Background Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. Objectives This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. Methods Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. Results From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. Conclusions COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.

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V entricular tachyarrhythmias are known sequelae among adults with repaired tetralogy of Fallot. The incidence of sustained ventricular tachycardia and sudden cardiac death in patients 35 years after corrective surgery is estimated at 11.9% and 8.3%, respectively.1 Studies have shown that right ventricular enlargement resulting from chronic pulmonic regurgitation is the most common hemodynamic substrate.2 QRS prolongation (>180 milliseconds) is one of the most sensitive ECG predictors and in turn correlates with right ventricular (RV) dilation.3 The exact mechanism is not as well studied as that of left ventricular arrhythmias, but it is purported that volume overload leads to RV myocardial stretching and RV fibrosis and hence serves as an arrhythmogenic focus.The anatomic location of the RV pathology leading to ventricular tachyarrhythmias after tetralogy of Fallot surgical repair is not well defined. The basal portions of the RV and the subpulmonic regions of the RV are difficult to visualize on transthoracic echocardiography. Furthermore, 2-dimensional echocardiography is not capable of myocardial scar imaging. Cardiac magnetic resonance imaging (MRI) is capable of not only visualizing all anatomic segments of the RV but also delineating areas of myocardial fibrosis with late-gadolinium-enhancement imaging.The value of cardiac MRI is demonstrated in this case of a 50-year-old man with a history of tetralogy of Fallot surgical repair as an infant. While playing recreational hockey, he suddenly developed presyncope with severe lightheadedness, dizziness, diaphoresis, and palpitations. On evaluation by emergency medical services personnel, he was found to have a wide-complex tachycardia at a rate of 300 bpm, suspicious for ventricular tachycardia (Figure 1). Normal sinus rhythm was restored after administration of amiodarone 150 mg IV. A postconversion ECG revealed normal sinus rhythm with a right bundle-branch block and a QRS duration of 175 milliseconds (Figure 2). The estimated total duration of the wide-complex tachycardia was 5 minutes. A transthoracic echocardiogram revealed evidence of both RV pressure and volume overload, with RV hypertrophy and abnormal septal motion.A cardiac MRI study showed moderate RV dilation with severe RV systolic dysfunction, noting a global RV ejection fraction of 20%. Left ventricular function was normal with a global LV ejection fraction of 62%. The basal portions of the RV and RV outflow tract were aneurysmal (Figures 3 and 4 and Movie I in the online-only Data Supplement). This was not as well appreciated on the transthoracic echocardiogram (Movie II in the online-only Data Supplement). The RV aneurysmal area was found to have discrete subendocardial fibrosis on lategadolinium delayed-hyperenhancement imaging ( Figure 5). Late-gadolinium-enhanced imaging also illustrated fibrosis at the LV-RV hinge point (Figure 6) but not in the left ventricle itself. Hence, the arrhythmogenic focus was thought to be the RV outflow tract. The patient subsequently underwent automatic implan...

Background: Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response. Methods: In this prospective, double-blind, placebo-controlled, crossover trial, 14 patients with a Fontan circulation were randomised to inhalation of placebo and Treprostinil. Incremental and constant work rate exercise tests were performed to assess the effect of Treprostinil on exercise tolerance. Venous pressures were measured throughout and liver stiffness at rest and immediately after peak exercise. Results: Mean age was 27.8 ± 7.9 years and 66% were females. Exercise acutely increased liver stiffness by 30% (mean shear wave speed: 2.38 ± 0.71 versus 2.89 ± 0.51 ms, p = 0.02). Peripheral venous pressures increased acutely during both incremental (12.1 ± 2.4 versus 22.6 ± 8.0 mmHg, p < 0.001) and constant work rate exercise (12.5 ± 2.5 versus 23.4 ± 5.2 mmHg, p < 0.001). Overall, Treprostinil failed to attenuate exercise-induced increases in liver stiffness. Compared with placebo, Treprostinil did not significantly impact venous pressure responses, VO2peak, nor exercise endurance times. Conclusions: Peripheral venous pressure increased acutely during exercise by an average of 88% above baseline and was not altered by administration of inhaled Treprostinil. Liver stiffness measured immediately post-exercise increased acutely by an average of 30%, with no attenuation following Treprostinil inhalation.