|Year : 2015 | Volume
| Issue : 2 | Page : 54-55
An Unusual First Presentation of Ebstein's Anomaly in a 70-year-old Patient
Amit Achliya1, Hitesh H Gulhane2
1 Assistant professor, Dr. Panjabrao Deshmukh Memorial Medical College and Research centre, Amravati, Maharashtra, India
2 Senior Resident, Dr. Panjabrao Deshmukh Memorial Medical College and Research centre, Amravati, Maharashtra, India
|Date of Web Publication||16-Jun-2015|
Dr. Hitesh H Gulhane
Department of General Medicine, Dr. Panjabrao Deshmukh Memorial Medical College and Research Centre, Shivaji Nagar, Amravati - 444 602, Maharashtra
Source of Support: None, Conflict of Interest: None
Ebstein's anomaly is a congenital heart defect. The septal and posterior leaflets of the tricuspid valve are displaced toward the apex of the right ventricle called as atrailazation of right ventricle. Most of them have interatrial communication. Ebstein's anomaly accounts for <1% of all cases of congenital heart disease. It has a high mortality rate in first few months of life and rare patients with Ebstein's anomaly live >70 years. A 70-year-old male presented with dyspnea on exertion was a case of Ebstein's anomaly with the late presentation in seventh decade of life.
Keywords: Atrailization of right ventricle, congenital heart disease, Ebstein′s anomaly
|How to cite this article:|
Achliya A, Gulhane HH. An Unusual First Presentation of Ebstein's Anomaly in a 70-year-old Patient. Heart India 2015;3:54-5
| Introduction|| |
Ebstein's anomaly initially described by Wilhelm Ebstein in 1866 is a rare congenital heart disorder occurring in ≈1 per 200,000 live births and accounting for <1% of all cases of congenital heart disease.  Ebstein's anomaly is a malformation of the tricuspid valve and right ventricle characterized by:
- Adherence of the septal and posterior leaflets to the underlying myocardium (failure of delamination, namely splitting of the tissue by detachment of the inner layer during embryologic development);
- Downward (apical) displacement of the functional annulus (septal > posterior > anterior);
- Dilation of the "atrialized" portion of the right ventricle, with various degrees of hypertrophy and thinning of the wall;
- Redundancy, fenestrations, and tethering of the anterior leaflet; and
- Dilation of the right atrioventricular junction (true tricuspid annulus). 
Few cases are reported in the literature to survived with Ebstein's till seventh decade. , Author report a case with an unusual the first presentation of Ebstein's anomaly in a 70-year-old patient.
| Case Report|| |
A 70-year-old male, was admitted to medicine ward, with (New York Heart Association) class III dyspnea for 12 months, split first heart sound and signs of right heart failure with concomitant atrial fibrillation on clinical examination. Electrocardiography suggestive of right bundle branch block with Bizarre morphologies of the terminal QRS pattern and atrial fibrillation, chest X-ray posterior-anterior view suggestive of globular cardiomegaly. Two-dimensional echo was done showing features of Ebstein's anomaly - apical displacement (>3 cm) and deformed tricuspid valve with atrialized right ventricle, moderate to severe tricuspid regurgitation and mild pulmonary hypertension [Figure 1]. Patient was treated with standard heart failure therapy including diuretics and digoxin and discharged in a stable condition.
|Figure 1: Two-dimensional echo image of Ebstein's anomaly showing atrailization of right ventricle|
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In the natural history of this congenital disease, only 5% of patients survive beyond the fifth decade.  This is a rare presentation of Ebstein's anomaly in seventh decade. Only five cases are reported the literature to survived with Ebstein's till seventh decade.
| References|| |
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