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Background Macrophage activating syndrome (MAS) is a form of hemophagocytic lymphohistiocytosis (HLH), a rare complication of autoimmune disease that is characterized by cytokine storm and multiorgan failure. Case summary A 32-year-old male presented with acutely decompensated pulmonary arterial hypertension and right heart failure secondary to MAS. The patient was immediately started on inhaled and intravenous epoprostenol, vasopressors and dexamethasone and anakinra were administered. Despite the therapies given, the patient's condition continued to decline, and he was placed on veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support. Over a few days, his clinical condition improved, and he was decannulated from VA-ECMO and later transitioned oral treprositinil and was discharged home. Due to its non-specific clinical manifestations, the diagnosis of MAS depends on high clinical suspicion and initial laboratory work up such as thrombocytopenia, transaminitis, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, etc. In our patient, MAS led to decompensated Pulmonary Arterial Hypertension (PAH) leading to right heart failure that was refractory to inhaled and intravenous epoprostenol and vasopressors and required VA-ECMO as a bridge to recovery while his MAS was managed by anakinra and dexamethasone. Conclusion MAS can result in acute decompensation of PAH and right heart failure. Besides RV failure management, immunosuppressants such as anakinra, etoposide, etc. should be utilized early in the management of MAS. In refractory right heart failure, VA-ECMO can be considered as a bridge to recovery. There is a paucity of literature supporting the utilization of VA-ECMO in the management of refractory right heart failure caused by MAS in adults and much of the data stems from pediatric studies. This case serves as a fine example of successful use of VA-ECMO in adult population.

BACKGROUND: Obese subjects are at higher risk of development and progression of ARDS. There are limited data regarding mechanical ventilation practices and use of adjunctive therapies in subjects with ARDS across different obesity classes. We hypothesized that the adherence to lung-protective ventilation would be worse with rising body mass index class in patients with ARDS. METHODS: We conducted a retrospective observational study of subjects with ARDS. We evaluated the differences in ventilator settings, airway pressures, gas exchange, use of rescue therapies, length of hospital stay, and mortality among subjects based on the obesity classes of the WHO. RESULTS: The study included 613 subjects with ARDS: 21.4% were normal weight, 25% were overweight, and 53.7% were obese; 33.3% of the obese subjects met criteria for class I-II obesity, while 20.4% were class III obese (morbid obesity). On day 1, 53% of subjects with class III obesity had tidal volumes > 8 mL/kg, compared to 26% of the subjects with normal weight. In addition, 48% of the morbidly obese subjects received at least one rescue therapy as compared to 37% of normal weight subjects and 36% of overweight subjects. There were significant differences in the use of rescue therapies among the groups. In a multivariable model, subjects with class III obesity were significantly more likely to receive tidal volume > 8 mL/kg predicted body weight on day 1 when compared with subjects with normal weight (odds ratio 3.14, 95% CI 1.78-5.57). There was no difference in length of stay in ICU or hospital, duration of mechanical ventilation, or adjusted ICU or hospital mortality among the 4 groups. CONCLUSIONS: In this study, the risk of exposure to higher tidal volumes and the need for specific rescue therapies rose with higher classes of obesity in subjects with ARDS. More research is needed to identify how to better implement lung-protective ventilation in patients with obesity.

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