Background. The pandemic of Novel Coronavirus Disease 2019 (COVID-19) is challenging, given the large number of hospitalized patients. Cardiovascular co-morbidities are linked to a higher mortality risk. Thus, patients with Congenital Heart Disease (CHD) might represent a high-risk population. Nevertheless, no data about them are available, yet. Hence, we conducted a nationwide survey to assess clinical characteristics and outcomes in patients with congenital heart disease affected by COVID-19. Methods and Results. This is a multi-centre, observational, nationwide survey, involving high-volume Italian CHD centres. COVID-19 diagnosis was defined as either “clinically suspected” or “confirmed”, where a severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) test had been performed and was positive. Cardiovascular comorbidities were observed among adult patients—atrial fibrillation (seven; 9%), hypertension (five; 7%), obesity (seven; 9%) and diabetes (one; 1%)—but were absent among children. Cardiovascular complications were mainly observed in the “confirmed” COVID-19⁺ group, consisting of heart failure (9%), palpitations/arrhythmias (3%), stroke/TIA (3%) and pulmonary hypertension (3%). Cardiovascular symptoms such as chest pain (1%), myocardial injury (1%) and pericardial effusion (1%) were also recorded. On the contrary, CHD patients from the clinically suspected COVID-19 group presented no severe symptoms or complications. Conclusions. Despite previous reports pointing to a higher case-fatality rate among patients with cardiovascular co-morbidities, we observed a mild COVID-19 clinical course in our cohort of CHD patients. Although these results should be confirmed in larger cohorts to investigate the underlying mechanisms, the findings of low cardiovascular complications rates and no deaths are reassuring for CHD patients.

Aims: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. Methods and results: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35–48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0–15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4–10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04–1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75–10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23–7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05–70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. Conclusion: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

Management of rhythm related issues might be particularly challenging in patients with congenital heart disease due to complex anatomy and restricted vascular access. The leadless technology appears a suitable and attractive alternative for this population. We describe a patient with single ventricle physiology who successfully underwent implantation of a leadless pacemaker.

We aimed to characterize the epidemiology, diagnostic peculiarities and outcome determinants of bacterial myocarditis. Two cases from our institution and literature reports were collected ending up with a total of 66 cases. In 37 (56%) patients, the diagnosis was confirmed by magnetic resonance and histopathological criteria. The other patients were classified as having possible myocarditis. Only occurrence of rhythm disturbances was associated with the specific diagnosis of myocarditis (p = 0.04). Thirty-two (48%) patients presented with severe sepsis that was associated with a worse prognosis. At multivariate analysis, left ventricular ejection fraction (LVEF) at admission and heart rhythm disturbances were associated with incomplete recovery (odds ratio (OR) 1.1, 95% (CI) 1.03-1.2, p = 0.004 and OR 6.6, 95% CI 1.35-32.5, p = 0.02, respectively). In summary, bacterial myocarditis is uncommon. Most commonly, it is secondary to septic dissemination of bacteria or to transient secondary myocardial toxicity.

To describe the long-term follow-up of patients with complex congenital heart disease who underwent subcutaneous implantable cardiac defibrillator (S-ICD), focusing on local complications, appropriate and inappropriate shocks. Patients with complex congenital heart disease underwent S-ICD implant in two centers with the conventional technique. Data at follow-up were retrieved from clinical notes and institutional database. Eight patients were implanted in two centres between 2010 and 2016. Median age at implant was 37.5 years (range 13-57). All patients who were deemed suitable for S-ICD implant passed the pre-procedural screening. Three patients were previously implanted with a anti-bradycardia device, one of whom with CRT. In one patient the device was explanted due to local infection. During the total median follow-up of 874 d, one patient had an appropriate and one inappropriate shock triggered by fast atrial tachycardia. None of the patients had inappropriate shocks secondary to T wave oversensing or electrical interference with anti- bradycardia devices. S-ICD appears to be effective and safe in patients with complex congenital heart disease.