Disease Information for Arrhythmogenic right ventr/dysplasia

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Clinical Manifestations
Signs & Symptoms
Cardiac Symptoms/Signs
Presyncope
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Diagnostic Test Results
Electrodiagnosis
Electrophysiologic/cardiac conduction study abnormal
EKG (ECG)
EKG/Wide QRS pattern tachycardia (ECG)
EKG/Widened QRS pattern (ECG)
EKG/Right atrial hypertrophy signs (ECG)
EKG/Changes/abnormalities (ECG)
CT Scan
MRI/Heart scan abnormal
X-RAY
Xray/Enlarged right atrium/Chest
Ultrasound
Echo/Right Atrium Chamber Large
Echo/Right atrium increased volume/diameter
Angiography
Angios/Right atrium enlarged/Heart
Heart cath/Left atrium enlarged
Heart cath/Right side abnormal
Associated Diseases & Rule outs
Rule Outs
Partial complex seizure
Associated Disease & Complications
Arrhythmogenic right ventr/dysplasia
Cardiogenic syncope
Death/Unanticipated
Sudden death/Child
Venticular tach/non sustained/recurrent
Wooley hair disease
Cardiac death, sudden
Right Atrial Hypertrophy
Sudden Death Young Athlete
Disease Mechanism & Classification
Class
CLASS/Cardiovascular (category)
CLASS/Heart disorder (ex)
Pathophysiology
Pathophysiology/Gene locus 14q23-q24
Pathophysiology/Gene locus chromosome 14
Pathophysiology/Gene Locus Identified/OMIM database
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Process
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Congenital/developmental (category)
PROCESS/Dysplasia/aplasia (ex)
PROCESS/Vegetative-Autonomic/Endocrine (category)
Synonyms
Synonym
Arrhythmogenic right ventricular dysplasia, Synonym/Naxos Familial variant RV dysplasia, Synonym/Naxos Wooley hair-RVDysplasia var
Treatment
Drug Therapy - Indication
SX/Implanted cardioverter/defibrillator (ICD)
Definition

Arrhythmic right ventricular dysplasia is characterized by focal fibrous-fatty replacement of the right ventricle; The right ventricular free wall and the atria are primarily involved, giving rise to ventricular and supraventricular arrhythmias, which are often the presenting symptom; Proposed pathology includes congenital hypoplasia of myocardial tissue and focal injury with fibrous replacement; Some patients present with left ventricular dysfunction, without initial recognition of the right ventricular abnormalities, which are often unappreciated on routine echocardiography; Currently, about one third of patients have evidence of familial involvement in an autosomal dominant pattern, with at least four genes currently identified on chromosomes 1 and 14; A well-known variant is Naxos syndrome originating from the Mediterranean area, in which the affected family members share strikingly curly hair and palmar hyperkeratosis; --------------------[Cecil 2004]---------------------

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External Links Related to Arrhythmogenic right ventr/dysplasia
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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