key: cord-268354-dq17uhzx
authors: Lastinger, Lauren T.; Daniels, Curt J.; Lee, Marc; Sabanayagam, Aarthi; Bradley, Elisa A.
title: Triage and management of the ACHD patient with COVID-19: A single center approach
date: 2020-06-19
journal: Int J Cardiol
DOI: 10.1016/j.ijcard.2020.06.023
sha: 
doc_id: 268354
cord_uid: dq17uhzx

With the recent emergence of SARS-CoV-2 and COVID-19, healthcare facilities and personnel are expected to rapidly triage and care for even the most complex medical conditions. Adults with congenital heart disease (ACHD) represent an often-intimidating group of complex cardiovascular disorders. Given that general internists and general cardiologists will often be asked to evaluate this group during the pandemic, we propose here an abbreviated triage algorithm that will assist in identifying the patient's overarching ACHD phenotype and baseline cardiac status. The strategy outlined allows for rapid triage and groups various anatomic CHD variants into overarching phenotypes, permitting care teams to quickly review key points in the management of moderate to severely complex ACHD patients.

Coronavirus disease -2019 (COVID-19) was first recognized in Wuhan, China in December 2019, and in a few short months, the SARS-CoV-2 virus has rapidly spread, resulting in a global pandemic [1] . The clinical presentation of COVID-19 typically includes: fever, cough, shortness of breath, fatigue and myalgias. Less commonly, those affected have demonstrated anosmia, sore throat, and nausea/vomiting. As a disease, COVID-19 is characterized predominantly by respiratory compromise, and in some cases evolves to acute respiratory distress syndrome (ARDS). Patients with underlying cardiovascular disease, diabetes mellitus, hypertension, and chronic lung disease appear to be at higher risk for the development of severe disease with ARDS [2] .

Limited data has been reported on outcomes in adults with congenital heart disease (ACHD) who contract SARS-CoV-2 and develop COVID-19 infection. Given that the most significant advances in interventional and surgical care of CHD evolved over the last 50 years, adults with CHD as a group, are younger than patients with acquired cardiovascular disease. However, ACHD patients demonstrate a higher prevalence of restrictive lung disease, acquired cardiovascular disease, and other general medical comorbidities [3, 4] . Therefore, the patient with ACHD and COVID-19 may be at risk for severe disease and poor outcomes.

As a group, CHD is a heterogenous cohort, and these patients are often defined primarily by the original anatomic defect (although sometimes by the palliative procedure) and physiologic functional class [5] . In the face of the current pandemic, these patients may present to primary care providers, the emergency department and other healthcare providers outside of their primary ACHD cardiologist. Furthermore, there is currently a lack of evidence available in COVID-19 infection in CHD patients to guide the evaluation and management of this complex group. We believe a rapid and concise triage algorithm for this group should include broad J o u r n a l P r e -p r o o f categorization of ACHD phenotype in order to ensure swift triage and care in the setting of known/suspected COVID-19. Here we propose an ACHD phenotype classification system to accurately characterize the ACHD patient with moderate-severely complex CHD. This "ACHD phenotype" will allow the non-ACHD physician evaluating a patient with known/suspected COVID-19 to rapidly recognize CHD anatomy and apply appropriate triage guidelines based upon the phenotype and baseline physiologic CHD level of compensation/decompensation.

Here we review a reasonable approach to identifying ACHD phenotypes and physiologic compensation to assist in the rapid triage of ACHD patients with known/suspected COVID-19.

We will focus the proposed triage system on ACHD patients with moderate or severely complex lesions, as this group is usually the most troublesome to understand, for those without a background in CHD. Recognizing there is often physiologic overlap between groups, we propose 5 broad "ACHD phenotypes" to include: Single Ventricle / Fontan Anatomy. Patients born with a single ventricle often undergo a series of surgeries that culminate in a Fontan procedure. In this physiology, deoxygenated blood empties passively to the lungs due to an absent subpulmonic ventricle. Pulmonary blood flow is dependent on adequate systemic venous pressure, and therefore so is cardiac output.

Increases in intrathoracic pressure, such as from positive end-expiratory pressure (PEEP) ventilation, may compromise pulmonary filling. Many patients with a Fontan palliation have either intentional or anatomic shunts in addition to coronary sinus incorporation to a single atrium, all of which result in some degree of resting hypoxia, highlighting the need to understand normal resting SpO2 in this group. Late after Fontan palliation, patients may develop "Fontan Failure", a syndrome that, to some extent, mimicks right heart failure, and may be associated with protein losing enteropathy. 

Once the practitioner is able to identify the overarching ACHD phenotype, physiologic assessment becomes important. Essentially the goal with rapid physiologic assessment is to determine the status of CHD at baselinecompensated, early decompensation or late decompensation. A careful review of prior history and imaging, as well documentation of baseline NYHA functional status and SpO2 is prudent. The presence of arrhythmia, residual hemodynamic lesions and extra-cardiac abnormalities indicate a more advanced physiological stage. Recent cardiopulmonary stress and VO2 testing can help clarify objective evidence of a patient's functional status. This must, however, be interpreted with caution, as it is well known J o u r n a l P r e -p r o o f that ACHD patients exhibit lower than predicted values when compared to age matched individuals [6] .

Compensated ACHD patients, regardless of physiological stage, will likely display only mild symptoms. Early decompensation should be suspect in patients with lower than usual SpO2 (>5 percentage points) or increased oxygen requirements, weight gain attributable to fluid retention (>5-8 pounds), worsening dyspnea on exertion, asymptomatic atrial or ventricular arrhythmias or an exacerbation of chronic non-cardiac end organ complications. These patients tend to also have low reserve, and in acute illness can decline rapidly and precipitously. Late decompensation is identified by the inability to oxygenate effectively without the use of noninvasive/mechanical ventilation, signs of poor perfusion from decompensated heart failure and multi-organ dysfunction as well as refractory and symptomatic arrhythmias requiring immediate pharmacological or electrical interventions.

Triage of the moderate-severely complex ACHD patient with known/suspected COVID-19 relies on a multi-step process that assesses patient characteristics in the following order: 1) Degree of COVID-19-specific symptoms, 2) Classification of COVID-19 symptom severity, 3) Assessment of general ACHD-based high-risk features and 4) Inpatient triage and management-specific key points based upon the over-arching ACHD phenotype.

The first step in triage of the moderate-severely complex ACHD patient with known/suspected COVID-19 is to assess whether or not the patient has symptoms consistent with COVID-19

infection. This patient is offered COVID-19 testing at the discretion of the institution's protocol, which typically relies on an assessment of symptoms and comorbid conditions. It is important to J o u r n a l P r e -p r o o f recognize that shortage in screening resources is often what dictates whom and how initial testing is allocated.

Once the patient is suspected or known to have COVID-19, we propose stratifying the next steps based upon the degree of symptoms from the virus (minimally, moderately and severely symptomatic). We propose that the mildly symptomatic ACHD patient with < 1 symptom is likely reasonably managed at home with appropriate telehealth follow-up within 48 hours. Severely symptomatic patients with evidence of end-organ dysfunction should be admitted to the hospital (intensive care unit, ICU). In those that are moderately symptomatic with > 2 symptoms, inpatient management is likely warranted but needs to be assessed on a case-by-case basis.

Step 3 is focused on the assessment of the moderately symptomatic patient and centers on whether or not the patient has baseline high-risk ACHD features* (Figure 1 ) or high-risk COVID-19 laboratory results [2] . At this step, the ACHD specialist may assist in determining if baseline ACHD features are stable (Compensated) or unstable (Decompensated). As outlined above, compensated patients typically have a stable (although often lower) SpO2, functional class and limited symptoms. Those with early decompensation may have demonstrated declines in exercise tolerance and/or symptoms in the preceding months or years. This is considered in order to determine which patients may benefit from higher levels of care beyond general ward hospital admission.

Step 4 is the final phase of ACHD phenotypic assessment. Recognition of the ACHD phenotype is used to help determine aspects of the patient's presentation that are normal for the type of underlying ACHD versus those that may be pathologic. Here, moderate and high-risk patients are assessed based upon their underlying phenotype to assist in determining adjunctive J o u r n a l P r e -p r o o f management strategies for CHD-specific symptoms with concurrent COVID-19 (Figure 1 , Supplemental Table 1 ).

The principles for intubation, oxygenation and mechanical ventilation for the COVID -19 positive ACHD patient begin with understanding the patient's baseline cardiac status with a focus on the following areas:

 Oxygen saturation: Patients with right-to-left shunts may have resting hypoxia (SpO2 ~ 85-90%); those with chronic cyanosis and or ES may be more pronounced (SpO2 ~ 75-80%).

 RHF phenotype (dilated RV with reduced RV systolic function): These patients may more easily develop hemodynamic instability with intubation.  Intracardiac Shunt: Such patients may present with worsening hypoxia due to increased right-to-left shunting that can occur due to low cardiac output (trade hypoxia for cardiac output). In this case, supporting the hemodynamic status will improve oxygenation (it is not a primary lung problem).

 Cardiac Preload: Several ACHD phenotypes (discussed above) are preload dependent and poor filling (dehydration) may affect the ability to provide adequate cardiac output, particularly in the setting of intubation/mechanical ventilation.

Considering this data, mechanical ventilation support strategies for ACHD patients should mirror those patients with significant cardiovascular and pulmonary disease who develop ARDS, with J o u r n a l P r e -p r o o f 

Clinical features of patients infected with 2019 novel coronavirus in Wuhan

Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study

Restrictive lung disease is an independent predictor of exercise intolerance in the adult with congenital heart disease

Risk of coronary artery disease in adults with congenital heart disease: A comparison with the general population

AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines

Exercise intolerance in adult congenital heart disease: comparative severity correlates and prognostic implication