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.

Turner syndrome (TS), a genetic abnormality affecting 1 in 2,500 people, is commonly associated with congenital heart disease (CHD). However, the surgical outcomes for TS patients have not been well described. This study reviewed the spectrum of CHD in TS at the authors' center. The authors report outcomes after coarctation of the aorta (CoA) repair or staged palliation of hypoplastic left heart syndrome (HLHS) and then compare the surgical outcomes with those of non-TS patients undergoing like repair. This retrospective chart review was conducted at the Children's Hospital of Wisconsin from 1999 to 2011. Of the 173 patients with TS, 77 (44.5 %) were found to have CHD. Left-sided obstructive lesions were the most common. However, the spectrum of CHD was wide and included systemic and pulmonary venous abnormalities as well as abnormalities of the coronary arteries. In the comparative analysis of CoA repair, the TS patients younger than 60 days had longer aortic cross-clamp times (24 vs. 16 min; p = 0.001) and longer hospital stays (12 vs. 6 days; p ≤ 0.0001) than the non-TS patients. At the follow-up assessment after 8.8 ± 9.1 years, 17 % of the TS patients had hypertension, but no patient had required reintervention, and no deaths had occurred. Finally, three of the four TS patients with HLHS died within the first year. The spectrum of CHD within TS is wide and not limited to bicuspid aortic valve or CoA. Additionally, patients with TS undergoing CoA repair may have a more challenging early postoperative course but experience outcomes similar to those of non-TS patients. Finally, patients who have TS combined with HLHS remain a challenging population with generally poor survival.

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