Biomaterial Database

Kearns-Sayre syndrome: association with long QT syndrome? 2002

Ahmad Rashid, and Michael H Kim

Department of Internal Medicine, Washington University in St. Louis, Missouri, USA.

Kearns-Sayre syndrome has associated cardiac findings, predominantly complete heart block, which has been implicated as a mechanism of sudden death in these individuals. A patient with Kearns-Sayre syndrome who had syncope and multiple cardiac arrests due to ventricular tachycardia in the setting of QT prolongation is described. Long QT syndrome is implicated as another possibility for sudden death in Kearns-Sayre syndrome. This potential association is unexplained, but now totals two reports in the small numbers of patients reported.

UI	MeSH Term	Description	Entries
D007625	Kearns-Sayre Syndrome	A mitochondrial disorder featuring the triad of chronic progressive EXTERNAL OPHTHALMOPLEGIA, cardiomyopathy (CARDIOMYOPATHIES) with conduction block (HEART BLOCK), and RETINITIS PIGMENTOSA. Disease onset is in the first or second decade. Elevated CSF protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. (Adams et al., Principles of Neurology, 6th ed, p984)	Kearns Syndrome,CPEO with Myopathy,CPEO with Ragged Red Fibers,Chronic Progressive External Ophthalmoplegia with Myopathy,Cpeo With Ragged-Red Fibers,Kearn-Sayre Mitochondrial Cytopathy,Kearns Sayre Syndrome,Kearns' Syndrome,Kearns-Sayre Mitochondrial Cytopathy,Kearns-Sayre-Shy-Daroff Syndrome,Oculocraniosomatic Syndrome,Ophthalmoplegia Plus Syndrome,Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy,Ophthalmoplegia, Progressive External, With Ragged-Red Fibers,Ophthalmoplegia-Plus Syndrome,CPEO with Myopathies,Cytopathies, Kearns-Sayre Mitochondrial,Cytopathy, Kearn-Sayre Mitochondrial,Cytopathy, Kearns-Sayre Mitochondrial,Kearn Sayre Mitochondrial Cytopathy,Kearn Syndrome,Kearns Sayre Mitochondrial Cytopathy,Kearns Sayre Shy Daroff Syndrome,Kearns-Sayre Mitochondrial Cytopathies,Mitochondrial Cytopathies, Kearns-Sayre,Mitochondrial Cytopathy, Kearn-Sayre,Mitochondrial Cytopathy, Kearns-Sayre,Myopathies, CPEO with,Myopathy, CPEO with,Oculocraniosomatic Syndromes,Ophthalmoplegia Plus Syndromes,Ophthalmoplegia-Plus Syndromes,Sayre Syndrome, Kearns,Syndrome, Kearns,Syndrome, Kearns Sayre,Syndrome, Kearns',Syndrome, Kearns-Sayre,Syndrome, Kearns-Sayre-Shy-Daroff,Syndrome, Oculocraniosomatic,Syndrome, Ophthalmoplegia Plus,Syndrome, Ophthalmoplegia-Plus,Syndromes, Ophthalmoplegia-Plus
D008133	Long QT Syndrome	A condition that is characterized by episodes of fainting (SYNCOPE) and varying degree of ventricular arrhythmia as indicated by the prolonged QT interval. The inherited forms are caused by mutation of genes encoding cardiac ion channel proteins. The two major forms are ROMANO-WARD SYNDROME and JERVELL-LANGE NIELSEN SYNDROME.	Electrocardiogram QT Prolonged
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
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
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