Mitral valve prolapse and conduction defects in Ehlers-Danlos syndrome. 1977

W R Cabeen, and M J Reza, and R B Kovick, and M S Stern

A case of type 3 Ehlers-Danlos syndrome with associated mitral valve prolapse, right bundle-branch block, and left anterior fascicular block was studied, including His bundle electrocardiography and echocardiography. Mitral valve prolapse and HV prolongation were reported. The present study suggests that the basic underlying collagen defect in this syndrome is responsible for these findings, and that the association may be more widespread in the syndrome than currently acknowledged. The possibility also is considered that the connective tissue abnormalities of Ehlers-Danlos syndrome involving the heart may be the cause of some cases of idiopathic conduction system disease or valvular insufficiency.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D008944 Mitral Valve Insufficiency Backflow of blood from the LEFT VENTRICLE into the LEFT ATRIUM due to imperfect closure of the MITRAL VALVE. This can lead to mitral valve regurgitation. Mitral Incompetence,Mitral Regurgitation,Mitral Valve Incompetence,Mitral Insufficiency,Mitral Valve Regurgitation,Incompetence, Mitral,Incompetence, Mitral Valve,Insufficiency, Mitral,Insufficiency, Mitral Valve,Regurgitation, Mitral,Regurgitation, Mitral Valve,Valve Incompetence, Mitral,Valve Insufficiency, Mitral,Valve Regurgitation, Mitral
D002037 Bundle-Branch Block A form of heart block in which the electrical stimulation of HEART VENTRICLES is interrupted at either one of the branches of BUNDLE OF HIS thus preventing the simultaneous depolarization of the two ventricles. Fascicular Block,Anterior Fascicular Block,Bundle Branch Block,Left Bundle-Branch Block,Posterior Fascicular Block,Right Bundle-Branch Block,Anterior Fascicular Blocks,Block, Anterior Fascicular,Block, Bundle Branch,Block, Bundle-Branch,Block, Fascicular,Block, Left Bundle-Branch,Block, Posterior Fascicular,Block, Right Bundle-Branch,Blocks, Anterior Fascicular,Blocks, Bundle Branch,Blocks, Bundle-Branch,Blocks, Fascicular,Blocks, Left Bundle-Branch,Blocks, Posterior Fascicular,Blocks, Right Bundle-Branch,Branch Block, Bundle,Branch Blocks, Bundle,Bundle Branch Blocks,Bundle-Branch Block, Left,Bundle-Branch Block, Right,Bundle-Branch Blocks,Bundle-Branch Blocks, Left,Bundle-Branch Blocks, Right,Fascicular Block, Anterior,Fascicular Block, Posterior,Fascicular Blocks,Fascicular Blocks, Anterior,Fascicular Blocks, Posterior,Left Bundle Branch Block,Left Bundle-Branch Blocks,Posterior Fascicular Blocks,Right Bundle Branch Block,Right Bundle-Branch Blocks
D004452 Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Echocardiography, Contrast,Echocardiography, Cross-Sectional,Echocardiography, M-Mode,Echocardiography, Transthoracic,Echocardiography, Two-Dimensional,Transthoracic Echocardiography,2-D Echocardiography,2D Echocardiography,Contrast Echocardiography,Cross-Sectional Echocardiography,Echocardiography, 2-D,Echocardiography, 2D,M-Mode Echocardiography,Two-Dimensional Echocardiography,2 D Echocardiography,Cross Sectional Echocardiography,Echocardiography, 2 D,Echocardiography, Cross Sectional,Echocardiography, M Mode,Echocardiography, Two Dimensional,M Mode Echocardiography,Two Dimensional Echocardiography
D004535 Ehlers-Danlos Syndrome A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability. Cutis Elastica,Ehlers Danlos Disease,Ehlers-Danlos Disease,Danlos Disease, Ehlers,Disease, Ehlers Danlos,Disease, Ehlers-Danlos,Ehlers Danlos Syndrome,Syndrome, Ehlers-Danlos
D004562 Electrocardiography Recording of the moment-to-moment electromotive forces of the HEART as projected onto various sites on the body's surface, delineated as a scalar function of time. The recording is monitored by a tracing on slow moving chart paper or by observing it on a cardioscope, which is a CATHODE RAY TUBE DISPLAY. 12-Lead ECG,12-Lead EKG,12-Lead Electrocardiography,Cardiography,ECG,EKG,Electrocardiogram,Electrocardiograph,12 Lead ECG,12 Lead EKG,12 Lead Electrocardiography,12-Lead ECGs,12-Lead EKGs,12-Lead Electrocardiographies,Cardiographies,ECG, 12-Lead,EKG, 12-Lead,Electrocardiograms,Electrocardiographies, 12-Lead,Electrocardiographs,Electrocardiography, 12-Lead
D006327 Heart Block Impaired conduction of cardiac impulse that can occur anywhere along the conduction pathway, such as between the SINOATRIAL NODE and the right atrium (SA block) or between atria and ventricles (AV block). Heart blocks can be classified by the duration, frequency, or completeness of conduction block. Reversibility depends on the degree of structural or functional defects. Auriculo-Ventricular Dissociation,A-V Dissociation,Atrioventricular Dissociation,A V Dissociation,A-V Dissociations,Atrioventricular Dissociations,Auriculo Ventricular Dissociation,Auriculo-Ventricular Dissociations,Block, Heart,Blocks, Heart,Dissociation, A-V,Dissociation, Atrioventricular,Dissociation, Auriculo-Ventricular,Dissociations, A-V,Dissociations, Atrioventricular,Dissociations, Auriculo-Ventricular,Heart Blocks
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001145 Arrhythmias, Cardiac Any disturbances of the normal rhythmic beating of the heart or MYOCARDIAL CONTRACTION. Cardiac arrhythmias can be classified by the abnormalities in HEART RATE, disorders of electrical impulse generation, or impulse conduction. Arrhythmia,Arrythmia,Cardiac Arrhythmia,Cardiac Arrhythmias,Cardiac Dysrhythmia,Arrhythmia, Cardiac,Dysrhythmia, Cardiac

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