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Increases in oxygen saturation following discharge from Fontan palliation – an indicator of resolution of pulmonary arteriovenous malformations?

Part of: Hypoplastic Left Heart Syndrome (HLHS) Functionally Univentricular Heart

Published online by Cambridge University Press:  11 March 2021

Hannah Van Galder ,
Amy M. Schaal ,
Mingen Feng ,
Amy Y. Pan ,
Michele A. Frommelt ,
Salil Ginde  and
Andrew D. Spearman Open the ORCID record for Andrew D. Spearman [Opens in a new window]
Hannah Van Galder
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin, Herma Heart Institute, 9000 West Wisconsin Avenue, Milwaukee, WI53226, USA
Amy M. Schaal
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin, Herma Heart Institute, 9000 West Wisconsin Avenue, Milwaukee, WI53226, USA
Mingen Feng
Affiliation:
Department of Pediatrics, Division of Quantitative Health Sciences, Medical College of Wisconsin, Children’s Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI53226, USA
Amy Y. Pan
Affiliation:
Department of Pediatrics, Division of Quantitative Health Sciences, Medical College of Wisconsin, Children’s Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI53226, USA Cardiovascular Center, Medical College of Wisconsin, 8701 West Watertown Plank Road, Milwaukee, WI53226, USA
Michele A. Frommelt
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin, Herma Heart Institute, 9000 West Wisconsin Avenue, Milwaukee, WI53226, USA Cardiovascular Center, Medical College of Wisconsin, 8701 West Watertown Plank Road, Milwaukee, WI53226, USA
Salil Ginde
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin, Herma Heart Institute, 9000 West Wisconsin Avenue, Milwaukee, WI53226, USA Cardiovascular Center, Medical College of Wisconsin, 8701 West Watertown Plank Road, Milwaukee, WI53226, USA Department of Medicine, Division of Cardiology, Medical College of Wisconsin, 8701 West Watertown Plank, Milwaukee, WI53226, USA
Andrew D. Spearman*
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin, Herma Heart Institute, 9000 West Wisconsin Avenue, Milwaukee, WI53226, USA Cardiovascular Center, Medical College of Wisconsin, 8701 West Watertown Plank Road, Milwaukee, WI53226, USA
*
Author for correspondence: Andrew D. Spearman, Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin, Herma Heart Institute, 9000 West Wisconsin Avenue, Milwaukee, WI, 53226, USA; Cardiovascular Center, Medical College of Wisconsin, 8701 West Watertown Plank Road, Milwaukee, WI, 53226, USA. Phone: 414.955.2274; Fax: 414.266.2963. E-mail: aspearman@mcw.edu
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Abstract

Background:

Pulmonary arteriovenous malformations in single ventricle congenital heart disease are poorly understood. Previous studies investigating pulmonary arteriovenous malformations predominantly focus on patients with heterotaxy syndrome and interrupted inferior caval vein. It is unknown if development and resolution of pulmonary arteriovenous malformations are similar for patients with and without heterotaxy syndrome.

Methods:

In this retrospective single-institution study, we identified patients with a history of single ventricle congenital heart disease and Fontan palliation. We then matched patients with heterotaxy syndrome (intact and interrupted inferior caval vein) and non-heterotaxy hypoplastic left heart syndrome. To compare development of pulmonary arteriovenous malformations, we identified the frequency of positive diagnoses pre-Fontan. To compare resolution of pulmonary arteriovenous malformations, we recorded oxygen saturation changes for 12 months following Fontan.

Results:

A total of 124 patients were included. Patients with heterotaxy and interrupted inferior caval vein were more likely to have a pre-Fontan contrast echocardiogram performed (p < 0.01) and more likely to be diagnosed with pulmonary arteriovenous malformations pre-Fontan (p < 0.01). There was no difference in oxygen saturation prior to Fontan, yet all patient groups had increased their oxygen saturations in the first year after Fontan discharge.

Conclusions:

Pulmonary arteriovenous malformations are variably diagnosed prior to Fontan palliation; however, all study groups had increased oxygen saturations after Fontan discharge, potentially indicating resolution of pulmonary arteriovenous malformations in all groups. The prevalence of pulmonary arteriovenous malformations pre-Fontan is likely underestimated. A quantitative, systematic approach to diagnosis and follow-up of pulmonary arteriovenous malformations is needed to better understand susceptibility and pathophysiology.

Keywords

Single ventriclepulmonary arteriovenous malformationscavopulmonary connectionKawashimaGlennFontan
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 11 , November 2021 , pp. 1807 - 1813
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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