Long term negative pressure ventilation: Rescue for the failing fontan?

Abstract

Current treatment strategies for single ventricle patients include non-intervention strategy, surgical palliation or primary transplantation. Surgical palliation includes a staged operative course culminating in the Fontan operation. With progress in surgical techniques, the survival has been improving. However, almost all of these Fontan patients will demonstrate pathophysiologic changes that ultimately constitute “Fontan failure physiology”. This article reviews the pathophysiologic changes, current approach to management of these patients and proposes a novel way of reversing some of the pathophysiologic changes by utilization of negative pressure ventilation.

Keywords: Fontan; Single ventricle physiology; Negative pressure ventilation; Cardiorespiratory interactions; Congenital heart disease

Core tip: In the current surgical era for congenital heart disease, palliation of single ventricle patients has become standard of care. However, pathophysiologic failure after the thrid stage of palliation (Fontan) is commonplace, with very few therapeutic options. Failing Fontan physiology is a management challenge. Herein, we review the pathophysiology of failing Fontan, current therapies and propose a novel way of treating the failing Fontan by utilizing negative pressure ventilation to reverse some of the maladapative changes. This is a hypothesis paper. We think, the ideas central to the manuscript are worth bringing out for intellectual discussion and wider testing.