Cardiac implications of thrombotic thrombocytopenic purpura

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder that essentially can affect any organ in the human body. The hallmark of the pathogenesis in TTP is the large von Willebrand factor multimers on platelet-mediated micro-thrombi formation, leading to microvascular thrombosis. Autopsy studies showed that cardiac arrest and myocardial infarction are the most common immediate causes of death in these patients. Clinical manifestations of cardiac involvement in TTP vary dramatically, from asymptomatic elevation of cardiac biomarkers, to heart failure, MI and sudden cardiac death. There is limited knowledge about optimal cardiac evaluation and management in patients with TTP. The absence of typical cardiac symptoms, combined with complicated multi-organ involvement in TTP, may contribute to the under-utilization of cardiac evaluation and treatment. Prompt diagnosis and timely initiation of effective therapy could be critically important in selected cases. Based on our experience and this review of the literature, we developed several recommendations for focused cardiac evaluation for patients with acute TTP: (1) patients with suspected or confirmed TTP should be screened for the potential presence of cardiac involvement with detailed history and physical, electrocardiogram and cardiac enzymes; (2) clinical deterioration of TTP patients warrants immediate cardiac reevaluation; (3) TTP patients with clinical evidence of cardiac involvement should be monitored for telemetry, cardiac biomarkers and evaluated with transthoracic echocardiography. These patients require urgent targeted TTP treatment as well as cardiac-specific treatment. Aspirin therapy is indicated for all TTP patients. Since epicardial coronary artery involvement is rare, cardiac catheterization is usually not required, given the high risk for hemorrhage and kidney injury; (4) we recommend evidence-based medical therapy for ischemic symptoms and heart failure. TTP patients with evidence of cardiac involvement would also benefit from routine cardiology follow up during remission.

Autopsy Findings in 32 Patients with COVID-19: A Single-Institution Experience

“…We believe that SARS-CoV-2-infected patients have a propensity to develop microthrombi in the myocardial vessels which correspond to microthrombi also seen in other organs. Microthrombi are not typically seen in the microcirculation of the heart in conditions other than thrombotic thrombocytopenic purpura and hemolytic uremic syndrome [ 26 , 27 ]. While frank myocardial ischemia was not frequently present, except in 1 case with well-documented MI, the elevated troponins and clinical myocardial dysfunction may be due to small-vessel thromboses in addition to general hypoxia.…”

Fatal Thrombotic Microangiopathy and Posterior Reversible Encephalopathy Syndrome in a Patient with Anti-melanoma Differentiation-associated Gene 5 Antibody-positive Dermatomyositis

“…Myocardial involvement was the most probable direct cause of death in this patient, as echocardiography revealed generalized myocardial hypomotility with asynergy. Cardiac manifestations of TTP vary widely, with prior research reporting symptoms such as asymptomatic elevation of cardiac biomarkers, chest pains, arrhythmias, heart failure, myocardial infarction, and even sudden cardiac death (24). Among patients with myositis complicated by TMA, heart failure was found to be the cause of death in 3 out of 13 patients (13).…”

Cerebral Infarction in Immune Thrombotic Thrombocytopenic Purpura Is Associated with Old Age, Hypertension, Smoking, and Anti-ADAMTS13 Ig, But Not with Mortality

“…4 The iTTP patients may present with a thrombotic event, either initially or during the course of treatment, including ischemic cerebral infarction (i.e., stroke), [5][6][7] and acute myocardial infarction (MI). [8][9][10] The clinical presentation may range from mild nonspecific symptoms, including weakness, fatigue, headache, nausea and vomiting to stroke, MI and acute renal failure, and death. 11,12 The classic pentad with fever, thrombocytopenia, hemolytic anemia, neurological signs, and renal failure occurs in less than 10% of iTTP patients.…”