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Macrophage activating syndrome causing decompensated right heart failure
Mwelwa Chizinga1, Saminder Singh Kalra1, Ayoub Innabi1
1University of Florida, Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, Gainesville, FL, USA.
Respiratory Medicine Case Reports
|August 17, 2021
Summary
Macrophage activating syndrome (MAS) can cause severe pulmonary arterial hypertension and right heart failure. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) offers a bridge to recovery in refractory cases, as demonstrated in this adult patient.
Area of Science:
- Cardiology
- Rheumatology
- Critical Care Medicine
Background:
- Macrophage activating syndrome (MAS) is a life-threatening hemophagocytic lymphohistiocytosis (HLH) complication of autoimmune diseases.
- MAS triggers cytokine storm and multiorgan failure, including cardiovascular compromise.
Observation:
- A 32-year-old male with MAS developed acutely decompensated pulmonary arterial hypertension (PAH) and right heart failure.
- Conventional therapies including epoprostenol and vasopressors were ineffective, necessitating veno-arterial extracorporeal membrane oxygenation (VA-ECMO).
Findings:
- MAS precipitated refractory right heart failure, requiring VA-ECMO as a bridge to recovery.
- Early immunosuppression with anakinra and dexamethasone was crucial for managing MAS.
- Successful VA-ECMO decannulation and transition to oral treprostinil were achieved.
Implications:
- This case highlights VA-ECMO's utility in managing adult MAS-induced refractory right heart failure.
- Early consideration of VA-ECMO and immunosuppressants is vital for severe MAS cases.
- Further research is needed to support VA-ECMO use in adult MAS patients.
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