Course of cardiac involvement in ECD and associated outcome. (A) Course of cardiac involvement in ECD according to the type of cardiac lesion. (B) Survival curve according to the course of cardiac involvement Kaplan-Meier survival curves for patients with regression (partial or complete) and without regression (stable or worsening) of their cardiac involvement during follow-up. (C) Survival curve according to treatment regimen. Kaplan-Meier survival for patients with and without vemurafenib as frontline therapy. The date of inclusion was taken as the date of last CMR. Follow-up was considered to end on the date of death or last medical visit. The last data were collected on 12 May 2021. The y-axis represents the number of patients. Median survival was 6 years, and the 5-year survival was 66%. HR, hazard ratio; LA, left atrium; LAVS, left atrioventricular sulcus; RA, right atrium; RAVS, right atrioventricular sulcus.