title: [Hidden intracardiac conduction disturbances and their spontaneous course in patients with progressive muscular dystrophy].
abstract: In patients with progressive muscular dystrophy (PMD) invasive electrophysiologic studies can detect hidden intracardiac conduction disturbances. The aim of this study was a long-term follow-up of these patients. Twelve consecutive patients (9 m, 3 f, age 28 +/- 4 yrs) without cardiac symptoms and with normal echocardiographic findings were included in the study. They suffered from different stages of PMD type Erb (n = 4), Becker-Kiener (n = 4), Duchenne (n = 2) and Landouzy-Déjerine (n = 2). At the beginning of the study all patients underwent an invasive electrophysiologic study (EPS). The follow-up of 5.5 yrs included regular clinical visits, ECGs, and Holter recordings (every 3 months) as well as an echocardiography every 6 months. In 4 patients the EPS revealed a hidden interatrial conduction disturbance (AHRS-ACS 120 +/- 18 ms), and in 10 pts an infrahisian conduction disturbance was found (HV max. 156 +/- 4 ms). Conduction defects were seen independently from the type of PMD and the stage of the disease. During the follow-up the initially hidden interatrial conduction disturbance became evident in the surface ECG in 2 of 4 pts. One of them developed paroxysmal atrial fibrillation. Five of 10 pts with an initially hidden infrahisian conduction disturbance developed an AV block grade I-III and in one case additionally a bundle branch block. Four of these pts--whose PMD showed progression or who developed congestive cardiomyopathy--needed pacemaker implantation because of a first-degree AV block + bifascicular bundle branch block (n = 1), a Mobitz II second-degree AV block (n = 1) or a third-degree AV block (n = 2). None of the pts with normal findings at the EPS showed abnormal p-waves, an AV block, or an intraventricular conduction disturbance during the follow-up. We conclude that intracardiac conduction disturbances, especially infrahisian defects including high-degree AV blocks, are a common finding in pts with PMD. Therefore a regular cardiological screening including an ECG and a Holter recording is reasonable in these patients.
authors.affiliation: II. Medizinische Klinik Johannes-Gutenberg-Universität, Mainz.
authors.forename: E
authors.initials: E
authors.lastname: Himmrich
authors.forename: S
authors.initials: S
authors.lastname: Popov
authors.forename: A
authors.initials: A
authors.lastname: Liebrich
authors.forename: S
authors.initials: S
authors.lastname: Rosocha
authors.forename: C
authors.initials: C
authors.lastname: Zellerhoff
authors.forename: B
authors.initials: B
authors.lastname: Nowak
authors.forename: O
authors.initials: O
authors.lastname: Przibille
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000209:etiology*; D001281:Atrial Fibrillation / Q000175:diagnosis / Q000209:etiology; D002037:Bundle-Branch Block / Q000175:diagnosis / Q000209:etiology; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis / Q000209:etiology; D004452:Echocardiography; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D004594:Electrophysiology; D005260:Female; D005500:Follow-Up Studies; D006327:Heart Block / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009136:Muscular Dystrophies / Q000150:complications / Q000503:physiopathology*; D010138:Pacemaker, Artificial; D013997:Time Factors