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

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.

Developing an adolescent and adult Fontan Management Programme

2021 ◽  
pp. 1-6
Author(s):  
Adam M. Lubert ◽  
Tarek Alsaied ◽  
Andrew T. Trout ◽  
Jonathan R. Dillman ◽  
Joseph J. Palermo ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Multiple Organ ◽  
Fontan Circulation ◽  
Management Programme ◽  
Adverse Outcomes ◽  
Cardiac Complications ◽  
Organ Systems ◽  
Adolescents And Adults ◽  
Care Models

Abstract Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

12th Annual C. Walton Lillehei Memorial Lecture in Cardiovascular Surgery: Fontan conversion – the Chicago experience

2011 ◽  
Vol 21 (S2) ◽  
pp. 169-176 ◽  
Author(s):  
Carl L. Backer
Keyword(s):  
Cardiac Transplantation ◽  
Operative Mortality ◽  
Cardiovascular Surgery ◽  
Fontan Procedure ◽  
Memorial Lecture ◽  
Univentricular Heart ◽  
Fontan Conversion ◽  
Double Inlet Left Ventricle ◽  
Arrhythmia Surgery

AbstractBetween 1994 and 2011, we performed 133 Fontan conversions with arrhythmia surgery. Most patients had tricuspid atresia or double-inlet left ventricle with prior atriopulmonary connection. Operative mortality was 1.5%, and mean length of stay was 14 days. A total of eight patients (6%) have had late cardiac transplantation. Freedom from arrhythmia recurrence is 85% at 10 years. For properly selected patients with a functionally univentricular heart who have had an atriopulmonary Fontan procedure, Fontan conversion with arrhythmia surgery significantly improves quality of life.

Abstract 16822: Growth Curves Describing a Single Ventricle Population After Fontan Procedure

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Bethany Runkel ◽  
Vincent Staggs ◽  
Chelsea Hosey Cojocari ◽  
Jonathan B Wagner
Keyword(s):  
Single Ventricle ◽  
Multiple Stressors ◽  
Pediatric Population ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Growth Patterns ◽  
Growth Charts ◽  
Genetic Syndrome ◽  
Adiposity Rebound ◽  
Extracardiac Conduit

Introduction: Complex single ventricle congenital heart disease (CHD) patients are subject to multiple stressors early in life that affect somatic growth. Pre-Fontan growth patterns are well-described, but post-Fontan growth has not been extensively studied in the current era. We sought to describe post-Fontan growth patterns by creating sex-specific body mass index (BMI)-for-age curves. Hypothesis: There is no difference in the growth of post-Fontan pediatric patients when compared to published normative data. Methods: A single-center, preexisting database was retrospectively queried for all patients who underwent Fontan procedure between 2006 and 2018. Patients with a genetic syndrome, a primary endocrine disorder, or significant prematurity were excluded. BMI-for-age curves were created for both male and female patients using anthropometric data extracted from the electronic medical record. Curves were then visually compared with Centers for Disease Control and Prevention (CDC) growth charts. Results: Of 227 patients who underwent primary Fontan operation, 37 were excluded. Of the remaining 190 patients, 59% were male. All had an extracardiac conduit, half had right-ventricular dominant CHD, and 15% had a Fontan fenestration. Median age and weight at surgery were 3.9 years and 15 kilograms, respectively. BMI curves were similar in appearance to CDC BMI-for-age growth charts, with adiposity rebound at age 6. BMI increased more rapidly in teen boys compared with girls, and a total of five patients (2.6%) had BMI values greater than 25 kg/m 2 at 16 years of age. Conclusion: Post-Fontan patients at our institution demonstrate BMI-for-age patterns similar to those of the general population during childhood and early adolescence, with adiposity rebound occurring at a typical age. Though excess weight gain does not completely spare patients with single ventricle CHD, overweight status appears to be less common than in the general pediatric population.

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

Abstract 78: Late Gadolinium Enhancement on Cardiac MRI Identifies Ventricular Dysfunction and Regional Myocardial Dyssynchrony in Patients with Univentricular Heart Diseases

2014 ◽  
Vol 115 (suppl_1) ◽  
Author(s):  
Michihiro Okuyama ◽  
Shuta Ishigami ◽  
Daiki Ousaka ◽  
Junko Kobayashi ◽  
Shingo Kasahara ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Myocardial Fibrosis ◽  
Prognostic Value ◽  
Circumferential Strain ◽  
Single Ventricle ◽  
Heart Diseases ◽  
Volume Index ◽  
Univentricular Heart ◽  
Gadolinium Enhancement ◽  
The Impact

Backgrounds: The impact of myocardial fibrosis on cardiac performance and clinical outcomes in patients with a functional single ventricle before stage-3 operation is unknown. Objective: The purpose of this study is to investigate the prognostic value of myocardial fibrosis identified by cardiac magnetic resonance imaging (cMRI) in patients with univentricular heart diseases. Methods: Consecutive 23 patients undergoing staged-3 surgical palliation with single ventricle physiology were prospectively scheduled to have cMRI study with late gadolinium enhancement (LGE) imaging and ventricle circumferential strain were examined. Results: Of 23 patients (mean age 3.3±0.9 years), 6 (26%) had positive late gadolinium enhancement (LGE+) in the ventricular myocardium, median percent LGE was 3.0% (interquartile range 3.0% to 14.0%). Pre-Fontan examinations revealed that patients with LGE+ had increased end-diastolic volume index (142.8 ml/BSA vs. 113.8 ml/BSA; P=0.02), increased end-systolic volume index (101.0 ml/BSA vs. 72.2 ml/BSA); P=0.01) compared with those without LGE (LGE-). Patients with LGE have shown to have lower ventricular circumferential strain compared with the area without LGE (basal: −1.9±1.9% vs. −4.0±3.0%, P=0.046; mid: −3.9±2.1% vs −8.0±3.9%, P=0.007; apical: −3.9±2.4% vs. −8.2±2.8%, P=0.004). In contrast to LGE- group, patients in LGE+ group had decreased right ventricular ejection fraction (27.7±8.8% vs. 38.2±8.4%; P=0.02) as well as higher levels of BNP (99.2±75.7 pg/ml vs. 32.6±44.3 pg/ml, P=0.02). In addition, patients with LGE+ had higher score of Ross classification (2.5±0.55 vs. 2.0±0, P=0.02) and New York University Pediatric Heart Failure Index (11.0±3.5 vs. 7.8±1.1, P=0.01) than in LGE- group. Age at stage-2 palliation was significantly older in patients with LGE+ group than LGE- subjects (16.8±16 months vs. 8.8±3.4 months, P=0.03). Conclusion: In this pre-stage-3 cMRI study, the age to stage-2 palliation may attribute to substantial myocardial fibrosis. The area of LGE was associated with impaired regional circumferential strain as well as disturbed ventricular performance. This novel strategy may provide a possible prognostic value of latent myocardial dysfunction after staged palliation.

Off-pump extracardiac Fontan completion: Surgical technique and pitfalls

2021 ◽  
Keyword(s):  
Surgical Technique ◽  
Laminar Flow ◽  
Single Ventricle ◽  
Fontan Procedure ◽  
Atrial Arrhythmias ◽  
Off Pump ◽  
Extracardiac Fontan

Completion of the extracardiac Fontan procedure is the final palliative stage for treating a functional single ventricle. It has been associated with a smaller incidence of atrial arrhythmias and more laminar flow in the Fontan pathway. We present our technique for the off-pump extracardiac Fontan procedure.

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.

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