An Unusual First Presentation of Ebstein's Anomaly in a 70-year-old Patient

Amit Achliya; Hitesh H Gulhane

doi:10.4103/2321-449X.158882

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CASE REPORT

Year : 2015 | Volume : 3 | 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

Correspondence Address:
Dr. Hitesh H Gulhane
Department of General Medicine, Dr. Panjabrao Deshmukh Memorial Medical College and Research Centre, Shivaji Nagar, Amravati - 444 602, Maharashtra
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2321-449X.158882

Abstract

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

How to cite this URL:
Achliya A, Gulhane HH. An Unusual First Presentation of Ebstein's Anomaly in a 70-year-old Patient. Heart India [serial online] 2015 [cited 2023 Mar 22];3:54-5. Available from: https://www.heartindia.net/text.asp?2015/3/2/54/158882

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. ^[1] 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). ^[2]

Few cases are reported in the literature to survived with Ebstein's till seventh decade. ^[3],[4] 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. ^[5] 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

1.	Lupo PJ, Langlois PH, Mitchell LE. Epidemiology of Ebstein anomaly: Prevalence and patterns in Texas, 1999-2005. Am J Med Genet A 2011;155A:1007-14.
2.	Frescura C, Angelini A, Daliento L, Thiene G. Morphological aspects of Ebstein′s anomaly in adults. Thorac Cardiovasc Surg 2000;48:203-8.
3.	Seward JB, Tajik AJ, Feist DJ, Smith HC. Ebstein′s anomaly in an 85-year-old man. Mayo Clin Proc 1979;54:193-6. [PUBMED]
4.	Makous N, Vander Veer JB. Ebstein′s anomaly and life expectancy. Report of a survival to over age 79. Am J Cardiol 1966;18:100-4. [PUBMED]
5.	Palmerini E, Federici D, Del Pasqua A, Bernazzali S, Lisi M, Chiavarelli M, et al. Echocardiographic Assessment of Ebstein′s Anomaly in a 60-Year-Old Man. Case Rep Med 2009;2009:653741.