Current Treatment Options of Fontan Arrhythmias: Etiology, Incidence, and Diagnosis
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Pediatric and Congenital Heart Disease (G Singh, Section Editor)
Current Treatment Options of Fontan Arrhythmias: Etiology, Incidence, and Diagnosis Mohammad Alnoor, MD Mayme Marshall, MD Seshadri Balaji, MBBS, MRCP (UK), PhD* Address * Department of Pediatrics, Division of Cardiology, Oregon Health and Science University, 707, SW Gaines Street, CDRC-P, Portland, OR, 97239, USA Email: [email protected]
Published online: 14 October 2020 * Springer Science+Business Media, LLC, part of Springer Nature 2020
This article is part of the Topical Collection on Pediatric and Congenital Heart Disease Keywords (6) Fontan I Arrhythmia I Single ventricle I Atrial flutter, supraventricular tachycardia
Abstract Purpose of the review Patients who undergo single ventricle palliation with a Fontan are at a high risk for developing arrhythmias. This review will address our understanding of pathophysiology, incidence, presentation, and type of arrhythmia, focusing on recent developments. Recent findings Patients with the initially described atriopulmonary connection type of Fontan have a high incidence of arrhythmias, with severe consequences. Arrhythmias are less of a problem with the two currently used surgical techniques, the lateral tunnel and the extracardiac conduit Fontan, with the extracardiac Fontan being potentially less arrhythmogenic in the long term. Summary There is no role for the atriopulmonary Fontan any longer. As the lateral tunnel and extracardiac conduit patients age, there will be a greater understanding of the unique complications specific to their unique anatomy.
Introduction Arrhythmias are arguably the most important complication in patients who undergo the Fontan operation. They
are an important cause of clinical deterioration and death, whether they occur immediately after surgery or many
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years later [1]. The relationship between arrhythmias (electrical problems) and hemodynamics (mechanical issues) is complex and can be compared with the chicken versus egg discussion. Mechanical problems like poor ventricular function and valve regurgitation predispose the patient to arrhythmias. In turn, arrhythmias can lead to poor ventricular function, and to the development of problems like protein-losing enteropathy (PLE), and thrombus formation. Once established, the vicious cycle of arrhythmias can
be hard to interrupt. Arrhythmia has been described as a strong “predictor of mortality” and “key marker of deterioration” with a 23-fold increased risk of death [1]. However, the Fontan is not one operation. At least two major modifications, namely the intracardiac lateral tunnel (ILT) and the extracardiac conduit (ECC), have been described since the original atriopulmonary connection (APC) type of Fontan, and they have had a significant impact on the incidence and severity of arrhythmias.
Incidence The incidence of early post-operative arrhythmias after the APC Fontan is estimated to be around 10–30% [2, 3]. This is however now only of historical int
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