Catheterization and Cardiovascular Interventions 00:00–00 (2014)

Original Studies Catheter Approach to Redirect Hepatic Venous Return for Treatment of Unilateral Pulmonary Arteriovenous Malformations After Fontan Yoav Dori,1† MD, PhD, Shyam Sathanandam,2† MD, Andrew C. Glatz,1 MD MSCE, Matthew J. Gillespie,1 MD, and Jonathan J. Rome,1* MD Objectives: The goal of this report is to describe a percutaneous approach to rerouting hepatic venous return in patients who developed progressive cyanosis due to unilateral pulmonary arteriovenous malformations (PAVM) after the total cavopulmonary connection (TCPC) operation. Background: Unilateral PAVM can develop in patients after TCPC operation when there is unequal distribution of hepatic venous return between the two lungs. This often results in progressive cyanosis and the need for surgical reintervention. A percutaneous based approach for rerouting hepatic venous return has never been described. Methods: We retrospectively reviewed the clinical data on four patients who underwent percutaneous rerouting procedures. One patient with a misaligned TCPC underwent realignment of the circuit with a bare metal stent. In three patients a combination of bare metal and covered stents were needed to achieve the desired results. Results: The rerouting procedures were successful in all patients with significant improvement in oxygen saturation from a median of 75% (range 55–80%) to a median of 90% (range 84–92%) (P 5 0.02). There were no recorded short term or intermediate term complications with maximum follow-up time of 43 months. Conclusions: Percutaneous rerouting of hepatic venous flow is feasible and should be considered when a surgical approach is not possible; this strategy may serve as a viable alternative to complex operative approaches in select cases. Furthermore studies are needed to determine the long-term efficacy of this procedure. VC 2013 Wiley Periodicals, Inc. Key words: congenital heart disease; pediatric intervention

INTRODUCTION

The development of pulmonary arteriovenous malformations (PAVM) after Fontan palliation is a wellrecognized complication and leads to significant morbidity [1,2]. Although the exact mechanism, by which PAVM develop is unknown, studies have shown that blood flow exiting the liver is needed to prevent their formation [1]. In most cases where PAVM develop they occur as a result of an absence or uneven distribution of hepatic venous return. PAVM develop in the lung with inadequate return of hepatic venous effluent [3,4]. Patients with heterotaxy syndrome of the polysplenia type with an interrupted inferior vena cava (IVC) and azygos continuation to a superior vena-cava (SVC) are at particularly high risk for development of unilateral C 2013 Wiley Periodicals, Inc. V

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Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Pennsylvania 2 Pediatric Cardiology, LeBonheur Children’s Medical Center, Memphis, Tennessee Conflict of interest: Neither I, nor any of my co-authors have a conflict of interest †

Both authors have contributed equally to this work

*Correspondence to: Jonathan J. Rome, MD; The Children’s Hospital of Philadelphia, 34th & Civic Center Blvd, Philadelphia, PA 19104. E-mail: [email protected] Received 17 July 2013; Revision accepted 28 November 2013 DOI: 10.1002/ccd.25326 Published online 00 Month 2013 in Wiley Online Library (wileyonlinelibrary.com)

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Dori et al.

PAVM after total cavopulmonary connection (TCPC) [1]. In these cases the venous return from the lower body drains through the azygos vein into a SVC that is often contralateral to the side of attachment of the conduit draining hepatic venous return. This unfavorable geometry often precludes hepatic effluent from reaching the contralateral lung. There have been several approaches reported for addressing this problem [5–9]. To date these strategies have mostly involved surgical rerouting procedures, which are complicated and not always successful [6]. Stent therapy for a stenotic or discontinuous central pulmonary artery (PA) segment has also been performed for treatment of PAVM. Although this has been effective in patients with normal venous drainage patterns, results have been less favorable in those with complex hepatic venous return [6,10]. A completely percutaneous methodology to reroute hepatic flow has not been reported. In this study, we describe our experience in a small series of patients where a percutaneous approach was used to reroute hepatic blood flow. We describe the geometrical arrangement, strategies used, and outcomes achieved. We believe that the general methodologies outlined in this study can be used in other instances where rerouting of flow is needed and should be considered as an alternative to the current surgical approaches. METHODS

This is a retrospective review of four patients who underwent catheter procedures for percutaneous rerouting of hepatic flow in our institution. In one of the cases misalignment of the TCPC circuit in a patient without heterotaxy was corrected by use of a single uncovered stent. In the three patients with heterotaxy, bare metal stents were used to relieve anatomic obstruction and provide a scaffold, into which covered stents were deployed that rerouted the hepatic flow to a location chosen based on careful planning such that hepatic effluent would mix with the incoming caval flow and redistribute into both lungs. STATISTICAL ANALYSIS

Demographic and procedural variables were summarized using standard descriptive statistics and expressed as median with range for skewed continuous variables, and count with percentage of total for categorical variables. Oxygen saturations were compared using Wilcoxon Signed Rank test. All statistical analyses were performed using SPSS v20. Statistical significance was established using a two-tailed P-value of

Catheter approach to redirect hepatic venous return for treatment of unilateral pulmonary arteriovenous malformations after fontan.

The goal of this report is to describe a percutaneous approach to rerouting hepatic venous return in patients who developed progressive cyanosis due t...
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