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Modern Day Care of Patients With Single Ventricle Heart Disease: Late Complications of Fontan Palliation

  • M. Abigail Simmons
  • Robert W. ElderEmail author
Cardiology (W Zuckerman and E Silver, Section Editors)
  • 2 Downloads
Part of the following topical collections:
  1. Cardiology

Abstract

Purpose of Review

The majority of individuals who have undergone Fontan palliation are surviving into adulthood, yet complications are common. This review will focus on late complications—cardiac and extra-cardiac—which may present insidiously.

Recent Findings

Recent meta-analysis of patients after Fontan operation suggests that over 80% will survive 20 years beyond surgery. This group is at risk for structural complications, arrhythmias, vascular complications, and heart failure. Not all forms of Fontan failure are the same, and categorization into separate entities such as failure with preserved versus reduced function may help guide therapies. Pulmonary vasodilators in particular may be useful to improve hemodynamics. Novel therapies aimed at lymphatic complications are emerging and promising. Late hepatic complications including ascites and liver cancer may be seen, and scoring systems may identify patients at higher risk.

Summary

Individuals who have undergone a Fontan surgery face challenges as they age which include both cardiac and non-cardiac systems.

Keywords

Fontan procedure Single ventricle Survival Cirrhosis Congenital heart disease Hepatocellular carcinoma Pulmonary vasodilators Lymphatic complications 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Section of Pediatric Cardiology, Department of Pediatrics and Internal MedicineYale University School of MedicineNew HavenUSA

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