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Fontan failure: phenotypes, evaluation, management, and future directions

Published online by Cambridge University Press:  22 June 2022

Ujjwal Kumar Chowdhury Open the ORCID record for Ujjwal Kumar Chowdhury [Opens in a new window] ,
Niwin George ,
Lakshmi Kumari Sankhyan ,
Doniparthi Pradeep ,
Chaitanya Chittimuri ,
Abhinavsingh Chauhan ,
Niraj Nirmal Pandey Open the ORCID record for Niraj Nirmal Pandey [Opens in a new window]  and
Shikha Goja
Ujjwal Kumar Chowdhury*
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Niwin George
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Lakshmi Kumari Sankhyan
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, Bilaspur, India
Doniparthi Pradeep
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Chaitanya Chittimuri
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Abhinavsingh Chauhan
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Niraj Nirmal Pandey
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Shikha Goja
Affiliation:
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
*
Author for correspondence: Dr U. K. Chowdhury, M.Ch., Diplomate NB, Department of Cardiothoracic and Vascular Surgery, AIIMS, New Delhi, 110029, India. Tel: +91 11 26594835; Fax: +91 11 26588641. E-mail: ujjwalchowdhury@gmail.com
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Abstract

Objectives:

Management of “failing” and “failed” Fontan circulation, particularly the indications, timing, and type of re-intervention, currently remains nebulous. Factors contributing to pathogenesis and mortality following Fontan procedure differ between children and adults.

Methods:

Since organ systems in individual patients are affected differently, we searched the extant literature for a “failing” and “failed” Fontan reviewing the clinical phenotypes, diagnostic modalities, pharmacological, non-pharmacological, and surgical techniques employed, and their outcomes.

Results:

A total of 410 investigations were synthesised. Although proper candidate selection, thoughtful technical modifications, timely deployment of mechanical support devices, tissue-engineered conduits, and Fontan takedown have decreased the peri-operative mortality from 9 to 15% and 1 to 3% per cent in recent series, pernicious changes in organ function are causing long-term patient attrition. In the setting of a failed Fontan circulation, literature documents three surgical options: Fontan revision, Fontan conversion, or cardiac transplantation. The reported morbidity of 25% and mortality of 8–10% among Fontan conversion continue to improve in select institutions. While operative mortality following cardiac transplantation for Fontan failure is 30% higher than for other CHDs, there is no difference in long-term survival with actuarial 10-year survival of around 54%. Mechanical circulatory assistance, stem cells, and tissue-engineered Fontan conduit for destination therapy or as a bridge to transplantation are in infancy for failing Fontan circulation.

Conclusions:

An individualised management strategy according to clinical phenotypes may delay the organ damage in patients with a failing Fontan circulation. At present, cardiac transplantation remains the last stage of palliation with gradually improving outcomes.

Keywords

CHDfunctionally univentricular heartFontanFontan failureheart failureportal hypertension

Information

Type
Review
Information
Cardiology in the Young , Volume 32 , Issue 10 , October 2022 , pp. 1554 - 1563
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

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Footnotes

All authors have contributed equally to this work

References

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