Pulmonary haemodynamics in Fontan physiology after lobectomy in a patient with a single ventricle associated with pulmonary sequestration

2016 ◽  
Vol 27 (2) ◽  
pp. 381-384
Author(s):  
Sayaka Mii ◽  
Kazushi Yasuda ◽  
Hiroomi Murayama
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Pulmonary Sequestration ◽  
Univentricular Heart ◽  
Lower Lobectomy ◽  
Pulmonary Haemodynamics ◽  
Fontan Physiology ◽  
Right Lower Lobectomy ◽  
Cavopulmonary Connection

AbstractA 2-year-old girl with a functionally univentricular heart associated with a pulmonary sequestration underwent right lower lobectomy after which increased lung volume with low mean pulmonary artery pressure and pulmonary vascular resistance was documented. A cardiac catheterisation performed after a subsequent total cavopulmonary connection demonstrated favourable Fontan haemodynamics. Lobectomy may have induced compensatory lung growth, contributing to the maintenance of haemodynamics favourable for the long-term success of the Fontan procedure.

scholarly journals LONG TERM OUTCOMES AFTER THE FONTAN PROCEDURE IN PATIENTS WITH SINGLE VENTRICLE: A META-ANALYSIS

2017 ◽  
Vol 69 (11) ◽  
pp. 622
Author(s):  
Ilana Schwartz ◽  
Courtney McCracken ◽  
Christopher Petit ◽  
Ritu Sachdeva
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Fontan Procedure ◽  
Long Term Outcomes

scholarly journals Transplant-Amenable Hepatocellular Carcinoma in a Fontan Patient

2019 ◽  
Vol 13 (2) ◽  
pp. 275-279 ◽  
Author(s):  
Brian Patrick Rutledge ◽  
Anupama Devara ◽  
Sindhuri Benjaram ◽  
Stephanie Judd ◽  
Murray Ehrinpreis
Keyword(s):  
Hepatocellular Carcinoma ◽  
At Risk ◽  
Liver Transplant ◽  
Fontan Procedure ◽  
Venous Pressure ◽  
Fontan Patient ◽  
Number Of Patients ◽  
Fontan Physiology ◽  
The Ideal

The Fontan circulation alters a patient’s physiology and imparts long-term risks related to chronically elevated systemic venous pressure. An increasing number of patients with Fontan physiology are surviving into adulthood and are at risk of hepatic sequalae. The ideal timeline and method of hepatic surveillance in the Fontan population remains to be defined. In this case, the patient was diagnosed with hepatocellular carcinoma more than 20 years after undergoing the Fontan procedure and was a candidate for combined heart-liver transplant. That her disease progressed prior to organ availability supports the argument for hepatic surveillance in this population.

Superior Cavopulmonary Connection: Its Physiology, Limitations, and Anesthetic Implications

2020 ◽  
Vol 24 (4) ◽  
pp. 337-348
Author(s):  
Ray S. Choi ◽  
James A. DiNardo ◽  
Morgan L. Brown
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Single Ventricle ◽  
Fontan Procedure ◽  
Intermediate Stage ◽  
Ventricular Volume ◽  
Volume Load ◽  
Modes Of Failure ◽  
Anesthetic Considerations ◽  
Cavopulmonary Connection

The superior cavopulmonary connection (SCPC) or “bidirectional Glenn” is an integral, intermediate stage in palliation of single ventricle patients to the Fontan procedure. The procedure, normally performed at 3 to 6 months of life, increases effective pulmonary blood flow and reduces the ventricular volume load in patients with single ventricle (parallel circulation) physiology. While the SCPC, with or without additional sources of pulmonary blood flow, cannot be considered a long-term palliation strategy, there are a subset of patients who require SCPC palliation for a longer interval than the typical patient. In this article, we will review the physiology of SCPC, the consequences of prolonged SCPC palliation, and modes of failure. We will also discuss strategies to augment pulmonary blood flow in the presence of an SCPC. The anesthetic considerations in SCPC patients will also be discussed, as these patients may present for noncardiac surgery from infancy to adulthood.

Functionally single ventricle, Fontan procedure – univentricular heart/circulation

2008 ◽  
pp. 135-144 ◽  
Author(s):  
Jana Popelová ◽  
Erwin Oechslin ◽  
Harald Kaemmerer ◽  
Martin G St John Sutton ◽  
Pavel Žáček
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Univentricular Heart

scholarly journals Fontan circulation and implications for future reproduction

2016 ◽  
Vol 10 (3) ◽  
pp. 104-106
Author(s):  
Niharika Mehta ◽  
Srilakshmi Mitta
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Cardiac Complications ◽  
Preconception Counseling ◽  
Passive Flow ◽  
History Of ◽  
Future Reproduction ◽  
Long Term Prognosis

This article summarizes and critiques four recent publications looking at preconception counseling, pregnancy outcomes, and cardiac complications in women with history of Fontan circulation. The Fontan procedure is a palliative strategy for single-ventricle type congenital heart disease and involves passive flow of venous return into the pulmonary circulation, bypassing the ventricles. Pregnancy in these patients is not without risk and preconception counseling and contraception practices vary widely. High rates of miscarriage, prematurity, and small-for-gestational-age babies are reported. Cardiac complications include mainly arrhythmias. Whether long-term prognosis in these patients is affected by pregnancy is not yet known.

scholarly journals Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection

2012 ◽  
Vol 23 (3) ◽  
pp. 335-343 ◽  
Author(s):  
Andrew M. Atz ◽  
Thomas G. Travison ◽  
Brian W. McCrindle ◽  
Lynn Mahony ◽  
Andrew C. Glatz ◽  
...  
Keyword(s):  
Fontan Procedure ◽  
Cross Sectional Study ◽  
Univentricular Heart ◽  
P Value ◽  
Cross Sectional ◽  
Ventricular Performance ◽  
Cavopulmonary Anastomosis ◽  
Bidirectional Cavopulmonary Anastomosis ◽  
Fontan Surgery ◽  
Cavopulmonary Connection

AbstractBackgroundA superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.MethodsWe compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114).ResultsA prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome.ConclusionsAfter controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.

Long-term follow-up after staged reconstruction or transplantation for patients with functionally univentricular heart

2004 ◽  
Vol 14 (S1) ◽  
pp. 115-126 ◽  
Author(s):  
Gil Wernovsky ◽  
Maryanne R. K. Chrisant
Keyword(s):  
Single Ventricle ◽  
Univentricular Heart ◽  
Right Heart ◽  
Long Term Follow Up ◽  
Chronic Hypoxemia ◽  
Staged Reconstruction ◽  
Right Heart Bypass

As recently as 35 years ago, children born with a functionally single ventricle were destined to a lifetime of multiple palliative operations, and disrupted growth and development. Physical signs of their cardiac disease were evidenced by chronic hypoxemia, polycythemia, clubbing, small stature, and severely reduced exercise ability. A normal lifestyle, with typical landmarks such as uninterrupted adolescence and potential childbearing, was unlikely. The successful application of right heart bypass by Fontan and Kreutzer in the early 1970s heralded an era of multiple medical and surgical breakthroughs that have systematically improved the quality of life and long-term outlook for these children.

Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis

Heart ◽  
2018 ◽  
Vol 104 (18) ◽  
pp. 1508-1514 ◽  
Author(s):  
Ilana Schwartz ◽  
Courtney E McCracken ◽  
Christopher J Petit ◽  
Ritu Sachdeva
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Fontan Procedure ◽  
Time Intervals ◽  
Protein Losing Enteropathy ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Physiology ◽  
Pacemaker Insertion

ObjectiveMore patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.MethodsPubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.ResultsNineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.ConclusionsSurvival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.

Modeling of Patient-Specific Fontan Physiology From MRI Images for CFD Testing of a Cavopulmonary Assist Device

2011 ◽  
Author(s):  
Jonathan DeGan ◽  
Jeffrey Kennington ◽  
Kameswararao Anupindi ◽  
Dinesh Shetty ◽  
Jun Chen ◽  
...  
Keyword(s):  
Inferior Vena ◽  
Fontan Procedure ◽  
Pulmonary Arteries ◽  
Patient Specific ◽  
Systemic Blood Flow ◽  
Congenital Condition ◽  
Left And Right ◽  
Fontan Physiology ◽  
Cavopulmonary Connection ◽  
Cavopulmonary Assist Device

Single ventricle heart disease is a congenital condition characterized by the inoperability of one ventricle of an infant’s heart. Those suffering from this condition face a series of palliative surgeries called the Fontan procedure, which bypasses the non-functional ventricle by creating a total cavopulmonary connection, or TCPC. This TCPC forms from the anastomosis of the superior and inferior vena cavae (SVC, IVC) to the left and right pulmonary arteries (LPA, RPA), thus allowing systemic blood flow to bypass the heart and flow passively to the lungs. The Fontan procedure creates this junction with three surgeries separated by months or years.

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