Isolated idiopathic right ventricular dilated cardiomyopathy. 2011

Swapan Samanta, and Rajesh Vijayverghia, and Kim Vaiphei
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh 160 012, India.

Isolated idiopathic right ventricular dilated cardiomyopathy is rare and is a diagnosis by exclusion. There is a distinct male predominance. The usual clinical presentations are syncope, ventricular tachycardia, left bundle branch block on ECG and right heart failure. Diagnosis is usually established based on the clinical and laboratory parameters. Confirmation of the same is done by studying the pathological features of the heart in an endomyocardial biopsy or at post-mortem. Herein we report a case of this rare entity which was diagnosed clinically and was further confirmed at autopsy.

UI MeSH Term Description Entries
D008297 Male Males
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
D002311 Cardiomyopathy, Dilated A form of CARDIAC MUSCLE disease that is characterized by ventricular dilation, VENTRICULAR DYSFUNCTION, and HEART FAILURE. Risk factors include SMOKING; ALCOHOL DRINKING; HYPERTENSION; INFECTION; PREGNANCY; and mutations in the LMNA gene encoding LAMIN TYPE A, a NUCLEAR LAMINA protein. Cardiomyopathy, Congestive,Congestive Cardiomyopathy,Dilated Cardiomyopathy,Cardiomyopathy, Dilated, 1a,Cardiomyopathy, Dilated, Autosomal Recessive,Cardiomyopathy, Dilated, CMD1A,Cardiomyopathy, Dilated, LMNA,Cardiomyopathy, Dilated, With Conduction Defect 1,Cardiomyopathy, Dilated, with Conduction Deffect1,Cardiomyopathy, Familial Idiopathic,Cardiomyopathy, Idiopathic Dilated,Cardiomyopathies, Congestive,Cardiomyopathies, Dilated,Cardiomyopathies, Familial Idiopathic,Cardiomyopathies, Idiopathic Dilated,Congestive Cardiomyopathies,Dilated Cardiomyopathies,Dilated Cardiomyopathies, Idiopathic,Dilated Cardiomyopathy, Idiopathic,Familial Idiopathic Cardiomyopathies,Familial Idiopathic Cardiomyopathy,Idiopathic Cardiomyopathies, Familial,Idiopathic Cardiomyopathy, Familial,Idiopathic Dilated Cardiomyopathies,Idiopathic Dilated Cardiomyopathy
D006352 Heart Ventricles The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation. Cardiac Ventricle,Cardiac Ventricles,Heart Ventricle,Left Ventricle,Right Ventricle,Left Ventricles,Right Ventricles,Ventricle, Cardiac,Ventricle, Heart,Ventricle, Left,Ventricle, Right,Ventricles, Cardiac,Ventricles, Heart,Ventricles, Left,Ventricles, Right
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013575 Syncope A transient loss of consciousness and postural tone caused by diminished blood flow to the brain (i.e., BRAIN ISCHEMIA). Presyncope refers to the sensation of lightheadedness and loss of strength that precedes a syncopal event or accompanies an incomplete syncope. (From Adams et al., Principles of Neurology, 6th ed, pp367-9) Drop Attack,Fainting,Presyncope,Syncope, Postural,Syncopal Episode,Syncopal Vertigo,Syncope, Cardiogenic,Syncope, Carotid Sinus,Syncope, Convulsive,Syncope, Deglutitional,Syncope, Effort,Syncope, Hyperventilation,Syncope, Micturition,Syncope, Situational,Syncope, Stokes-Adams,Syncope, Tussive,Attack, Drop,Cardiogenic Syncope,Cardiogenic Syncopes,Carotid Sinus Syncope,Carotid Sinus Syncopes,Convulsive Syncope,Convulsive Syncopes,Deglutitional Syncope,Deglutitional Syncopes,Drop Attacks,Effort Syncope,Effort Syncopes,Episode, Syncopal,Hyperventilation Syncope,Hyperventilation Syncopes,Micturition Syncope,Micturition Syncopes,Postural Syncope,Postural Syncopes,Presyncopes,Situational Syncope,Situational Syncopes,Stokes-Adams Syncope,Stokes-Adams Syncopes,Syncopal Episodes,Syncope, Stokes Adams,Syncopes,Syncopes, Cardiogenic,Syncopes, Carotid Sinus,Syncopes, Convulsive,Syncopes, Deglutitional,Syncopes, Effort,Syncopes, Hyperventilation,Syncopes, Micturition,Syncopes, Postural,Syncopes, Situational,Syncopes, Stokes-Adams,Syncopes, Tussive,Tussive Syncope,Tussive Syncopes,Vertigo, Syncopal,Vertigos, Syncopal
D013902 Radiography, Thoracic X-ray visualization of the chest and organs of the thoracic cavity. It is not restricted to visualization of the lungs. Thoracic Radiography,Radiographies, Thoracic,Thoracic Radiographies
D017180 Tachycardia, Ventricular An abnormally rapid ventricular rhythm usually in excess of 150 beats per minute. It is generated within the ventricle below the BUNDLE OF HIS, either as autonomic impulse formation or reentrant impulse conduction. Depending on the etiology, onset of ventricular tachycardia can be paroxysmal (sudden) or nonparoxysmal, its wide QRS complexes can be uniform or polymorphic, and the ventricular beating may be independent of the atrial beating (AV dissociation). Idiopathic Ventricular Tachycardia,Nonsustained Ventricular Tachycardia,Paroxysmal Supraventricular Tachycardia,Ventricular Tachyarrhythmias,Ventricular Tachycardia,Idiopathic Ventricular Tachycardias,Nonsustained Ventricular Tachycardias,Paroxysmal Supraventricular Tachycardias,Supraventricular Tachycardia, Paroxysmal,Tachyarrhythmia, Ventricular,Tachycardia, Idiopathic Ventricular,Tachycardia, Nonsustained Ventricular,Tachycardia, Paroxysmal Supraventricular,Ventricular Tachyarrhythmia,Ventricular Tachycardia, Idiopathic,Ventricular Tachycardia, Nonsustained,Ventricular Tachycardias
D018497 Ventricular Dysfunction, Right A condition in which the RIGHT VENTRICLE of the heart was functionally impaired. This condition usually leads to HEART FAILURE or MYOCARDIAL INFARCTION, and other cardiovascular complications. Diagnosis is made by measuring the diminished ejection fraction and a depressed level of motility of the right ventricular wall. Right Ventricular Dysfunction,Dysfunction, Right Ventricular,Dysfunctions, Right Ventricular,Right Ventricular Dysfunctions,Ventricular Dysfunctions, Right

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