The electrocardiogram in Ebstein's anomaly of the tricuspid valve

doi:10.1016/0002-8703(55)90250-7

American Heart Journal

Volume 50, Issue 1, July 1955, Pages 13-23

https://doi.org/10.1016/0002-8703(55)90250-7 Get rights and content

Abstract

1.
1. The electrocardiograms of eight cases of Ebstein's anomaly of the tricuspid valve have been studied. These were characterized by complete right bundle branch block in all cases, and by small deflections (R, R′, and R + S) in Leads V₁ and commonly in Leads V₂, V₃, and V₄. Tall and prolonged P waves occurred commonly and were considered to be evidence of right auricular hypertrophy and dilatation. Prolongation of the P-R interval and supraventricular tachycardia were also observed.
2.
2. It was considered that the small deflections of the QRS complex in the right chest leads were due to the thinness of the proximal chamber of the right ventricle which is a characteristic and constant feature of Ebstein's disease. This feature of the electrocardiogram in Ebstein's anomaly is only rarely found in complete or incomplete right bundle branch block from other causes and is considered to be of value in the diagnosis of this condition.
3.
3. The association of electrocardiographic evidence of right atrial hypertrophy in the absence of evidence of right ventricular hypertrophy (small R or R′ waves in the right chest leads) is uncommon in other types of congenital heart disease and is a characteristic feature of Ebstein's anomaly.

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Cited by (16)

Ebstein Anomaly in the Adult Patient
2020, Cardiology Clinics
Citation Excerpt :
PR prolongation, resulting from atrial dilation and delayed intra-atrial conduction26 Low-voltage QRS in right-sided chest leads, secondary to generalized right ventricular myopathy27 Supraventricular tachycardia and atrial arrhythmias
Noninvasive electrocardiographic imaging (ECGI) of epicardial activation before and after catheter ablation of the accessory pathway in a patient with Ebstein anomaly
2008, Heart Rhythm
Citation Excerpt :
Ebstein anomaly1 is characterized by abnormal development of the tricuspid valve with the septal (and often posterior) leaflets of the valve displaced into the right ventricle (RV). The abnormal development of the tricuspid valve often is associated with several conduction abnormalities, including delayed intra-atrial conduction, right bundle branch block (RBBB),2,3 and ventricular pre-excitation.4 Absence of an RBBB pattern during sinus rhythm on a baseline electrocardiogram (ECG) suggests the presence of an atrioventricular (AV) accessory pathway (AP) in patients with Ebstein anomaly.
The Electrocardiogram in Congenital Heart Disease
2008, Chou's Electrocardiography in Clinical Practice: Adult and Pediatric
Tachycardia in Ebstein's anomaly
2003, Heart and Lung: Journal of Acute and Critical Care
Two-dimensional echocardiographic spectrum of ebstein's anomaly: Detailed anatomic assessment
1984, Journal of the American College of Cardiology
This report details the two-dimensional echocardiography assessment of 41 patients with Ebstein's anomaly. The anatomic spectrum of Ebstein's anomaly is correlated with surgical and autopsy observations in 66% of these patients. Morphologic abnormalities of the tricuspid valve and right heart structures were uniquely assessed by noninvasive two-dimensional echocardiography examination. Features of the tricuspid valve that best related to decreased functional capacity were: 1) absence of the septal leaflet, and 2) pronounced tethering, restriction of motion and displacement of the anterior leaflet of the tricuspid valve.
This 4½ year experience suggests that two-dimensional echocardiography can replace angiography as the procedure of choice for diagnostic afld morphologic assessment of Ebstein's anomaly. Cardiac catheterization is necessary only when specific hemodynamic questions or clinical inconsistencies exist.
Ebstein's anomaly. Clinical profile and natural history
1971, The American Journal of Cardiology
The clinical features, cardiac catheterization data and natural history of 55 patients with Ebstein's anomaly are presented; 18 had a significant associated cardiac anomaly. The clinical features are usually clear-cut. Cardiac catheterization and angiography demonstrate downward displacement of the tricuspid valve, a large right atrium, no important elevation of the right ventricular pressures, decreased pulmonary blood flow and right to left shunt through the foramen ovale. Right ventricular outflow obstruction may be masked by the low level of pulmonary blood flow and resistance. Intracardiac electrocardiogram is usually diagnostic.
The prognosis in these children is relatively poor in the presence of (1) complicating cardiac lesions, (2) more marked cyanosis and (3), to a lesser extent, extreme cardiomegaly. The appearance of congestive heart failure foretells early death (<4 years). Atrial arrhythmias are common and Wolff-Parkinson-White syndrome may be encountered, but fatality from arrhythmia is unlikely. Sudden death occurs but is unusual. A loud systolic murmur, right ventricular hypertrophy or an atypical roentgenogram should prompt an early study to exclude an associated lesion. Medical management results in about 50 percent survival up to 13 years of age. The usefulness of surgery for this lesion is unproved and is usually reserved for complicating lesions or critical situations.
In neonates presenting with Ebstein's anomaly the incidence of important associated cardiac anomalies is higher, and the clinical picture more variable because of the physiologic adjustments of birth. Cyanosis may be severe only to improve later. The electrocardiogram may show atypical features and exhibit the classic pattern within weeks. Radiographic cardiomegaly may be dramatically out of proportion to the apparent well-being of the infant. Surprisingly gross cardiomegaly is compatible with survival.

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^☆: Presented in abstract to The Society for Experimental Biology, Johannesburg, and published in South Africa J. Med. Sc. 18; 88–89, 1953.

^∗: Eli Lilly Medical Research Fellow (1952); present address, Medical School, University of the Witwatersrand, Johannesburg, South Africa.

^∗∗: Children's Hospital of Pittsburgh, 125 De Soto Street, Pittsburgh 13, Pa.

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Original communicationThe electrocardiogram in Ebstein's anomaly of the tricuspid valve☆

Abstract

Am. Heart J.

Am. Heart J.

Am. Heart J.

Am. J. Med.

Am. Heart J.

Am. J. Med.

Am. Heart J.

Ebstein's Anomaly of the Tricuspid Valve. Report of Three Cases and Analysis of the Clinical Syndrome

Circulation

Maladie d'Ebstein: Essai de diagnostic clinique

Arch. mal. coeur

Ebstein's Disease

Guy's Hosp. Rep.

Ebstein's Disease. A Case Diagnosed Clinically

Guy's Hosp. Rep.

Ebstein's Disease. Report of the First Case Diagnosed During Life

Am. J. M. Sc.

Electrocardiogram in Congenital Heart Disease

Circulation

Essentials of Electrocardiography

Arch. Inst. cardiol. México

The Normal Human Ventricular Gradient. Factors Which Affect Its Direction and Its Relation to the Mean QRS Axis

Am. Heart J.

The Interpretation of the Ventricular Complex of the Electrocardiogram

Advances Int. Med.

Original communication
The electrocardiogram in Ebstein's anomaly of the tricuspid valve☆