Electrocardiographic Abnormalities in Patients With Hutchinson-Gilford Progeria Syndrome

Abstract

We read with great interest the study by Prakash et al,1 which aimed to describe the prevalence and natural history of cardiac abnormalities in patients with Hutchinson-Gilford progeria syndrome (HGPS). They conducted a cross-sectional study to describe several cardiovascular parameters in, to our knowledge, the largest cohort to date of patients with HGPS. They reported that electrocardiographic (ECG) abnormalities were rare (2 of 27 patients) and only presented during the second decade of life. The latter is in sharp contrast with our recent report,2 which demonstrated overt repolarization abnormalities in 7 of 15 patients with HGPS at advanced disease stages. In our study, patients with HGPS exhibited significant T-wave flattening, which was exacerbated as the disease progressed. More specifically, we analyzed the T-wave alterations rigorously using a newly developed, rate-independent T-flattening score that enabled us to quantify T-wave flatness using lead II and precordial V5 tracings.3 Importantly, similar to patients with HGPS, progeroid mice showed age-dependent repolarization abnormalities manifested as significant T-wave flattening compared with control mice.2