Ann Thorac Surg 2007;83:304-306
© 2007 The Society of Thoracic Surgeons
Case Reports
Ebstein’s Anomaly of the Tricuspid Valve in Combination of Tetralogy of Fallot: Total Correction in Infancy
Chao-Han Lai, MDa,
Jing-Ming Wu, MDb,
Yu-Jen Yang, MD, PhDa,*
a Division of Cardiovascular Surgery, Department of Surgery, National Cheng Kung University Hospital, Tainan, Taiwan
b Department of Pediatrics, National Cheng Kung University Hospital, and Cardiovascular Research Center, Medical College, National Chen Kung University, Tainan, Taiwan
Accepted for publication April 24, 2006.
* Address correspondence to Dr Yang, Department of Surgery, National Cheng Kung University Hospital, National Cheng Kung University, 138, Sheng-Li Rd, Tainan 704, Taiwan (Email: yyji5483130{at}yahoo.com.tw).
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Abstract
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Ebstein’s anomaly of the tricuspid valve in combination with Tetralogy of Fallot is extremely rare. We report a patient with these combined entities in a baby presented with cyanosis and right heart failure soon after birth. Total correction toward biventricular repair was successfully conducted when he was 7 months old. For management of a patient with such rare complex anomalies, we recommend Carpentier’s technique for Ebstein’s anomaly to restore the morphology of the right ventricle, and "one and half ventricle repair" can be a salvage procedure if the right ventricle is not functionally tolerant.
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Introduction
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Ebstein’s anomaly of the tricuspid valve, featured as downward displacement of the septal and posterior leaflets, resulting in an atrialized portion of the right ventricle, is an uncommon congenital malformation [1, 2]. The combination of tetralogy of Fallot (TOF) and Ebstein’s anomaly is extremely rare [3]. In this report, an infant with Ebstein’s anomaly and TOF, manifesting as cyanosis and right heart failure soon after birth, received successful correction of TOF and valvuloplasty of the tricuspid valve at the age of 7 months.
A male newborn was referred to our hospital because of cyanosis, heart murmur, and dyspnea noted soon after birth. On physical examination, he presented tachypnea with subcostal retraction, marked hepatomegaly, and general cyanosis. A grade III/VI systolic ejection murmur was audible over the left upper sternal border. Chest roentgenogram showed decreased bilateral lung marking and marked cardiomegaly with 95% cardiothoracic ratio, especially with the right atrial enlargement. Electrocardiogram showed right atrial enlargement, right ventricular hypertrophy, and nonsustained atrial flutter. Echocardiographic studies revealed the typical features of TOF (Fig 1), Ebstein’s anomaly, and an interatrial shunt. The posterior and septal leaflets were displaced 0.7 cm downward from the tricuspid annulus, resulting in severe tricuspid regurgitation (TR) (Fig 2). Although the true right ventricle was mildly underdeveloped, the contractility was not compromised.
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Fig 1. Preoperative echocardiography showed features of tetralogy of Fallot, including (A) overriding aorta and ventricular septal defect and (B) severe pulmonary stenosis with pressure gradient of 70 mm Hg (arrow).
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Fig 2. Preoperative echocardiography also showed the concomitant Ebstein’s anomaly featured as a downward displacement of the tricuspid valve (arrow) and severe tricuspid regurgitation with a pressure gradient of 70 mm Hg.
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Medication was prescribed for right heart failure, yet the symptoms of respiratory distress and heart failure became more severe during the follow-up period. Therefore surgical intervention was advised at the age of 7 months, while body weight was 6 kg. The cardiopulmonary bypass was set up with single aortic and bi-caval cannulation. After the aorta was cross clamped and the blood cardioplegic solution was infused, the right atrium was opened parallel to the atrioventricular groove. The anterior leaflet was large and freely movable; however the posterior and septal leaflets were displaced downward about 1 cm from the annulus level. The septal leaflet was hypoplastic and adhered to the septum. Pathology of the atrialized portion of the right ventricle and tricuspid valve leaflets was comparable with that of type B according to Carpentier’s classification [1]. The ventricular septal defect came into sight when the anterior and posterior leaflets of the tricuspid valve were detached from the annulus. However, the outlet extension of the perimembranous ventricular septal defect made its upper margin difficult to repair by this approach. Thus we made the longitudinal incision on the right ventricle outflow tract and performed infundibulectomy. The ventricular septal defect was patch repaired with multiple interrupted stitches. The pulmonary valve was bicuspid with commissural fusion, and the pulmonary annulus was hypoplastic. Therefore, after pulmonary valvotomy, the right ventricle outflow tract was enlarged with a transannular patch. The tricuspid valve was reconstructed using Carpentier’s technique: the atrialized ventricle was plicated vertically with four mattress stitches reinforced with pledgets. The detached leaflets were reattached clockwise to the new annulus to cover the tricuspid orifice beyond the plication site and ending below the coronary sinus, but no prosthetic ring was used [1, 2]. After rewarming, the patient was smoothly weaned from cardiopulmonary bypass with a small amount of inotropic agents.
Postoperative echocardiography demonstrated only mild pulmonary stenosis (PS), and mild to moderate TR (Fig 3). The cardiothoracic ratio on chest roentgenogram was reduced to 78%. The postoperative course was uneventful, and he was discharged on postoperative day 10. One month after surgery, mild pulmonary stenosis with a 22 mm Hg pressure gradient between the right ventricle and main pulmonary artery was shown in follow-up echocardiography. Also a mild to moderate degree of TR (the regurgitation jet extending over one third but less than one half of the atrium) with a pressure gradient of 28 mm Hg between the right ventricle and right atrium was shown. The patient was asymptomatic with a moderate amount of diuretics.
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Fig 3. Postoperative echocardiography showed a mild to moderate tricuspid regurgitation with a pressure gradient of 27 mm Hg.
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Comment
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Ebstein’s anomaly comprises only about 1% of all congenital heart lesions [1]. It is frequently associated with various cardiac anomalies; however, coexistence with TOF is extremely rare. There has been very scarce surgical experience for patients with such a combination. Chan and colleagues [3] reported the surgical correction in a 7-month-old girl with TOF in the presence of Ebstein’s anomaly, but the tricuspid valve was not touched because the TR was only mild. In our patient, right heart failure due to severe TR necessitated total correction for both lesions in infancy. Successful surgical management for both lesions has not been previously described in the English literature.
Clinical presentation of Ebstein’s anomaly varies; it depends mainly on the displacement of the tricuspid valve. The most severe form usually has symptoms presenting in the neonatal stage or early infancy, whereas patients with mild displacement may remain asymptomatic until late adult life. The symptoms of our patient should be moderately severe because the pathologic features were compatible with that of type B according to Carpentier’s classification. However, systemic pressure of the right ventricle resulting from coexistence of TOF aggravated the severity of TR and heart failure. Thus, intractable right heart failure ensued even after maximal medication and made early surgical intervention necessary.
How to manage the patient surgically merits some discussion. The right complex heart brought about both heart failure and cyanosis. A palliative aortopulmonary shunt was not advisable because it would not prevent heart failure. Starnes procedure has been reserved only for those with the most severe form, and inevitably the patients had to accept the single ventricle physiology [4]. Even though the right ventricle was slightly hypoplastic in the preoperative evaluation, we preferred the biventricular repair because the right ventricular function seemed adequate. We adopted Carpentier’s technique because the vertical plication may restore the morphology and function of right ventricle better, yet the prosthetic ring was not used due to the infant’s growth potential [2]. Fortunately, the patient could be weaned from the pump smoothly after total correction of both lesions. The hemodynamic data were satisfactory and transesophageal echocardiography showed only mild TR. Therefore the salvage "one and one half ventricle repair" procedure [5] that is designed to unload the hypoplastic and dysplastic right ventricle by means of a bi-directional cavopulmonary shunt was not used. Albeit, long term follow-up is indeed necessary because the postoperative echocardiography revealed mild pulmonary stenosis and mild to moderate TR.
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References
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- Carpentier, A Chauvaud, S Mace, L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve J Thorac Cardiovasc Surg 1988;96:92-101.[Abstract]
- Quaegebeur, JM Sreeram, N Fraser, AG, et al. Surgery for Ebstein’s anomaly: the clinical and echocardiographic evaluation of a new technique J Am Coll Cardiol 1991;17:722-728.[Abstract]
- Chan, KC Walker, DR Gibbs, JL. Surgical correction of tetralogy of Fallot in the presence of Ebstein’s anomaly of the tricuspid valve Int J Cardiol 1989;25:242-243.[Medline]
- Starnes, VA Pitlick, PT Bernstein, D Griffin, ML Choy, M Shumway, N. Ebstein’s anomaly appearing in the neonate: a new surgical approach J Thorac Cardiovasc Surg 1991;101:1082-1087.[Abstract]
- Stellin, G Vida, VL Milanesi, O, et al. Surgical treatment of complex cardiac anomalies: the "one and one half ventricle repair" Eur J Cardiothorac Surg 2002;22:1043-1049.[Abstract/Free Full Text]
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Abstract
Introduction
