Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure

Poh, Chin; Zannino, Diana; Weintraub, Robert G.; Winlaw, David S.; Grigg, Leeanne; Cordina, Rachael; Hornung, Tim; Bullock, Andrew; Justo, Robert; Gentles, Thomas L.; Verrall, Charlotte E; Plessis, Karin du; Celermajer, David S.; d’Udekem, Yves

doi:10.1016/j.ijcard.2017.01.057

Cited by 45 publications

(42 citation statements)

References 18 publications

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“…The high rate of complications, most often atrial arrhythmias, that occur after the Fontan operation has been well recognised for many years [43,44,292]. Atrial arrhythmias, due predominantly to macro re-entrant circuits, are understood to result from both postoperative right atrial scarring and progressive right atrial dilatation [293].…”

Section: Overviewmentioning

confidence: 99%

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Zentner

Celermajer

Gentles

et al. 2020

Heart, Lung and Circulation

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The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival.

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Section: Overviewmentioning

confidence: 99%

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Zentner

Celermajer

Gentles

et al. 2020

Heart, Lung and Circulation

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“…In older cohorts, higher re-operation rates have been reported 24 , 57 , 58 . The most common surgical re-intervention procedures, in order of incidence, are pacemaker implantation in 9 to 23% of patients 23 , 24 , Fontan revision or conversion in 3 to 18% of patients 9 , 24 , 59 , and atrioventricular (AV) valve repair in 1 to 14% of patients 24 , 59 , 60 .…”

Section: State Of the Artmentioning

confidence: 99%

“…In the “early days” of this procedure, it was attempted to replace the function of the right ventricle with the right atrium by connecting the right atrium to the pulmonary artery. Although short-term results were unprecedented, this strategy caused dilation of the right atrium, resulting in arrhythmia and thromboembolism due to sluggish blood flow 9 . Modifications of this surgery are referred to as atriopulmonary connections (APCs).…”

Section: Introductionmentioning

confidence: 99%

State of the art of the Fontan strategy for treatment of univentricular heart disease

et al. 2018

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In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining challenges in the Fontan field are outlined.

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“…A significant proportion of adult CHD patients are unemployed (33%) and many are neurodevelopmentally or physically challenged. 3,19 Moreover, many lack an organized transition to ongoing cardiac care, either due to individual barriers or lack of adequate health care delivery systems that support transition. The authors' finding of near-universal coverage is likely due to the fact that only a dichotomous factor (private vs public) to assess insurance status (rather than inclusion of both primary and secondary insurance) was utilized, 1 which severely limits the validity of inferences correlating insurance with economic viability.…”

Section: Clarity or Obscurity-the Socioeconomic Fontan Paradoxmentioning

confidence: 99%

Resolving the Fontan paradox: Addressing socioeconomic and racial disparities in patients with a single ventricle

Binwale¹,

Reemsten²,

Federman

et al. 2018

The Journal of Thoracic and Cardiovascular Surgery

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for the members of the Southwest Congenital Cardiac Consortium Feature Editor's Note-The Fontan operation, in many respects, is the signature operation for congenital heart surgeons. At our Heart Center, palliative operations leading up to the Fontan procedure, the Fontan procedure itself, and post-Fontan surgical interventions account for 20% of our congenital cardiac surgery case volume. Over the past 45 years, the Fontan operation has evolved steadily with numerous technical modifications as surgeons have sought to improve patient outcomes. The resources and attention devoted by heart centers to the totality of care of Fontan patients is enormous. In a recent issue of The Journal of the American College of Cardiology, Atz and colleagues from the Pediatric Heart Network Investigators reported on nearly 500 patients undergoing a thorough health assessment 9 years after their previous landmark Fontan crosssectional study. The authors noted a concerning finding in these patients: Exercise performance decreased over the years and this was associated with worse functional health status. We are fortunate to present an invited expert opinion summarizing and augmenting this article by Yves d'Udekem. d'Udekem is a passionate advocate for Fontan patients. He is the founder and chair of the Australian and New Zealand Fontan Registry. He estimates that there are currently 70,000 patients with a Fontan circulation. d'Udekem has reviewed the findings of Atz and colleagues and added to this his own extensive experience with the very robust Australian and New Zealand Fontan Registry. That comprehensive database goes back 35 years, and they have found that a high level of physical activity and exercise training optimizes outcomes after the Fontan procedure. This analysis will further advance steps we can take to improve the lives of this complex cohort of patients.

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Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure

Cited by 45 publications

References 18 publications

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

State of the art of the Fontan strategy for treatment of univentricular heart disease

Resolving the Fontan paradox: Addressing socioeconomic and racial disparities in patients with a single ventricle

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