Abstract

Background: Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH) have attacks of complement-mediated hemolysis that can be spontaneous or secondary to precipitating factors such as infections. During these episodes, these patients have an increased risk of thrombosis, typically venous and rarely arterial. We present a rare case of cardiac arrest due to ventricular fibrillation (VF) and inferior STEMI in a patient with PNH and a recent COVID infection. Case: 63 years old male patient with a medical history of PNH presented with typical chest pain, fever, cough, jaundice, and dark-colored urine. On examination, he was hemodynamically stable with jaundiced eyes. A few minutes later, he had cardiac arrest due to VF. He achieved return of spontaneous circulation (ROSC) after two defibrillation shocks. EKG showed inferior STEMI. Labs showed hemoglobin of 6.4 g/dl, elevated cardiac markers, serum LDH, and indirect bilirubin. Serum haptoglobin was < 1 mg/dl. His COVID 19 PCR test was positive. Decision making: He received 2 units of packed RBCs and underwent a coronary angiogram (CA) which revealed total proximal occlusion of the right coronary artery (Figure A). He underwent successful PCI by two drug-eluting stents (figure B). His peripheral blood immunophenotyping and flow cytometry was positive for loss of glycosylphosphatidylinositol-linked antigens and decreased expression of CD 59/14/24. He was started on Ravulizumab, a humanized monoclonal antibody complement five inhibitor. Conclusion: PNH and COVID increase the risk of thrombosis. Thrombosis mechanism in PNH includes platelets and coagulation system activation via complement cascade and impairment of the fibrinolytic system. COVID infection increases the risk of thrombosis by endothelial cell dysfunction and release of cytokines. Due to the patient’s acute presentation, he needed to undergo immediate CA and lifesaving PCI regardless of his hematological disorder or active COVID-19 infection.

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