Recurrent Episodes of ST-Elevation Myocardial Infarction in a 19-Year-Old Male with Fontan Circulation

Case Report

Austin J Clin Cardiolog. 2021; 7(2): 1078.

Recurrent Episodes of ST-Elevation Myocardial Infarction in a 19-Year-Old Male with Fontan Circulation

Alghammass MA¹*, Drakos SG¹, Lal AK², Martinez HD³ and Kemeyou L¹

¹Division of Cardiovascular Medicine, Department of Internal Medicine, University of Utah, USA

²Division of Pediatric Cardiology, Department of Pediatrics, University of Utah, USA

³Division of Palliative Care, University of Utah, USA

*Corresponding author: Alghammass MA, Department of Internal Medicine, Division of Cardiovascular Medicine, University of Utah, 30 N Medical Dr. Room 4a100, Salt Lake City, Utah 84132, USA

Received: May 24, 2021; Accepted: June 12, 2021; Published: June 19, 2021


We describe a case of a 19-year-old male with history of single ventricle physiology status post-Fontan procedure at the age of two who developed thromboembolic phenomena involving his splenic, renal and coronary arteries resulting in multiple infarcts and recurrent in-hospital acute ST-Segment Elevation Myocardial Infarction (STEMI) treated by emergent Percutaneous Coronary Intervention (PCI). This case highlights multiple aspects and challenges of managing young patients with congenital heart disease.

Keywords: Congenital heart disease; Heart failure; Thromboembolism; Anticoagulation; Acute coronary syndrome


ECG: Electrocardiogram; LAD: Left Anterior Descending Artery; STEMI: ST-Segment Elevation Myocardial Infarction; DOAC: Direct Oral Anticoagulants; CVICU: Cardiovascular Intensive Care Unit

Case Presentation

19-year-old male with a history of single ventricle physiology (S,L,L) including L-transposition of the great arteries, pulmonary valve atresia, left atrioventricular valve atresia, large unrestrictive VSD, unrestrictive secundum ASD, and left pulmonary artery stenosis status post staged corrective heart surgery with eventually extracardiac Fontan at the age of two who presented to our emergency department complaining of abdominal pain for five days, associated with nausea and vomiting. Prior to his presentation to our facility, he was recently discharged from a local hospital after evaluation of chest pain with left sided weakness; he was found to have severe systolic and diastolic dysfunction, and age indeterminate thromboembolic stroke. His cardiac catheterization during this admission did not show any coronary vasculature abnormalities. He was discharged on aspirin, furosemide and rivaroxaban due to patient’s refusal to be treated with warfarin. His medications history also includes daily prophylactic aspirin since his Fontan procedure, but he reported not taking it.

On exam he was vitally stable with left upper quadrant tenderness. Labs notable for normal complete blood count and kidney function. B-type natriuretic peptide resulted 1235pg/mL (normal range: ≤100pg/mL). Chest radiograph showed evidence of pulmonary edema and CT abdomen/pelvis revealed acute splenic infarct and left renal infarct. He was admitted to the Cardiovascular Intensive Care Unit (CVICU) for management of acute exacerbation of heart failure, and acute infarcts involving the spleen and left renal arteries. He was started on infusions of milrinone and furosemide; as well as heparin infusion for management of heart failure and thromboemboli respectively. Our adult congenital heart disease and hematology teams were also involved in his inpatient care.

Shortly following admission, he reported acute severe nonradiating substernal pressure-like chest pain. Clinical examination was notable for tachycardia. Electrocardiogram (ECG) showed ST elevation in the anterolateral leads (Figure 1). Patient was immediately taken to the cardiac catheterization laboratory where coronary angiogram showed situs inversus, and the morphologic left coronary artery demonstrated a thrombotic occlusion in the mid Left Anterior Descending Artery (LAD) (Figure 2). A thrombectomy was performed and coronary flow was re-established; however, this intervention did not restore distal flow. Stenting was not performed given the embolic nature of the lesion. The final angiography shot showed TIMI3 flow in all vessels with persistent limited flow and occlusion in the distal LAD (Figure 3). The patient was transferred back to the CVICU and continued on antiplatelet therapy, with resolution of symptoms and ECG changes (Figure 4). Troponin peaked at 16.5ng/mL (normal range: ≤0.03ng/mL).