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Original Articles: Pediatric Cardiac

Invited Commentary

Cardiothoracic Surgery, Children's Hospital of Wisconsin, MS 715 Children's Hospital of Wisconsin, 9000 West Wisconsin Ave, Milwaukee, WI 53226

(Email: jtweddell{at}chw.org).

Pulmonary arteriovenous fistulas (PAVFs) are pathologic channels that permit pulmonary arterial blood to traverse the lung without participating in gas exchange, resulting in cyanosis [1]. Evidence for the role of hepatic venous blood in both inhibiting the development and promoting the regression of PAVFs is substantial. The PAVFs are seen in single ventricle patients who have undergone superior cavopulmonary shunt (BCPS), in patients with two-ventricle anatomy with hepatic venous drainage to the pulmonary venous atrium, and among patients with liver failure [1–6]. Restoration of hepatic venous blood to the pulmonary circuit results in improvement.

The current article by Kim and colleagues [7] looks at 33 patients with PAVFs after BCPS. Consistent with previous reports, severely affected patients were likely to have an interrupted inferior vena cava (IVC) and heterotaxy syndrome. After completion Fontan, PAVFs resolved in all but 5 patients with these common and challenging anatomic features; bilateral superior vena cava (SVC) and interrupted IVC with azygous or hemi-azygous continuation to the SVC contralateral to the Fontan extracardiac conduit (EC). Within this anatomic subgroup exists the potential for venous blood from the liver and its hepatic factor to be unevenly distributed, and inadequate hepatic factor enters the lung opposite the EC and PAVFs in the contralateral lung persist and worsen. Even forewarned of this potential hazard equalizing the output from the EC may be very difficult. The cardiac mass may prevent placement of the EC on the same side as the SVC receiving the IVC. Although a more central placement of the cephalad end of the EC would seem a potential solution, the flow from the SVC with the addition of IVC drainage may simply be too great to permit any of the hepatic venous blood from entering the contralateral lung. One potential solution suggested by the authors is construction of a more medial position of the BCPS anastomosis of the SVC receiving the IVC return.

Kim and colleagues [7] have carefully analyzed this large series of patients with PAVFs, and the conclusions are clear: (1) after the Kawashima procedure, patients should proceed to completion Fontan on a timeline such as other single ventricles, (2) the presence of PAVFs are an indication for completion Fontan, and (3) patients with bilateral SVC and interrupted IVC will require special attention both at the time of BCPS and completion Fontan to prevent creating the substrate for PAVFs.

	References

Top References

 

1. Srivastava D, Preminger T, Lock JE, et al. Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease Circulation 1995;92:1217-1222.[Abstract/Free Full Text]
2. Knight WB, Mee RBB. A cure for pulmonary arteriovenous fistulas? Ann Thorac Surg 1995;59:999-1001.[Abstract/Free Full Text]
3. Duncan BW, Kneebone JM, Chi EY, et al. A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease J Thorac Cardiovasc Surg 1999;117:931-938.[Abstract/Free Full Text]
4. Duncan BW, Desai S. Pulmonary arteriovenous malformations after cavopulmonary anastomosis Ann Thorac Surg 2003;76:1759-1766.[Abstract/Free Full Text]
5. Lee J, Menkis AH, Rosenberg HC. Reversal of pulmonary arteriovenous malformation after diversion of anomalous hepatic drainage Ann Thorac Surg 1998;65:848-849.[Abstract/Free Full Text]
6. Agnoletti G, Borghi A, Annecchino FP, Crupi G. Regression of pulmonary fistulas in congenital heart disease after redirection of hepatic venous flow to the lungs Ann Thorac Surg 2001;72:909-911.[Abstract/Free Full Text]
7. Kim SJ, Bae EJ, Lee JY, et al. Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas Ann Thorac Surg 2009;87:548-554.[Abstract/Free Full Text]

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): James S. Tweddell Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Tweddell, J. S. Search for Related Content PubMed Articles by Tweddell, J. S. Related Collections Congenital - cyanotic Related Article