Surgical repair of a ventricular septal rupture and CABG in a 67-year-old female was complicated by fatal macrophage activation syndrome (ferritin 32155 ng/mL, 4H score 185).
Case Report (n=1)
This case report describes a rare and fatal instance of macrophage activation syndrome potentially triggered by cardiopulmonary bypass during ventricular septal rupture repair and CABG.
Abstract Introduction Macrophage activation syndrome (MAS) is a rare, severe, hyperinflammatory state with an incidence estimated to be 1.2 cases per million individuals. It is characterized by fever, pancytopenia, hepatosplenomegaly, and hyperferritinemia. It has a high mortality, being fatal in 20-53% of cases. Primary MAS is a result of a lack of natural killer cell cytotoxicity, rendering NK cells unable to control host cell proliferation. Secondary MAS is typically triggered by infectious, autoimmune, or malignant etiologies. Both subtypes result in uncontrolled activation of macrophages and T-cells. Here, we present a case of MAS in the setting of surgically repaired ventricular septal rupture (VSR) and coronary artery bypass graft (CABG). Case A 67-year-old female with rheumatoid arthritis (RA) on immunosuppressive medications and interstitial lung disease presented to the emergency room after experiencing chest pain and malaise. Work-up revealed significantly elevated troponin and elevated lactic acid. Chest CTA suggested an apical VSR. This was confirmed on doppler echocardiography. Due to continuing deterioration, the patient was taken to the cardiac catheterization lab for mechanical support. Initially, an Impella CP was placed. Due to worsening cardiogenic shock, her mechanical support was increased to an Impella 5.5 and RP. After stabilization, she was taken for VSR repair and CABG. Her post-operative course was complicated by MAS and significant acidosis, requiring continuous renal replacement therapy. Diagnosis of MAS in this patient was based on a history of rheumatoid arthritis, elevated ferritin level at 32155 ng/mL, a 4H score of 185, and significantly elevated CD25. The patient was treated with steroids; however, her condition continued to worsen and the patient’s family opted to withdraw care. Discussion Secondary MAS is typically triggered by infection, autoimmune disease, or malignant etiologies.This is a unique case of MAS triggered by CABG and VSR repair. Furthermore, cardiopulmonary bypass (CPB) is known to cause significant vasoplegia and systemic inflammatory response postoperatively via surgical trauma, exposure to the CPB circuit, and reperfusion injury. There may be a connection between the pathophysiology of vasoplegia caused by CPB and the etiology of the hyperinflammatory state that characterizes MAS; however, more research is required to establish a definitive connection. This abstract is funded by: None
Wood et al. (Fri,) conducted a case report in Macrophage activation syndrome complicating ventricular septal rupture repair and CABG (n=1). Ventricular septal rupture repair and CABG was evaluated. Surgical repair of a ventricular septal rupture and CABG in a 67-year-old female was complicated by fatal macrophage activation syndrome (ferritin 32155 ng/mL, 4H score 185).