key: cord-325609-n6dpac6i
authors: Dawson, Kathryn L.; Vincent, Logan L.; Krieger, Eric V.; Stout, Karen K.; Buber, Jonathan
title: Acute increase in deaths among adult congenital heart disease patients during COVID-19 - single center experience.
date: 2020-06-13
journal: JACC Case Rep
DOI: 10.1016/j.jaccas.2020.06.013
sha: 
doc_id: 325609
cord_uid: n6dpac6i

Abstract Fear of acquiring severe acute respiratory syndrome coronavirus 2 infection is a major contributor to under-utilization of the healthcare system during the current pandemic. In this report, we describe 4 cases of unexpected deaths that occurred within a short time period in adult congenital heart disease patients without warning symptoms.

As the prevalence of adult congenital heart disease (ACHD) increases, awareness of their unique risk factors for adverse health outcomes and the need for ongoing care in adulthood is critical (1, 2) . Caring for this population requires understanding of the dichotomy that underlies their interaction with healthcare: ACHD patients often have complex disease that requires frequent contact with their providers, but they simultaneously have frequent gaps in their care. Common etiologies for these times away from care include poor insight into disease severity, financial/health insurance issues, and decreased parental oversight in adulthood (2) . By recognizing increased acute-setting mortality rates among ACHD patients during the mandated stay-at-home period, this report seeks to demonstrate the effect of the ongoing severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related pandemic on mortality even in uninfected ACHD patients.

Over the 12-month period preceding the SARS-CoV-2 related stay-at-home order, a total of 4 patients followed by the ACHD service at the University of Washington Medical Center with defects of various severities died in an acute setting. In this report we describe a series of 4 acute-setting ACHD deaths that occurred within a single week at the time of effective stay-athome order. Acute onset death was defined as death that occurred either out of the hospital or within 24 hours of presentation to a medical facility. With the exception of 1 patient (case #4) who was called but did not answer the phone 2 days prior to being found dead at home, none of the patients had a missed clinic appointment since the initiation of the stay-at-home order, and none made contact with the medical system to report concerning symptoms prior to their deaths.

A 46-year-old woman with a history of aortic coarctation who underwent surgical repair at age 28 using an extra anatomic Dacron graft arrived to the emergency department (ED) due to a witnessed out of hospital arrest. The documented rhythm on the defibrillator during resuscitation was a narrow complex tachycardia. Cardiopulmonary resuscitation (CPR) was performed by her husband and emergency medical services, with eventual return of spontaneous circulation (ROSC). Her admission laboratory studies revealed a mildly elevated troponin I, NT-pro BNP, white blood count levels, and markedly elevated lactic acid and liver transaminases levels. Renal function was normal, and rapid SARS-CoV-2 testing was negative. A non-contrast head computerized tomography (CT) was unrevealing, and a chest CT demonstrated normal postsurgical findings and a patent Dacron graft. A transthoracic echocardiogram (TTE) performed showed mild to moderate RV dysfunction. A cooling protocol was initiated but she subsequently developed status epilepticus refractory to therapy. A brain magnetic resonance imaging (MRI) study revealed diffuse anoxic brain injury. The patient's family elected to transition to comfort care measures.

A 31-year-old man with a history of congenital aortic stenosis presented to the ED with rapidly performed and consistent with brain death, so supportive measures were withdrawn.

A 59-year-old man with a history of tetralogy of Fallot, hypertrophic cardiomyopathy and atrial fibrillation presented to the ED with chest pain and syncope. His past surgical history consisted of patch closure of the ventricular septal defect and right ventricular outflow tract enlargement during childhood, followed by repeat pulmonary valvuloplasty 19 years prior to his presentation.

In the emergency department, the ICD was interrogated and no events in the preceding two months were seen. Serial troponin levels were within the normal limits, a BNP levels was elevated at 2400 pg/mL and creatinine levels were elevated to more than twice the patient's baseline. The patient was monitored and was given a diuretic for volume overload. He was being prepared for discharge when he suffered an asystolic cardiac arrest. Code Blue was called and over 20 minutes of resuscitation attempts were performed. Asystole persisted without ROSC and he was pronounced deceased after prolonged resuscitation attempts.

A 48-year-old man with history of congenital aortic stenosis was found deceased at home. His surgical history consisted of childhood aortic valvotomy followed by a Bentall procedure with a 23mm mechanical valve. Two years prior to his death he had an episode of ventricular tachycardia (VT), and was found to have moderate-severe left ventricular systolic dysfunction.

He underwent an ICD implantation and ventricular function normalized on guideline-directed medical therapy. Three months prior to his death he underwent transcatheter VT and atrial flutter ablation due to recurrent arrhythmic episodes. On an echocardiogram performed following the ablation procedure, the left ventricular and mechanical aortic valve function were normal. Two days prior to being found, the patient's cardiologist had called him regarding a missed clinic visit but never received a return call.

Several news articles from across the United States and early publications internationally on the SARS-CoV-2 pandemic highlight the decline in ED visits. In Portugal, March 2020 brought 48% less emergency department visits than forecasted (3). Emergency department visits in England were down 49% in the third week of March 2020 as compared to the final week of February 2020 (4). Similarly, early reports from six institutions in the Seattle area (not including ours) showed that while there had been an increase in emergency department visits for respiratory illnesses, there was a 10-40% decline in overall emergency department visits (5).

Patients with chronic medical illnesses and those at risk for acute cardiac compromise have remained at risk despite SARS-CoV-2, and this case series highlights this reality.

Though ACHD mortality at our institution varies from month-to-month and year-to-year, four out-of-hospital deaths in a single week is equal to the cumulative number of hospital deaths in our ACHD population over the preceding year. What is even more concerning is that none of these patients had high-risk features that would put them at increased risk amongst ACHD patients. No patient had residual cyanotic heart disease, single ventricle physiology or concurrent pulmonary hypertension that would put them at even higher risk for complications during a respiratory pandemic. These patients had clinically stable conditions and routine follow-up care at an established ACHD center. None had sought care prior to their presentations or expressed symptoms to medical providers. This case series highlights the need for close follow-up, particularly in high-risk populations even if their disease burden has remained stable. While we are unable to definitively say that fear of SARS-CoV-2 lead to delayed hospital presentations in each of these cases, we believe that they do reflect a growing trend of healthcare avoidance by cardiology patients with non-SARS-CoV-2 emergencies and potentially life-threatening consequences of these delays in care.

The SARS-CoV-2 pandemic and associated shelter-in-place orders, combined with public fear of contraction of the virus, has led to an overall reduction in emergency department visits despite increasing visits for respiratory illnesses. We believe that this change in emergency department volume, combined with the acute increase in mortality reported in this case series, emphasizes the potential adverse outcomes of delayed presentations in medically complex patients. This includes our ACHD population. These cases highlight the need for public education regarding the imperative to present for medical care when symptoms would have merited emergency treatment prior to the pandemic, particularly amongst our most vulnerable populations. It also highlights the need for routine follow-up care for patients with congenital heart disease, even in the presence of clinical stability, to assess for subclinical symptom burdens that may herald future acute presentations. 

Unexpected mortality among ACHD patients appears to have acutely increased at a single academic ACHD center during the SARS-CoV-2 pandemic

The ongoing SARS-CoV-2 pandemic appears to be contributing to increased ACHD mortality by delaying patient contact with healthcare

Routine follow-up care for high risk groups, including those with congenital heart disease, during the SARS-CoV-2 pandemic is critical to ensure appropriate triage and care for vulnerable populations. Even with prior clinical stability these populations remain at risk for acute cardiovascular complications and increased mortality

Projected growth of the adult congenital heart disease population in the United States to 2050: An integrative systems modeling approach

Prevalence and predictors of gaps in care among adult congenital heart disease patients: HEART-ACHD (The Health, Education, and Access Research Trial)

The Demand for Hospital Emergency Services: Trends during the First Month of COVID-19 Response

Covid-19: A&E visits in England fall by 25% in week after lockdown

COVID-19 in Seattle-Early lessons learned