In this prospective study of consecutive patients aged ≤60 years presenting with unexplained AVB, we found that 11/32 (34%) had previously undiagnosed CS. Among patients with CS, 3/11 had adverse clinical outcomes compared with 0/21 (P = 0.011). Our data suggest that all patients aged ≤60 years with unexplained AVB should be investigated for CS. Moreover, patients diagnosed with CS should be closely followed.