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

2017 ◽  
Vol 4 (4) ◽  
pp. 675-678 ◽  
Author(s):  
Phuoc Duong ◽  
Louise Coats ◽  
John O'Sullivan ◽  
David Crossland ◽  
Beate Haugk ◽  
...  
Author(s):  
Leigh C. Reardon ◽  
Jeannette P. Lin ◽  
Glen S. VanArsdell ◽  
Fady M. Kaldas ◽  
Gentian Lluri ◽  
...  

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.


Children ◽  
2021 ◽  
Vol 8 (6) ◽  
pp. 441
Author(s):  
P. Syamasundar Rao

In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.


2018 ◽  
Vol 9 (1) ◽  
pp. 31-37 ◽  
Author(s):  
Syed M. Peer ◽  
Kristopher B. Deatrick ◽  
Thomas J. Johnson ◽  
Jonathan W. Haft ◽  
Francis D. Pagani ◽  
...  

Background: Mechanical circulatory support (MCS) of a failing Fontan circulation remains challenging. We hypothesized that MCS can be provided by converting the Fontan circulation into a mechanically assisted single ventricle parallel circulation (MASVC). Methods: A porcine model of functionally univentricular circulation was created under cardiopulmonary bypass (CPB) by performing an atrial septectomy, tricuspid valvectomy, and interrupting antegrade pulmonary blood flow. A centrifugal flow pump was placed with inflow from the common atrium. Eight millimeter Dacron grafts anastomosed to the ascending aorta and main pulmonary artery supplied systemic (Qs) and pulmonary (Qp) blood flow. Ultrasonic flow probes were used to measure Qs and Qp after weaning from CPB. The Qp/Qs ratio was regulated using an adjustable clamp. Hemodynamic and laboratory data were recorded. Results: All four animals were successfully weaned from CPB onto the MASVC for a duration of two hours. Mechanically assisted single ventricle parallel circulation achieved satisfactory hemodynamics. As anticipated, the arterial oxygen saturation and partial pressure of oxygen in arterial blood were lower in the MASVC compared to baseline biventricular circulation. At the conclusion of the study, there was a trend towards a decrease in the mixed venous saturation with increasing oxygen extraction compared to the baseline. Serum lactate levels increased after weaning from CPB and did not return to baseline after two hours of support. Conclusion: Mechanically assisted single ventricle parallel circulation can be established in a single ventricle animal model. This strategy could potentially provide MCS of a single ventricle circulation. Studies with longer duration of support are required to assess adequacy of support and long-term sustainability.


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