Myocarditis and right atrial thrombus in a patient with Thrombotic thrombocytopenic purpura- CASE REPORT

Background: Thrombotic thrombocytopenic purpura is a very rare form of thrombotic microangiopathy with an annual incidence ranging between 1.5-6 cases per million adult individuals. Cardiac involvement in thrombotic thrombocytopenic purpura may be diagnosed in up to a quarter of these patients, often associated with an adverse prognosis. Though cardiac microvascular thrombosis is most commonly noted, macrovascular thrombosis may also occur. Case Presentation: We report about a young male patient with obesity and hypertension who presented with recurrent ischemic strokes. He was found to be having severe hemolytic anemia along with severe thrombocytopenia. A diagnosis of thrombotic thrombocytopenic purpura was confirmed due to almost undetectable levels of a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 levels. His troponin I level was also elevated while having no cardiac symptoms. He was diagnosed with asymptomatic myocarditis along with right atrial thrombosis which was confirmed with transesophageal echocardiography and cardiac magnetic resonance imaging. This is probably the first reported case of concomitant myocarditis with intracardiac thrombosis in a patient of thrombotic thrombocytopenic purpura. Prompt initiation of plasma exchange therapy followed by corticosteroids, rituximab and aspirin led to disease remission. Conclusion: Intensivists and cardiologists must be aware of the varied presentations associated with thrombotic thrombocytopenic purpura. Early diagnosis and cardiac evaluation is essential for ensuring a favorable outcome in these critically ill patients.