Hepatic Coma Secondary to Fontan Conduit Thrombosis.

Introduction: The Fontan procedure is used to treat congenital heart defects and has improved long-term survival. Long-term complications include liver disease due to congestive hepatopathy. Fontan-related cirrhosis can manifest with ascites, gastrointestinal bleeding and encephalopathy. Case description: A 43-year-old man with history of Fontan surgery was admitted with COVID-19 pneumonia. There was rapid clinical and neurological deterioration, with coma and shock. CT imaging showed thrombosis of the Fontan conduit. The patient was successfully subjected to recanalization of the Fontan circulation, with progressive improvement of coma. Discussion: Fontan-associated liver disease is a major complication following the Fontan procedure. Clinicians must be aware of this pathology during patient follow-up. LEARNING POINTS: The Fontan procedure has improved the long-term survival of patients with single ventricle pathology.Fontan-associated liver disease is a result of haemodynamic changes associated with Fontan circulation; complications include hepatic encephalopathy and coma.Patients with Fontan circulation should be evaluated over time in order prevent and treat systemic complications.

Acute Massive Thrombus Following Fontan Procedure

Background Patients with hypoplastic left heart syndrome (HLHS) undergo a Fontan procedure as part of single ventricle surgical palliation. Post-Fontan, sluggish blood flow and an imbalance in coagulant factor proteins may predispose to thrombus formation. Other risk factors may include chylothorax as well as acute and chronic inflammation. Currently, there is no standardized surveillance strategy to detect thrombus in Fontan patients. Case A 34-month old male with HLHS underwent an extracardiac non-fenestrated Fontan complicated by chylothorax treated with 5 days of IV steroids and diuretics. He was on therapeutic aspirin. After progressive worsening of right pleural effusion, a chest tube was placed three weeks post-Fontan with continued chylous output. Stool alpha 1 antitrypsin was negative. Decision-making Given persistent chylothorax, a repeat echocardiogram was performed revealing a large mass in the Fontan circuit less than one month post-op. Cardiac CT showed occlusive thrombus filling the entirety of the Fontan conduit extending into hepatic veins and bilateral pulmonary arteries. He underwent extensive surgical thrombectomy and Fontan conduit revision. Hypercoagulable work-up revealed elevated factor 8 and von Willebrand factor activity which persisted more than one month post-op. Patient's history was also significant for COVID-19 infection 6 months prior. He was initially anticoagulated with bivalirudin with tirofiban initiated for antiplatelet therapy. He was ultimately transitioned to rivaroxaban, pentoxifylline and aspirin with chylothorax resolution over one month without thrombus recurrence. Conclusion Development of risk stratification tools to identify patients at higher risk for thrombi formation post-Fontan may facilitate patient selection for more aggressive anticoagulation. Consideration of elevated factor 8 as well as persistent or recurrent chylothorax may be beneficial, as increased thrombosis risk has been reported for both conditions in Fontan patients.Copyright © 2023 American College of Cardiology Foundation

Implementation of Virtual Fontan Heart Camps During a Pandemic.

BACKGROUND: Children with a Fontan operation represent a unique form of congenital heart disease (CHD) that requires multiple cardiac surgeries and procedures with an uncertain long-term outcome. Given the rarity of the types of CHD that require this procedure, many children with a Fontan do not know any others like them. METHODS: With the cancelation of medically supervised heart camps due to the COVID-19 pandemic, we have organized several physician-led virtual day camps for children with a Fontan operation to connect with others in their province and across Canada. The aim of this study was to describe the implementation and evaluation of these camps via the use of an anonymous online survey immediately after the event and reminders on days 2 and 4 postevent. RESULTS: Fifty-one children have participated in at least 1 of our camps. Registration data showed that 70% of participants did not know anyone else with a Fontan. Postcamp evaluations showed that 86% to 94% learned something new about their heart and 95% to 100% felt more connected to other children like them. CONCLUSION: We have demonstrated the implementation of a virtual heart camp to expand the support network for children with a Fontan. These experiences may help to promote healthy psychosocial adjustments through inclusion and relatedness.

The impact of the COVID-19 pandemic on individuals with Fontan circulation: Focus on gaps in care.

Background: Gaps in subspecialty cardiology care could potentially delay identification and care for multi-organ complications common in patients with Fontan circulation. This study analyzed the frequency of gaps in care for individuals with Fontan circulation during the COVID-19 pandemic and associated demographic and clinical factors. Methods: This retrospective study evaluated individuals with Fontan circulation followed at our center since 2010. A gap in care was defined as an absence of any formal cardiology provider-patient contact (clinic visit or telehealth) for >15 months. Results: Over a third of 308 patients with Fontan circulation experienced at least one gap in care between 2010 and 2022, and 77 experienced a gap in care during the COVID-19 pandemic. Of this latter group, 27 (35%) had never experienced a prior gap in cardiology care until the pandemic. Those who experienced gaps in care during the pandemic were on average older (18.0 [IQR 9.6-25.6] vs. 14.2 [7.2-21.2] years, p = 0.01), more likely to be of Black/African American race (23.4% vs 7.4%, p = 0.001), and less likely to have a diagnosis of protein-losing enteropathy or plastic bronchitis (0% vs. 8.6%, p = 0.005). Those without a gap in care during the pandemic were more likely to have utilized telehealth visits (13% vs 3%, p = 0.02). Conclusion: Gaps in care are common and appear to have been exacerbated by the COVID-19 pandemic in those with a Fontan circulation. Such gaps are particularly common among African American and adult patients, and may potentially be mitigated by expanding telehealth access.

Congenital cardiac liver cirrhosis with combined hepatocellular-cholangiocarcinoma-a case report.

Background: Cardiac liver cirrhosis secondary to Fontan procedure has been associated with hepatocellular carcinoma at a younger age. However, Fontan associated liver disease and combined hepatocellular-cholangiocarcinoma has not been previously reported. Combined hepatocellular-cholangiocarcinoma is a rare cancer that accounts for 2-5% of primary liver tumors and poses significant diagnostic and treatment challenges. This case highlights these needs and potential screening and treatment considerations. Herein we describe a case of combined hepatocellular-cholangiocarcinoma in a patient with autism, congenital heart disease, and Fontan procedure. Case Description: The patient is a 27-year-old male who presented with a liver mass detected on MRI performed in the context of a rising alpha-fetoprotein during a screening visit. Biopsy of the mass revealed a combined hepatocellular-cholangiocarcinoma which was staged as localized. Due to the COVID-19 pandemic and subsequent halt of all elective surgeries, the patient received local therapy with chemoembolization followed by pembrolizumab. The disease progressed though, and therapy was changed to gemcitabine plus cisplatin. Patient received 2 cycles of therapy, after which he and his family decided to transfer medical care to Memorial Sloan Kettering. Next generation sequencing of the tumor revealed TP53 and FGFR2 mutations. By then patient was also found to have lung metastasis. To help address the hepatocellular carcinoma, lenvatinib was added. Patient had sustainable disease control for about a year, yet eventually developed thrombocytopenia complicated by an episode of gastrointestinal bleeding. With a worsening performance status, adverse events of the treatment, and recurrent hospitalizations, a goals of care discussion with his family led to the discontinuation of active cancer therapy and patient was started on best supportive care. Patient remained in active follow-up until the time of this report and passed away less than a year from initiating best supportive care alone. Conclusions: This challenging case raises awareness towards screening and monitoring all patients with Fontan procedure for Fontan associated liver disease and liver cancers, including combined hepatocellular-cholangiocarcinoma. To the best of our knowledge, this is the first description of combined hepatocellular-cholangiocarcinoma occurring in the context of cardiac cirrhosis. The management difficulties that led to altering the goals of care, is another reminder of the dynamic nature of the care oncologists would provide.

SARS-Cov-2 infection in patients with univentricular heart after fontan operation

Background and Aim: COVID-19 pandemic caused by SARS-Cov-2 coronavirus affects all groups of patients. Although pediatric pop-ulation seems to be less affected with milder or asymptomatic course of SARS-CoV-2 infection, there are few groups of patients with potential high risk of severe or fatal course of coronavirus dis-ease. These include children with congenital heart defects. The aim of this study was to evaluate the course of SARS-Cov-2 infection in patients with univentricular heart after Fontan operation. Method(s): From September 2020 to May 2021 (before vccination started in pediatric population in Poland) we screen all 38 Fontan patients admitted to Cardiology Department, Polish Mother's Memorial Hospital Research Institute for SARS-Cov2 antibodies. Result(s): We found positive SARS-Cov-2 antibodies in 21 unvac-cinated Fontan patients (55% of all hospitalized Fontan patients), 15 boys (71%) and 6 girls in the age 3-22 years (mean 11 years). 14 patients (67%) had hypoplastic left heart syndrome. Course of SARS-CoV-2 infection: asymptomatic course in 11(52%) patients, fever in 7 (33%) patients, cough 4 (19%) patients, diar-rhoea in 2 patients, loss of smell and taste-1 patient. One, 18 years old patient suffered from Covid fog symptoms (impairment of sus-tained attention and memory problems), he hasn't notice any SARS-Cov-2 symptoms but the level of antiobodies was high. Only 3 patients were hospitalized in acute SARS Cov2 infection: 2 due beacause of need for intravenous rehydratation during severe diarrhoea, 1 because of JET (junctional ectopic tachycardia) during fever. There was no case of PIMS (pediatric inflammatory multi-system syndrome) in study group. Medications used in study group: aspirin in 19 (90 %), warfarin in 2, spironolactone in 18 (86%), sildenafil in 9 (43%), angiotensyn-converting enzyme inhibitors in 17 (81%), beta-blockers in 4 (19%) of patients. Conclusion(s): 1. In our study severe congenital heart defect such as univentricular heart was not a risk factor of severe course of SARS-Cov-2 infection. 2. Absence of PIMS in analized group of patients may be connected with changed immunologic response in Fontan patients and chronic use of ASA (acetylsalicylic acid). 3. The impact of SARS CoV 2 infection on patients with congenital heart defects needs further studies.

Physical activity of single ventricle patients during the covid-19 pandemic

Background and Aim: It is well known that Fontan patients tend to a sedentary lifestyle with low physical activity levels and their objec-tive exercise capacity is reduced. Currently the COVID-19 pandemic has a significant impact on the physical activity levels of the whole population. This study investigates physical activity lev-els of Fontan patients during the COVID-pandemic, and their relationship to exercise capacity, heart rates, cardiac function at cardiovascular magnetic resonance (CMR), and biomarkers. Method(s): CMR, exercise testing, 24h-ECG, and blood samples were prospectively performed in 38 Fontan patients, of which 15 were females (40%) and 18 (47%) had a single left ventricle (LV). Time interval from Fontan operation was in median (IQR) 10 (8-15) years. Physical activity was assessed by accelerom-eter during 7 consecutive days of regular school/work. Moderate intensity physical activity was defined as gt;2296 counts/minute and vigorous physical activity as gt;4012 counts/minute. Patients with moderate-to-vigorous physical activity (MVPA) below 60minutes per day were categorized as inactive as recom-mended by the WHO. Parameters of exercise capacity included maximal oxygen uptake, maximum work rate, and maximal heart rate. Result(s): Daily MVPA was in median (IQR) 40.2 (27.6-56.5) minutes and 7/18 (39%) patients reached the recommended 60minutes/day of MVPA. Daily minutes of MVPA did not cor-relate with gender, age, single left ventricle, years from Fontan sur-gery, mean heart rate, ventricular volumes, and ejection fraction at CMR, cardiac biomarkers, or exercise capacity. Conclusion(s): Only 39% of the Fontan patients meet the recommen-dation for daily MVPA. The MVPA during the pandemic in our patients' cohort is lower than the one reported in Fontan patients before the pandemic. These current changes in behavioral habits may increase the risk for habitual sedentary lifestyles in Fontan patients.

COVID-19 in a Child With Transposition of the Great Arteries S/P Fontan Palliation: A Case Report and Literature Review.

Background: Although adult patients with cardiovascular disease are at higher risk of adverse outcomes such as death or severe infection, limited data exist regarding pediatric patients with congenital heart disease. We would like to report our experience with COVID-19 in a pediatric patient with Fontan circulation. Furthermore, we present a review of patients with Fontan palliation and COVID-19 previously reported in the literature to summarize the clinical characteristics of this population. Case Presentation: A 9-year-old boy with dextro-transposition of the great arteries, ventricular septal defect, pulmonary stenosis, patent foramen ovale, and borderline left ventricle post bidirectional Glenn shunt and Fontan palliation presented with paroxysmal cough in the context of COVID-19. The coagulation profile was beyond the normal limits, and the patient began to receive anticoagulant aspirin. On the 5th day, the patient presented with fever, sore throat, and fatigue. The oxygen saturations dropped to 93%, and he received nasal catheter oxygen inhalation. On the 7th day, computed tomography of the chest revealed little emerging flaky exudation in the posterior basal segment of the left lower lobe. Nasal cannula was removed on the 12th day, and the coagulation profile returned to normal on the 16th day. After two consecutively negative SARS-CoV-2 viral RNA tests (on the 18th and 19th days, interval ≥ 24 h), he was discharged from the hospital on the 21st day. Literature review indicated that COVID-19 with Fontan palliation seemed to be more common in male adults. Disease presentation varied from mild upper respiratory tract infection to severe pneumonia. Complications were not uncommon in this population. The treatments varied depending on the specific factors. Fortunately, most patients reported a favorable prognosis. Conclusion: Although patients with COVID-19 and Fontan circulation might have the risk of adverse outcomes due to multiple mechanisms, most patients have a favorable prognosis.

Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation-Initial Results From a Prospective Study.

BACKGROUND: Patients with congenital heart disease (CHD)-including those after Fontan operation-are encouraged to be physically active. AIM: To prospectively determine the effects of an individually adapted, home-based cycle ergometer endurance training in combination with inspiratory muscle training (IMT) in pediatric and adult Fontan patients. We, herein, report the results of the initial 10-months follow-up (phase 1). METHODS: 18 patients (median age 16.5 years; range 10-43 years) completed baseline check-ups, and 4 and 10 months follow-up visits, which each included cardiopulmonary exercise testing (CPET), bodyplethysmography (including measurement of respiratory muscle strength), and a quality of life questionnaire (PedsQL™). The training program consisted of a home-based cycle ergometer endurance training on a "Magbike® AM-5i/3i" (DKN Technology®, Clermont-Ferrand, France) and IMT with a handheld "POWERbreathe® Medic plus" device. Patients performed 90 min of endurance training per week in addition to IMT (30 breaths per day, 6-7 times per week). After the first 4 months, patients underwent additional interval training. RESULTS: After 10 months of training, we observed significant increases in maximum relative workload (W/kg, p = 0.003) and in maximum inspiratory (MIP, p = 0.002) and expiratory (MEP, p = 0.008) pressures. Peak VO2 values did not increase significantly as compared to baseline (p = 0.12) in the entire cohort (n = 18), but reached statistical significance in a subgroup analysis of teenage/adult patients (n = 14; p = 0.03). Patients' subjective quality of life did not show any significant changes after 10 months of training. DISCUSSION: In Fontan patients, an individually adapted home-based training is safe and associated with improvements in some CPET variables. However, these improvements did not translate into an improved QoL after 10 months. With an unclear, but most likely negative, impact of the COVID-19 pandemic, improvements in QoL may become evident during further follow-up (phase 2 of the study).

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address the extracardiac anomalies in fetuses with congenital heart disease, post COVID-19 vaccination myocarditis, the use of cardiac magnetic resonance after the Fontan operation, congenitally corrected transposition in adults, robitically assisted congenital heart surgery and the increased risk of congenital heart surgery in patients receiving tracheal surgery during the same admission.

Fifty years of pediatric cardiology (1971-2021): from Fontan's surgery to the present day

Pediatric cardiology has matured profoundly over the last 50 years, paralleled by a similar development in pediatric cardiac surgery and cardiac anesthesia. This field of medicine provides structured and effective care for a very heterogeneous group of diseases including congenital heart disease, cardiomyopathies and heart failure, myocarditis, rheumatic heart disease, inherited and acquired arrhythmias, Kawasaki disease and more recently multisystemic inflammatory syndrome of children related to primary infection by SARS-CoV-2. This review summarizes achievements and results in selected topics of pediatric cardiology and cardiac surgery with focused attention to the diagnosis and management of congenital heart diseases.

Mechanical thrombectomy of COVID-19 DVT with congenital heart disease leading to phlegmasia cerulea dolens: a case report.

BACKGROUND: COVID-19 and Fontan physiology have each been associated with an elevated risk of venous thromboembolism (VTE), however little is known about the risks and potential consequences of having both. CASE PRESENTATION: A 51 year old male with tricuspid atresia status post Fontan and extracardiac Glenn shunt, atrial flutter, and sinus sick syndrome presented with phlegmasia cerulea dolens (PCD) of the left lower extremity in spite of supratherapeutic INR in the context of symptomatic COVID-10 pneumonia. He was treated with single session, catheter directed mechanical thrombectomy that was well-tolerated. CONCLUSIONS: This report of acute PCD despite therapeutic anticoagulation with a Vitamin K antagonist, managed with emergent mechanical thrombectomy, calls to attention the importance of altered flow dynamics in COVID positive patients with Fontan circulation that may compound these independent risk factors for developing deep venous thrombosis with the potential for even higher morbidity.

Fibrin airway cast obstruction: Experience, classification, and treatment guideline from Denver.

BACKGROUND AND OBJECTIVES: Plastic bronchitis (PB) is a condition characterized by the formation of thick airway casts leading to acute and often life-threatening airway obstruction. PB occurs mainly in pediatric patients with congenital heart disease (CHO) who have undergone staged surgical palliation (Glenn, Fontan), but can also occur after chemical inhalation, H1N1, severe COVID-19, sickle cell disease, severe asthma, and other diseases. Mortality risk from PB can be up to 40%-60%, and no treatment guideline exist. The objectives herein are to develop a standardized evaluation, classification, and treatment guideline for PB patients presenting with tracheobronchial casts, based on our experience with PB at the Children's Hospital of Colorado in Denver. METHODS: We describe 11 patients with CHO-associated PB (post-Fontan [n = 9], pre-Fontan [n = 2]) who presented with their initial episodes. We utilized histopathological analysis of tracheobronchial casts to guide treatment in these patients, utilizing our hospital-wide guideline document and classification system. RESULTS: We found that 100% of post-Fontan PB patients had fibrinous airway casts, while pre-Fontan PB casts were fibrinous only in one of two patients (50%). Utilizing histopathology as a guide to therapy, PB patients with fibrin airway casts were treated with airway-delivered fibrinolytics and anticoagulants, as well as aggressive airway clearance and other supportive care measures. These therapies resulted in successful cast resolution and improved survival in post-Fontan PB patients. CONCLUSION: We have shown an improved outcome in PB patients whose treatment plan was based on Denver's PB classification schema and standardized treatment guideline based on tracheobronchial cast histopathology.

Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease.

The aim of the current study is to investigate hospitalization outcomes of COVID-19 positive children and adults with moderate or severe congenital heart disease to children and adults without congenital heart disease. Retrospective review using the Vizient Clinical Data Base for admissions of patients with an ICD-10 code for COVID-19 from April 2020 to March 2021. Admissions with COVID-19 and with and without moderate or severe congenital heart disease (CHD) were stratified into pediatric (< 18 years) and adult (≥ 18 years) and hospital outcomes were compared. There were 9478 pediatric COVID-19 admissions, 160 (1.7%) with CHD, and 658,230 adult COVID-19 admissions, 389 (0.06%) with CHD. Pediatric admissions with COVID-19 and CHD were younger (1 vs 11 years), had longer length of stay (22 vs 6 days), higher complication rates (6.9 vs 1.1%), higher mortality rates (3.8, 0.8%), and higher costs ($54,619 vs 10,731; p < 0.001 for all). Adult admissions with COVID-19 and CHD were younger (53 vs 64 years, p < 0.001), had longer length of stay (12 vs 9 days, p < 0.001), higher complication rates (8 vs 4.8%, p = 0.003), and higher costs ($23,551 vs 13,311, p < 0.001). This appears to be the first study to report the increased hospital morbidities and costs for patients with CHD affected by COVID-19. Our hope is that these findings will help counsel patients moving forward during the pandemic.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) outcomes for COVID-19 infection in adults with congenital heart disease which showed no increased mortality compared to the general population, (2) hepatorenal dysfunction before transplantation in patients with Fontan is associated with increased mortality, (3) abnormal Von Willebrand factor metabolism and angiopoietin signaling may contribute to pulmonary AVM formation in children with a Glenn circulation, (4) low cardiac output after the Norwood procedure which improves with higher hemoglobin and with milrionone, (5) a comparison of staged versus complete repair in neonatal tetralogy of Fallot reveiling the pros and cons of each strategy, (6) the long-term outcomes of early repair of complete atrioventricular canal show no difference in outcomes in patients who were repaired below 3 months of life.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) The impact of COVID-19 in individuals with congenital heart disease through the life span. Patients with a genetic syndrome and adults at advanced physiological stage were at highest risk for moderate/severe infection. (2) Echocardiographic findings of the multisystem inflammatory syndrome in children showing a high prevalence of myocardial injury and systolic dysfunction that improves in the subacute phase. (3) A score assessment of the Fontan associated liver disease which correlated with the risk for Fontan failure. (4) Grown-up congenital heart surgery in 1093 consecutive cases showed that the 30 day mortality may underestimate the mortaility and that the 6 months mortality is likely a better measure in this population. (5) Cone versus conventional repair for Ebstein's anomaly showed better midterm results and freedom from tricuspid regurgitation after the cone operation. (6) Association between race/ethnicity, illness severity, and mortality in children undergoing cardiac surgery. The study showed that the African American race associated with increased disease severity and thus higher postoperative mortality compared to the caucausian race.

A case of COVID-19 in a patient with a univentricular heart post total cavopulmonary connection (Fontan) surgery.

Coronavirus disease 2019 (COVID-19) has caused a global pandemic which has affected patients and healthcare systems around the world. Patients with underlying health conditions seem to be more severely affected. There are limited reports of patients with univentricular circulations and COVID 19; thus, we report a case of COVID-19 in a patient with a univentricular circulation.