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Review
. 2024 May 22;13(11):3049.
doi: 10.3390/jcm13113049.

Contemporary Management of the Failing Fontan

Prashanth Venkatesh  1 , Hans Gao  1 , Islam Abudayyeh  2 , Ramdas G Pai  3 , Padmini Varadarajan  3
Affiliations
Review

Contemporary Management of the Failing Fontan

Prashanth Venkatesh et al. J Clin Med. .

Abstract

Adult patients with congenital heart disease have now surpassed the pediatric population due to advances in surgery and improved survival. One such complex congenital heart disease seen in adult patients is the Fontan circulation. These patients have complex physiology and are at risk for several complications, including thrombosis of the Fontan pathway, pulmonary vascular disease, heart failure, atrial arrhythmias, atrioventricular valve regurgitation, and protein-losing enteropathy. This review discusses the commonly encountered phenotypes of Fontan circulatory failure and their contemporary management.

Keywords: Fontan circulation; Fontan failure; congenital heart disease; single ventricle physiology.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Hypoplastic left ventricle with extracardiac Fontan.
Figure 2
Figure 2
Chest X-ray of a patient with hypoplastic left heart and extracardiac Fontan with temporary mechanical circulatory support as bridge to heart transplant.
Figure 3
Figure 3
Selective angiography of extracardiac Fontan and left pulmonary artery status post multiple stents.
Figure 4
Figure 4
Selective angiography of bidirectional right-sided Glenn shunt (A) and extracardiac Fontan (B).
Figure 5
Figure 5
Transesophageal echocardiogram demonstrating an enlarged common atrium with a deformed atrioventricular valve (AV) (A) and severe AV valve regurgitation (B).
Figure 6
Figure 6
Transesophageal echocardiogram demonstrating two MitraClips XTW (A), and an improvement in atrioventricular (AV) regurgitation severity (B).
Figure 7
Figure 7
Fluoroscopic image showing the MitraClips in Position.
See this image and copyright information in PMC

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