of Interest: None

PMID: 20808638

Rights and PermissionsRights and Permissions


  [Figure 1], [Figure 2]