Impaired Pulmonary Perfusion Associated With Thymus Hyperplasia in an Infant Candidate for Fontan Operation

Background: Although the avoidance of cardiopulmonary bypass during the Fontan procedure has potential advantages, using cardiopulmonary bypass during this procedure has no adverse effects in terms of morbidity and mortality rates. In this study, we assessed the postoperative outcomes of our first 9 patients who have undergone extracardiac Fontan operation by the same surgeon using cardiopulmonary bypass.Methods: Between September 2011 and April 2013, 9 consecutive patients (3 males and 6 females) underwent extra-cardiac Fontan operation. All operations were performed under cardiopulmonary bypass at normothermia by the same surgeon. The age of patients ranged between 4 and 17 (9.8 ± 4.2) years. Previous operations performed on these patients were modified Blalock-Taussig shunt procedure in 2 patients, bidirectional cavopulmonary shunt operation in 6 patients, and pulmonary arterial banding in 1 patient. Except 2 patients who required intracardiac intervention, cross-clamping was not applied. In all patients, the extracardiac Fontan procedure was carried out by interposing an appropriately sized tube graft between the infe-rior vena cava and right pulmonary artery.Results: The mean intraoperative Fontan pressure and transpulmonary gradient were 12.3 ± 2.5 and 6.9 ± 2.2 mm Hg, respectively. Intraoperative fenestration was not required. There was no mortality and 7 patients were discharged with-out complications. Complications included persistent pleural effusion in 1 patient and a transient neurological event in 1 patient. All patients were weaned off mechanical ventila-tion within 24 hours. The mean arterial oxygen saturation increased from 76.1% ± 5.3% to 93.5% ± 2.2%. All patients were in sinus rhythm postoperatively. Five patients required blood and blood-product transfusions. The mean intensive care unit and hospital stay periods were 2.9 ± 1.7 and 8.2 ± 1.9 days, respectively.Conclusions: The extracardiac Fontan operation per-formed using cardiopulmonary bypass provides satisfactory results in short-term follow-up and is associated with favor-able postoperative hemodynamics and morbidity rates.

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Faculty Opinions recommendation of 40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.725846316.793514388 ◽

2016 ◽

Author(s):

Larry Latson ◽

Athar Qureshi

Keyword(s):

Fontan Operation ◽

Long Term Outcomes

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Impact of the Fontan Operation on Organ Systems

Cardiovascular & Haematological Disorders - Drug Targets ◽

10.2174/1871529x19666190211165124 ◽

2019 ◽

Vol 19 (3) ◽

pp. 205-214

Author(s):

Miriam Michel ◽

Manuela Zlamy ◽

Andreas Entenmann ◽

Karin Pichler ◽

Sabine Scholl-Bürgi ◽

...

Keyword(s):

Systematic Study ◽

Fontan Operation ◽

Gastrointestinal System ◽

Adjunct Treatment ◽

Organ Systems ◽

Fontan Surgery ◽

Fontan Patients

: In patients having undergone the Fontan operation, besides the well discussed changes in the cardiac, pulmonary and gastrointestinal system, alterations of further organ systems including the hematologic, immunologic, endocrinological and metabolic are reported. As a medical adjunct to Fontan surgery, the systematic study of the central role of the liver as a metabolizing and synthesizing organ should allow for a better understanding of the pathomechanism underlying the typical problems in Fontan patients, and in this context, the profiling of endocrinological and metabolic patterns might offer a tool for the optimization of Fontan follow-up, targeted monitoring and specific adjunct treatment.

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Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome

Journal of the Formosan Medical Association ◽

10.1016/j.jfma.2021.01.014 ◽

2021 ◽

Author(s):

Hsin-Chia Lin ◽

Mei-Hwan Wu ◽

Jou-Kou Wang ◽

Ming-Tai Lin ◽

Chun-An Chen ◽

...

Keyword(s):

Fontan Operation ◽

Perioperative Outcomes ◽

Heterotaxy Syndrome

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Ventricular function and dyssynchrony in children with a functional single right ventricle using real time three‐dimensional echocardiography after fontan operation

Echocardiography ◽

10.1111/echo.15148 ◽

2021 ◽

Author(s):

Shu‐wen Zhong ◽

Yu‐Qi Zhang ◽

Li‐jun Chen ◽

Zhi‐Fang Zhang ◽

Lan‐Ping Wu ◽

...

Keyword(s):

Right Ventricle ◽

Real Time ◽

Ventricular Function ◽

Fontan Operation ◽

Three Dimensional ◽

Dimensional Echocardiography ◽

Three Dimensional Echocardiography

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Forward Flow Through the Pulmonary Valve After Bidirectional Cavopulmonary Shunt Benefits Patients at Fontan Operation

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2015.05.041 ◽

2015 ◽

Vol 100 (4) ◽

pp. 1390-1397 ◽

Cited By ~ 3

Author(s):

Koichi Sughimoto ◽

Diana Zannino ◽

Jacob Mathew ◽

Robert G. Weintraub ◽

Christian P. Brizard ◽

...

Keyword(s):

Pulmonary Valve ◽

Fontan Operation ◽

Forward Flow ◽

Bidirectional Cavopulmonary Shunt ◽

Flow Through ◽

Cavopulmonary Shunt

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Prognostic Value of Liver and Spleen Stiffness in Patients with Fontan Associated Liver Disease (FALD): A Case Series with Histopathologic Comparison

Journal of Cardiovascular Development and Disease ◽

10.3390/jcdd8030030 ◽

2021 ◽

Vol 8 (3) ◽

pp. 30

Author(s):

Massimo Padalino ◽

Liliana Chemello ◽

Luisa Cavalletto ◽

Annalisa Angelini ◽

Marny Fedrigo

Keyword(s):

Portal Hypertension ◽

Liver Disease ◽

Liver Fibrosis ◽

Prognostic Value ◽

Fontan Operation ◽

Vena Cava ◽

Case Series ◽

Morbidity And Mortality ◽

Post Mortem ◽

Spleen Stiffness

The Fontan operation is the current surgical procedure to treat single-ventricle congenital heart disease, by splitting the systemic and pulmonary circulations and thus permitting lifespan to adulthood for the majority of newborns. However, emerging data are showing that Fontan-associated liver disease (FALD) is an increasing related cause of morbidity and mortality in patients with the Fontan circuit. We described the clinical, laboratory, and transient elastography (TE) findings in a case series of adults with the Fontan circuit, and also correlated data with post-mortem histological features, aimed to define the prognostic value of TE in the staging of FALD. All patients presented signs of a long-standing Fontan failure, characterized by reoperation need, systemic ventricle dysfunction, and FALD stigmata (liver and spleen enlargement, portal vein and inferior vena cava dilation, and abnormal liver function tests). Liver and spleen stiffness (LS and SS) values were indicative of significant liver fibrosis/cirrhosis and the presence of suggestive portal hypertension (LS mean 35.9; range 27.3–44.7 kPa; SS mean 42.1, range 32.2–54.5 kPa). Post-mortem evaluations confirmed a gross hepatic architecture distortion in all cases. All patients died from severe complications related to liver dysfunction and bleeding. TE correlated well with pathological findings and FALD severity. We propose this validated and harmless technique to monitor liver fibrosis extension and portal hypertension over time in Fontan patients, and to identify the optimal timing for surgical reoperations or orthotopic-heart transplantation (OHT), avoiding a higher risk of morbidity and mortality in cases with severe FALD.

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Myocardial Deformation in Fontan Patients Assessed by Cardiac Magnetic Resonance Feature Tracking: Correlation with Function, Clinical Course, and Biomarkers

Pediatric Cardiology ◽

10.1007/s00246-021-02650-w ◽

2021 ◽

Author(s):

Alessia Callegari ◽

Simona Marcora ◽

Barbara Burkhardt ◽

Michael Voutat ◽

Christian Johannes Kellenberger ◽

...

Keyword(s):

Ventricular Function ◽

Feature Tracking ◽

Heart Diseases ◽

Fontan Operation ◽

Myocardial Deformation ◽

Time Interval ◽

Ventricular Volumes ◽

Atrioventricular Valve Regurgitation ◽

Cine Mr ◽

Fontan Patients

AbstractCardiac MR (CMR) is a standard modality for assessing ventricular function of single ventricles. CMR feature-tracking (CMR-FT) is a novel application enabling strain measurement on cine MR images and is used in patients with congenital heart diseases. We sought to assess the feasibility of CMR-FT in Fontan patients and analyze the correlation between CMR-FT strain values and conventional CMR volumetric parameters, clinical findings, and biomarkers. Global circumferential (GCS) and longitudinal (GLS) strain were retrospectively measured by CMR-FT on Steady-State Free Precession cine images. Data regarding post-operative course at Fontan operation, and medication, exercise capacity, invasive hemodynamics, and blood biomarkers at a time interval ± 6 months from CMR were collected. Forty-seven patients underwent CMR 11 ± 6 years after the Fontan operation; age at CMR was 15 ± 7 years. End-diastolic volume (EDV) of the SV was 93 ± 37 ml/m2, end-systolic volume (ESV) was 46 ± 23 ml/m2, and ejection fraction (EF) was 51 ± 11%. Twenty (42%) patients had a single right ventricle (SRV). In single left ventricle (SLV), GCS was higher (p < 0.001), but GLS was lower (p = 0.04) than in SRV. GCS correlated positively with EDV (p = 0.005), ESV (p < 0.001), and EF (p ≤ 0.0001). GLS correlated positively with EF (p = 0.002), but not with ventricular volumes. Impaired GCS correlated with decreased ventricular function (p = 0.03) and atrioventricular valve regurgitation (p = 0.04) at echocardiography, direct atriopulmonary connection (p = 0.02), post-operative complications (p = 0.05), and presence of a rudimentary ventricle (p = 0.01). A reduced GCS was associated with increased NT-pro-BNP (p = 0.05). Myocardial deformation can be measured by CMR-FT in Fontan patients. SLVs have higher GCS, but lower GLS than SRVs. GCS correlates with ventricular volumes and EF, whereas GLS correlates with EF only. Myocardial deformation shows a relationship with several clinical parameters and NT-pro-BNP.

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