Pulsatile Glenn as long-term palliation for single ventricle physiology patients

2018 ◽  
Vol 13 (6) ◽  
pp. 927-934 ◽  
Author(s):  
Martin A. Chacon-Portillo ◽  
Rodrigo Zea-Vera ◽  
Huirong Zhu ◽  
Heather A. Dickerson ◽  
Iki Adachi ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Single Ventricle Physiology

LONG TERM OUTCOMES OF ADULTS WITH SINGLE VENTRICLE PHYSIOLOGY NOT UNDERGOING FONTAN REPAIR: A MULTICENTRE EXPERIENCE

2021 ◽  
Author(s):  
Francisco Buendía-Fuentes ◽  
Blanca Gordon-Ramírez ◽  
Laura Dos Subirà ◽  
Pablo Merás ◽  
Pastora Gallego ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Long Term Outcomes ◽  
Single Ventricle Physiology

scholarly journals IS A PULSATILE GLENN AN ADEQUATE ALTERNATIVE FOR LONG-TERM PALLIATION IN PATIENTS WITH SINGLE VENTRICLE PHYSIOLOGY?

2017 ◽  
Vol 69 (11) ◽  
pp. 621
Author(s):  
Carlos M. Mery ◽  
Martin A. Chacon-Portillo ◽  
Huirong Zhu ◽  
Heather Dickerson ◽  
Iki Adachi ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Single Ventricle Physiology

Long term outcome of single ventricle physiology with pulmonary restriction not undergoing Fontan repair

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
F Buendia ◽  
B Ramirez ◽  
P Gallego ◽  
J.M Oliver ◽  
S Montserrat ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Single Ventricle Physiology ◽  
Pulmonary Flow ◽  
Adult Congenital ◽  
Group 3 ◽  
Group 2

Abstract Background Patients with univentricular physiology who do not complete the palliation to Fontan are a heterogeneous group with unknown long term outcome. Aims This study aimed at describing the clinical course and long-term survival of patients with SV physiology with restricted pulmonary flow that had not undergone a Fontan type of repair. Methods From the prospectively maintained databases of the adult congenital cardiac units of five tertiary referral centers, data from all SV physiology patients were obtained. Patients completing a Fontan type palliation or developing Eisenmenger physiology and segmental pulmonary hypertension were excluded. Baseline data were recorded on the first visit at adult congenital heart disease (ACHD) unit. The primary end point was death. Results 101 patients (50.5% females) were identified. Mean age at end of follow up was 39.3±11.3 years. Of these, 45 (44.6%) were unoperated (group 1, restricted forward pulmonary flow with or without pulmonary banding), 38 (37.6%) had undergone a cavopulmonary shunt as a definitive palliation (group 2) and 18 (17.8%) had aortopulmonary shunts (group 3). The main diagnosis was double inlet left ventricle (DILV) (N: 52, 51.5%) and most of the ventricle was left (82.2%). The principal reason for not performing a Fontan repair was mean pulmonary artery pressure >18 mmHg. At initial visit at the ACHD unit patients were 32.2±11.1 years of age. 35% of the patients were in NYHA class III-IV, with no differences between groups. However, patients in group 2 had worse oxygen saturation (p=002) and higher haemoglobin (p=0.037). After a mean follow-up of 7.3±4.1 years, mortality was 20.8% (21 patients), being sudden death (7p, 6.9%) the most frequent cause. Patients in group 3 showed worse ventricular function (p=0.0001) and a trend to higher mortality that did not reach statistical significance (HR 2.7, CI 95% 0.91–8.14, P=0.07). Conclusions Patients with single ventricle physiology not undergoing Fontan repair are a population of high risk, with sudden death as main driver of mortality. Patients palliated with aortopulmonary shunts are prone to worse ventricular function and a trend to higher mortality. Funding Acknowledgement Type of funding source: Public hospital(s)

scholarly journals Value of Follow-Up N-Terminal Probrain Natriuretic Peptide (NT-proBNP) after a Modified Fontan Procedure

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Jianbin Li ◽  
Li Ma ◽  
Minghui Zou ◽  
Wenlei Li ◽  
Xinxin Chen ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Natriuretic Peptide ◽  
Single Ventricle ◽  
Fontan Procedure ◽  
Left Ventricular ◽  
Single Ventricle Physiology ◽  
Long Term Follow Up ◽  
The Right

Objective. To assess the value of N-terminal probrain natriuretic peptide (NT-proBNP) in short-term and long-term follow-up after a modified Fontan procedure. Methods. We retrospectively enrolled children who had undergone a modified Fontan procedure in the Heart Center of Guangzhou Women and Children’s Medical Center from January 2014 to September 2020 and collected data on NT-proBNP values before bidirectional Glenn procedure, before Fontan procedure, and on 1, 3, 7, 30, 90, and 180 days and 1, 2, 3, 4, 5, and 6 year after Fontan procedure. The relationship between changes in NT-proBNP levels and the outcomes in children was analyzed. Results. A total of 108 children (78 boys and 30 girls, mean age: 54.62 ± 29.38 weeks) were included in the analysis. According to one-way analysis of variance, the left ventricular type and biventricular type of single ventricle physiology showed shorter duration on cardiopulmonary bypass during the operation and lower levels of NT-proBNP after the operation than the right ventricular type and univentricular type physiology. Conclusion. NT-proBNP is a good indicator for mid and long-term follow-up after a modified Fontan procedure. The left ventricular type and biventricular type of single ventricle physiology show better mid and long-term benefits from the modified Fontan procedure than the right ventricular type and univentricular type physiology.

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

2021 ◽  
Author(s):  
Leigh C. Reardon ◽  
Jeannette P. Lin ◽  
Glen S. VanArsdell ◽  
Fady M. Kaldas ◽  
Gentian Lluri ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Venous Hypertension ◽  
Term Survival ◽  
Single Ventricle Physiology ◽  
Challenges And Opportunities ◽  
Fontan Physiology ◽  
Failing Fontan

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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