|Year : 2013 | Volume
| Issue : 3 | Page : 130-132
Ebstein's anomaly with severe aortic stenosis and syncope: Implications in management
Vijayakumar Subban, Anitha Lakshmanan, Latchumanadhas Kalidoss, Ulhas M Pandurangi, Ajit S Mullasari
Department of Cardiology, Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India
|Date of Web Publication||22-Nov-2013|
Department of Cardiology, Institute of Cardiovascular Diseases, Madras Medical Mission, 4A, J.J. Nagar, Mogappair, Chennai - 600 037
Ebstein's anomaly is a rare congenital heart disease involving the right side of the heart with typical malformations of the tricuspid valve and the right ventricle. Associated left heart anomalies, particularly aortic valve disease, are extremely rare. We report here an unusual case of Ebstein's anomaly of the tricuspid valve and severe aortic stenosis who presented to us with recurrent syncopal episodes. The patient needed to undergo electrophysiological evaluation before aortic valve replacement to rule out arrhythmic causes of syncope.
Keywords: Aortic stenosis, Ebstein′s anomaly, electrophysiological study, syncope
|How to cite this article:|
Subban V, Lakshmanan A, Kalidoss L, Pandurangi UM, Mullasari AS. Ebstein's anomaly with severe aortic stenosis and syncope: Implications in management. Sahel Med J 2013;16:130-2
|How to cite this URL:|
Subban V, Lakshmanan A, Kalidoss L, Pandurangi UM, Mullasari AS. Ebstein's anomaly with severe aortic stenosis and syncope: Implications in management. Sahel Med J [serial online] 2013 [cited 2021 Sep 27];16:130-2. Available from: https://www.smjonline.org/text.asp?2013/16/3/130/121927
| Introduction|| |
Ebstein's anomaly is a rare congenital heart disease involving the right side of the heart with typical malformations of the tricuspid valve and the right ventricle. Associated left heart anomalies particularly aortic valve disease isextremely rare. , We report here an unusual case of Ebstein's anomaly of the tricuspid valve and severe aortic stenosis who presented to us with syncopal episodes.
| Case Report|| |
A 69-year-old gentleman presented to us with a history of three episodes of non-exertional syncope. He gave no history of palpitations, dyspnea or angina. His physical examination revealed a pulse rate of 70 per minute, which was regular, and blood pressure of 120/80 mmHg. There were no signs of congestive heart failure. His first and second heart sounds were widely split. There was a 3/6 pansystolic murmur in the left lower sternal border and a 4/6 ejection systolic murmur in the right second intercostal space conducting to both the carotids. Chest radiography revealed cardiomegaly with right atrial enlargement and clear lung fields. Electrocardiogram (ECG) [Figure 1] showed sinus rhythm with a rate of 70 per minute, PR interval of 160 msec and splintered QRS complexes in the precordial leads and left axis deviation of QRS in the limb leads. Echocardiography revealed Ebstein's anomaly of the tricuspid valve with 22 mm apical displacement of the septal tricuspid leaflet and severe low-pressure tricuspid regurgitation [Figure 2]a. It also showed severe calcific aortic stenosis with a peak gradient of 80 mmHg and a mean gradient of 55 mmHg and good biventricular function [Figure 2]b and c. The patient was advised to undergo aortic valve replacement and repair of the tricuspid valve.
|Figure 1: Surface 12-lead electrocardiogram. Note the splintered QRS complexes|
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|Figure 2: (a) Apical four-chamber view showing apical displacement of the septal leafl et of the tricuspid valve characteristic of Ebstein's anomaly. TV - tricuspid valve. MV - mitral valve (b) Parasternal short-axis view at the aortic valve level. Note the calcifi ed bicuspid aortic leafl ets. AV - aortic valve (c) Continuous wave Doppler showing a peak gradient of 80 mmHg and a mean gradient of 55 mmHg across the aortic valve|
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An electrophysiological study (EPS) was performed before surgery to rule out accessory pathways and inducible tachyarrhythmias that could be responsible for his syncopal episodes. The EPS was essentially normal with no evidence of accessory pathway. His AH and HV intervals were 70 msec and 40 msec, respectively. VA conduction was concentric and decremental. On adenosine, there was AV block without pre-excitation. No sustained tachycardia could be induced by programmed atrial and ventricular stimulation even with isoprenaline. Severe aortic stenosis in this patient was considered responsible for his syncopal episodes. As this patient was awaiting surgery, he had a sudden death at home.
| Discussion|| |
Ebstein's anomaly is found in 0.5% of all cases of congenital heart diseases, and represents about 40% of the congenital malformations of the tricuspid valve.  Ebstein's anomaly is often associated with other abnormalities of the heart.  In a study of 76 patients with Ebstein's anomaly at the Mayo Clinic, abnormalities of the left ventricular myocardium resembling non-compaction have been reported in 18% of the patients. Mitral valve abnormalities such as mitral valve prolapse occurred in 15% of the patients and bicuspid aortic valve occurred in 8% of the patients.  However, obstructive lesions of the left heart requiring interventions are rare. Different authors have reported significant coarctation of aorta in association with Ebstein's anomaly. ,,
Patients with Ebstein's anomaly have a high risk for developing tachyarrhythmias, which are a common cause of morbidity and death. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring with direct muscular connections thus creating a potential substrate for accessory atrioventricular connection and ventricular pre-excitation.  Syncope was reported as a presenting symptom only in 10% of the Ebstein patients by Attenhofer et al.  The usual mechanisms of syncope are accessory pathway-mediated tachycardia, atrial or ventricular tachyarrhythmias  and, occasionally, high-degree atrioventricular blocks.  Ventricular pre-excitation on surface ECG is seen in 5-25% of the patients with Ebstein's anomaly. Accessory atrioventricular connections are usually located on the same side as the malformed valve and, more often, they are multiple. Nearly 30% of the patients who have symptoms of palpitations may have inducible atrioventricular re-entrant tachycardia. Enlarged right atrium predisposes Ebstein's patients to develop atrial fibrillation. In the presence of an accessory pathway, atrial fibrillation may result in very fast ventricular rates, resulting in syncope or sudden cardiac death.  Syncope is an ominous symptom in patients with aortic stenosis. It occurs in 15-30% of the symptomatic patients with severe aortic stenosis. Average survival after the onset of syncope is 2-3 years with a high risk of sudden death. Syncope is most commonly due to reduced cerebral perfusion that occurs during exertion when the arterial pressure declines consequent to systemic vasodilatation in the presence of a fixed cardiac output.  Syncope has also been attributed to malfunction of baroreceptor mechanism in severe aortic stenosis as well as to a vasodepressor response to a greatly elevated left ventricular systolic pressure during exercise.  Syncope at rest may be due to transient ventricular fibrillation or transient atrial fibrillation with a loss of atrial contribution to the left ventricular filing leading to a precipitous decline in cardiac output. High-grade atrioventricular blocks due to extension of the calcification of the valve into the conduction system can also cause syncope.  In a patient with both Ebstein's anomaly and severe aortic stenosis, it is difficult to implicate the cause of syncope with certainty. Our patient was advised aortic valve replacement. Pre-operatively, he underwent EPS to rule out the coexistence of accessory pathways as well as inducible tachyarrhythmias. The absence of any electrophysiological abnormality implicated aortic stenosis as the probable cause of syncope. Sudden death of the patient while awaiting surgery underlines the ominous prognostic significance of syncope in aortic stenosis.
Evaluation of syncope may be difficult in the presence of a combination of abnormalities that would produce the same symptom. Although EPS can be useful to find out significant electrical abnormalities, it may not induce all the arrhythmias responsible for syncope. Early surgery in such patients may be life-saving.
| References|| |
|1.||Hauck AJ, Freeman DP, Ackermann DM, Danielson GK, Edwards WD. Surgcial pathology of the tricuspid valve: A study of 363 cases spanning 25 years. Mayo Clin Proc 1988;63:851-63. |
|2.||Monibi AA, Neches WH, Lenox CC, Park SC, Mathews RA, Zuberbuhler JR. Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve. Circulation 1978;57:303-6. |
|3.||Attenhofer Jost CH, Connolly HM, O'Leary PW, Warne CA, Tajik AJ, Seward JB. Left heart lesions in patients with Ebstein's anomaly. Mayo Clin Proc 2005;80:361-8. |
|4.||Salkar HR, Salkar RG, Sengupta PP. Ebstein's Anomaly with coarctation of the aorta-An unusual association. Indian Heart J 1996;48:283-4. |
|5.||Rajasekhar D, Raju R, Reddy DC. Ebstein's anomaly of the tricuspid valve: Association with coarctation of aorta. Indian Heart J 2000;52:599-600. |
|6.||Ebaid M, Azeka E, Ikari NM, Sosa EA, Marcial MB, Atik E. Ebstein's Anomaly with coarctation of the aorta-an unusual association. Arq Bras Cardiol 1999;73:219-24. |
|7.||Cappato R, Schluter M, Weiss C, Antz M, Koschyk DH, Hofmann T, Kuck KH. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly. Circulation-1996;94:376-83. |
|8.||Allen MR, Hayes DL, Warnes CA, Danielson GK. Permanent pacing in Ebstein's anomaly. Pacing Clin Electrophysiol 1997;20:1243-6. |
|9.||Khositseth A, Danielson GK, Dearani JA, Munger TM, Porter CJ. Supraventricular tachyarrhythmias in Ebstein anomaly: Management and outcome. J Thorac Cardiovasc Surg 2004;128:826-33. |
|10.||Richards AM, Nicholls MG, Ikram H, Hamilton EJ, Richards RD. Syncope in aortic valvular stenosis. Lancet 1984;2:1113-6. |
|11.||Mark AL, Kioschos JM, Abboud FM, Heistad DD, Schmid PG. Abnormal vascular responses to exercise in patients with aortic stenosis. J Clin Invest 1973;52:1138-46. |
|12.||Ablaza SG, Blanco G, Maranhao V, Morse DP, Nichols HT. Calcific aortic valvular disease associated with complete heart block: Case reports of successful correction. Dis Chest 1968;54:457-60. |
[Figure 1], [Figure 2]