P0885 - Heart Failure in a Previously Healthy Adolescent Presenting with Lower Extremity Rash

Introduction
Cardiac involvement is a potential sequela of many rheumatologic conditions, including lupus and vasculitides. Intracardiac thrombus formation is rare and can be seen in the presence of severe ventricular dysfunction, as well as with predisposing systemic illnesses, such as rheumatologic/inflammatory conditions, including those with antiphospholipid antibody (ALPA). Catastrophic antiphospholipid antibody syndrome (CAPS) is the most feared entity in these patients. This diffuse thrombotic disease of the microvasculature has high mortality.

Case Description
Patient is a previously healthy 13 year-old-female who presented as a transfer from an outside ED with 2-3 weeks of cough and three days of bilateral leg rash concern for “covid toes.” She described tingling and a cold sensation in her legs for several days, which acutely worsened while on the treadmill, prompting the family to seek evaluation. She denied dyspnea or chest pain, though she did report new onset fatigue with exertion. A chest x-ray (CXR) demonstrated a pleural effusion and cardiomegaly. Her creatinine was mildly elevated and SARS-COV-2 was negative. On exam, her legs were erythematous-to-purple and cool with 1+ distal pulses. Her symptoms and CXR prompted an electrocardiogram, which revealed right axis deviation, low voltages and diffuse t-wave flattening. Echocardiography demonstrated biventricular dilation and severe dysfunction in addition to a large left ventricular (LV) thrombus. She was transferred to the Cardiac ICU for initiation of heparin, nicardipine and milrinone. She underwent a kidney biopsy on hospital day 4, which revealed focal segmental glomerulosclerosis. Her course was complicated by systemic embolization of LV thrombus 5 days after admission, resulting in ischemic arterial infarcts in the kidneys, spleen, abdominal vasculature and occlusive thromboses in the bilateral lower extremities. She underwent emergent suction thrombectomy and tPA with successful restoration of distal extremity perfusion. On hospital day 6, she underwent plasma exchange while awaiting results of ALPA screen.

Discussion
Her presentation was concerning for the possibility of myocarditis and resultant ventricular dysfunction secondary to an underlying rheumatologic/systemic inflammatory syndrome Due to the high mortality associated with CAPS, plasma exchange was initiated prior to ALPA results. Ultimately, her ALPA screen was negative and further evaluation is currently underway.

Conclusion
Pediatric patients with severe biventricular dysfunction may have minimal symptoms, and a high index of suspicion for cardiac dysfunction in patients with consistent symptoms and concerning laboratory/imaging findings is key. In the presence of documented thrombus, or suspected sequelae of thrombus where CAPS is suspected, treatment initiation with anticoagulants, anti-inflammatory medications or plasma exchange may be required before definitive diagnosis can be established.