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Valve replacement for Ebstein’s anomaly of the tricuspid valve
Valve replacement for Ebstein's anomaly of the tricuspid valve The role of valve replacement in the treatment of Ebstein' s anomaly of the tricuspid valve remains controversial. Between 1965 and 1977, five patients with Ebstein:s anomaly ranging in age from 11 to 57 years (mean 29) underwent tricuspid valve replacement in our institution. All of the patients were cyanotic but one, three were in Functional Class 111, and two were in Class IV (New York Heart Association classification). The valve was placed above the coronary sinus. Plication of the atrialized portion of right ventricle was required in only one patient. Four patients had additional closure of an atrial septal defect. There were no deaths. On follow-up (range 1 to 13 years), four patients are in Functional Class 1 and one is in Class ll. None is cyanotic. Four patients are in sinus rhythm and one has a pacemaker because of postoperative complete heart block. We conclude that valve replacement and, when necessary, plication of the atrialired right ventricle produce excellent clinical improvement.
Edward L. Bove, M.D., and Marvin M. Kirsh, M.D., Ann Arbor, Mich.
T
he ideal surgical management of Ebstein's anomaly is not yet established. The choice today between valve replacement and reconstruction remains controversial. Barnard;' Lillehei," Kitamura," and their associates reported favorable results following tricuspid valve replacement, whereas McFaul and colleagues- found that replacement of the malformed tricuspid valve was almost always unsuccessful. Because Ebstein's anomaly is a relatively rare congenital malformation, there are no reported series with a large number of patients undergoing operation. Since 1965, seven patients underwent operation for Ebstein's anomaly at the University of Michigan Medical Center. Two asymptomatic patients underwent annuloplastic repair, and five patients underwent tricuspid valve replacement. The present study was undertaken to assess the long-term results following valve replacement for Ebstein's anomaly.
Technique of operation and operative findings
The operative approach was through a median sternotomy incision. All patients were noted to have a dilated right atrium. Standard bicaval cannulation techniques were utilized. The ascending aorta or the right From the Department of Surgery, Section of Thoracic Surgery, The University of Michigan Medical Center, Ann Arbor, Mich. Received for publication Jan. 31, 1979. Accepted for publication April 17, 1979. Address for reprints: Marvin M. Kirsh, M.D., Professor of Surgery, Section of Thoracic Surgery, The University of Michigan Medical Center, Ann Arbor, Mich. 48109.
femoral artery was the site of cannulation for arterial inflow. Only three patients had the typical thin-walled atrialized right ventricular segment with a paradoxical bulge. This area was obliterated with additional mattress sutures over two long Teflon felt strips in one patient. Plication was not felt necessary in the remaining two patients. Abnormalities of the tricuspid valve were consistent with Ebstein's anomaly in all five patients. Four patients had an atrial communication; in one there was a patent foramen ovale and in three an ostiurn secundum atrial septal defect. The leaflets of the tricuspid valve along with the chordae tendineae and papillary muscles were excised. A Starr-Edwards ball valve was used in three patients and a porcine heterograft prosthesis (Hancock Laboratories, Inc., Anaheim, Calif.) was used in two patients. The technique of valve replacement was that described by Barnard and Schrire.! The prosthetic valve was sutured with buttressed Teflon sutures into the normal atrioventricular anulus, except at the region of the coronary sinus, where the sutures were placed above the coronary sinus to avoid injury to the atrioventricular conduction system. The coronary sinus opening was beneath the prosthesis after insertion of the valve. The atriotomy was closed with the use of Teflon felt strips to buttress the suture line. Patients
The preoperative clinical status, age of the patient, and the postoperative condition of our five patients are listed in Table I. They ranged in age from 11 to 57
0022-5223/79/080229+04$00.40/0 © 1979 The C. V. Mosby Co.
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The Journal of
Rove and Kirsh
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Thoracic and Cardiovascular Surgery
Table I Atrialized Age (yr)
Preop. NYHA Class
11
III
Yes
NSR
Yes
2
17
III
Yes
NSR, WPW(B)
Yes
3 4
57 36
IV III
No Yes
NSR,RBBB NSR,RBBB
No Yes
5
27
IV
Yes
NSR, RBBB, WPW (B)
No
Case No.
RV
Cyanosis
Preop. ECG
segment
Operative procedure Hancock valve (35 mm), pIication of atrialized segment, closure of PFO Starr-Edwards valve (4M), closure of ASD Hancock valve (33 mm) Starr-Edwards valve (4M), ASD closed with perforated patch Starr-Edwards valve (4M), closure of ASD
Postop. NYHA Class
Postop. ECG NSR,RBBB
3
NSR
9
NSR CHB, pacemaker
II
Followup (yr)
NSR, tachyarrhythmias
I 13
8
Legend: NYHA, New York Heart Association. ECG, Electrocardiogram. RV, Right ventricular. NSR, Normal sinus rhythm. WPW (B), Wolfe-Parkinson-White
syndrome (type B). RBBB, Right bundle branch block. PFO, Patent foramen ovale. ASD, Atrial septal defect. CHB, Complete heart block.
years (mean 29.6 years). At the time of operation, three patients were in Functional Class III and two patients were in Functional Class IV (New York Heart Association classification). All were cyanotic but one. The indication for operation was progressive cyanosis in four patients and severe exercise intolerance in the remaining patient. In all patients, the chest roentgenogram revealed marked cardiomegaly with diminished pulmonary vascular markings. Normal sinus rhythm was present in all patients. Right bundle branch block was noted in three patients and the Wolff-Parkinson- White syndrome (type B) in two patients. Four patients experienced recurrent episodes of supraventricular tachycardia. All patients were treated with digoxin and quinidine preoperatively. Cardiac catheterizations was performed in all patients, and the typical findings of Ebstein's anomaly were present on the angiograms: an enlarged right atrium, displaced tricuspid valve, and in four patients a right-to-left shunt at the atrial level. In three patients there was a dilated atrialized right ventricle and in all patients an area demonstrating a ventricular electrocardiogram with an atrial pressure tracing was present. In addition, the right ventricular pressures were normal in all patients. Results All patients survived operation. At follow-up I, 3, 8, 9, and 13 years after operation, respectively, the patients were acyanotic and clinically improved, with four being in Functional Class I and one in Functional Class II. At present, four patients are in sinus rhythm and one has a pacemaker because of postoperative complete heart block. Only one patient still experiences
intermittent supraventricular tachycardia which, although present preoperatively, is now well tolerated. On chest roentgenogram there has been a marked decrease in heart size in all patients. Discussion Ebstein's anomaly is characterized by the downward displacement of a malformed tricuspid valve. Displacement of the hypoplastic septal and posterior leaflets into the right ventricle partitions the right side of the heart into three chambers: an atrium, a proximal "atrialized" ventricular segment, and a diminutive distal ventricular ejecting chamber. The proximal part usually is dilated and thin walled, and the tricuspid valve almost always is incompetent. The anterior leaflet is large and sail-like and is attached normally to the anulus. However, at times there may be anomalous attachments of its chordae tendineae and papillary muscles, producing right ventricular outflow tract obstruction. An interatrial communication is frequently present and may result in significant right-to-left shunting. The physiological derangements in Ebstein's anomaly result from a combination of abnormalities. The primary hemodynamic disturbance consists of inadequate right ventricular output. This occurs because the small right ventricular pumping chamber distal to the insertion of the tricuspid valve cannot receive a normal volume of blood in diastole, During systole, the incompetent tricuspid valve allows free regurgitation of blood back into the right atrium. Because of the decreased volume and distensibility of this distal pumping chamber, the receiving chamber, composed of the right atrium and "atrialized" right ventricle, is unable to empty itself completely. In some instances, the right
Volume 78 Number 2 August, 1979
ventricular output is further reduced by outflow obstruction resulting from the abnormal insertion of the chordae tendineae. The resultant right atrial hypertension produces right-to-left shunting through any associated defects in the atrial septum, further decreasing forward flow. In general, the surgical treatment of Ebstein's anomaly should accomplish the following: (1) relocation of the tricuspid valve in the correct anatomic position; (2) correction of regurgitation or stenosis; (3) closure of interatrial septal defects; (4) removal of right ventricular outflow tract obstruction; and (5) improvement in the functional capacity of the right ventricle. The surgical approach to these goals has been through valve replacement or reconstruction. Methods incorporating both of these approaches also have been utilized. The reconstructive technique devised by Hardy and colleagues" excludes the atrialized ventricle by simultaneous plication and tricuspid valve annuloplasty. Although excellent clinical results have been obtained with this technique, it is not always applicable because of the wide spectrum of deviations in anatomy that exist in Ebstein's anomaly." Bahnson and associates? described variations of the defect in which reconstruction was not feasible because of fusion of the septal and posterior leaflets to the ventricular wall and bizarre attachments of the chordae tendineae and papillary muscle. Reconstruction also may not be possible because of a small-sized anterior leaflet. If repair were attempted under these circumstances, the patient would be left with residual tricuspid insufficiency and an unsatisfactory result. In 1963 Barnard and Schrire! reported the case histories of two patients with Ebstein's anomaly in whom successful correction was achieved by valve replacement. Shortly thereafter, Lillehei," Cartwright," and their co-workers also reported successful valve replacement for Ebstein's malformation. However, since that time valve replacement for Ebstein's anomaly has met with variable success. In an international cooperative study of patients with Ebstein's anomaly, Watson? found that nine of 16 patients undergoing valve replacement died postoperatively. Similar poor results recently have been reported from the Mayo Clinic by McFaul's group"; i.e., four of their five patients died after undergoing prosthetic valve replacement. On the other hand, a review of the literature by Kitamura and co-workers" revealed that only three of 25 patients died following valve replacement for Ebstein's anomaly. There were no long-term follow-up studies in any of the successfully treated patients. We have achieved excellent results with valve re-
Ebstein's anomaly of tricuspid valve
231
placement. All of our patients survived the operation and remained in markedly improved condition at longterm follow-up (range I to 13 years). Four patients are in Functional Class I and one is in Class II. None is cyanotic. The reasons for the difference in our results following valve replacement are not readily apparent. Although Starr'? has stated that the caged-ball valve is not suitable for tricuspid valve replacement because of a high incidence of thrombus formation, we have not encountered any embolic problems with the caged-ball prosthesis in the tricuspid position. A porcine prosthesis was used in the two most recently treated patients only because of our preference for this valve. LiIIehei and associates" have advocated that closure of the interatrial septal defect be delayed to avoid postoperative congestive heart failure. On the basis of our experience and that of others, we believe that delayed closure is not necessary since heart failure rarely occurs after satisfactory repair or replacement of the tricuspid valve. There is a divergence of opinion concerning the atrialized ventricle and its role in determining right ventricular output. Hardy, 5 Timmis, II Jugdutt;'" and others believe that the atrialized ventricle interferes with filling of the lower portion of the ventricle and with ejection of blood because of paradoxical distention; they contend that it must be obliterated at the time of surgical correction. On the other hand, Barnard and Schrire' felt that although the atrialized ventricle does not appear to contract well during ventricular systole, this condition does not interfere with right ventricular function and need not be corrected. Senoo's group'" achieved excellent clinical results in three patients with Ebstein's anomaly by replacement of the valve alone, without plicating the atrialized ventricle. We could not determine the role of plication of the atrialized ventricle from our experience, because obliteration of this chamber was deemed necessary in only one patient. Perhaps the diverse results are due to the variability of the size of the atrialized ventricle, which sometimes is small and thick walled and exhibits minimal paradoxical motion. Atrial arrhythmias are common in patients with Ebstein's anomaly and may further compromise an already reduced cardiac output. This was true in four of our patients who required frequent hospitalization for treatment. Although there may be no decrease in the frequency of arrhythmias, by correcting the hemodynamic abnormalities tricuspid valve replacement enables the patient to tolerate them better. This was the case in our patient who continued to experience arrhythmias postoperatively.
The Journal of
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Bove and Kirsh
On the basis of this study, we conclude that valve replacement and, when necessary, plication of the atrialized ventricle produces satisfactory and longlasting clinical improvement in patients with Ebstein's malformation. REFERENCES
2
3
4
5
6
Barnard CN, Schrire V: Surgical correction of Ebstein's malformation with prosthetic tricuspid valve. Surgery 54:302-308, 1963 Lillehei CW, Kalke BR, Carlson RG: Evolution of corrective surgery for Ebstein's anomaly. Circulation 35: Suppl 111-118, 1967 Kitamura S, Johnson JL, Redington JV, Mendez A, Zubiate P, Kay JH: Surgery for Ebstein's anomaly. Ann Thorac Surg 11:320-330, 1971 McFaul RC, Davis Z, Giuliani ER, Ritter DG, Danielson GK: Ebsteiu's malformation. Surgical experience at the Mayo Clinic. J THORAC CARDIOVASC SURG 72:910-915, 1976 Hardy KL, May lA, Webster CA, Kimball KG: Ebstein's anomaly. A functional concept and successful definitive repair. J THORAC CARDIOVASC SURG. 48:927-940, 1964 Najafi H, Hunter JA, Dye WS, Javid H, Julian OC; Ebstein's malformation of the tricuspid valve. Surgical management. Ann Thorac Surg 4:334-343, 1967
Thoracic and Cardiovascular Surgery
7 Bahnson HT, Bauersfeld SR, Smith JW: Pathological anatomy and surgical correction of Ebstein's anomaly. Circulation 31:Suppl 1:3-8, 1965 8 Cartwright RS, Smeloff EA, Cayler 00, Fong WY, Huntley AC, Blake JR, McFall RA: Total correction of Ebstein's anomaly by means of tricuspid replacement. J THORAC CARDIOVASC SURG 47:755-761, 1964 9 Watson H: Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. Br Heart J 36:417-427, 1974 10 Starr A: Panel discussion: Cardiac valve substitution. Caged-ball. Held at the Annual Meeting of the American College of Cardiology, New Orleans, La., February, 1970 II Timmis HH, Hardy JD, Watson 00: The surgical management of Ebstein's anomaly. J THORAC CARDIOVASC SURG 53:385-391, 1966 12 Jugdutt BI, Brooks CH, Stems LP, Callaghan JC, Rossall RE: Surgical treatment of Ebstein's anomaly. J THORAC CARDIOVASC SURG 73: 114-119, 1977 13 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 74: 243-248, 1976
Edward L. Bove, M.D., and Marvin M. Kirsh, M.D., Ann Arbor, Mich.
T
he ideal surgical management of Ebstein's anomaly is not yet established. The choice today between valve replacement and reconstruction remains controversial. Barnard;' Lillehei," Kitamura," and their associates reported favorable results following tricuspid valve replacement, whereas McFaul and colleagues- found that replacement of the malformed tricuspid valve was almost always unsuccessful. Because Ebstein's anomaly is a relatively rare congenital malformation, there are no reported series with a large number of patients undergoing operation. Since 1965, seven patients underwent operation for Ebstein's anomaly at the University of Michigan Medical Center. Two asymptomatic patients underwent annuloplastic repair, and five patients underwent tricuspid valve replacement. The present study was undertaken to assess the long-term results following valve replacement for Ebstein's anomaly.
Technique of operation and operative findings
The operative approach was through a median sternotomy incision. All patients were noted to have a dilated right atrium. Standard bicaval cannulation techniques were utilized. The ascending aorta or the right From the Department of Surgery, Section of Thoracic Surgery, The University of Michigan Medical Center, Ann Arbor, Mich. Received for publication Jan. 31, 1979. Accepted for publication April 17, 1979. Address for reprints: Marvin M. Kirsh, M.D., Professor of Surgery, Section of Thoracic Surgery, The University of Michigan Medical Center, Ann Arbor, Mich. 48109.
femoral artery was the site of cannulation for arterial inflow. Only three patients had the typical thin-walled atrialized right ventricular segment with a paradoxical bulge. This area was obliterated with additional mattress sutures over two long Teflon felt strips in one patient. Plication was not felt necessary in the remaining two patients. Abnormalities of the tricuspid valve were consistent with Ebstein's anomaly in all five patients. Four patients had an atrial communication; in one there was a patent foramen ovale and in three an ostiurn secundum atrial septal defect. The leaflets of the tricuspid valve along with the chordae tendineae and papillary muscles were excised. A Starr-Edwards ball valve was used in three patients and a porcine heterograft prosthesis (Hancock Laboratories, Inc., Anaheim, Calif.) was used in two patients. The technique of valve replacement was that described by Barnard and Schrire.! The prosthetic valve was sutured with buttressed Teflon sutures into the normal atrioventricular anulus, except at the region of the coronary sinus, where the sutures were placed above the coronary sinus to avoid injury to the atrioventricular conduction system. The coronary sinus opening was beneath the prosthesis after insertion of the valve. The atriotomy was closed with the use of Teflon felt strips to buttress the suture line. Patients
The preoperative clinical status, age of the patient, and the postoperative condition of our five patients are listed in Table I. They ranged in age from 11 to 57
0022-5223/79/080229+04$00.40/0 © 1979 The C. V. Mosby Co.
229
The Journal of
Rove and Kirsh
230
Thoracic and Cardiovascular Surgery
Table I Atrialized Age (yr)
Preop. NYHA Class
11
III
Yes
NSR
Yes
2
17
III
Yes
NSR, WPW(B)
Yes
3 4
57 36
IV III
No Yes
NSR,RBBB NSR,RBBB
No Yes
5
27
IV
Yes
NSR, RBBB, WPW (B)
No
Case No.
RV
Cyanosis
Preop. ECG
segment
Operative procedure Hancock valve (35 mm), pIication of atrialized segment, closure of PFO Starr-Edwards valve (4M), closure of ASD Hancock valve (33 mm) Starr-Edwards valve (4M), ASD closed with perforated patch Starr-Edwards valve (4M), closure of ASD
Postop. NYHA Class
Postop. ECG NSR,RBBB
3
NSR
9
NSR CHB, pacemaker
II
Followup (yr)
NSR, tachyarrhythmias
I 13
8
Legend: NYHA, New York Heart Association. ECG, Electrocardiogram. RV, Right ventricular. NSR, Normal sinus rhythm. WPW (B), Wolfe-Parkinson-White
syndrome (type B). RBBB, Right bundle branch block. PFO, Patent foramen ovale. ASD, Atrial septal defect. CHB, Complete heart block.
years (mean 29.6 years). At the time of operation, three patients were in Functional Class III and two patients were in Functional Class IV (New York Heart Association classification). All were cyanotic but one. The indication for operation was progressive cyanosis in four patients and severe exercise intolerance in the remaining patient. In all patients, the chest roentgenogram revealed marked cardiomegaly with diminished pulmonary vascular markings. Normal sinus rhythm was present in all patients. Right bundle branch block was noted in three patients and the Wolff-Parkinson- White syndrome (type B) in two patients. Four patients experienced recurrent episodes of supraventricular tachycardia. All patients were treated with digoxin and quinidine preoperatively. Cardiac catheterizations was performed in all patients, and the typical findings of Ebstein's anomaly were present on the angiograms: an enlarged right atrium, displaced tricuspid valve, and in four patients a right-to-left shunt at the atrial level. In three patients there was a dilated atrialized right ventricle and in all patients an area demonstrating a ventricular electrocardiogram with an atrial pressure tracing was present. In addition, the right ventricular pressures were normal in all patients. Results All patients survived operation. At follow-up I, 3, 8, 9, and 13 years after operation, respectively, the patients were acyanotic and clinically improved, with four being in Functional Class I and one in Functional Class II. At present, four patients are in sinus rhythm and one has a pacemaker because of postoperative complete heart block. Only one patient still experiences
intermittent supraventricular tachycardia which, although present preoperatively, is now well tolerated. On chest roentgenogram there has been a marked decrease in heart size in all patients. Discussion Ebstein's anomaly is characterized by the downward displacement of a malformed tricuspid valve. Displacement of the hypoplastic septal and posterior leaflets into the right ventricle partitions the right side of the heart into three chambers: an atrium, a proximal "atrialized" ventricular segment, and a diminutive distal ventricular ejecting chamber. The proximal part usually is dilated and thin walled, and the tricuspid valve almost always is incompetent. The anterior leaflet is large and sail-like and is attached normally to the anulus. However, at times there may be anomalous attachments of its chordae tendineae and papillary muscles, producing right ventricular outflow tract obstruction. An interatrial communication is frequently present and may result in significant right-to-left shunting. The physiological derangements in Ebstein's anomaly result from a combination of abnormalities. The primary hemodynamic disturbance consists of inadequate right ventricular output. This occurs because the small right ventricular pumping chamber distal to the insertion of the tricuspid valve cannot receive a normal volume of blood in diastole, During systole, the incompetent tricuspid valve allows free regurgitation of blood back into the right atrium. Because of the decreased volume and distensibility of this distal pumping chamber, the receiving chamber, composed of the right atrium and "atrialized" right ventricle, is unable to empty itself completely. In some instances, the right
Volume 78 Number 2 August, 1979
ventricular output is further reduced by outflow obstruction resulting from the abnormal insertion of the chordae tendineae. The resultant right atrial hypertension produces right-to-left shunting through any associated defects in the atrial septum, further decreasing forward flow. In general, the surgical treatment of Ebstein's anomaly should accomplish the following: (1) relocation of the tricuspid valve in the correct anatomic position; (2) correction of regurgitation or stenosis; (3) closure of interatrial septal defects; (4) removal of right ventricular outflow tract obstruction; and (5) improvement in the functional capacity of the right ventricle. The surgical approach to these goals has been through valve replacement or reconstruction. Methods incorporating both of these approaches also have been utilized. The reconstructive technique devised by Hardy and colleagues" excludes the atrialized ventricle by simultaneous plication and tricuspid valve annuloplasty. Although excellent clinical results have been obtained with this technique, it is not always applicable because of the wide spectrum of deviations in anatomy that exist in Ebstein's anomaly." Bahnson and associates? described variations of the defect in which reconstruction was not feasible because of fusion of the septal and posterior leaflets to the ventricular wall and bizarre attachments of the chordae tendineae and papillary muscle. Reconstruction also may not be possible because of a small-sized anterior leaflet. If repair were attempted under these circumstances, the patient would be left with residual tricuspid insufficiency and an unsatisfactory result. In 1963 Barnard and Schrire! reported the case histories of two patients with Ebstein's anomaly in whom successful correction was achieved by valve replacement. Shortly thereafter, Lillehei," Cartwright," and their co-workers also reported successful valve replacement for Ebstein's malformation. However, since that time valve replacement for Ebstein's anomaly has met with variable success. In an international cooperative study of patients with Ebstein's anomaly, Watson? found that nine of 16 patients undergoing valve replacement died postoperatively. Similar poor results recently have been reported from the Mayo Clinic by McFaul's group"; i.e., four of their five patients died after undergoing prosthetic valve replacement. On the other hand, a review of the literature by Kitamura and co-workers" revealed that only three of 25 patients died following valve replacement for Ebstein's anomaly. There were no long-term follow-up studies in any of the successfully treated patients. We have achieved excellent results with valve re-
Ebstein's anomaly of tricuspid valve
231
placement. All of our patients survived the operation and remained in markedly improved condition at longterm follow-up (range I to 13 years). Four patients are in Functional Class I and one is in Class II. None is cyanotic. The reasons for the difference in our results following valve replacement are not readily apparent. Although Starr'? has stated that the caged-ball valve is not suitable for tricuspid valve replacement because of a high incidence of thrombus formation, we have not encountered any embolic problems with the caged-ball prosthesis in the tricuspid position. A porcine prosthesis was used in the two most recently treated patients only because of our preference for this valve. LiIIehei and associates" have advocated that closure of the interatrial septal defect be delayed to avoid postoperative congestive heart failure. On the basis of our experience and that of others, we believe that delayed closure is not necessary since heart failure rarely occurs after satisfactory repair or replacement of the tricuspid valve. There is a divergence of opinion concerning the atrialized ventricle and its role in determining right ventricular output. Hardy, 5 Timmis, II Jugdutt;'" and others believe that the atrialized ventricle interferes with filling of the lower portion of the ventricle and with ejection of blood because of paradoxical distention; they contend that it must be obliterated at the time of surgical correction. On the other hand, Barnard and Schrire' felt that although the atrialized ventricle does not appear to contract well during ventricular systole, this condition does not interfere with right ventricular function and need not be corrected. Senoo's group'" achieved excellent clinical results in three patients with Ebstein's anomaly by replacement of the valve alone, without plicating the atrialized ventricle. We could not determine the role of plication of the atrialized ventricle from our experience, because obliteration of this chamber was deemed necessary in only one patient. Perhaps the diverse results are due to the variability of the size of the atrialized ventricle, which sometimes is small and thick walled and exhibits minimal paradoxical motion. Atrial arrhythmias are common in patients with Ebstein's anomaly and may further compromise an already reduced cardiac output. This was true in four of our patients who required frequent hospitalization for treatment. Although there may be no decrease in the frequency of arrhythmias, by correcting the hemodynamic abnormalities tricuspid valve replacement enables the patient to tolerate them better. This was the case in our patient who continued to experience arrhythmias postoperatively.
The Journal of
232
Bove and Kirsh
On the basis of this study, we conclude that valve replacement and, when necessary, plication of the atrialized ventricle produces satisfactory and longlasting clinical improvement in patients with Ebstein's malformation. REFERENCES
2
3
4
5
6
Barnard CN, Schrire V: Surgical correction of Ebstein's malformation with prosthetic tricuspid valve. Surgery 54:302-308, 1963 Lillehei CW, Kalke BR, Carlson RG: Evolution of corrective surgery for Ebstein's anomaly. Circulation 35: Suppl 111-118, 1967 Kitamura S, Johnson JL, Redington JV, Mendez A, Zubiate P, Kay JH: Surgery for Ebstein's anomaly. Ann Thorac Surg 11:320-330, 1971 McFaul RC, Davis Z, Giuliani ER, Ritter DG, Danielson GK: Ebsteiu's malformation. Surgical experience at the Mayo Clinic. J THORAC CARDIOVASC SURG 72:910-915, 1976 Hardy KL, May lA, Webster CA, Kimball KG: Ebstein's anomaly. A functional concept and successful definitive repair. J THORAC CARDIOVASC SURG. 48:927-940, 1964 Najafi H, Hunter JA, Dye WS, Javid H, Julian OC; Ebstein's malformation of the tricuspid valve. Surgical management. Ann Thorac Surg 4:334-343, 1967
Thoracic and Cardiovascular Surgery
7 Bahnson HT, Bauersfeld SR, Smith JW: Pathological anatomy and surgical correction of Ebstein's anomaly. Circulation 31:Suppl 1:3-8, 1965 8 Cartwright RS, Smeloff EA, Cayler 00, Fong WY, Huntley AC, Blake JR, McFall RA: Total correction of Ebstein's anomaly by means of tricuspid replacement. J THORAC CARDIOVASC SURG 47:755-761, 1964 9 Watson H: Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. Br Heart J 36:417-427, 1974 10 Starr A: Panel discussion: Cardiac valve substitution. Caged-ball. Held at the Annual Meeting of the American College of Cardiology, New Orleans, La., February, 1970 II Timmis HH, Hardy JD, Watson 00: The surgical management of Ebstein's anomaly. J THORAC CARDIOVASC SURG 53:385-391, 1966 12 Jugdutt BI, Brooks CH, Stems LP, Callaghan JC, Rossall RE: Surgical treatment of Ebstein's anomaly. J THORAC CARDIOVASC SURG 73: 114-119, 1977 13 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 74: 243-248, 1976