open access

Vol 18, No 2 (2011)
Review articles
Published online: 2011-03-10
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Challenges of management and therapy in patients with a functionally single ventricle after Fontan operation

Olga Trojnarska, Aleksandra Ciepłucha
Cardiol J 2011;18(2):119-127.

open access

Vol 18, No 2 (2011)
Review articles
Published online: 2011-03-10

Abstract

Forty years ago, Fontan and Baudet performed the first life-saving operation on a patient with a functionally single ventricle. This multi-stage procedure established the connection between systemic venous circulation and pulmonary arteries. As a consequence, the pulmonary circulation is supplied in a passive way, whereas the single ventricle pumps the blood into the systemic circulation only.
Over the years, the technique of creating the abovementioned vascular connections has undergone several modifications. Due to the fundamental non-physiological hemodynamic relations between arterial pulmonary and systemic venous pressures, numerous complications can be observed in these patients including: supraventricular arrhythmias, thromboemboli, hepatic dysfunction, protein-losing enteropathy, heart failure, worsening cyanosis, systemic venous collateralization, and pulmonary arteriovenous malformations, as well as connective tissue lesions in bronchi.
Although based on an ingenious concept, the operation remains of a palliative character. Occasionally, heart transplantation is the ultimate resolution. Pharmacological therapy, and surgical conversion, often appear to be ineffective. However, this procedure has enabled many patients to reach adulthood and enjoy their lives to the full. This fact poses a great challenge for cardiologists wishing to become more knowledgeable and experienced as regards such patients, if we are not to waste such fabulous surgical achievements. (Cardiol J 2011; 18, 2: 119-127)

Abstract

Forty years ago, Fontan and Baudet performed the first life-saving operation on a patient with a functionally single ventricle. This multi-stage procedure established the connection between systemic venous circulation and pulmonary arteries. As a consequence, the pulmonary circulation is supplied in a passive way, whereas the single ventricle pumps the blood into the systemic circulation only.
Over the years, the technique of creating the abovementioned vascular connections has undergone several modifications. Due to the fundamental non-physiological hemodynamic relations between arterial pulmonary and systemic venous pressures, numerous complications can be observed in these patients including: supraventricular arrhythmias, thromboemboli, hepatic dysfunction, protein-losing enteropathy, heart failure, worsening cyanosis, systemic venous collateralization, and pulmonary arteriovenous malformations, as well as connective tissue lesions in bronchi.
Although based on an ingenious concept, the operation remains of a palliative character. Occasionally, heart transplantation is the ultimate resolution. Pharmacological therapy, and surgical conversion, often appear to be ineffective. However, this procedure has enabled many patients to reach adulthood and enjoy their lives to the full. This fact poses a great challenge for cardiologists wishing to become more knowledgeable and experienced as regards such patients, if we are not to waste such fabulous surgical achievements. (Cardiol J 2011; 18, 2: 119-127)
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Keywords

Fontan operation; long-term follow-up; complications; management

About this article
Title

Challenges of management and therapy in patients with a functionally single ventricle after Fontan operation

Journal

Cardiology Journal

Issue

Vol 18, No 2 (2011)

Pages

119-127

Published online

2011-03-10

Bibliographic record

Cardiol J 2011;18(2):119-127.

Keywords

Fontan operation
long-term follow-up
complications
management

Authors

Olga Trojnarska
Aleksandra Ciepłucha

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