JOURNAL ARTICLE
RESEARCH SUPPORT, NON-U.S. GOV'T
RESEARCH SUPPORT, U.S. GOV'T, P.H.S.
Electrophysiologic abnormalities in AL (primary) amyloidosis with cardiac involvement.
Journal of the American College of Cardiology 1997 October
OBJECTIVES: This study sought to determine the spectrum of electrophysiologic abnormalities found in patients with cardiac involvement due to AL (primary) amyloidosis and to evaluate the prognostic implications, particularly in relation to subsequent sudden death.
BACKGROUND: Only case reports, but no series of invasive electrophysiologic studies, exist in patients with cardiac AL.
METHODS: Twenty-five patients with biopsy-proven AL and cardiac involvement underwent standard invasive electrophysiologic studies.
RESULTS: The function of the sinus and the atrioventricular node was preserved in most patients, but the infra-His (HV) conduction times were usually abnormal. The mean (+/-SD) HV interval for the 25 patients was 79 +/- 18 ms (range 50 to 110), and 23 patients (92%) had an abnormally prolonged interval (> 55 ms). Marked HV prolongation (> or = 80 ms) occurred in 12 patients, 6 of whom had an interval > or = 100 ms. Among the 23 patients who died during follow-up, HV prolongation was the sole independent predictor of sudden death by multivariate analysis (p = 0.05).
CONCLUSIONS: Patients with cardiac AL are prone to disease in the His-Purkinje system. Prolongation of the HV interval is common and may not be suspected from the surface electrocardiogram in the presence of a narrow QRS complex. These patients have a high prevalence of sudden death, of which the HV interval is an independent predictor. The association of HV prolongation and sudden death is probably multifactorial, representing either a marker of severe myocardial infiltration with an increased propensity to lethal ventricular arrhythmias or electromechanical dissociation, or indicating severe conduction system disease eventually leading to complete atrioventricular block and bradycardic death.
BACKGROUND: Only case reports, but no series of invasive electrophysiologic studies, exist in patients with cardiac AL.
METHODS: Twenty-five patients with biopsy-proven AL and cardiac involvement underwent standard invasive electrophysiologic studies.
RESULTS: The function of the sinus and the atrioventricular node was preserved in most patients, but the infra-His (HV) conduction times were usually abnormal. The mean (+/-SD) HV interval for the 25 patients was 79 +/- 18 ms (range 50 to 110), and 23 patients (92%) had an abnormally prolonged interval (> 55 ms). Marked HV prolongation (> or = 80 ms) occurred in 12 patients, 6 of whom had an interval > or = 100 ms. Among the 23 patients who died during follow-up, HV prolongation was the sole independent predictor of sudden death by multivariate analysis (p = 0.05).
CONCLUSIONS: Patients with cardiac AL are prone to disease in the His-Purkinje system. Prolongation of the HV interval is common and may not be suspected from the surface electrocardiogram in the presence of a narrow QRS complex. These patients have a high prevalence of sudden death, of which the HV interval is an independent predictor. The association of HV prolongation and sudden death is probably multifactorial, representing either a marker of severe myocardial infiltration with an increased propensity to lethal ventricular arrhythmias or electromechanical dissociation, or indicating severe conduction system disease eventually leading to complete atrioventricular block and bradycardic death.
Full text links
Trending Papers
Bacteremia with gram positive bacteria - when and how do I need to look for endocarditis?Clinical Microbiology and Infection 2023 August 32
Abdominal wall closure.British Journal of Surgery 2023 September 16
Antireflux surgery versus antireflux medication and risk of esophageal adenocarcinoma in patients with Barrett's esophagus.Gastroenterology 2023 September 9
Diagnosis and management of prolactin-secreting pituitary adenomas: a Pituitary Society international Consensus Statement.Nature Reviews. Endocrinology 2023 September 6
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
Read by QxMD is copyright © 2021 QxMD Software Inc. All rights reserved. By using this service, you agree to our terms of use and privacy policy.
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app