Ebstein Anomaly Associated With Unusual Conduction Delay and Sick Sinus Syndrome-A Case Report-

Abstract

bstein anomaly is characterized by apical displacement of the septal or posterior leaflet of the tricuspid valve and may be related to abnormal cell death. Furthermore, conduction abnormalities associated with this anomaly are often confined to the atrioventricular junction. We report a rare case of Ebstein anomaly associated with an unusual conduction delay in the whole heart, as well as systolic dysfunction of both ventricles, which indicates that the entire myocardium can be impaired globally in this anomaly. Case ReportA 64-year-old man was admitted to our hospital because he had often felt palpitation and dyspnea on effort, and he had facial edema. On admission, the 12-lead electrocardiogram (ECG) revealed atrial tachycardia with 2:1 conduction and intraventricular conduction delay; his heart rate was 75 beats/min (atrial rate: 150 beats/min) (Fig 1). The cardiothoracic ratio was 61% on chest rentogenogram. Echocardiography showed severe enlargement of the right atrium and ventricle, decreased systolic function of both ventricles (end-diastolic and end-systolic left ventricular dimensions were 48 mm and 41 mm, respectively), apical displacement of the septal leaflet of the tricuspid valve by 16 mm (10.5 mm/m 2 ), and mild tricuspid regurgitation with a peak velocity of 0.78 m/s and pressure gradient of 2.4 mmHg (Fig 2). The diagnosis was Ebstein anomaly.An electrophysiological study revealed that the atrial rhythm was common atrial flutter. Atrial mapping showed a counterclockwise rotation of excitation along the tricusCirculation Journal Vol.68, July 2004 pid annulus; the atrial flutter cycle length was 400 ms (Fig 3). The post pacing interval after an extra-stimulation at the proximal coronary sinus and the low lateral right atrium was the same as the atrial flutter cycle length. Right atrial mapping showed that the slowest atrial conduction existed between RA3 and RA1 (Fig 3), located on the inferior vena cava -tricuspid annulus isthmus where low A 64-year-old man was admitted to our hospital because of palpitation, dyspnea on effort, and facial edema. The echocardiographic diagnosis was Ebstein anomaly. Although the 12-lead electrocardiogram showed an atrial rate of 150 beats/min and no typical flutter wave, the electrophysiological study showed counterclockwise rotation of excitation along the tricuspid annulus. Because of sinus arrest and syncope, a permanent pacemaker was implanted, but the right atrium was not captured by electrical stimulation at 5V/0.4 ms, except for the orifice of coronary sinus, and the intracardiac P wave was only 0.2 mV or less. This is a rare case of Ebstein anomaly characterized by unusually prolonged conduction in the atrium, the basis of which was global myocardial damage, including the ventricles.

Širdies ritmo ir laidumo sutrikimai sergant Ebšteino anomalija

“…Taèiau taip pat apraðyta atvejø, kai ligoniams, sergantiems EA ir kartu turintiems WPW sindromà, ilgainiui atsirado bradikardija ir sinusinio mazgo silpnumas, buvo implantuoti EKS. Vieni autoriai mano, kad tai gali bûti nulemta genetiniø veiksniø, kitø manymu, vystosi EA sukelta paþeistø làsteliø apoptozë [8,9].…”