title: Clinical profile and natural history of Ebstein's anomaly of tricuspid valve.
abstract: There were 63 patients of Ebstein's anomaly of tricuspid valve encountered from 1976 to 1991; 28 (44.4%) were male and 35 (55.6%) female. Their age at presentation ranged from 3 months to 51 years. Five (7.9%) patients were asymptomatic, 48 (76.2%) had class II-III exertional dyspnoea, palpitation or both. Thirty patients (47.6%) had cyanosis. Electrocardiogram showed paroxysmal atrial fibrillation in two, chronic atrial fibrillation in four (6.3%), paroxysmal supraventricular tachycardia in seven, atrial or ventricular ectopic beats in five (7.9%), 2:1 atrioventricular block in one (1.6%), complete atrioventricular block in two (3.2%) and type B WPW syndrome in nine patients (14.3%). Chest X-ray showed diminished vascularity in 22 (34.9%). Diagnosis was established by cardiac catheterization and or echocardiography. Atrialized right ventricular chamber was demonstrated in 51 (80.9%) by angiography and in 40 (63.5%) by electrophysiology. Patients were followed up for 1-172 months. Seventeen patients (26.9%) required surgery. Three patients (4.8%) died during medical follow-up, and five (7.9%) died following surgery. Survival probability for 46 medical patients was 88.9% at 172 months. Factors affecting survival were pulmonary blood flow, cyanosis, clubbing and systemic arterial oxygen saturation.
authors: Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
authors: P K
authors: PK
authors: Jaiswal
authors: K G
authors: KG
authors: Balakrishnan
authors: A
authors: A
authors: Saha
authors: C G
authors: CG
authors: Venkitachalam
authors: J
authors: J
authors: Tharakan
authors: T
authors: T
authors: Titus
mesh_terms: D000293:Adolescent; D000328:Adult; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D003490:Cyanosis / Q000150:complications; D004437:Ebstein Anomaly / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006334:Heart Function Tests; D006347:Heart Sounds; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D015993:Life Tables; D008297:Male; D008875:Middle Aged; D010101:Oxygen Consumption; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D014261:Tricuspid Valve / Q000601:surgery*