key: cord-0704451-895ld36e
authors: Gallego, Pastora; Ruperti-Repilado, Francisco Javier; Schwerzmann, Markus
title: Adults with congenital heart disease during the coronavirus disease 2019 (COVID-19) pandemic: are they at risk?
date: 2020-06-30
journal: Rev Esp Cardiol (Engl Ed)
DOI: 10.1016/j.rec.2020.06.016
sha: 9df271b6a32dd62fea2eadfb5ffc449e3c251fe2
doc_id: 704451
cord_uid: 895ld36e

nan

J o u r n a l P r e -p r o o f inflammatory disease with an impact on the vascular and hematopoietic system and hypercoagulability is common among hospitalized COVID-19 patients. 5 SARS-CoV-2 may interact with the cardiovascular system on multiple levels, increasing morbidity in patients with underlying cardiovascular conditions and provoking myocardial injury and dysfunction 6 (figure 1). 7 Since the early stages of the pandemic, higher mortality rates among COVID-19 patients with acquired cardiovascular disease, such as hypertension, coronary artery disease or diabetes, have been reported compared with patients without acquired cardiovascular disease, and heart failure may be precipitated by acute illness in patients with pre-existing cardiovascular disease. 8 Over the past few decades, a new cardiovascular population of adults with congenital heart disease (ACHD) has emerged in developed countries due to medical and surgical advances. Unfortunately, a complete cure of congenital cardiac defects in childhood is exceptional and many of them face a life-long increased risk in cardiovascular complications (such as heart failure, arrhythmias, pulmonary circulation disorders, andeven death) due to hemodynamic residuae 9,10 or sequelae of previous repair strategies. Therefore, it is reasonable to assume that some of these ACHD patients will be at increased risk of adverse outcomes if they contract COVID-19.

ACHD patients now outnumber children with congenital heart disease (CHD). The available evidence suggests an overall ACHD prevalence of about 3000 per million. Included in this group are patients with "mild" defects (eg, repaired atrial septal defect or ligated ductus arteriosus), but most have moderate or severe CHD whose natural history has been forever changed by therapeutic interventions. Analyses of registry data of adults with various forms of CHD from Spain and Switzerland have allowed for detailed phenotyping of patients followed by J o u r n a l P r e -p r o o f respectively, and 32% as having mild defects. 11 In the Swiss Adult Congenital Heart Disease Registry (SACHER), up to 16% of 2836 patients had cyanotic or other complex congenital heart disease, 15% had lesions affecting the right heart (ie, tetralogy of Fallot or Ebsteinanomaly), 13% had complex diseases of the left ventricular outflow tract, 25% had valvular lesions, and 22% had a shunt pathology. 12 As expected, most Spanish and Swiss ACHD patients had undergone previous repair procedures (77% and 71%, respectively) and 47% of those had had 1 or more reinterventions. 11, 12 When considering the potential outcome of COVID-19 in ACHD patients, competing risks have to been taken into account. First, age per se is an independent risk factor for COVID-19-related mortality. 13, 14 Immune responses in older adults are slower, less coordinated, and less efficient than in younger adults, making them more susceptible to coronavirus infections. 15 In addition, circulating plasma concentrations of angiotensin-converting enzyme II are higher in adults than children and are also higher in men than in women. 16 COVID-19 invades cells by binding to angiotensin-converting enzyme II, which is expressed on the surface of alveolar cells in the lungs, making older men potentially vulnerable to SARS-CoV-2. On the other hand, typical ACHD patients with their median age of 35 years may be less susceptible or have a milder COVID-19 course, due to the protective effects of their age, independently of the underlying defect complexity. In addition, although the number of ACHD patients older than 60 years is steadily increasing, acquired cardiovascular risk factors for fatal COVID-19 outcomes are infrequently found in the ACHD population. In the Spanish cohort, 75% of patients were younger than 45 years, and the prevalence of hypertension, diabetes and ischemic heart disease was only 14%, 2.7%, and 1.5%, respectively (unpublished data). Thus, many ACHD patients without major comorbidities may not be at higher risk for a poor outcome if they contract COVID-19 than the corresponding general population. appropriate management, with a potentially direct effect on morbidity and mortality. We know from young ACHD patients with loss of follow-up during the transition period from pediatric to adult care that a lapse of care is associated with more late complications and a higher need for urgent cardiac interventions. 19 The desire to minimize the risk of coronavirus exposure for a given ACHD patient must obviously be weighed against the need for in-person visits, particularly in those with decompensated congestive heart failure or arrhythmias. If possible, scheduled clinic visits should be converted to telehealth visits. Accustomed since their childhood to being surrounded by technological devices, the young ACHD population may be particularly inclined to the use of mobile health technologies. A wide range of clinical data, including visual examination, auscultatory sounds, physiological parameters and echocardiographic images, can be transmitted in real-time across telemedicine links, facilitating accurate diagnosis, effective decision making and promoting access to specialist opinion. Such telemedicine programs can continue to provide state of the art, high quality, personalized care for individuals in the community beyond the coronavirus pandemic.

In conclusion, the current COVID-19 pandemic poses unprecedented challenges and changes to both patients and specialists. Assumptions derived from theoretical reasoning and extrapolation of data from patients with acquired heart disease might not be sufficiently accurate for risk stratification in ACHD. Predicting the risk of poor outcomes remains challenging due to the variety of cardiac defects and repair strategies. The COVID-19 pandemic may represent a special burden for patients with severe CHD, such as cyanotic patients, patients with overt heart failure or associated pulmonary vascular disorders, and the Page 7 of 10 J o u r n a l P r e -p r o o f involvement of other organs may adversely impact the prognosis. However, fairly young ACHD patients without comorbidities may not be particularly vulnerable to COVID-19. Since they are also exposed to the potentially far-reaching socioeconomic impact of this pandemic, our recommendations for these patients needs to be based on sound clinical evidence. Therefore, national and international collaborations aiming to address this issue must be fostered and are on their way. Permission obtained from © The European Society of Cardiology 2020. All rights reserved.

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