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Ann Thorac Surg 1998;65:540
© 1998 The Society of Thoracic Surgeons

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Case Reports

Tricuspid Valve Closure for Neonatal Ebstein’s Anomaly

Department of Thoracic and Cardiovascular Surgery, Tohoku University School of Medicine, Sendai, Japan
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan

Accepted for publication September 13, 1997.

Dr Endo, Department of Thoracic and Cardiovascular Surgery, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-77, Japan.

	Abstract

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A respirator-dependent 11-day-old boy with Ebstein’s anomaly is presented. His cardiac anomaly with progressive cardiomegaly was first noticed at 24 weeks’ gestation on fetal echocardiography. After birth, he required mechanical ventilation because of massive tricuspid regurgitation and restricted pulmonary blood flow. The operation of tricuspid valve patch closure, resection of right atrial wall, and central shunt successfully weaned him from the respirator on postoperative day 13. Cavopulmonary anastomosis was performed without problem when the patient was 8 months of age.

	Introduction

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Ebstein’s anomaly with severe cardiomegaly at birth carries a poor prognosis [1] [2]. These patients are always dependent on the patent ductus arteriosus because of reduced forward flow through the pulmonary valve associated with severe tricuspid valve regurgitation. Those cases are also complicated with volume reduction of both lungs due to the compression by an enlarged heart [3]. This volume reduction was thought to cause underdevelopment of the lung parenchyma.

Cardiomegaly was noticed at 24 weeks’ gestation by fetal echocardiography demonstrating severe tricuspid regurgitation associated with an anomalously large anterior leaflet, downward displacement of the septal leaflet, and a dilated right atrium (Fig 1); the abnormality had not been detected at 19 weeks. The cardio:thoracic area ratio was 0.40 at 26 weeks and increased to 0.47 at 28 weeks’ gestation.

View larger version (87K): [in this window] [in a new window]   (A) Fetal echocardiogram at 30 weeks’ gestation demonstrating the downward displacement of septal leaflet of tricuspid valve and the enlarged right atrium. (B) Postnatal, preoperative echocardiogram showing the further enlarged right atrium. (LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.)

View larger version (155K): [in this window] [in a new window]   Chest roentgenogram showing severe cardiomegaly, with a cardiothoracic ratio of 0.92.

He came back to us to undergo the bidirectional Glenn shunt when he was 8 months old and weighed 8.2 kg. The main pulmonary artery was not closed to keep the pathway of the coronary sinus blood to the pulmonary artery. After discharge, he has been doing well and waiting for the appropriate time for a modified Fontan operation.

	Comment

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Surgical treatment of the patient with symptomatic Ebstein’s anomaly has been performed to stabilize the pulmonary blood flow and to get rid of tricuspid regurgitation. However, the neonate whose condition is complicated with a very enlarged heart has another critical problem of lung hypoplasia. Hornberger and associates [3] reported that 6 of 11 autopsy cases of fetuses and neonates with Ebstein’s anomaly had hypoplastic lungs that weighed less than half of the normal lung for their body weights.

Tanaka and colleagues [4] examined lung:body weight ratios and radial alveolar counts in the lungs from 3 autopsy cases with neonatal Ebstein’s anomaly, and revealed that the lung parenchyma was reasonably matured even though the lung was compressed by the enlarged heart. They suggested that the surgical relief of lung compression might improve respiratory function in full-term neonates even if the cardiothoracic ratio was more than 0.9. On the other hand, the hypoplastic lung caused by diaphragmatic hernia displayed underdevelopment of lung parenchyma. The difference in lung parenchyma between these two disorders is probably explained by the fact that the lung is compressed by the enlarged heart at a relatively late stage of gestation, after the age of alveolar development from 24 weeks’ gestation [5], in the Ebstein’s anomaly, compared with early lung compression in the case of diaphragmatic hernia [6] [7]. In the present case, cardiomegaly progressed after 24 weeks’ gestation on fetal echocardiography. Thus, we presumed that the lung could expand and improve its function when the compression by the heart was relieved. In fact, a marked improvement of respiratory state was obtained after the operation.

Surgically creating tricuspid atresia by closing the tricuspid valve, resecting the right atrial wall, and placing an aortopulmonary shunt was advocated by Starnes and associates [8] as a palliation for symptomatic infants with Ebstein’s anomaly. This procedure corrected tricuspid regurgitation and cardiac enlargement in addition to establishing a reliable pulmonary blood flow. We agree that this method simplifies the cardiopulmonary condition of those critically ill neonates and improves the prognosis of this disorder, although it abandons biventricular repair as a correction. We also emphasize that downsizing of the right atrium by generous resection is necessary to allow the lung to expand well and normalize its function.

	References

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1. Roberson DA, Silverman NH Ebstein’s anomaly: echocardiographic and clinical feature in the fetus and neonate. J Am Coll Cardiol 1989;14:1300-1307.[Abstract]
2. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RKH, Deanfield JE Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 1992;19:1041-1046.[Abstract]
3. Hornberger LK, Sahn DJ, Kleiman CS, Copel JA, Reed KL Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome. J Am Coll Cardiol 1991;17:167-173.[Abstract]
4. Tanaka T, Kan T, Yamada M, et al. Three cases of neonatal Ebstein’s anomaly with remarkable cardiomegaly. Jpn J Pediatr Cardiol 1994;10:375-379.
5. Wigglesworth JS Pathology of the lung in the fetus and neonate, with particular reference to problems of growth and maturation. Histopathology 1987;11:671-689.[Medline]
6. Wigglesworth JS, Desai R Fetal lung hypoplasia: biochemical and structural variation and their possible significance. Arch Dis Child 1981;56:606-615.[Abstract/Free Full Text]
7. George DK, Cooney TP, Chiu BK, Thurlbeck WM Hypoplasia and immaturity of the terminal lung unit (acinus) in congenital diaphragmatic hernia. Am Rev Respir Dis 1987;136:947-950.[Medline]
8. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE Ebstein’s anomaly appearing in the neonate: a new surgical approach. J Thorac Cardiovasc Surg 1991;101:1082-1087.[Abstract]

This article has been cited by other articles:

				T.-J. Yun, S.-H. Lee, and J.-K. Ko Neonatal stenotic Ebstein's anomaly: A novel technique of right ventricular exclusion J. Thorac. Cardiovasc. Surg., February 1, 2006; 131(2): 469 - 471. [Full Text] [PDF]

				C. J. Knott-Craig, E. D. Overholt, K. E. Ward, J. M. Ringewald, S. S. Baker, and J. D. Razook Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up Ann. Thorac. Surg., June 1, 2002; 73(6): 1786 - 1793. [Abstract] [Full Text] [PDF]

				C. J. Knott-Craig, E. D. Overholt, K. E. Ward, and J. D. Razook Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up Ann. Thorac. Surg., May 1, 2000; 69(5): 1505 - 1510. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Masato Endo Mikio Ohmi Takashi Tanaka Koichi Tabayashi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Endo, M. Articles by Tabayashi, K. Search for Related Content PubMed PubMed Citation Articles by Endo, M. Articles by Tabayashi, K.