| Right ventricular dysplasia: the Mayo Clinic experience. | |
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MedLine Citation:
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PMID: 7776713 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To determine the initial clinical manifestations and echocardiographic features of right ventricular dysplasia as encountered in a major cardiovascular referral center in the United States. DESIGN: We conducted a retrospective study of cases of right ventricular dysplasia diagnosed at the Mayo Clinic between January 1978 and January 1993. MATERIAL AND METHODS: In an institutional data-base search, we identified 20 patients with right ventricular dysplasia. Echocardiographic, electrophysiologic, Holter monitoring, cardiac catheterization, and endomyocardial biopsy results were analyzed. The mean duration of follow-up was 7 years. RESULTS: In the 12 female and 8 male patients (mean age, 30 years; range, 3 to 60), the initial manifestations of right ventricular dysplasia included ventricular arrhythmia (45%), congestive heart failure (25%), heart murmur (10%), asymptomatic (10%), complete heart block (5%), and sudden death (5%). First-order relatives were affected in 30% of the patients. Ventricular tachycardia with morphologic features of left bundle branch block was inducible in seven of nine patients. On Holter monitoring, all but 2 of 15 patients studied had frequent ventricular ectopic activity (Lown grade 2 or more). Characteristic fatty infiltration of the myocardium was present in 7 of 13 right ventricular biopsy specimens. Inordinate right ventricular enlargement was present in 60% of the patients at first echocardiographic assessment and in two other patients on follow-up assessment. Variable left ventricular involvement was noted in 50% of the cases. During the follow-up period, four patients died: two died suddenly, one died of congestive heart failure, and one died of respiratory failure after a coronary artery bypass operation. Of the 16 living patients, 8 are doing well, 3 have an implanted cardiac defibrillator, 3 are receiving antiarrhythmic agents, and 2 have undergone cardiac transplantation because of progressive biventricular failure. CONCLUSION: Patients with right ventricular dysplasia have varied initial manifestations and a high frequency of serious cardiovascular symptoms and complications. |
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Authors:
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I J Kullo; W D Edwards; J B Seward |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Mayo Clinic proceedings. Mayo Clinic Volume: 70 ISSN: 0025-6196 ISO Abbreviation: Mayo Clin. Proc. Publication Date: 1995 Jun |
Date Detail:
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Created Date: 1995-07-07 Completed Date: 1995-07-07 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0405543 Medline TA: Mayo Clin Proc Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 541-8 Citation Subset: AIM; IM |
Affiliation:
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Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Child Child, Preschool Death, Sudden, Cardiac / etiology Echocardiography Electrocardiography, Ambulatory Female Follow-Up Studies Heart Block / diagnosis, etiology, physiopathology Heart Catheterization Heart Failure / diagnosis, etiology, physiopathology Heart Murmurs / diagnosis, etiology, physiopathology Heart Ventricles / pathology* Humans Hypertrophy, Right Ventricular / complications, diagnosis*, physiopathology Male Middle Aged Retrospective Studies Tachycardia, Ventricular / diagnosis, etiology, physiopathology Ventricular Dysfunction, Right / complications, diagnosis*, physiopathology Ventricular Function, Right |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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