A FATAL INTERSECTION: ANTIPHOSPHOLIPID ANTIBODY SYNDROME AND CARDIOGENIC SHOCK

Document Type

Conference Proceeding

Publication Date

4-1-2025

Publication Title

J Am Coll Cardiol

Keywords

steroid, adult, anticoagulation, antiphospholipid syndrome, cannulation, cardiogenic shock, cardiomyopathy, case report, catastrophic antiphospholipid syndrome, cesarean section, clinical article, conference abstract, coronary angiography, diagnosis, drug therapy, dyspnea, echocardiograph, echocardiography, extracorporeal oxygenation, female, granulation tissue, heart failure, heart left atrium, heart muscle biopsy, heart muscle ischemia, heart output, heart transplantation, heart ventricle function, HELLP syndrome, hemodynamics, hemolysis, hospitalization, human, hypertransaminasemia, intravenous catheter, intravenous drug administration, ischemic heart disease, left ventricular assist device, lung wedge pressure, oral drug administration, plasmapheresis, platelet count, systemic lupus erythematosus, therapy, valvular heart disease, vein thrombosis, veno-arterial ECMO

Abstract

Background: Cardiac involvement in antiphospholipid syndrome (APS) manifests as valvular heart disease, ventricular dysfunction, and thrombosis. Mechanical support (MCS) and advanced heart failure (HF) therapies may be necessary in patients who do not respond to conventional management. Case A 32-year-old woman with a history of APS and systemic lupus erythematosus presented with shortness of breath and was admitted for cardiogenic shock. One month prior, she underwent cesarean section delivery at 26-weeks of gestation for hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Echocardiogram revealed biventricular dysfunction. Invasive coronary angiography was negative for coronary artery ischemia or dissection. She was medically managed for decompensated HF but developed worsening shock despite vasopressor and inotropic support. Hemodynamic assessment revealed biventricular dysfunction (RA pressure 18 mmHg, wedge pressure 30 mmHg), with a reduced cardiac output of 3.54 L/min. Due to worsening hemodynamics, a peripherally cannulated transcaval left atrial veno-arterial extracorporeal membrane oxygenation (ECMO) was placed. Further evaluation for HF etiologies was unremarkable with a bland endomyocardial biopsy. Despite therapeutic systemic anticoagulation, her course was complicated by extensive thrombosis involving the arterial and venous catheters, and ECMO inflow cannula at the IVC extending into the left atrium. Decision-making Our patient developed refractory cardiogenic shock and extensive arterial and venous thrombosis concerning for catastrophic APS. She was treated with plasmapheresis, systemic steroids, and therapeutic anticoagulation with a modified higher intensity protocol. She underwent orthotropic heart transplantation ten days after hospitalization. Biopsy of the explanted heart revealed myocardium with extensive transmural necrosis and granulation tissue. Conclusion APS associated cardiomyopathy confers a poor prognosis and early MCS evaluation should be considered. Management requires a multidisciplinary approach that could include hematologic interventions and advanced HF therapies.

Volume

85

Issue

12

First Page

4348

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