Orthotopic Heart and Combined Heart Liver Transplantation: the Ultimate Treatment Option for Failing Fontan Physiology

Abstract Purpose of the Review This is a comprehensive update on failing Fontan physiology and the role of heart and combined heart and liver transplantation in the current era. Recent Findings Single ventricle physiology encompasses a series of rare congenital cardiac abnormalities that are characterized by absence of or hypoplasia of one ventricle. This effectively results in a single ventricular pumping chamber. These abnormalities are rarely compatible with long-term survival if left without surgical palliation in the first few years of life. Surgical treatment of single ventricle physiology has evolved over the past 60 years and is characterized by numerous creative innovations. These include the development of arteriopulmonary shunts, the evolution of partial cavopulmonary connections, and the eventual development of the “Fontan” operation. Regardless of the type of Fontan modification, the long-term consequences of the Fontan operation are predominantly related to chronic central venous hypertension and the multi-organ consequences thereof. Atrial arrhythmias can further compromise this circulation.Patients with single ventricle physiology represent a special sub-segment of congenital cardiac transplants and are arguably the most challenging patients considered for transplantation. Summary This review describes in detail the challenges and opportunities of heart and liver transplantation in Fontan patients, as viewed and managed by the experienced team at the Ahmanson/UCLA Adult Congenital Heart Center.

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Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy

Cardiology in the Young ◽

10.1017/s1047951115002309 ◽

2015 ◽

Vol 25 (8) ◽

pp. 1489-1492 ◽

Cited By ~ 17

Author(s):

Brian S. Snarr ◽

Stephen M. Paridon ◽

Jack Rychik ◽

David J. Goldberg

Keyword(s):

Single Ventricle ◽

Fontan Operation ◽

Vasodilator Therapy ◽

Pulmonary Vasodilators ◽

Pulmonary Vasodilator ◽

Fontan Physiology ◽

Fontan Surgery ◽

Failing Fontan ◽

Fontan Patients

AbstractThe Fontan operation is the final step of palliation for patients with a functionally single ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a successful surgical strategy that has improved single ventricle mortality. In recent years, we have become more aware of the limitations and long-term consequences of the Fontan physiology. Pulmonary vascular resistance plays an important role in total cavopulmonary circulation, and has been identified as a potential therapeutic target to mitigate Fontan sequelae. In this review, we will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasodilators as a treatment strategy for Fontan patients.

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Rebuttal

Cardiology in the Young ◽

10.1017/s1047951104006432 ◽

2004 ◽

Vol 14 (S1) ◽

pp. 114-114 ◽

Cited By ~ 1

Author(s):

Leonard L. Bailey

Keyword(s):

Coronary Disease ◽

Reconstructive Surgery ◽

Fontan Circulation ◽

Actuarial Survival ◽

Term Survival ◽

Long Term Survival ◽

Fontan Physiology ◽

Failing Fontan

The portrayal of a beautiful youngster performing uninhibited acrobatics based on Fontan physiology, as presented by Marshall Jacobs, is a brilliant and beautiful thing for us to see. It is, perhaps, all about will over physiology. But it is, nevertheless, happening for that child. Marshall mentioned the need for re-transplantation, whether the beginning strategy was transplantation or reconstructive surgery. Indeed, a relatively small percentage of children transplanted will require re-transplantation because of severe graft coronary disease. Remarkably, in the Loma Linda experience, 10-year actuarial survival for 26 patients following elective re-transplantation is over 85%, exceeding overall actuarial survival at 10 years for children following primary transplantation. Many of the transplanted infants, however, seem to be realistically hopeful that one heart will last their entire lifetime. Of course, the hope is that their's will be a long and healthy lifetime. The requirement for late transplantation following Fontan procedures, however, seems almost inevitable. We'll simply have to keep these children with Fontan circulation under surveillance to see when, in the course of their lives, transplantation will become necessary. Unfortunately, operative and long-term survival among children who are transplanted for failing Fontan physiology have, as yet, been somewhat suboptimal.

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Combined heart-liver transplantation for failing Fontan circulation in a late survivor with single-ventricle physiology

ESC Heart Failure ◽

10.1002/ehf2.12202 ◽

2017 ◽

Vol 4 (4) ◽

pp. 675-678 ◽

Cited By ~ 6

Author(s):

Phuoc Duong ◽

Louise Coats ◽

John O'Sullivan ◽

David Crossland ◽

Beate Haugk ◽

...

Keyword(s):

Liver Transplantation ◽

Single Ventricle ◽

Fontan Circulation ◽

Single Ventricle Physiology ◽

Failing Fontan

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Surgical revisions and mechanical support of the failing Fontan

Cardiology in the Young ◽

10.1017/s1047951113001674 ◽

2013 ◽

Vol 23 (6) ◽

pp. 847-851 ◽

Cited By ~ 3

Author(s):

Stephanie Fuller

Keyword(s):

Single Ventricle ◽

Fontan Operation ◽

Mechanical Support ◽

Protein Losing Enteropathy ◽

Management Of Complications ◽

Fontan Failure ◽

Mechanical Devices ◽

Surgical Treatments ◽

Failing Fontan

AbstractDespite its many anatomic modifications, Fontan failure remains prevalent in adult patients living with single-ventricle physiology. Although the Fontan operation has created a generation of survivors of complex single-ventricle disease, recognition of the limitation of this circulation is increasing. Most commonly, survivors suffer from ventricular dysfunction, protein-losing enteropathy, plastic bronchitis and arrhythmias. Although several medical therapies exist to palliate these long-term complications, surgical treatments are becoming increasingly useful, including the introduction of mechanical devices and cardiac transplantation. Currently, there are a variety of options available for mechanical support of the failing Fontan. Further developments in mechanical support technology will be required to tailor-make specific devices for this unique population. Owing to the fact that availability of donor hearts for transplantation is limited, mechanical support will play an increasingly important role in the management of complications of the Fontan circulation.

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Review on Mechanical Support and Cell-Based Therapies for the Prevention and Recovery of the Failed Fontan-Kreutzer Circulation

Frontiers in Pediatrics ◽

10.3389/fped.2020.627660 ◽

2021 ◽

Vol 8 ◽

Author(s):

Margaret R. Ferrari ◽

Michael V. Di Maria ◽

Jeffrey G. Jacot

Keyword(s):

Single Ventricle ◽

Technology Development ◽

Fontan Circulation ◽

Circulatory Support ◽

Short Term ◽

Term Survival ◽

Fontan Physiology ◽

Mechanical Assist ◽

Short Term Survival

Though the current staged surgical strategy for palliation of single ventricle heart disease, culminating in a Fontan circulation, has increased short-term survival, mounting evidence has shown that the single ventricle, especially a morphologic right ventricle (RV), is inadequate for long-term circulatory support. In addition to high rates of ventricular failure, high central venous pressures (CVP) lead to liver fibrosis or cirrhosis, lymphatic dysfunction, kidney failure, and other comorbidities. In this review, we discuss the complications seen with Fontan physiology, including causes of ventricular and multi-organ failure. We then evaluate the clinical use, results, and limitations of long-term mechanical assist devices intended to reduce RV work and high CVP, as well as biological therapies for failed Fontan circulations. Finally, we discuss experimental tissue engineering solutions designed to prevent Fontan circulation failure and evaluate knowledge gaps and needed technology development to realize a more robust single ventricle therapy.

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