Dilated Cardiomyopathy Due to Thrombotic Microangiopathy as the only Manifestation of Antiphospholipid Syndrome: A Case Report

Abstract

Dilated cardiomyopathy due to thrombotic microangiopathy has been rarely reported as a clinical manifestation of antiphospholipid syndrome (APS). We describe the case of a 39-year-old woman affected by systemic lupus erythematosus (SLE) and positive antiphospholipid antibodies (aPL) who presented with orthopnea and peripheral oedema. Diagnosis of dilated cardiomyopathy due to myocardial thrombotic microangiopathy was made and treatment with anticoagulants prevented the worsening of the clinical condition. Interestingly, at variance with other cases, our patient showed no extracardiac signs of APS. The review of the current literature has confirmed that dilated cardiomyopathy due to thrombotic microangiopathy is a rare manifestation of APS.

Cardiac manifestations other than valvulopathy in antiphospholipid syndrome: long-time echocardiography follow-up study

“…We did not find any case of infarct with normal angiography, so we did not suspect myocardial thrombotic microangiopathy, a rare entity described in APS [20][21][22] and in catastrophic APS causing severe heart failure with high mortality, 23,24 almost always associated with renal, pulmonary and cerebral thrombotic involvement. We did not find any case of infarct with normal angiography, so we did not suspect myocardial thrombotic microangiopathy, a rare entity described in APS [20][21][22] and in catastrophic APS causing severe heart failure with high mortality, 23,24 almost always associated with renal, pulmonary and cerebral thrombotic involvement.…”

“…Left ventricular dilated myocardiopathy and systolic dysfunction in our APS cohort was exclusively secondary to valvulopathy and atherosclerotic coronary artery disease. We did not find any case of infarct with normal angiography, so we did not suspect myocardial thrombotic microangiopathy, a rare entity described in APS [20][21][22] and in catastrophic APS causing severe heart failure with high mortality, 23,24 almost always associated with renal, pulmonary and cerebral thrombotic involvement. Therefore we would say that LV dilatation and systolic dysfunction would rarely be secondary to microvascular aPL-associated thrombosis of the myocardium and classical risk pathologies would account for most of the cases.…”

Primary antiphospholipid syndrome, hypertrophic non-obstructive cardiomyopathy and hypotelorism

“…Case reports5 have indicated the association of APS with dilated cardiomyopathy. The most likely underlying pathomechanism is intramyocardial microvascular thrombosis with subsequent myocardial necrosis.…”

An Approach to Differential Diagnosis of Antiphospholipid Antibody Syndrome and Related Conditions

“…Heart valves dysfunctions, generally of the mitral valve, ranging from mild valve thickening to the typical nonbacterial thrombotic lesions (Libman-Sacks endocarditis) have been demonstrated by echocardiographic studies [ 41 ]. Cardiomyopathy in APS patients is of a quite rare occurrence and a convincing pathogenetic relationship with circulating aPL has not been clearly demonstrated [ 42 ].…”