ABSTRACT
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
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.