A 54 year old female with a diagnosis of Erdheim-Chester disease under therapy with dabrafenib that presents with clinical signs of heart failure. After discontinuing the offending medication and initiating Guideline Directed Medical Therapy for Heart Failure with Reduced ejection Fraction, the clinical picture improved. ECD is a type of histiocytosis characterized by a multisystem infiltration, creating a wide range of pathological findings and presentations. Deposited histiocytes can induce a local cytokine cascade, eventually resulting in tissue damage. Our patient had imaging findings of tissue infiltration in the aorta and the mitral valve. This disease is driven by the MAPK pathway, and therefore, BRAF and MEK inhibitors have been established as the first line therapeutic. Inhibition of the MAPK pathway negatively interferes with cardiovascular signaling and has the potential to induce dysfunction. Dabrafenifb’s cardiotoxicity mechanism is still incompletely understood, and therefore the reversibility has not been established. There are no current therapeutic guidelines that match our patient’s clinical scenario, and therefore it is our responsibility to report the case, the installed management and the outcome in order to expand our scientific understanding of the condition.

Santiago Saenz Ancira obtained his medical degree with honors from the Mexican Faculty of Medicine of La Salle University. He is an independent researcher with experience in cardiology related scientific publications in high impact journals, including Frontiers, the European Heart Journal and the Journal of the American College of Cardiology. He has over 10 publications and over 15 participations in international medical conferences, including the WSC 2023, the AHA Scientific Sessions 2023, the ACC Latin America/Cardio Academic 2022, the AKI& CRRT 2022 and the ASN Kidney Week 2022.  He was a main collaborator in many of the scientific protocols currently taking place at the National Institute of Cardiology “Ignacio Chavez” at Mexico City.