scholarly journals MACITENTAN USE AS SERIAL THERAPY IN FAILING FONTAN PHYSIOLOGY

2017 ◽  
Vol 69 (11) ◽  
pp. 632
Author(s):  
Tabitha Grace Moe
2009 ◽  
Vol 33 (11) ◽  
pp. 977-987 ◽  
Author(s):  
Sonya S. Bhavsar ◽  
Jugal Y. Kapadia ◽  
Steven G. Chopski ◽  
Amy L. Throckmorton

2004 ◽  
Vol 14 (S1) ◽  
pp. 114-114 ◽  
Author(s):  
Leonard L. Bailey

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.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ilias P Doulamis ◽  
Supreet P Marathe ◽  
Breanna L Piekarski ◽  
Rebecca S Beroukhim ◽  
Gerald R Marx ◽  
...  

Hypothesis: Biventricular conversion (BiVC) following takedown of Fontan circulation is feasible and results in improved hemodynamics. Methods: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to BiV circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mmHg. Results: There were 23 patients (median age: 10.0 (7.5-13.0) years); 15 (65%) had failing Fontan physiology and 8 (35%) underwent elective takedown of their Fontan circulation. Of the 15 patients with failing Fontan physiology, 4 had exercise intolerance or cyanosis, 3 had hepatic congestion or cirrhosis, 3 had end-organ damage and 1 patient had protein losing enteropathy; the rest 4 patients had no other sign of SVP complications. A subset of patients (n=6) underwent recruitment of the non-dominant ventricle prior to conversion. HLHS (p<0.01) and sub-/aortic stenosis (p<0.01) were more common in these patients. BiVC with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (p<0.01), indexed end-systolic volume (p<0.01) and ventricular mass (p<0.01) of the non-dominant ventricle (14 RV, 9 LV). There were 1 (4%) early and 4 (17%) late deaths. All who underwent elective BiVC survived, while 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% CI: 37-90%) (Figure 1). The overall, 1-year reintervention free survival was 44.1% (95% CI: 21-65%). Left dominant atrioventricular canal defect (p<0.01) and early year of BiVC (p=0.02) were significant predictors for mortality. Conclusions: BiVC is feasible in patients with failing Fontans, and has promising outcomes after elective takedown of Fontan circulation. A staged approach for ventricular recruitment does not seem inferior to primary BiVC. The optimal timing for BiVC in Fontan patients needs further evaluation.


2013 ◽  
Vol 34 (6) ◽  
pp. 1357-1365 ◽  
Author(s):  
Amy L. Throckmorton ◽  
Sergio Lopez-Isaza ◽  
Emily A. Downs ◽  
Steven G. Chopski ◽  
James J. Gangemi ◽  
...  

2009 ◽  
Vol 4 (2) ◽  
pp. 107-111 ◽  
Author(s):  
Gira S. Morchi ◽  
D. Dunbar Ivy ◽  
Mark C. Duster ◽  
Lori Claussen ◽  
Kak-Chen Chan ◽  
...  

2011 ◽  
Vol 2 (2) ◽  
pp. 243-248 ◽  
Author(s):  
Lucian A. Durham ◽  
Joseph A. Dearani ◽  
Harold M. Burkhart ◽  
Lyle D. Joyce ◽  
Frank Cetta ◽  
...  

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Caroline Eden ◽  
Hugo Clifford ◽  
Arthur Wang ◽  
Asif Mohammed ◽  
Peter Yim

Abstract Background Anesthetic management of an adult with failing Fontan physiology is complicated given inherent anatomical and physiological alterations. Neurosurgical interventions including thromboembolectomy may be particularly challenging given importance of blood pressure control and cerebral perfusion. Case Presentation We describe a 29 year old patient born with double outlet right ventricle (DORV) with mitral valve atresia who after multi-staged surgeries earlier in life, presented with failing Fontan physiology. She was admitted to the hospital almost 29 years after her initial surgeries to undergo workup for a dual heart and liver transplant in the context of a failing Fontan with elevated end diastolic pressures, NYHA III heart failure symptoms, and liver cirrhosis from congestive hepatopathy. During the workup in the context of holding anticoagulation for invasive procedures, she developed a middle cerebral artery (MCA) stroke requiring a thromboembolectomy via left carotid artery approach. Discussion and Conclusions This case posed many challenges to the anesthesiologist including airway control, hemodynamic and cardiopulmonary monitoring, evaluation of perfusion, vascular access, and management of anticoagulation in an adult patient in heart and liver failure with Fontan physiology undergoing thromboembolectomy for MCA embolic stroke.


1998 ◽  
Vol 65 (1) ◽  
pp. 268-270 ◽  
Author(s):  
Helena T Henneveld ◽  
Paul Hutter ◽  
Francois J Hitchcock ◽  
Narayanswami Sreeram

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.


2015 ◽  
Vol 25 (8) ◽  
pp. 1489-1492 ◽  
Author(s):  
Brian S. Snarr ◽  
Stephen M. Paridon ◽  
Jack Rychik ◽  
David J. Goldberg

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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