Table 3.
First-degree relatives mutation carriers (n = 1) | More distant relatives mutation carriers (n = 3) | Obliged mutation carriers (n = 4) | |
---|---|---|---|
TFC 2010 or clinical data if TFC not available | Woman with borderline AC at 42 years old, normal CMR. | Man with definitive AC (LDAC) since 21 years old with severe LV LGE. Woman with definitive AC (LDAC) since 19 years old with severe LV LGE. Woman with possible AC at 63 years old with mild LV LGE. |
Woman with DCM since her first pregnancy at 33 years old, dead at 54 years old. Man with cardiomyopathy, dead at 70 years old. Man with no history of heart disease, dead at 48 years old. Woman with no history of heart disease, SCD at 61 years old. |
Clinical management | β-blockers, regular follow-up, cascade screening extended to her offspring. | β-blockers, heart failure optimal medical treatment, two ICDs, regular follow-up, genetic counselling for future pregnancies. | – |
TFC 2010: Task Force Criteria released in 2010; AC: arrhythmogenic cardiomyopathy; CMR: cardiac magnetic resonance; LDAC: left ventricular dominant arrhythmogenic cardiomyopathy; LV: left ventricle; LGE: late gadolinium enhancement; DCM: dilated cardiomyopathy; ICD: implanted cardioverter-defibrillator; SCD: sudden cardiac death.