Polycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathy

► Pkd2 mutant zebrafish display reduced cardiac output, atrioventricular block, and edema. ► Pkd2 mutant hearts show prolonged Ca2 + transients and Ca2 + alternans. ► Pacing increases resting Ca2 + levels and decreases Ca2 + cycling in pkd2 mutant hearts. ► ADPKD is associated with an increased prevalence of IDCM. ► Patients with PKD2 mutations are more likely than PKD1 patients to suffer from IDCM.