Endocardial Fibroelastosis With Coronary Artery Thromboembolus and Myocardial Infarction

Abstract

We report a case of an 18-month-old male, born to a woman with third trimester febrile illness, who had a history of congestive heart failure and respiratory distress, cardiomegaly, and electrocardiographic (ECG) findings suggestive of cardiomyopathy and myocarditis. After gradual improvement in heart size and function with pharmacologic therapy, he developed a terminal episode of respiratory distress and cardiogenic shock, with ECG findings of an anterolateral infarct. At autopsy it was found that endocardial fibroelastosis with mural thrombi in the left ventricle had been complicated by thromboembolism to the left anterior descending coronary artery, resulting in transmural infarction of the anteroseptal region of the left ventricle. Myocardial infarction is a potential but unusual thromboembolic complication of endocardial fibroelastosis. A high index of suspicion for coronary artery thromboemboli should be maintained in pediatric patients with cardiomyopathy and suspected myocardial infarction.

Incremental Value of 3D and Contrast Echocardiography in Evaluation of Endocardial Fibroelastosis and Multiple Cardiovascular Thrombi

“…It is one of the causes of heart failure in infants and children [6,7]. Patients with EFE are prone to endocardial thrombosis, which may be due to enlarged cardiac cavity, systolic dysfunction, and altered endothelium lining [8,9]. Previous studies have also reported that a thrombus can easily form at the sites of akinetic, dyskinetic, or aneurysmal segments that are potentially at risk for thromboembolism [10].…”

“…A total of seven patients diagnosed with EFE with thrombosis were identi ed. A summary of these patients is presented in Table 1 [8,9,[11][12][13]. There were one female and six males, with the age at diagnosis ranging from fetal to 68 years old.…”

Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions

“…stenosis and hypoplastic left heart syndrome [28]; nonetheless, the exact aetiology is unknown. The underlying pathophysiology of endocardial fibroelastosis is the deposition of acellular fibrocartilaginous tissue in the subendothelial layer of the endocardium predominantly involving the inflow tracts and apices of ventricles [29], and intraventricular thrombi formation are an additional complication that CMR can detect [30].…”

Cardiovascular Disease in Primary Sjögren’s Syndrome: Raising Clinicians’ Awareness

“…Deposition of acellular fibrous and cartilaginous tissue in the subendothelial layer of the endocardium, more frequently involving the inflow tracts and apices of both ventricles leads to severe left ventricular diastolic impairment with restrictive cardiomyopathy, with a possible evolution towards dilated cardiomyopathy ( 87 , 88 ). Other features are papillary muscles shortening with severe mitral regurgitation and mural thrombus formation ( 89 , 90 ). CMR is also a valuable tool for diagnosing EFE in children born from anti-SSA/SSB antibodies positive mothers.…”