J Thorac Cardiovasc Surg 1994;108:193-194
© 1994 Mosby, Inc.
Successful correction of Ebstein's anomaly with rheumatic mitral valvular disease
Premanand Ponoth, MS, MCh,
S. Rajan, MS, MCh,
Suresh G. Rao, MS, MCh,
K. M. Cherian, MS, FRACS
Institute of Cardiovascular Diseases
Vadapalani, Madras, India
To the Editor:
Anomalies of the left side of the heart associated with Ebstein's malformation of the tricuspid valve occur occasionally. Mitral valve abnormalities that have been reported include valvular stenosis, parachute mitral valve, mitral valve prolapse, supravalvular ring, cleft mitral leaflet in association with endocardial cushion defects, coarctation of the aorta, and other defects. A combination of Ebstein's anomaly with rheumatic mitral valvular disease is still a rarity. We report here a case of rheumatic mitral valvular lesion and Ebstein's anomaly, successfully treated with tricuspid and mitral valve replacements. Successful treatment of this combination of lesions has not been reported previously to our knowledge.
An 11-year-old Kenyan boy weighing 25.5 kg was admitted with a history of frequent cough and palpitations of 7 years' duration. There was a history of dyspnea on exertion and paroxysmal nocturnal dyspnea since the age of 3 years, and of pain on the left side of the chest, puffiness of the face, and edema of the lower limbs since the age of 3
months. Clinical examination revealed clubbing and pallor. Heart rate was 98 beats/min and regular. Jugular venous pulsations were within normal limits. Blood pressure was 90/70 mm Hg. Apex beat was in the left sixth intercostal space, outside the midclavicular line. The first heart sound was normal; the pulmonary component of the second heart sound was loud. Auscultation findings suggested mitral stenosis with regurgitation and tricuspid regurgitation. The liver was 4 cm below the right costal margin and pulsatile. Results of routine blood and urine investigations were within normal limits. A chest radiograph showed significant cardiomegaly, with a narrow pedicle and normal pulmonary blood flow. There was mitralization of the left heart border. Electrocardiogram showed normal sinus rhythm, with right and left atrial enlargement. Echocardiography revealed trabeculated right ventricle with Ebstein's anomaly of the tricuspid valve. There was moderate tricuspid regurgitation. The mitral valve had a restricted opening, with doming of the leaflets associated with mitral regurgitation. Cardiac catheterization confirmed the echocardiographic findings, and the characteristic cloverleaf appearance of the tricuspid valve could be discerned (Fig. 1). The catheterization data are given in 
Table I. The diagnosis of Ebstein's anomaly with moderate tricuspid regurgitation and rheumatic mitral stenosis with regurgitation (Fig. 2) was established. The patient was taken in for surgical correction. Surgical exposure was through a median sternotomy. Cardiopulmonary bypass was achieved by cannulation of the aorta, superior vena cava, and inferior vena cava. The patient was cooled to 25° C. Cold blood cardioplegia was used to achieve cardioplegic arrest; topical hypothermia with normal saline solution was used in addition. The tricuspid anulus was extremely large, with a perforated anterior leaflet. It was therefore decided to replace the valve with a 3M Starr-Edwards valve (Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.) attached with continuous 2-0 polypropylene sutures in a supracoronary position. The aneurysmal part of the atrialized right ventricular portion was also plicated. The mitral valve was excised and replaced with a 2M Starr-Edwards valve. This was sutured into position with continuous 2-0 polypropylene sutures. Cardiac rhythm was obtained by a single direct-current shock after routine deairing and release of the aortic crossclamp. Chest closure was achieved in the usual manner after inserting a right atrial line, a left atrial line, and atrioventricular pacing wires. Except for transient rhythm disturbance in the immediate postoperative period, which was well controlled by antiarrhythmics, postoperative recovery was uneventful. The patient was discharged on the tenth postoperative day in sinus rhythm. Ten months after the operation, he continues to do well.
Ebstein's anomaly alone constitutes only 1% 1 of the congenital heart diseases, with a wide spectrum of deformity of the tricuspid valve. Ebstein's anomaly usually occurs as an isolated lesion. 2 Association of Ebstein's anomaly with a rheumatic mitral valve lesion was reported from India by Mohan and Tandon. 3 Medical treatment has little to offer the patient except for the control of arrhythmias and congestive heart failure. It has been our experience that valves suitable for repair are rare, especially when there is significant tricuspid regurgitation. Repair based on a large anterior tricuspid leaflet is preferred to valve replacement whenever feasible because it avoids the problem of prosthetic valve dysfunction, anticoagulation, and (in children) the need for a later replacement because of growth. 4 Melo and colleagues 5 and Najafi and associates 6 report excellent long-term results in Ebstein's anomaly with the Starr-Edwards valve. 6 In cases with associated mitral stenosis and pulmonary hypertension with tricuspid regurgitation, which could be aggravated by Ebstein's anomaly, replacement of the tricuspid valve may be preferable to repair.
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