Conference proceeding
Myotonic dystrophy and cardiac conduction system abnormalities: How physician awareness affects the patient outcome?
HEART DISEASE: PATHOPHYSIOLOGY, EVALUATION AND MANAGEMENT, pp.217-221
01 Jan 2012
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
In this case series, we present 7 consecutive patients with myotonic dystrophy presenting with recurrent lightheadedness, dizziness, near-syncope and fatigue. All had EKG evidence for intraventricular conduction delay but only 5 had first-degree atrioventricular block. The HV intervals were greater than 72 msec in 5 patients. Four had inducible sustained ventricular tachycardia during electrophysiology studies requiring immediate cardioversion and received ICD. Two underwent permanent pacemaker placement due to complete heart block and infra-Hissian block. These electrophysiological findings are typical in patients with myotonic dystrophy.
Five out of 7 patients were referred for arrhythmia evaluation in the last 2 years, suggesting an unusual surge of referrals, which may be attributed to an increased awareness on the part of primary care physicians to the cardiac manifestations of myotonic dystrophy.
Since one-third of the cardiac deaths in these patients are sudden, prophylactic pacemaker placement is recommended, despite minimal conduction system abnormality. However, the common practice is to identify patients at high risk of conduction abnormalities by electrophysiology studies and to then provide those patients at increased risk with prophylactic invasive strategies.
There is a clear need for identifying the subgroup of patients with myotonic dystrophy and to intervene on behalf of those at high-risk of cardiac death with prophylactic invasive strategies. Identifying such patients may be difficult unless there is an increase in awareness among our primary care physicians.
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Details
- Title
- Myotonic dystrophy and cardiac conduction system abnormalities: How physician awareness affects the patient outcome?
- Creators
- S. B. Thapamagar - Drexel Univ, Easton Hosp, Internal Med Residency Program, Easton, PA 18042 USAN. Singh - Drexel Univ, Easton Hosp, Internal Med Residency Program, Easton, PA 18042 USAS. R. Walker - Drexel UniversityK. Khalighi - Drexel Univ, Easton Hosp, Internal Med Residency Program, Easton, PA 18042 USA
- Contributors
- A Kimchi (Editor)
- Publication Details
- HEART DISEASE: PATHOPHYSIOLOGY, EVALUATION AND MANAGEMENT, pp.217-221
- Publisher
- Medimond S R L
- Number of pages
- 5
- Resource Type
- Conference proceeding
- Language
- English
- Academic Unit
- General Internal Medicine; Medicine (Graduate)
- Identifiers
- 991019170511704721
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InCites Highlights
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- Web of Science research areas
- Cardiac & Cardiovascular Systems