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Long-term results after surgical correction of Ebstein’s anomaly
Long-term results after surgical correction of Ebstein's anomaly Report of two cases Two patients with Ebstein's anomaly had valve replacement with Starr-Edwards cloth-covered valves 11 and 8 years ago. They have been asymptomatic and very active despite the absence of anticoagulation. These cases provide some of the long-term results needed for proper evaluation of the different methods of operative treatment of Ebstein's anomaly.
J. Melo, M.D.,* A. Saylam, M.D.,* R. Knight, M.D.,** and A. Starr, M.D.,*** Portland, Ore.
T he method of choice for surgical treatment of Ebstein's anomaly is not finalized. Many reports indicate poor results with mechanical prostheses, but they also have short follow-up periods. We present our long-term results with two patients, II and 8 years after tricuspid valve replacement with caged-ball valves, without anticoagulation.
Case reports The total experience with operative treatment of Ebstein's anomaly at the University of Oregon Health Sciences Center is three cases. One patient had a previous, unsuccessful Hardy procedure at another center and was referred for operation in severe congestive heart failure. Following reoperation, it was impossible to wean him from cardiopulmonary bypass and he died. The other two cases constitute the basis of this report. CASE l. A 21-year-old Caucasian man was given the diagnosis of Ebstein 's malformation in 1960. He remained in Functional Class I until 1967, when atrial flutter with varying stages of heart block and secondary congestive heart failure developed. Despite successful cardioversion, congestive
From the Division of Cardiopulmonary Surgery, the University of Oregon Health Sciences Center, Portland, Ore. Received for publication Feb. 21, 1979. Accepted for publication March 21, 1979. Address for reprints: A. Starr, M.D., Division of Cardiopulmonary Surgery, 3181 S.W. Sam Jackson Park Rd., Portland, Ore. 97201.
*Fellow. **ChiefResident. ***Professor and Chief of Cardiopulmonary Surgery.
heart failure persisted and progressive enlargement of the heart was noted on chest x-ray films. Heart catheterization showed a right atrial pressure of 21 mm Hg. He was operated upon in October of 1967. Classic findings of Ebstein's anomaly without associated malformations were observed. The atrialized portion of the right ventricle was fairly small. The tricuspid valve was excised and replaced with a StarrEdwards cloth-covered ball valve (Model 6310, 30 mm anulus diameter) with 3-0 interrupted Dacron sutures. The valve was placed in the normal anatomic position. No attempt was made to correct the atrialized ventricle. After operation, the right atrial pressure dropped to 10 mm Hg. The postoperative course was uneventful. A chest roentgenogram 3 months after the operation showed a sizeable decrease in heart size, with a cardiothoracic ratio of 0.5. Eleven years postoperatively, the patient is asymptomatic and plays basketball regularly. The cardiothoracic ratio is still 0.5 (Fig. I). He has never received anticoagulants. CAS E 2. A 15-year-old cyanotic Caucasian girl was referred because of two fainting episodes. Cardiomegaly was present on the chest x-ray film. Electrocardiographic studies revealed nodal tachycardia and heart catheterization showed Ebstein's anomaly (arterial oxygen saturation of 62%, with normal pressures). She was operated upon in June of 1969. A patent foramen ovale was closed. The tricuspid valve was excised and replaced with a Starr-Edwards cloth-covered ball valve (Model 6310, 32 mm anulus diameter) with 2-0 Dacron stitches. Along the posterior and lateral walls of the tricuspid anulus, these sutures were placed first into the anatomic anulus and next into the downward displaced zone. When these sutures were placed through the prosthesis and tied, they effectively plicated the atrialized portion of the ventricle. The postoperative course was benign despite permanent right bundle branch block. Seven years later, 1976, she was delivered of a healthy baby. Eight years after the operation, she is carrying on a normal life, including skiing. She, too, has never received anticoagulants.
0022-5223/79/080233+03$00.30/0 © 1979 The C. V. Mosby Co.
233
The Journal of Thoracic and Cardiovascular Surgery
234 Melo et al.
Fig. 1. Case I. Chest roentgenogram II years after tricuspid valve replacement for Ebstein's anomaly.
Discussion The choice of treatment for Ebstein's anomaly is dependent upon its natural history relative to the age of presentation of the patients. 1-6 Our two patients were older than 15 years. In this age group, 60% of the patients are in Functional Class I or II, and neither cardiomegaly nor ventricular paroxysmal arrhythmia is predictive of sudden death." Medical management is the most appropriate treatment for these patients. Surgical treatment is indicated only for those adults with congestive heart failure unresponsive to medical treatment, paradoxical embolus, or severe cyanosis.": 7. 8 Several surgical approaches are currently used to correct this anomaly. The trend seems to be toward a corrective procedure without valve replacement. This avoids the danger of thrombosis of prosthetic valves in the tricuspid position and achieves, as well, a better hemodynamic result. 5. 8 If tricuspid valve replacement is necessary, tissue valves are the current choice. Because of these reports, we also favor this approach.v J" Even so, we think that more information about longterm results is necessary for its proper evaluation. The results of our past experience, performed under a different surgical approach, are surprising. They prove that good long-term results can be obtained with a ball-valve prosthesis, even without anticoagulation.
2 3
4 5
6 7 8
REFERENCES Sekelj P, Benfey B: Historical landmarks. Ebstein's anomaly of the tricuspid valve. Am Heart J 88:108-114, 1971 Kumar ED, Fyler D, Miettinen as, Nadas AS: Ebsteiri's anomaly. Clinical profile and natural history. Am J CardioI28:84-95, 1971 Watson H: Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. An international cooperative study of 505 cases. Br Heart J 36:417427, 1974 Genton E, Blount SG: The spectrum of Ebstein's anomaly. Am Heart J 73:395-425, 1967 Deutsch V, Wexler L, Blieden LC, Yahini JH, Neufeld HN: Ebsteiri's anomaly of tricuspid valve. Critical review of roentgenological features and additional angiographic signs. Am J Roentgenol Radium Ther Nucl Med 125: 395-411, 1975 Farooki ZQ, Henry JG, Green EW: Echocardiographic spectrum of Ebsteiri's anomaly of the tricuspid valve. Circulation 53:63-68, 1976 Roe BB: Ebsteins anomaly, Gibbon's Surgery of the Chest, ed 3, DC Sabiston, FC Spencer, eds., Philadelphia, 1976, W. B. Saunders Company, pp 1170-1175 McFaul R, Davis Z, Guiliani ER, Ritter DG, Danielson G: Ebstein' s malformation. Surgical experience at the Mayo CLinic. J THoRAc CARDIOVASC SURG 72:910-915, 1976
Volume 78
Ebstein's anomaly
Number 2
235
August. 1979
9 Jugdutt BI, Brooks CH, Stems LP, Callaghan JC, Rossall RE: Surgical treatment of Ebstein's anomaly. J THORAC CARDIOVASC SURG 73:115-119, 1977 10 Senoo Y, Ohishi K, Nawa S, Teramoto S, Sunada T: Total correction of Ebstein's anomaly by replacement with a biological aortic valve without plication of the atrialized ventricle. J THORAC CARDIOVASC SURG 72: 243-248, 1976 II Ross D, Sommerville J: Surgical correction of Ebstein's anomaly. Lancet 2:280-284, 1970
12 Hardy KL, May lA, Webster CA, Kimball KG: Ebstein's anomaly. A functional concept and a successful definitive repair. J THORAC CARDIOVASC SURG 48:927-940, 1964 13 Bernard CN, Schrire V: Surgical correction of Ebsteiri's malformation with prosthetic tricuspid valve. Surgery 54:302-308, 1963 14 Hansen JF, Leth A, Dorph S, Wennevold A: The prognosis in Ebsteiri's disease of the heart. Long-term follow-up of 22 patients. Acta Med Scand 201:331-335, 1977
Copyright information The appearance of a code at the bottom of the first page of an original article in this journal indicates the copyright owner's consent that copies of the article may be made for personal or internal use, or for the personal or internal use of specific clients. This consent is given on the condition, however, that the copier pay the stated per copy fee through the Copyright Clearance Center, Inc., P.O. Box 765, Schenectady, N. Y. 12301,518-3744430, for copying beyond that permitted by Sections 107 or 108 of the U. S. Copyright Law. This consent does not extend to other kinds of copying, such as copying for general distribution, for advertising or promotional purposes, for creating new collective works, or for resale.
J. Melo, M.D.,* A. Saylam, M.D.,* R. Knight, M.D.,** and A. Starr, M.D.,*** Portland, Ore.
T he method of choice for surgical treatment of Ebstein's anomaly is not finalized. Many reports indicate poor results with mechanical prostheses, but they also have short follow-up periods. We present our long-term results with two patients, II and 8 years after tricuspid valve replacement with caged-ball valves, without anticoagulation.
Case reports The total experience with operative treatment of Ebstein's anomaly at the University of Oregon Health Sciences Center is three cases. One patient had a previous, unsuccessful Hardy procedure at another center and was referred for operation in severe congestive heart failure. Following reoperation, it was impossible to wean him from cardiopulmonary bypass and he died. The other two cases constitute the basis of this report. CASE l. A 21-year-old Caucasian man was given the diagnosis of Ebstein 's malformation in 1960. He remained in Functional Class I until 1967, when atrial flutter with varying stages of heart block and secondary congestive heart failure developed. Despite successful cardioversion, congestive
From the Division of Cardiopulmonary Surgery, the University of Oregon Health Sciences Center, Portland, Ore. Received for publication Feb. 21, 1979. Accepted for publication March 21, 1979. Address for reprints: A. Starr, M.D., Division of Cardiopulmonary Surgery, 3181 S.W. Sam Jackson Park Rd., Portland, Ore. 97201.
*Fellow. **ChiefResident. ***Professor and Chief of Cardiopulmonary Surgery.
heart failure persisted and progressive enlargement of the heart was noted on chest x-ray films. Heart catheterization showed a right atrial pressure of 21 mm Hg. He was operated upon in October of 1967. Classic findings of Ebstein's anomaly without associated malformations were observed. The atrialized portion of the right ventricle was fairly small. The tricuspid valve was excised and replaced with a StarrEdwards cloth-covered ball valve (Model 6310, 30 mm anulus diameter) with 3-0 interrupted Dacron sutures. The valve was placed in the normal anatomic position. No attempt was made to correct the atrialized ventricle. After operation, the right atrial pressure dropped to 10 mm Hg. The postoperative course was uneventful. A chest roentgenogram 3 months after the operation showed a sizeable decrease in heart size, with a cardiothoracic ratio of 0.5. Eleven years postoperatively, the patient is asymptomatic and plays basketball regularly. The cardiothoracic ratio is still 0.5 (Fig. I). He has never received anticoagulants. CAS E 2. A 15-year-old cyanotic Caucasian girl was referred because of two fainting episodes. Cardiomegaly was present on the chest x-ray film. Electrocardiographic studies revealed nodal tachycardia and heart catheterization showed Ebstein's anomaly (arterial oxygen saturation of 62%, with normal pressures). She was operated upon in June of 1969. A patent foramen ovale was closed. The tricuspid valve was excised and replaced with a Starr-Edwards cloth-covered ball valve (Model 6310, 32 mm anulus diameter) with 2-0 Dacron stitches. Along the posterior and lateral walls of the tricuspid anulus, these sutures were placed first into the anatomic anulus and next into the downward displaced zone. When these sutures were placed through the prosthesis and tied, they effectively plicated the atrialized portion of the ventricle. The postoperative course was benign despite permanent right bundle branch block. Seven years later, 1976, she was delivered of a healthy baby. Eight years after the operation, she is carrying on a normal life, including skiing. She, too, has never received anticoagulants.
0022-5223/79/080233+03$00.30/0 © 1979 The C. V. Mosby Co.
233
The Journal of Thoracic and Cardiovascular Surgery
234 Melo et al.
Fig. 1. Case I. Chest roentgenogram II years after tricuspid valve replacement for Ebstein's anomaly.
Discussion The choice of treatment for Ebstein's anomaly is dependent upon its natural history relative to the age of presentation of the patients. 1-6 Our two patients were older than 15 years. In this age group, 60% of the patients are in Functional Class I or II, and neither cardiomegaly nor ventricular paroxysmal arrhythmia is predictive of sudden death." Medical management is the most appropriate treatment for these patients. Surgical treatment is indicated only for those adults with congestive heart failure unresponsive to medical treatment, paradoxical embolus, or severe cyanosis.": 7. 8 Several surgical approaches are currently used to correct this anomaly. The trend seems to be toward a corrective procedure without valve replacement. This avoids the danger of thrombosis of prosthetic valves in the tricuspid position and achieves, as well, a better hemodynamic result. 5. 8 If tricuspid valve replacement is necessary, tissue valves are the current choice. Because of these reports, we also favor this approach.v J" Even so, we think that more information about longterm results is necessary for its proper evaluation. The results of our past experience, performed under a different surgical approach, are surprising. They prove that good long-term results can be obtained with a ball-valve prosthesis, even without anticoagulation.
2 3
4 5
6 7 8
REFERENCES Sekelj P, Benfey B: Historical landmarks. Ebstein's anomaly of the tricuspid valve. Am Heart J 88:108-114, 1971 Kumar ED, Fyler D, Miettinen as, Nadas AS: Ebsteiri's anomaly. Clinical profile and natural history. Am J CardioI28:84-95, 1971 Watson H: Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. An international cooperative study of 505 cases. Br Heart J 36:417427, 1974 Genton E, Blount SG: The spectrum of Ebstein's anomaly. Am Heart J 73:395-425, 1967 Deutsch V, Wexler L, Blieden LC, Yahini JH, Neufeld HN: Ebsteiri's anomaly of tricuspid valve. Critical review of roentgenological features and additional angiographic signs. Am J Roentgenol Radium Ther Nucl Med 125: 395-411, 1975 Farooki ZQ, Henry JG, Green EW: Echocardiographic spectrum of Ebsteiri's anomaly of the tricuspid valve. Circulation 53:63-68, 1976 Roe BB: Ebsteins anomaly, Gibbon's Surgery of the Chest, ed 3, DC Sabiston, FC Spencer, eds., Philadelphia, 1976, W. B. Saunders Company, pp 1170-1175 McFaul R, Davis Z, Guiliani ER, Ritter DG, Danielson G: Ebstein' s malformation. Surgical experience at the Mayo CLinic. J THoRAc CARDIOVASC SURG 72:910-915, 1976
Volume 78
Ebstein's anomaly
Number 2
235
August. 1979
9 Jugdutt BI, Brooks CH, Stems LP, Callaghan JC, Rossall RE: Surgical treatment of Ebstein's anomaly. J THORAC CARDIOVASC SURG 73:115-119, 1977 10 Senoo Y, Ohishi K, Nawa S, Teramoto S, Sunada T: Total correction of Ebstein's anomaly by replacement with a biological aortic valve without plication of the atrialized ventricle. J THORAC CARDIOVASC SURG 72: 243-248, 1976 II Ross D, Sommerville J: Surgical correction of Ebstein's anomaly. Lancet 2:280-284, 1970
12 Hardy KL, May lA, Webster CA, Kimball KG: Ebstein's anomaly. A functional concept and a successful definitive repair. J THORAC CARDIOVASC SURG 48:927-940, 1964 13 Bernard CN, Schrire V: Surgical correction of Ebsteiri's malformation with prosthetic tricuspid valve. Surgery 54:302-308, 1963 14 Hansen JF, Leth A, Dorph S, Wennevold A: The prognosis in Ebsteiri's disease of the heart. Long-term follow-up of 22 patients. Acta Med Scand 201:331-335, 1977
Copyright information The appearance of a code at the bottom of the first page of an original article in this journal indicates the copyright owner's consent that copies of the article may be made for personal or internal use, or for the personal or internal use of specific clients. This consent is given on the condition, however, that the copier pay the stated per copy fee through the Copyright Clearance Center, Inc., P.O. Box 765, Schenectady, N. Y. 12301,518-3744430, for copying beyond that permitted by Sections 107 or 108 of the U. S. Copyright Law. This consent does not extend to other kinds of copying, such as copying for general distribution, for advertising or promotional purposes, for creating new collective works, or for resale.