[Right cardiac insufficiency disclosing systemic mastocytosis]

Area of Science:

• Cardiology
• Dermatology
• Hematology

Background:

• Mast cell activation disorders can present with diverse systemic manifestations.
• Cutaneous-systemic mast cell disease (CSMCD) involves skin and internal organ involvement.
• Cardiac dysfunction is an under-recognized complication of systemic mast cell disease.

Observation:

• A 54-year-old woman presented with right heart failure.
• The patient was diagnosed with contemporaneous cutaneous-systemic mast cell disease.
• Cardiac symptoms improved alongside vasomotor symptoms of mast cell disease.

Findings:

• The study investigated the link between mast cell mediators and cardiovascular manifestations.
• Pathogenetic hypotheses for cardiac involvement in CSMCD were explored.
• Concomitant regression of cardiac and vasomotor symptoms suggests a shared pathway.

Implications:

• This case underscores the importance of considering systemic mast cell disease in patients with unexplained cardiac failure.
• Understanding mast cell mediators may reveal novel therapeutic targets for cardiac complications.
• Further research is needed to elucidate the precise mechanisms of cardiovascular involvement in CSMCD.