A baby girl with TBX1 haploinsufficiency who died after elective cardiac repair surgery due to suspected enteroviral meningitis

Introduction: TBX1 haploinsufficiency is an inborn error of immunity with the phenotype of DiGeorge Syndrome. DiGeorge Syndrome has variable immunodeficiency associated with grade of thymic hypoplasia ranging from mild with no infections to severe requiring thymus implant. Enterovirus is an example of an opportunistic infection that can be fatal in these patients. Case Presentation: A 1 year old girl with TBX1 haploinsufficiency complicated by Tetralogy of Fallot, pulmonary atresia, high arched palate, and vesicovaginal fistula presented for elective cardiac repair surgery from another country due to failure to thrive and cyanosis. She had no prior infectious history but was on sulfamethoxazole-trimethoprim for prophylaxis. She was asymptomatic with a negative COVID test but no other infectious studies performed. Immediately postoperatively, she was febrile and nasal respiratory viral panel was positive for rhinovirus/enterovirus with increased procalcitonin and leukocytosis with left shift. She decompensated with multi-organ failure and cardiac arrest on postoperative day two. She was cannulated to veno-arterial extracorporeal membrane oxygenation (ECMO). Pre-operatively, she had a normal absolute lymphocyte count. No thymus tissue was observed in surgery. She had profound CD3 lymphopenia to 130 cells/cmm when critically ill. Enteroviral meningitis was suspected as no infectious, cardiac, or other pathology could be identified causing decompensation. Enteroviral serum polymerase chain reaction (PCR) test was negative while lumbar puncture deferred due to clinical status. She was treated with immunoglobulin. Offlabel investigational drug pocapavir was considered but deferred to patient's irreversible neurological status. The patient was disconnected from ECMO and expired. Discussion(s): Though we cannot confirm that this patient had enteroviral meningitis, invasive enteroviral infections are associated with elevated transaminases, coagulopathy, and seizures all present in our patient. There has also been reported negative serum enteroviral PCR but positive CSF enteroviral PCR in an immunodeficient patient. Additionally, this case highlights the importance of immunologic evaluation in patients with DiGeorge Syndrome and questions if asymptomatic viral screening for viruses like enterovirus should be considered pre-operatively in patients with inborn errors of immunity. This case highlights potential treatment options for invasive enteroviral infections in patients with inborn errors of immunity: high dose immunoglobulin, fluoxetine, and pocapavir.Copyright © 2023 Elsevier Inc.

Impact of coronavirus disease 2019 on adult patients with congenital heart disease

Background: COVID-19 is responsible for a worldwide pandemic, causing more than 18,000 deaths to date in Portugal. Data already exists regarding the increased risk of adverse events in patients with cardiovascular diseases, however the impact of SARS-CoV-2 infection in patients (P) with congenital heart disease (CHD) is still under investigation. Purpose(s): To study the impact of COVID-19 in a adult patients with CHD Methods: Adult patients seen at the CHD outpatient's clinic at a tertiary centre, who became infected with SARS-CoV-2 infection up to December 2021 were included. Assessment of patients' symptoms, need for hospitalization and admission in an intensive care unit was assessed based on medical records. Result(s): We identified seventy-nine patients (pts) with COVID-19 infection. Symptoms were present in 67 (84%). The median age was 44 (15) years, 52% were females. Eight P (10%) had complex cyanotic disease;seven Tetralogy of Fallot;five (6%) transposition of great arteries;eight (10%) right ventricle obstacle;two (3%) atrioventricular canal defect;sixteen (20%) atrial septal defect;nine (11%) ventricular septal defect;eight (10%) aortic coarctation;two (3%) had Eisenmenger syndrome. 49% of P had previous surgery or percutaneous procedure. 63% of P were at New York Heart Association (NYHA) class of I and 30% at NYHA II. Mild symptoms were reported by 56 P (71%). Ten adults (7,9%) experienced moderate symptoms (dyspnea and hypoxia) that led to hospitalization for oxygen therapy, none required mechanical ventilation. One death was reported in an 83-year-old patient with non-corrected interventricular communication and compromised biventricular function. There was a significant association between the gravity of CHD and hospitalizations (p=0.02). Conclusion(s): Our pts had mainly mild to moderate symptoms and did not appear to have a disproportionately negative outcome;the need for hospitalization was more frequent in patients with higher CHD gravity. These findings are in line with the emerging data regarding COVID-19 in CHD P, and may be in part explained by the patient's young age and functional status.

Hidden in plain sight: A case of arrhythmia and sudden cardiac failure masked by COVID-19 positivity

Background and Aim: Whilst most commonly recognised as a res-piratory pathogen, COVID-19 can also result in a variety of extrapulmonary manifestations including myocardial dysfunction and arrhythmia. We report a case of a 15 year old girl with repaired atrioventricular septal defect, presenting with arrhythmia and sud-den severe cardiac failure masked by COVID-19 positivity. Method(s): A 15 year old girl with repaired atrioventricular septal defect and tetralogy of Fallot, under regular follow up with asymp-tomatic moderate to severe atrioventricular valve dysfunction, pre-sented with one month's history of progressive breathlessness to her local hospital. Onset of illness coincided with typical COVID-19 symptoms;her family, attributing her deterioration to this, delayed seeking medical help. She was rapidly referred to our unit. The heart failure severity, which included diffuse dependent oedema, large pleural effusion and severe biventricular dysfunc-tion, could not be attributed to major valve function change. Moreover, deterioration had occurred rapidly without apparent ongoing cause, after extensive diagnostics. Tachyarrhythmia was suspected;initial adenosine challenge via peripheral vein was inconclusive. Diuretics, inotrope and empirical administration of amiodarone provided limited response;the patient was therefore ventilated, enabling further adenosine challenge via central line, which revealed atrial flutter. Shortly after conversion to sinus rhythm and pleural drainage, her biventricular function improved to near normal. Result(s): Redo surgery was undertaken. Firstly, this included mechanical valve replacement of left and right atrioventricular valves and ablation of the isthmus. Secondly, a permanent pace-maker was inserted a few days later. The patient made a quick and uneventful recovery and was discharged on day 10 with good biventricular function on minimal medical therapy and no symptoms. Conclusion(s): Our case highlights delayed presentation as a hidden effect of the COVID-19 pandemic, and that sudden deterioration in stable children with repaired congenital heart disease should prompt the clinician to exclude all reversible causes of de-stabili-sation, and in particular to maintain high suspicion of arrhythmia.

Initiating a pediatric cardiac surgery program in europe during the COVID-19 pandemic: Methods, challenges and outcomes

Background and Aim: Before 2020, no pediatric cardiac surgery pro-gram was available at our institution, despite being a university hospital providing tertiary care for 6 million inhabitants. Our goal is to describe the preparation and the first year of expe-rience of our pediatric cardiac surgery program, which will even-tually cater for 300 patients annually. Method(s): The project was supported by European funds (INTERREG program). Medical and nursing staff training was performed via a transborder collaboration. Significant investments were necessary to reach the required standards for the facilities (operating rooms, pediatric intensive care beds) and equipment (cardiopulmonary bypass and ECMO machines, ultrasound sys-tems etc.). A multidisciplinary team was built over 3 years. The pediatric ECMO program was started a year prior to the surgical program. In parallel, a program dedicated to the study and care of neurological impact of congenital heart diseases and interventions was set up. Importantly, a progressive upscale was devised: only children with a weight gt;5 kg requiring non-complex surgeries were operated on during the first year. Result(s): The first year of experience was marked by challenges caused by the successive COVID-19 waves, such as restricted access to the operating room and a subsequent slow-down in the progression of the schedule. Fifty-nine patients constituted the cohort of the first year (October 2020-October 2021). In addition to low-risk procedures (left-to-right shunts closures etc.), cases included 6 tetralogy of Fallot repairs, 1 Ross procedure and 2 bilateral cavopulmonary connections. There were no early or late deaths. Median age was 6.3 years old (1.8-9.8) and median weight was 18.5 kg (10.0-32.0). Fourteen patients (23.7%) were operated on with a weight lt;10 kg. Bypass cases represented 72.9% (43 patients) of all cases. Median cardiopulmonary bypass and cross-clamping times were 88 (52-153) and 51 (26-98) minutes respectively. Median intensive care and hospital stays were 3 (2.0-6.7) and 6 days (5-11) respectively. Conclusion(s): Despite COVID-19-related difficulties, our pediatric cardiac surgery program achieved excellent outcomes in selected patients. Institutional support, meticulous planning, team cohesion and perseverance are keys for successful initiation of a program requiring such high technicality.

Impact of coronavirus disease 2019 on functional capacity in paediatric congenital heart disease assessed by cardiopulmonary exercise test

Background and Aim: Since December 2019 the novel coronavirus disease 2019 (COVID-19) has been burdening all health systems worldwide. However, cardiopulmonary repercussions in paediat-ric patients with congenital heart disease (CHD) are unknown. The aim of this study is to compare changes in cardiopulmonary exercise test (CPET) in this patients before and after COVID-19. Method(s): Prospective observational study was lead comparing CPET results after COVID-19 in paediatric patients with stable CHD who had a previous routine CPET. All underwent for stand-ardised CPET, using Godfrey ramp protocol as recommended by the European Society of Cardiology (ESC). Measured variables, expressed by predicted values, were: forced vital capacity (FVC), forced expiratory volume (FEV1), ratio of minute venti-lation to carbon dioxide production (VE/VO2 slope), peak oxy-gen consumption (peak VO2), oxygen uptake efficiency slope (OUES), oxygen pulse (O2 pulse) and peak heart rate (pHR). Wilcoxon test was used to compare continuous variables for related samples. Result(s): Ten patients (6 boys, 60%;mean age 11,4 +/- 2,4 years) with hemodynamically stable CHD (3 Tetralogy of Fallot, 30%;2 trans-position of the great arteries, 20%;2 dilated cardiomyopathy, 20%;2 Kawasaki disease, 20%;1 cardiac tumor, 10%) were selected to repeat a post-COVID CPET. All of them had mild COVID and could follow ambulatory treatment. Comparing before/post COVID tests, there were no significantly changes in predicted res-piratory parameters: FVC (90,6 +/- 7,4 vs 98,1 +/- 23,9%;p = 0,799), FEV1 (89,5 +/- 13,8 vs 94,5 +/- 8,8%;p = 0,475), VE/CO2 slope (31,6 +/- 3,7 vs 30,6 +/- 3,9degree, p = 0,203). In the same way, no significantly changes were seen in cardiovascular predicted parameters: oxygen pulse (97,3 +/- 19,2 vs 98,5 +/- 17,4%, p = 0,798), peak VO2 (82,4 +/- 19,4 vs 76,8 +/- 13,7;p = 0,123) and OUES (1,79 +/- 0,4 vs 2,01 +/- 0,6;p = 0,066). Respect peak VO2, there was a non-significant slightly decrease in post-COVID test (82,4 +/- 19,4 vs 76,8 +/- 13,7;p = 0,123). Conclusion(s): In our series, post-COVID CPET results showed that paediatric patients with hemodynamically stable CHD had no impairment in their functional capacity in relation to Sars-CoV-2 disease. Contrary to adults with previous cardiovascular disease, children should have mild infections without sequelae in cardio-pulmonary function.

Detrimental Intrauterine Stress Exposure and Post-natal Outcomes in Prenatally Diagnosed Congenital Heart Disease

Background: Prenatal diagnosis of congenital heart disease (CHD) is a stressful event that leads to anxiety, depression and traumatic stress in expectant parents. Cortisol is elevated in times of stress, and when present in mothers, crosses the placenta and leads to suppression of the fetus' own production of cortisol. When those neonates go on to require stressful procedures in the first months of life, some are unable to effectively mount a cortisol mediated stress response which can lead to poor outcomes and even death. We sought to investigate the relationship between maternal stress during pregnancy, and neonatal outcomes. Methods: We conducted a retrospective chart review of pregnancies complicated by a fetal diagnosis of critical CHD (including transposition of the great arteries, tetralogy of Fallot, total anomalous pulmonary venous return, and coarctation) who were born between 5/1/2019 and 5/1/2021. Maternal data included demographics and medical comorbidities. Composite maternal prenatal stress score (PSS) was calculated based upon 1) prenatal mental health diagnoses, 2) housing/food insecurity 3) income insecurity, 4) social support/child care, 5) legal involvement, 6) transportation issues and 7) other stressors. Categories ranged from 0 (no concerns) to 3 (significant concerns). Infant charts were reviewed for postnatal, and post-operative outcomes including infection, inotropic support, and exogenous steroid treatment. Results: 41 maternal-fetal dyads met inclusion criteria. Demographic and catheter based intervention at a median of 8 (2-54) days of life. 13 patients had single ventricle anatomy (8 initial surgical palliation, 5 catheterization). Mothers with higher composite PSS were more likely to have infants that required steroids after CHD surgery compared to mothers with lower scores (p=.01) (figure 1). Surgical patients needing bypass were more likely to require post-operative steroids than those not requiring bypass (18/22 vs 0/4, p<.005). None of the catheter-based interventions (including those with high risk single ventricle anatomy) required steroids (p <.0001). Maternal individual stress sub-categories, severity of prenatal CHD diagnosis, and counseling during the COVID-19 era did not correlate with steroid treatment. Finally, PSS did not correlate with individual outcomes such as birthweight, inotropic support, infection or hypoglycemia. Conclusion: Maternal prenatal stress is multifactorial and higher composite maternal prenatal stress scores are correlated with post-bypass steroid requirements, suggesting that a stressful intrauterine environment can be associated with worse postoperative outcomes for the neonate.

Inter-Rater Reliability and Agreement of Fetal Echocardiography Measurements Among Investigators From Multiple Sites

Background: Single institutions specialising in fetal cardiology often have too few cases to develop robust prognostic indicators for specific conditions. The Australia and New Zealand (ANZ) Fetal Cardiology Working Group instigated a multicentre study to examine fetal risk factors for early postnatal intervention in Tetralogy of Fallot (ToF). Centralised data analysis was not possible due to COVID-19 travel restrictions and ethical constraints related to sharing of retrospectively acquired images. A study of inter-observer agreement of standardised in utero cardiac measurements was undertaken to assess the feasibility of combining data from multiple centres. Methods: Ten fetuses with ToF were randomly identified. Deidentified images were distributed securely to ten ANZ fetal cardiologists. The pulmonary valve (PV) annulus, main pulmonary artery (MPA), branch pulmonary arteries (BPA), aortic valve (AV) annulus, ascending aorta (AA), and ductus arteriosus (DA) were measured in triplicate following a defined protocol. Inter-rater reliability was assessed using a two-way random effects model to calculate the intra-class coefficient (ICC). Results: Measurements were available for seven ANZ fetal cardiologists. There was moderate inter-observer agreement for PV (ICC 0.74, 95% CI 0.49–0.91) and AV (0.71, 95% CI 0.48–0.90), and good agreement for MPA (0.81, 95% CI 0.62–0.94) and AA (0.91, 95% CI 0.80–0.97). Inadequate data were available for BPA and DA analysis. Multi-variate analysis found no patient or investigator factors that influenced measurement variability. Conclusion: Fetal cardiac structures can be measured using a defined measurement protocol by multiple investigators with at least moderate agreement. Analysis of large datasets by multiple investigators is a reasonable alternative to centralised data analysis.

TREATMENT SUCCESS IN A RESISTANT CASE OF INSOMNIA WITH AN IRREGULAR CIRCADIAN RHYTHM DISORDER

Introduction: A 19-year-old non-verbal male with history of CHARGE syndrome, severe autism, intellectual disability, coloboma with blindness OD and severely imparied vision OS, deafness, self-injurious and aggressive behavior, Tetralogy of Fallot status post repair, pulmonary valve replacement, hypertension, hypothyroidism, megacolon, gastrostomy tube dependence, eosinophilic esophagitis and chronic kidney disease with an irregular sleep cycle who has failed multiple medications for insomnia has shown treatment success with suvorexant. Report of Cases: This patient's sleep schedule ranges from 1.5 to 5 hour segments at various times of day or night including naps at school with occasional longer periods of sleep up to 10 hours and longer periods of wakefulness up to 22 hours who has been treated with the following medications: trazodone, clonidine, hydroxyzine, diphenhydramine, quetiapine, gabapentin, mirtazapine, eszopiclone, melatonin and ramelteon. His behavioral problems have been treated with olanzapine. He continued to be aggressive and difficult to direct. His parents reported exhaustion. Then, suvorexant 5mg was added at bedtime while the following sleep medications were continued: gabapentin total daily dose of 1500mg (300mg in morning and 3pm;900mg at bedtime, 300mg one hour later if still awake), ramelteon 8mg, mirtazapine 7.5mg and olanzapine 10mg at bedtime and bid prn aggressive behavior. He also takes the following daily medications: bisacodyl, polyethylene glycol, simethicone, hyoscyamine, cholecalciferol, aspirin, levothyroxine, hypoallergenic nutritional formula, starch and albuterol prn. With the addition of suvorexant 5mg, he had been able to get 9.5 hours of consolidated sleep at night with improvement in his behavior until he contracted Covid-19 and regressed. The suvorexant dose was increased to 10mg which again improved his insomnia and behavior. Conclusion: Various medications have either not worked at all or have worked suboptimally for insomnia in this medically complex patient who has an irregular Circadian rhythm disorder. Adding an orexin receptor antagonist as a novel mechanism to his regimen has shown promise. At this time, this patient has been stable for one month with suvorexant 10mg at bedtime after regression on the 5mg dose that coincided with a Covid-19 infection. We are proceeding with cautious optimism.

Investigating the clinical characteristics of paediatric COVID-19 in Cape Town, South Africa: Initial results from the University of Cape Town (UCT), Department of Paediatrics and Child Health, COVID-19 paediatric repository

Introduction: Studies show that children account for only 1-5% of diagnosed COVID-19 cases, they have milder disease than adults and deaths are extremely rare. The complete clinical picture of pediatric COVID-19 has not yet been fully reported or defined. Additionally, the South African pediatric population has unique clinical characteristics and risk implications and needs investigating. We aimed to characterize COVID-19 in Cape Town children. Methods: The UCT COVID-19 pediatric repository is a prospective cohort recruited via convenience sampling at 3 Western Cape Hospitals. All patients ≤ 18 years who test COVID-19 positive are eligible for inclusion in the study. Results: To date 227 participants, 56%(125/227) male with median age 2 years (IQR:0-6), have been enrolled. Only 28(12%) participants were in contact with a confirmed COVID-19 positive case, 67% of these, were first degree relatives, 28% second degree relatives and 6% health care workers. Comorbidities were present in 125(56%) participants. Of 32 recorded comorbidities, congenital heart disease (CHD), found in 7% of participants, ranked third. CHD subtypes included PDA (4), Tetralogy of Fallot (3), AVSD (2), Pulmonary atresia with VSD (2), truncus arteriosus (1), Coarctation of the Aorta (1), Congenital aortic valve stenosis (1), and ASD (1). Other cardiac comorbidities were, cardiomyopathy (2), primary pulmonary hypertension (1) and rhabdomyoma (1). On presentation 173 (76%) were symptomatic. Predominant symptoms included cough 40%, history of fever 36%, documented fever 34%, difficulty breathing 28%, and nausea or vomiting 20%. On examination, 65% had abnormal heart rates, 47% abnormal respiratory rates, 35% were in respiratory distress and 24% were hypoxic. Of the 227 patients, 169(74%) were admitted to hospital and 33 (15%) were admitted to ICU. In the ICU 79% of patients required non-invasive and 24% invasive ventilation, median length of ICU admission was 3 days (IQR:2-7.5). During admission 38(17%) patients developed COVID-19 complications: secondary infection 10%, sepsis 4%, MIS-C 2%, and myocarditis or new onset heart failure 1%) and 2(0.9%) died, including one patient with AVSD, who presented with severe pulmonary hypertension and acute heart failure post cardiac surgery. Conclusion: We present the initial findings of the UCT pediatric COVID-19 registry. We anticipate that these data will help to complete the clinical picture of COVID-19 in the South African pediatric population.

The Experience of the Cooperation in Science and Technology European Network for Innovative Diagnosis and Treatment of Chronic Neutropenias (COST EuNet-INNOCHRON) Action and the Sweden Experience in the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) Era

[Formula presented] Background-Aim: Infection from SARS-CoV-2 has emerged as new pathological entity within the global medical community. One of the earliest questions was in relation to the ability of the immunocompromised patients to clear the infection. In COST EuNet-INNOCHRON we were interested in the impact of SARS-CoV-2 infection in patients with different types of chronic neutropenia (CNP). The aim of the current study is to understand the impact of SARS-CoV-2 infection and to identify any possible characteristic patterns of the clinical course in patients with CNP. Patients and Methods: The COST EuNet-INNOCHRON Action in collaboration with the European Haematology Association - Scientific Working Group (EHA-SWG) on Granulocytes and Constitutional Marrow Failure Syndromes has conducted an online survey on SARS-CoV-2 infection in patients with CNP. The EuNet-INNOCHRON participants from different countries got access to an on-line platform fulfilling the General Data Protection Regulation (GDPR) and could register adult and paediatric CNP patients who had been infected by SARS-CoV-2 from March 2020 to June 2021. Data on demographic characteristics, type of CNP, patients' background and SARS-CoV-2 infection history (symptoms, laboratory features, radiological appearance, therapeutic approach and outcome) were collected. Results: Twenty-six patients with diagnosis of CNP, 7 males and 19 females were registered. Patient age distribution as follows: 16 patients >18 years old (y.o.)5 patients 5-18 y.o, 4 patients < 5 y.o whereas age was not available for one of the patients. Nine of the patients were diagnosed with idiopathic CNP, 7 patients with congenital neutropenia (6 of them with severe congenital neutropenia), 3 with secondary CNP, 2 with suspected autoimmune neutropenia of infancy (although antineutrophil Ab were negative), one with autoimmune neutropenia, one with drug induced neutropenia and 3 with other types of CNP. Twelve patients were on treatment with G-CSF and 6 patients had a history of previous viral or bacterial infections. Clonal Cytopenia(s) of Undetermined Significance (CCUS) was excluded in the eight patients who were investigated. Twenty-four out of 26 patients had positive PCR and one was found incidentally with positive antibodies for SARS-CoV-2. One more patient was symptomatic with history of close contact with SARS-CoV-2 infected family members. The commonest observed symptoms were fever >38 oC (19 patients), cough (10 patients), rhinorrhoea (10 patients), sore throat (6 patients), musculoskeletal pains (7 patients), taste/smell loss (5 patients), headache (5 patients), dyspnoea (4 patients), chest pain (one patient) and none of them had gastrointestinal symptoms. No other associated respiratory viral or bacterial infections were reported. Four patients who had one or more underlying conditions (immune deficiency, heart/respiratory/kidney disease) were admitted in hospital and needed anti SARS-CoV-2 treatment. Two of them had non-invasive ventilation and one of them needed admission in intensive care unit (ICU);both recovered. Another patient with Fallot's tetralogy needed mechanical ventilation in ICU and sadly passed away. No other deaths were observed. Deterioration of the pre-existing neutropenia was seen in two patients, two patients developed thrombocytopenia, one patient developed worsening lymphopenia and one anaemia. Twelve patients had chest X-ray and consolidation was found in two of them. All three patients who had chest CT scans were found with ground-glass changes. During the observation period (up to two months), no re-infection from SARS-CoV-2 was found. The Stockholm, Sweden experience is similar to the above data. One hundred fifty-four patients with CNP were followed up, for 10 months (March 1 to December 31, 2020) for SARS-CoV-2. Seventeen of these (i.e. 11 %) were infected. None needed hospitalization and there were no fatalities. Conclusion: Although the relative susceptibility of neutropenic patients to contract SARS-CoV-2 needs to be assessed with further studies the clinical course and severity of SARS-CoV-2 infection doesn't seem to be worse in CNP patients (regardless the type of neutropenia and the need for GCSF treatment) compared to the general population. Also, like what has been observed in non-neutropenic patients, underlying comorbidities is a significant risk factor for severe disease and adverse outcome. Disclosures: Dale: X4 Pharmaceuticals: Consultancy, Honoraria, Research Funding. Palmblad: Chiesi Ltd Sweden: Honoraria;Roche Sweden: Speakers Bureau;Chiesi Ltd Candada,: Honoraria.

Impact of COVID-19 in adult patients with congenital heart disease

Background: COVID-19 is responsible for a worldwide pandemic, causing more than 13 000 deaths to date in Portugal. Data already exists regarding the increased risk of adverse events in patients with cardiovascular diseases, however the impact of SARS-CoV-2 infection in patients (P) with congenital heart disease (CHD) is still under investigation. Aims: To evaluate the impact of COVID-19 in adult patients with congenital heart disease in our tertiary centre Methods: Adult patients seen at the CHD outpatient's clinic at a tertiary centre, who became infected with SARS-CoV-2 infection up to February 2021 were included. Assessment of patients' symptoms, need for hospitalization and admission in an intensive care unit was assessed based on medical records. Results: We identified 36 patients (pts) with COVID-19 infection. Symptoms were present in 31 (86%). The median age was 39 (32-49) years, 58% were females. Seven P (19%) had complex cyanotic disease;three (8%) Tetralogy of Fallot;three (8%) transposition of great arteries (one after Senning procedure and 2 after arterial switch);six (14%) right ventricle obstacle;two (8%) atrioventricular canal defect;four (11%) atrial septal defect;five (14%) ventricular septal defect;five (14%) aortic coarctation;two aortopathies (one submitted do David procedure);one subaortic stenosis;two (6%) had Eisenmenger syndrome. The majority (61%) of P had previous surgery and 58% were at New York Heart Association class of I. Mild symptoms were reported by 24 P (67%). Seven adults experienced moderate symptoms (dyspnea and hypoxia) that led to hospitalization for oxygen therapy, although none required mechanical ventilation. One death was reported. There was a significant association between the gravity of CHD and hospitalizations (p=0.012). Conclusion: Our pts had mainly mild to moderate symptoms and did not appear to have a disproportionately negative outcome;the need for hospitalization was more frequent in patients with higher CHD gravity. These findings are in line with the emerging data regarding COVID-19 in CHD P, and may be in part explained by the patient's young age and functional status.