Abstract Aims Transthyretin amyloid cardiomyopathy (ATTR-CM) is characterized by increased wall thickness, diastolic dysfunction and progressive heart failure symptoms. The disease may infiltrate the conduction system leading to conduction disturbances requiring an implantation of permanent cardiac pacemaker (PM), but the extent is unknown. Here, we report the prevalence of ATTR-CM in patients ≥65 years with PM. Methods and results In this prospective, cross-sectional single-centre study patients were recruited from our out-patient pacemaker clinic. Eligibility criteria were age above 65 years, permanent cardiac pacemaker and competent to give informed consent. Patients underwent echocardiography at the pacemaker visit and were referred to 99mTc-DPD-scintigraphy (DPD) and blood samples if septum thickness was ≥12 mm, defined as left ventricular hypertrophy (LVH). Fifty eight of the 128 patients had LVH on echocardiography. Eleven patients had a DPD-scintigraphy based diagnosis of ATTR-CM, which represent 19% of patients with LVH and 9% of the total cohort. Patients diagnosed with ATTR-CM had higher concentrations of cardiac biomarkers (P < 0.001), higher E/E′ (P = 0.001), and lower global longitudinal strain (P = 0.003) on echocardiography and more heart failure symptoms (P = 0.001). Conclusions The prevalence of ATTR-CM in elderly patients with PM and LVH on echocardiography was 19%.