Narrative review of single ventricle: where are we after 40 years?

Abstract

Background and Objective: Key medical and surgical advances have been made in the longitudinal management of patients with "functionally" single ventricle physiology, with the principles of Fontan circulation applied to other complex congenital heart defects. The purpose of this article is to review all of the innovations, starting from fetal life, that led to a change of strategy for single ventricle.Methods: Our literature review included all full articles published in English language on the Cochrane, MedLine, and Embase with references to "single ventricle" and "univentricular hearts", including the initial history of the treatments for this congenital heart defects as well as the innovations reported within the last decades.

Cardiac and Liver Fibrosis Assessed by Multiparametric MRI in Patients with Fontan Circulation

“…Although the long-term consequences of the Fontan procedure are a growing concern due to circuit failure [5,6], most patients in this cross-sectional study were in NYHA functional class I and had preserved ventricular function. Patients with Fontan had increased myocardial and liver native T1 and ECV, and increased MRE derived liver stiffness compared to controls.…”

“…The systolic ventricular function may remain preserved for several years following this surgery [3], although it tends to decline over time. Up to 30-50% of adult patients with Fontan circulation are expected to develop circuit failure [4][5][6][7].…”

The Functioning of the Frog’s Single Ventricle

“…The life expectancy of children born with "functionally" uni-ventricular hearts is severely reduced, as well as the long-term outcomes are negatively affected by heart failure and cyanosis, substantially compromising the quality of life [1][2][3][4][5][6][7][8][9][10][11][12][13]. Reports of patients with single ventricle surviving to the adult life either without any surgical treatment or after limited palliative procedures without undergoing Fontan completion are exceptionally rare [12,[14][15][16][17][18][19][20][21][22][23].…”

“…Reports of patients with single ventricle surviving to the adult life either without any surgical treatment or after limited palliative procedures without undergoing Fontan completion are exceptionally rare [12,[14][15][16][17][18][19][20][21][22][23]. In patients born with this type of complex congenital heart defect at least 2-3 staged operations are needed to achieve Fontan circulation, with the single ventricular chamber pumping the oxygenated blood to the body (systemic circulation), while the desaturated blood returning from the superior and inferior vena cava by pressure difference passively flows directly through the lungs (pulmonary circulation) [1][2][3][4][5][6][7][8][9][10][11][12][13]. Abnormally high systemic venous pressure is a consequence of the arrangement of this type of circulation and the chronic elevation of systemic venous pressure may cause liver failure, renal failure, protein-losing enteropathy and plastic bronchitis [4][5][6][7][8][9][10][11]13,24,25].…”

“…In patients born with this type of complex congenital heart defect at least 2-3 staged operations are needed to achieve Fontan circulation, with the single ventricular chamber pumping the oxygenated blood to the body (systemic circulation), while the desaturated blood returning from the superior and inferior vena cava by pressure difference passively flows directly through the lungs (pulmonary circulation) [1][2][3][4][5][6][7][8][9][10][11][12][13]. Abnormally high systemic venous pressure is a consequence of the arrangement of this type of circulation and the chronic elevation of systemic venous pressure may cause liver failure, renal failure, protein-losing enteropathy and plastic bronchitis [4][5][6][7][8][9][10][11]13,24,25]. The only available approaches to prevent or treat failing Fontan circulation are mechanical assistance or heart and heart and liver transplantation [28,29].…”