(220) Stress Cardiomyopathy in HIV-related Thrombotic Thrombocytopenic Purpura Reversed by Plasmapheresis

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy associated with organ ischemia and dysfunction. Acquired deficiency of a‐disintegrin‐and‐metalloproteinase‐with‐thrombospondin‐motifs 13 (ADAMTS‐13) protease leads to TTP and may be due to certain medications, autoimmune disorders and viral infections including HIV, coronavirus, cytomegalovirus and hepatitis. HIV-associated TTP (HIV-TTP) is usually seen in untreated or advanced HIV patients rendering it a higher morbidity and mortality.
Severe TTP has also been implicated in acute stress/takotsubo cardiomyopathy in lupus and pregnancy. We report the first case of TTP associated cardiomyopathy and acute heart failure in an HIV patient that resolved with plasmapheresis.

Description: A 48 y-o man with untreated HIV for 12 years presented with loss of consciousness requiring intubation. He had headaches, fever and altered mentation foe 1 week. Labs showed hemoglobin 5.2 g/dl, 3+ schistocytes, reticulocyte 13%, WBC 9 k/cumm, platelets of 18 K/cumm, normal PT, PTT and fibrinogen. Creatinine 0.8 mg/dl. LDH 1912 u/l. Neuroimaging showed no ischemic or hemorrhagic. His HIV viral load was 174,000 copies/ml, CD4 158/mm3. There were no signs of bleeding on CT scans of chest and abdomen. ADAMTS-13 was < 5%. Echocardiogram showed dilated left ventricle with poor systolic function and ejection fraction 18%. Troponin was 1300 ng/ml. EKG had T wave inversion. Workup for acute bacterial and viral infections was negative and empiric antibiotics were stopped.
Plasma exchange of ~3000 ml was performed daily for 7 days following which the patient recovered clinically and biochemically (LDH < 200, platelets > 150, absent schistocytes, Hgb > 9). Echocardiogram showed EF of 74% and return of LV function on Day 10.

Discussion: Microthrombosis in TTP is from excess von Willebrand factor (VWF) due to ADAMTS-13 deficiency. Acute myocardial injury may be mediated via endothelial & complement activation with accumulation of proinflammatory C5b‐9 membrane attack complexes causing endotheliitis causing apoptosis and activation of inflammatory pathways. These leads to platelet‐rich VWF thrombi occlusion of the coronary/endocardial microcirculation.
Ours is a unique case of HIV-TTP acute cardiomyopathy that resolved completely with plasmapheresis.