Myocardial necrosis in patients with thrombotic thrombocytopenic purpura: pathophysiology and rationale for specific therapy

Abstract

Myocardial necrosis is now recognized as a common feature of thrombotic thrombocytopenic purpura (TTP), usually due to platelet plugging in the microvasculature. Despite reports that TTP patients with myocardial damage have higher morbidity and mortality, there are no established guidelines for managing these high‐risk patients. The universal occurrence of thrombocytopenia and variable findings including renal dysfunction present unique challenges in this setting. Established therapies including plasma exchange and immunosuppression are the mainstay of therapy for all TTP patients. For the subset of patients with myocardial damage, therapy with more potent antiplatelet drugs, agents that enhance NO availability and alleviate vasospasm and drugs that suppress von Willebrand factor levels may have additional benefit. However, clinical trials are needed to optimize therapy for this subset of TTP patients.

Cardiovascular Manifestations in Patients With Thrombotic Thrombocytopenic Purpura: A Single‐center Experience

“…3 -5 Myocardial necrosis is now recognized as a common feature of TTP, usually due to platelet plugging in the microvasculature. 6 Presented at the American College of Chest Physicians Annual Meeting, San Diego, California, October 31-November 5, 2009. The present article reports the incidence of myocardial infarction, supraventricular tachyarrhythmias, congestive heart failure, and mortality in patients admitted to an academic institution with the diagnosis of TTP.…”

“…16 Sane et al recommend that an echocardiogram should be obtained for all patients with a positive troponin or elevated brain natriuretic peptide level. 6 There is some evidence of recovery of left ventricular function over 1 to 4 weeks with plasmapheresis. 10,17 Cardiac involvement in patients with TTP is common and may be an important cause of continuing mortality.…”

ST segment elevation myocardial infarction as a presenting feature of thrombotic thrombocytopenic purpura

“…Pathologically, focal areas of necrosis and hemorrhage may be seen in the pancreas, adrenals, heart, brain and kidneys. [3–5] Although myocardial injury and necrosis are observed in a large number of patients with TTP, it is infrequently the initial presentation,[6] and most likely thought to be due to microthrombi from massive platelet aggregation than plaque rupture–thrombosis cascade. [7] Various studies have determined the incidence of myocardial infarction in TTP to range from 15–41%.…”

Cardiac involvement in thrombotic microangiopathies

“…В настоя-щее время сердце рассматривается как один из основных (наряду с головным мозгом) органов-мишеней микроциркуляторного тромбообразования при ТТП, а его поражение -как неблаго-приятный прогностический фактор и важная причина смерти у этого контингента [3,7]. При аутопсийном исследовании в боль-шинстве случаев выявляют тромбоз интрамиокардиальных арте-риол и капилляров и очаги некроза миокарда в отсутствие пато-логии коронарных артерий, реже -кровоизлияния в миокард и очаговый фиброз миокарда [7,32,33].…”

“…Поражение сердца наиболее часто представлено при АГУС ИМ, кардиомиопатией и СН [35,36]. Однако, как и при ТТП, описаны выпот в полость перикарда с тампонадой сердца, нарушения ритма и проводимости, кардиогенный шок, синкопальные состояния, не связанные с поражением легких и ЦНС, а также внезапная смерть [3,32,36]. Последняя, по-видимому, также может быть обусловлена аритмиями, поскольку при аутопсийном исследовании миокарда умерших пациентов с ТТП выявлен тромбоз сосудов в области атриовентрикулярного узла и пучка Гиса [7,37,38].…”