Complications and management of functional single ventricle patients with Fontan circulation: From surgeon’s point of view

Abstract

Fontan surgery by step-wise completing the isolation of originally mixed pulmonary and systemic circulation provides an operative approach for functional single-ventricle patients not amenable to biventricular repair and allows their survival into adulthood. In the absence of a subpulmonic pumping chamber, however, the unphysiological Fontan circulation consequently results in diminished cardiac output and elevated central venous pressure, in which multiple short-term or long-term complications may develop. Current understanding of the Fontan-associated complications, particularly toward etiology and pathophysiology, is extremely incomplete. What’s more, ongoing efforts have been made to manage these complications to weaken the Fontan-associated adverse impact and improve the life quality, but strategies are ill-defined. Herein, this review summarizes recent studies on cardiac and non-cardiac complications associated with Fontan circulation, focusing on significance or severity, etiology, pathophysiology, prevalence, risk factors, surveillance, or diagnosis. From the perspective of surgeons, we also discuss the management of the Fontan circulation based on current evidence, including post-operative administration of antithrombotic agents, ablation, pacemaker implantation, mechanical circulatory support, and final orthotopic heart transplantation, etc., to standardize diagnosis and treatment in the future.

Case Report: Remote magnetic navigation and accessory pathways ablation in a single ventricle young adult with complex corrective surgeries

“…In cases of venous occlusion where vascular access is not possible, the transthoracic percutaneous route may be an option in reaching the atrium and thus be able to map and ablate (24). Rarely ablation procedures can be performed through atrial conducts (22).…”

Ventricular Morphology and Outcomes in Fontan Circulation without Hypoplastic Left Heart Syndrome: A Single-Center's Experience

“…Single ventricle (SV) deficits are rare congenital heart defects characterized by either one severely underdeveloped ventricle, or the absence of the ventricular septum, resulting in a broad range of cardiac structural abnormalities [ 1 , 2 ]. This life-threatening congenital heart defect necessitates prompt intervention, commonly through the Fontan operation [ 2 , 3 , 4 ].…”

“…Single ventricle (SV) deficits are rare congenital heart defects characterized by either one severely underdeveloped ventricle, or the absence of the ventricular septum, resulting in a broad range of cardiac structural abnormalities [ 1 , 2 ]. This life-threatening congenital heart defect necessitates prompt intervention, commonly through the Fontan operation [ 2 , 3 , 4 ]. This Fontan procedure, which may be executed in either one or multiple stages as a total cavopulmonary connection, provides palliation and treatment, achieving satisfactory long-term survival for SV patients [ 3 , 4 ].…”