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Transatrial-transpulmonary repair of tetralogy of Fallot
J
THoRAc CARDIOVASC SURG
1987;93:919-24
Transatrial-transpulmonary repair of tetralogy of Fallot Classicaltetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 61 of 70 patients treated surgically on one surgical service between July 1981 and December1985. Their ages ranged from 2.5 monthsto 57 years (mean52.9 ± 104.2 months). The ventricularseptal defect was closedthrough the right atrium and tricuspid valve in 60 patients and via the pulmonary artery in one patient with a subpulmonary defect.A pulmonary arteriotomy was made in 38 patients to expose the stenotic pulmonary valve for valvotomy and to begin tbe infundibular mobilization and resection. It was extended through a small pulmonary anulus primarily in 15 patients and secondarilyin one, a minimal distance upon the right ventricular infundibulum to enlarge the outflow tract to 2 standard deviations larger than the mean normal pulmonary valve anulus diameter. There were no hospital or late deaths or reoperations among the entire group. The mean postrepair peak right ventricular/left ventricular pressure measured in the operating room was 0.52 ± 0.165 for the entire group of 61 patients which indicates similar relief of pulmonary stenosis to that obtained by the classical transventricular repair. At follow-up between 2.3 and 53.8 months (mean 22.6) each patient was in New York Heart Association Class [ and without cardiac medication. Postoperative cardiac catheterization studies performed in nine patients between 4 and 28 months after operation showed no residual ventricular septal defects and a mean peak right/left ventricular pressure of 0.42 ± 0.13. This series indicates that successful repair of tetralogy of Fallot can be accomplished in most patients by a transatrial-transpulmonary approach, whichmay provide improved right ventricular function over the long term.
Albert D. Pacifico, M.D., Mark E. Sand, M.D., Lionel M. Bargeron, Jr., M.D., and Edward C. Colvin, M.D., Birmingham. Ala.
HUdspeth, Cordell, and Johnston I in 1963 and Edmunds and associates' in 1976 demonstrated that tetralogy of Fallot could be repaired without a right ventriculotomy. This, the experimental demonstration that right ventricular function is reduced by a right ventriculotomy, and concerns regarding the postoperative long-term function of the right ventricle made us enthusiastic about repairing tetralogy via a transatrialtranspulmonary approach." When the pulmonary valve anulus must be enlarged, the incision through it is carried only minimally on the right ventricular infundibulum. From the University of Alabama at Birmingham and the Alabama Congenital Heart Disease Diagnosis and Treatment Center, Birmingham, Ala. 35294. Received for publication April 25, 1986. Accepted for publication May 29, 1986. Address for reprints: Dr. A. D. Pacifico, University of Alabama at Birmingham, Division of Cardiothoracic Surgery, University Station, Birmingham, Ala. 35294.
Material and methods We have reviewed all tetralogy repairs done on one surgical service (A.D.P.) at the University of Alabama at Birmingham (UAB) between July 1, 1981 (the inception of this repair policy) and Dec. 31, 1985. One service was selected because of the bias of the operating surgeon toward this technique and therefore to provide information regarding the frequency with which this type of repair may be accomplished in the overall anatomical spectrum of classical tetralogy of Fallot. Seventy patients with classical tetralogy of Fallot underwent complete repair during the study period. Patients with associated pulmonary atresia, absent pulmonary valve syndrome, and origin of a branch pulmonary artery from the ascending aortic (hemitruncus) were excluded. Operations were performed through a median sternotomy with standard cardiopulmonary bypass methods with separate cannulation of each vena cava, moderate (28° C) hypothermia, and myocardial protection with cold sanguineous cardioplegic solution." Total circulatory arrest was not employed. Nine patients 919
The Journal of Thoracic and Cardiovascular Surgery
9 2 0 Pacifico et al.
Fig. 1. Right ventricular angiocardiogram showing the tubular infundibulum of a patient aged 3 years. The pulmonary anulus was measured intraoperatively and had a Z value of -0.7. A classical transventricular repair was employed in which a vertical infundibular incision widened with a Dacron patch was used. The postrepair P RV/ LV was 0.59.
Fig. 3. Preoperative right ventricular angiocardiogram of a 2-month-old patient who underwent repair by the transatrialtranspulmonary approach. After pulmonary valvotomy, the pulmonary valve anulus had a Z value of 0 and the immediate postrepair P RV/ LV was 0.31.
Table I. Transatrial-transpulmonary repair of tetralogy of Fallot (July 1, 1981 to Dec. 31, 1985) Limited transanular patch
Patients Age (mo)
No.
:<3 >3::::6 > 6:::: 12 >12::::18 > 18:::: 24 > 24:::: 48 > 48
1 4
Total
I
%
No.
I
%*
13 8 13 15
1.6 6.6 11 21 13 21 25
0 2 2 4 3 5 0
0 50 29 31 38 38 0
61
100
16
26
7
Legend: Range 2.5 months to 56.8 years (mean 52.9 ± 104.2 months) *p = 0.20 for difference in incidence of transanular patch by age group (chi square).
Fig. 2. Preoperative right ventricular angiocardiogram of a lO-month-old patient who underwent repair by the transatrial approach. The pulmonary valve anulus had a Z value of +I and the postrepair P RV/ LV measured in the operating room was 0.32.
underwent the classical transventricular repair, because of a narrow tubular infundibulum (Fig. 1) in six, the need for orthotopic placement of a cryopreserved aortic homograft valve in the pulmonary position in two with
peripheral pulmonary arterial problems, and because of marked right ventricular hypertrophy with a small pulmonary valve anulus in one 44-year-old patient. The ages of the 61 patients who underwent transatrial/ transpulmonary repair are shown in Table I. Representative preoperative right ventricular angiocardiograrns are shown in Figs. 2 to 5. Sixteen patients had a previous classical or modified Blalock-Taussig shunt, which was unilateral in 13 and bilateral in three. Repair of tetralogy of Fallot was accomplished by relieving infundibular and valvular pulmonary stenosis through a
Volume 93 Number 6
Tetralogy of Fallot
June 1987
Fig. 4. The right ventricular angiocardiogram of a 16-
month-old patient is shown. Repair was accomplished by transatrial-transpulmonary exposure and the pulmonaryvalve anulus had a Z value of - 3.5. The pulmonary artery incision was carried through the valve anulus for a distance of 8 mm onto the right ventricle. After infundibular resection, a Hegar dilator with a Z value of +2 could be passed into the right ventricle. This area was enlarged with a pericardial patch. Additional infundibular resection and VSD closure were performed via the right atrial approach and the immediate postrepair PRV/ LV was 0.55. transatrial approach in 32, transpulmonary approach in five, and a combined transatrial and transpulmonary exposure in 24. The ventricular septal defect (VSD) was closed through the right atrium and tricuspid valve in 60 and via the pulmonary artery in one patient with a subpulmonary VSD. Additional procedures performed at the time of tetralogy repair are shown in Table II. A pulmonary arteriotomy was made in 38 patients and extended through the pulmonary anulus primarily in 15 and secondarily in one. The incision onto the infundibulum from the valve anulus was measured in 13 patients and was between 1 and 14 mm, mean 8 mm (Fig. 6). The pulmonary arteriotomy was closed primarily in 16 patients and widened with a patch of pericardium in 22, including the 16 in whom a limited transannular patch was placed. The pulmonary valve anulus was initially measured with a Hegar dilator and related to the mean normal valve (Table 111).7.8 It was larger than -1.5 standard deviations from the mean normal valve in 45 without transannular patching and smaller than this value in 14 of the 16 patients in whom the pulmonary valve anulus was enlarged by patching (Table IV). The pulmonary
921
Fig. 5. Right ventricular angiocardiogram of an l8-monthold patient who underwent transatrial-transpulmonary repair. The pulmonary valve anulus had a Z value of - 3 and the longitudinal incision in the main pulmonary artery was extended through the origin of the left pulmonary artery and across the valve anulus for a distance of 14 mm onto the right ventricle. Through this opening, a Hegar dilator with a Z value of +2 was passed into the right venticle. The entire area was widened with a patch of pericardium. Additional infundibular resection and VSD closurewere accomplished via a right atrial approach and the immediate postrepair PRV/ LV was 0.44.
valve was not stenotic in 22 patients; in the remaining patients, valvotomy was performed via a pulmonary arteriotomy in 32 and via the right atrium and tricuspid valve in seven. Six patients had pulmonary artery problems consisting of bifurcation stenosis in one, stenosis of the origin of either branch in four, and hypoplasia of the left pulmonary artery in one. In this group of 61 patients the elapsed time of cardiopulmonary bypass ranged from 25 to 94 minutes (mean 50 minutes) and the aortic cross-clamp time ranged from 14 to 61 minutes (mean 32 minutes). The family and/or physician of each patient was contacted in January 1986 to collect follow-up information between 2.3 and 53.8 months (mean 22.6) postoperatively.
Results There were no hospital or late deaths or reoperations among the entire group of 70 patients. Peak right ventricular/left ventricular pressure (PR V/ LV) was measured in the operating room at the termination of cardiopulmonary bypass in each of the 61 patients who underwent transatrial-transpulmonary repair. The mean
922
The Journal of Thoracic and Cardiovascular
Pacifico et al.
Surgery
Fig. 6. The incision in the main pulmonary artery, which is used for pulmonary valvotomy and initial infundibular mobilization and resection, is shown (left). When the anulus must be enlarged it is extended onto the right ventricular infundibulum (right) for the minimal distance required to pass a Hegar dilator with a Z value of +2 into the ventricle. The incision is then widened with a patch.
Table II. Additional surgical procedures Procedure Repair of left pulmonary artery stenosis VSD enlargement Excision of tricuspid valve excrescences restricting VSD Repair of right pulmonary artery occlusion Reconstruction of pulmonary artery confluence Tricuspid valve annuloplasty Tunnel repair of partial anomalous pulmonary venous connection of right superior pulmonary vein to superior vena cava Repair of aortopulmonary window Repair of aortic incompetence Coronary artery bypass grafting x5
No. 2 1 I
Legend: VSD. Ventricular septal defect. No. of patients = 61.
value was 0.52 ± 0.165 for the entire group of 61 patients, 0.50 ± 0.143 among the 45 patients without transannular patching, and 0.56 ± 0.212 among 16 with transannu1ar patching (p = 0.21). The distribution of limited transannular patching according to age at repair is shown in Table I. Postoperative complications included reentry for bleeding in one, removal of a retained monitoring catheter in one, chylothorax in one, transient atrioventricular dissociation in one, and sternal infection in another. At follow-up, each patient was in New York Heart
Association (NYHA) Functional Class I and without cardiac medication. Postoperative cardiac catheterization studies were performed in nine patients between 4 and 28 months postoperatively. No residual VSDs were identified and the P RV/ LV ranged from 0.30 to 0.65, mean 0.42 ± 0.13.
Comment
In this surgical experience, transatria1-transpu1monary repair was successfully performed in nearly 90% of patients with classical tetralogy of Fallot. The method was useful even in infants; 19%of patients were younger than 12 months of age and 54% were younger than 2 years of age. A limited transannu1ar patch was placed primarily in 15 patients (25%) and secondarily in one (1.6%). The incision through the pulmonary valve anulus onto the right ventricular infundibulum was made long enough to enlarge the right ventricular outlet to the upper limit of normal (Z value = +2 for pulmonary valve anulus) and in the 13 patients in whom it was measured, the length varied from 1 to 14 mm, mean 8 mm. The surgical method described provides effective relief of right ventricular outflow tract obstruction in most patients. The mean postrepair P RV/ LV of 0.52 ± 0.165 for the entire group of 61 patients was similar to the mean postrepair P RV/ LV of 0.56 ± 0.195 of 391 patients who underwent transventricular repair of tetralogy at the UAB between 1967 and 1982, p = 0.13 (unpublished data). Transatrial-transpulmonary relief of infundibular stenosis is not possible when the infun-
Volume 93 Number 6 June 1987
Tetralogy of Fallot
Table ill. Mean normal pulmonary valve anulus diameter* Body surface area (m2)
Diameteri
0.25 0.30 0.35 0.40 0.45 0.50 0.60 0.70 0.80
8.4 9.3 10.1 10.7 11.3 11.9 12.8 13.5 14.2 14.8 15.3
0.90
1.00 'Modified from Rowlatt, Rimaldi, and Lev.' t Approximate standard deviation for each = ± 1.2.
923
Table IV. Prerepair Hegar measurement of pulmonary anulus (N = 61) Standard deviations away from predicted mean normal value (2 value)
No transannular patch (No.)
2.0 1.0 0.5
o
-1 -1.5 -2.0 -2.5 -3.0 2: -3.5
Limited transannular patch (No.)
2 4
1 32 5 1
I' I
o 4
2 6 2
'Less than -I after closure of subpulmonary ventricular septal defect.
dibulum has a long and narrow (tubular) configuration, as shown in Fig, 1. This type of infundibulum must be enlarged by longitudinal incision and the insertion of an enlarging patch. Exposure of the stenotic pulmonary valve is probably best achieved via a pulmonary arteriotomy. The parietal insertion of the infundibular septum and more distal bands of the septal insertion are also nicely exposed by this approach through the pulmonary valve anulus. The VSD is easily exposed and closed through a right atriotomy and the exposure is facilitated by separate caval cannulation and the use of cold cardioplegic arrest. 6 Through this approach the septal insertion of the infundibular septum is nicely exposed and released. Transatrial exposure of the inferior aspect of the parietal insertion is facilitated by placement of a traction suture on the infundibular septum, but more distal bands are less well visualized. They are more easily managed through the pulmonary arteriotomy. Early and intermediate term results of this method of repair of the tetralogy of Fallot were similar to the classical transventricular repair as regards hospital and late mortality (absent in this series) and adequacy of relief of pulmonary stenosis. Each patient at follow-up was in NYHA Class I and without cardiac medication. Twenty-two (36%) of the 61 patients in this series had a normal pulmonary valve, and theoretically with adequate relief of pulmonary stenosis, a competent pulmonary valve, and an intact right ventricle, they should have nearly normal right ventricular function. Overall, 74% of the group of 61 patients did not have transannular patching. Similarly, the majority of these should be without severe pulmonary incompetence and perhaps
have a greater probability of preserved right ventricular function over the very long term. In addition, the remaining 26% who had limited transannular patching and thus resultant pulmonary insufficiency are left with a more intact right ventricle than that which is present after transventricular repair. Kawashima and colleagues," using similar surgical methods that included preservation or reconstruction of the pulmonary valve, have demonstrated a smaller right ventricular enddiastolic volume index, higher right ventricular ejection fraction during isoproterenol infusion, and fewer ventricular arrhythmias than in patients operated on by the conventional method. Additional studies, however, and late follow-up are required to assess the function of the right ventricle and to determine if differences exist between this method and the classical transventricu1ar technique of repair. These results, however, support continuation of this method for repair of the tetralogy of Fallot. REFERENCES 1. Hudspeth AS, Cordell AR, Johnston FR. Transatrial approach to total correction of tetralogy of Fallot. Circulation 1963;27:796-800. 2. Edmunds LH Jr, Saxena NC, Friedman S, Rashkind WJ, Dodd PF. Transatrial repair of tetralogy of Fallot. Surgery 1976;80:681-8. 3. March HW, Ross JK, Weirich WL, Gerbode F. The influence of the ventriculotomy site on the contraction and function of the right ventricle. Circulation 1961;24:5727. 4. Sanger PW, Robicsek F, Taylor FH, Davis SC. A method of preventing myocardial damage by using a modified ventriculotomy incision. Ann Surg 1962;155:874-82. 5. Stirling GR, Stanley PH, Lillehei CWo The effects of
9 2 4 Pacifico et al.
cardiac bypass and ventriculotomy upon right ventricular function. Surg Forum 1957;8:433-8. 6. Pacifico AD. Low flow bypass: the University of Alabama technique. In: Congenital heart surgery: current techniques and controversies, Moulton AL, ed., Pasadena, California: Appleton Davies, Inc., 1984:187-192. 7. Rowlatt UF, Rimoldi HJA, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am 1963;10:499-588.
The Journal of Thoracic and Cardiovascular Surgery
8. Pacifico AD, Kirklin JW, Blackstone EH. Surgical management of pulmonary stenosis in tetralogy of Fallot. J THORAC CARDIOVASC SURG 1977;74:382-95. 9. Kawashima Y, Matsuda H, Hirose H, Nakano S, Shirakura R, Kobayashi J. Ninety consecutive corrective operations for tetralogy of Fallot with or without minimal right ventriculotomy. J THORAC CARDIOVASC SURG 1985; 90:856-63.
Bound volumes available to subscribers Bound volumes of THE JOURNAL OFTHORACIC AND CARDIOVASCULAR SURGERY are available to subscribers (only) for the 1987 issues from the Publisher, at a cost of $49.00 ($66.00 international) for Vol. 93 (January-June) and Vol. 94 (July-December). Shipping charges are included. Each bound volume contains a subject and author index and all advertising is removed. Copies are shipped within 60 days after publication of the last issue of the volume. The binding is durable buckram with the JOURNAL name, volume number, and year stamped in gold on the spine. Payment must accompany all orders. Contact The C. V. Mosby Company, Circulation Department, 11830 Westline Industrial Drive, St. Louis, Missouri 63146, USA; phone (800) 325-4177, ext. 351. Subscriptions must be in force to qualify. Bound volumes are not available in place of a regular JOURNAL subscription.
THoRAc CARDIOVASC SURG
1987;93:919-24
Transatrial-transpulmonary repair of tetralogy of Fallot Classicaltetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 61 of 70 patients treated surgically on one surgical service between July 1981 and December1985. Their ages ranged from 2.5 monthsto 57 years (mean52.9 ± 104.2 months). The ventricularseptal defect was closedthrough the right atrium and tricuspid valve in 60 patients and via the pulmonary artery in one patient with a subpulmonary defect.A pulmonary arteriotomy was made in 38 patients to expose the stenotic pulmonary valve for valvotomy and to begin tbe infundibular mobilization and resection. It was extended through a small pulmonary anulus primarily in 15 patients and secondarilyin one, a minimal distance upon the right ventricular infundibulum to enlarge the outflow tract to 2 standard deviations larger than the mean normal pulmonary valve anulus diameter. There were no hospital or late deaths or reoperations among the entire group. The mean postrepair peak right ventricular/left ventricular pressure measured in the operating room was 0.52 ± 0.165 for the entire group of 61 patients which indicates similar relief of pulmonary stenosis to that obtained by the classical transventricular repair. At follow-up between 2.3 and 53.8 months (mean 22.6) each patient was in New York Heart Association Class [ and without cardiac medication. Postoperative cardiac catheterization studies performed in nine patients between 4 and 28 months after operation showed no residual ventricular septal defects and a mean peak right/left ventricular pressure of 0.42 ± 0.13. This series indicates that successful repair of tetralogy of Fallot can be accomplished in most patients by a transatrial-transpulmonary approach, whichmay provide improved right ventricular function over the long term.
Albert D. Pacifico, M.D., Mark E. Sand, M.D., Lionel M. Bargeron, Jr., M.D., and Edward C. Colvin, M.D., Birmingham. Ala.
HUdspeth, Cordell, and Johnston I in 1963 and Edmunds and associates' in 1976 demonstrated that tetralogy of Fallot could be repaired without a right ventriculotomy. This, the experimental demonstration that right ventricular function is reduced by a right ventriculotomy, and concerns regarding the postoperative long-term function of the right ventricle made us enthusiastic about repairing tetralogy via a transatrialtranspulmonary approach." When the pulmonary valve anulus must be enlarged, the incision through it is carried only minimally on the right ventricular infundibulum. From the University of Alabama at Birmingham and the Alabama Congenital Heart Disease Diagnosis and Treatment Center, Birmingham, Ala. 35294. Received for publication April 25, 1986. Accepted for publication May 29, 1986. Address for reprints: Dr. A. D. Pacifico, University of Alabama at Birmingham, Division of Cardiothoracic Surgery, University Station, Birmingham, Ala. 35294.
Material and methods We have reviewed all tetralogy repairs done on one surgical service (A.D.P.) at the University of Alabama at Birmingham (UAB) between July 1, 1981 (the inception of this repair policy) and Dec. 31, 1985. One service was selected because of the bias of the operating surgeon toward this technique and therefore to provide information regarding the frequency with which this type of repair may be accomplished in the overall anatomical spectrum of classical tetralogy of Fallot. Seventy patients with classical tetralogy of Fallot underwent complete repair during the study period. Patients with associated pulmonary atresia, absent pulmonary valve syndrome, and origin of a branch pulmonary artery from the ascending aortic (hemitruncus) were excluded. Operations were performed through a median sternotomy with standard cardiopulmonary bypass methods with separate cannulation of each vena cava, moderate (28° C) hypothermia, and myocardial protection with cold sanguineous cardioplegic solution." Total circulatory arrest was not employed. Nine patients 919
The Journal of Thoracic and Cardiovascular Surgery
9 2 0 Pacifico et al.
Fig. 1. Right ventricular angiocardiogram showing the tubular infundibulum of a patient aged 3 years. The pulmonary anulus was measured intraoperatively and had a Z value of -0.7. A classical transventricular repair was employed in which a vertical infundibular incision widened with a Dacron patch was used. The postrepair P RV/ LV was 0.59.
Fig. 3. Preoperative right ventricular angiocardiogram of a 2-month-old patient who underwent repair by the transatrialtranspulmonary approach. After pulmonary valvotomy, the pulmonary valve anulus had a Z value of 0 and the immediate postrepair P RV/ LV was 0.31.
Table I. Transatrial-transpulmonary repair of tetralogy of Fallot (July 1, 1981 to Dec. 31, 1985) Limited transanular patch
Patients Age (mo)
No.
:<3 >3::::6 > 6:::: 12 >12::::18 > 18:::: 24 > 24:::: 48 > 48
1 4
Total
I
%
No.
I
%*
13 8 13 15
1.6 6.6 11 21 13 21 25
0 2 2 4 3 5 0
0 50 29 31 38 38 0
61
100
16
26
7
Legend: Range 2.5 months to 56.8 years (mean 52.9 ± 104.2 months) *p = 0.20 for difference in incidence of transanular patch by age group (chi square).
Fig. 2. Preoperative right ventricular angiocardiogram of a lO-month-old patient who underwent repair by the transatrial approach. The pulmonary valve anulus had a Z value of +I and the postrepair P RV/ LV measured in the operating room was 0.32.
underwent the classical transventricular repair, because of a narrow tubular infundibulum (Fig. 1) in six, the need for orthotopic placement of a cryopreserved aortic homograft valve in the pulmonary position in two with
peripheral pulmonary arterial problems, and because of marked right ventricular hypertrophy with a small pulmonary valve anulus in one 44-year-old patient. The ages of the 61 patients who underwent transatrial/ transpulmonary repair are shown in Table I. Representative preoperative right ventricular angiocardiograrns are shown in Figs. 2 to 5. Sixteen patients had a previous classical or modified Blalock-Taussig shunt, which was unilateral in 13 and bilateral in three. Repair of tetralogy of Fallot was accomplished by relieving infundibular and valvular pulmonary stenosis through a
Volume 93 Number 6
Tetralogy of Fallot
June 1987
Fig. 4. The right ventricular angiocardiogram of a 16-
month-old patient is shown. Repair was accomplished by transatrial-transpulmonary exposure and the pulmonaryvalve anulus had a Z value of - 3.5. The pulmonary artery incision was carried through the valve anulus for a distance of 8 mm onto the right ventricle. After infundibular resection, a Hegar dilator with a Z value of +2 could be passed into the right ventricle. This area was enlarged with a pericardial patch. Additional infundibular resection and VSD closure were performed via the right atrial approach and the immediate postrepair PRV/ LV was 0.55. transatrial approach in 32, transpulmonary approach in five, and a combined transatrial and transpulmonary exposure in 24. The ventricular septal defect (VSD) was closed through the right atrium and tricuspid valve in 60 and via the pulmonary artery in one patient with a subpulmonary VSD. Additional procedures performed at the time of tetralogy repair are shown in Table II. A pulmonary arteriotomy was made in 38 patients and extended through the pulmonary anulus primarily in 15 and secondarily in one. The incision onto the infundibulum from the valve anulus was measured in 13 patients and was between 1 and 14 mm, mean 8 mm (Fig. 6). The pulmonary arteriotomy was closed primarily in 16 patients and widened with a patch of pericardium in 22, including the 16 in whom a limited transannular patch was placed. The pulmonary valve anulus was initially measured with a Hegar dilator and related to the mean normal valve (Table 111).7.8 It was larger than -1.5 standard deviations from the mean normal valve in 45 without transannular patching and smaller than this value in 14 of the 16 patients in whom the pulmonary valve anulus was enlarged by patching (Table IV). The pulmonary
921
Fig. 5. Right ventricular angiocardiogram of an l8-monthold patient who underwent transatrial-transpulmonary repair. The pulmonary valve anulus had a Z value of - 3 and the longitudinal incision in the main pulmonary artery was extended through the origin of the left pulmonary artery and across the valve anulus for a distance of 14 mm onto the right ventricle. Through this opening, a Hegar dilator with a Z value of +2 was passed into the right venticle. The entire area was widened with a patch of pericardium. Additional infundibular resection and VSD closurewere accomplished via a right atrial approach and the immediate postrepair PRV/ LV was 0.44.
valve was not stenotic in 22 patients; in the remaining patients, valvotomy was performed via a pulmonary arteriotomy in 32 and via the right atrium and tricuspid valve in seven. Six patients had pulmonary artery problems consisting of bifurcation stenosis in one, stenosis of the origin of either branch in four, and hypoplasia of the left pulmonary artery in one. In this group of 61 patients the elapsed time of cardiopulmonary bypass ranged from 25 to 94 minutes (mean 50 minutes) and the aortic cross-clamp time ranged from 14 to 61 minutes (mean 32 minutes). The family and/or physician of each patient was contacted in January 1986 to collect follow-up information between 2.3 and 53.8 months (mean 22.6) postoperatively.
Results There were no hospital or late deaths or reoperations among the entire group of 70 patients. Peak right ventricular/left ventricular pressure (PR V/ LV) was measured in the operating room at the termination of cardiopulmonary bypass in each of the 61 patients who underwent transatrial-transpulmonary repair. The mean
922
The Journal of Thoracic and Cardiovascular
Pacifico et al.
Surgery
Fig. 6. The incision in the main pulmonary artery, which is used for pulmonary valvotomy and initial infundibular mobilization and resection, is shown (left). When the anulus must be enlarged it is extended onto the right ventricular infundibulum (right) for the minimal distance required to pass a Hegar dilator with a Z value of +2 into the ventricle. The incision is then widened with a patch.
Table II. Additional surgical procedures Procedure Repair of left pulmonary artery stenosis VSD enlargement Excision of tricuspid valve excrescences restricting VSD Repair of right pulmonary artery occlusion Reconstruction of pulmonary artery confluence Tricuspid valve annuloplasty Tunnel repair of partial anomalous pulmonary venous connection of right superior pulmonary vein to superior vena cava Repair of aortopulmonary window Repair of aortic incompetence Coronary artery bypass grafting x5
No. 2 1 I
Legend: VSD. Ventricular septal defect. No. of patients = 61.
value was 0.52 ± 0.165 for the entire group of 61 patients, 0.50 ± 0.143 among the 45 patients without transannular patching, and 0.56 ± 0.212 among 16 with transannu1ar patching (p = 0.21). The distribution of limited transannular patching according to age at repair is shown in Table I. Postoperative complications included reentry for bleeding in one, removal of a retained monitoring catheter in one, chylothorax in one, transient atrioventricular dissociation in one, and sternal infection in another. At follow-up, each patient was in New York Heart
Association (NYHA) Functional Class I and without cardiac medication. Postoperative cardiac catheterization studies were performed in nine patients between 4 and 28 months postoperatively. No residual VSDs were identified and the P RV/ LV ranged from 0.30 to 0.65, mean 0.42 ± 0.13.
Comment
In this surgical experience, transatria1-transpu1monary repair was successfully performed in nearly 90% of patients with classical tetralogy of Fallot. The method was useful even in infants; 19%of patients were younger than 12 months of age and 54% were younger than 2 years of age. A limited transannu1ar patch was placed primarily in 15 patients (25%) and secondarily in one (1.6%). The incision through the pulmonary valve anulus onto the right ventricular infundibulum was made long enough to enlarge the right ventricular outlet to the upper limit of normal (Z value = +2 for pulmonary valve anulus) and in the 13 patients in whom it was measured, the length varied from 1 to 14 mm, mean 8 mm. The surgical method described provides effective relief of right ventricular outflow tract obstruction in most patients. The mean postrepair P RV/ LV of 0.52 ± 0.165 for the entire group of 61 patients was similar to the mean postrepair P RV/ LV of 0.56 ± 0.195 of 391 patients who underwent transventricular repair of tetralogy at the UAB between 1967 and 1982, p = 0.13 (unpublished data). Transatrial-transpulmonary relief of infundibular stenosis is not possible when the infun-
Volume 93 Number 6 June 1987
Tetralogy of Fallot
Table ill. Mean normal pulmonary valve anulus diameter* Body surface area (m2)
Diameteri
0.25 0.30 0.35 0.40 0.45 0.50 0.60 0.70 0.80
8.4 9.3 10.1 10.7 11.3 11.9 12.8 13.5 14.2 14.8 15.3
0.90
1.00 'Modified from Rowlatt, Rimaldi, and Lev.' t Approximate standard deviation for each = ± 1.2.
923
Table IV. Prerepair Hegar measurement of pulmonary anulus (N = 61) Standard deviations away from predicted mean normal value (2 value)
No transannular patch (No.)
2.0 1.0 0.5
o
-1 -1.5 -2.0 -2.5 -3.0 2: -3.5
Limited transannular patch (No.)
2 4
1 32 5 1
I' I
o 4
2 6 2
'Less than -I after closure of subpulmonary ventricular septal defect.
dibulum has a long and narrow (tubular) configuration, as shown in Fig, 1. This type of infundibulum must be enlarged by longitudinal incision and the insertion of an enlarging patch. Exposure of the stenotic pulmonary valve is probably best achieved via a pulmonary arteriotomy. The parietal insertion of the infundibular septum and more distal bands of the septal insertion are also nicely exposed by this approach through the pulmonary valve anulus. The VSD is easily exposed and closed through a right atriotomy and the exposure is facilitated by separate caval cannulation and the use of cold cardioplegic arrest. 6 Through this approach the septal insertion of the infundibular septum is nicely exposed and released. Transatrial exposure of the inferior aspect of the parietal insertion is facilitated by placement of a traction suture on the infundibular septum, but more distal bands are less well visualized. They are more easily managed through the pulmonary arteriotomy. Early and intermediate term results of this method of repair of the tetralogy of Fallot were similar to the classical transventricular repair as regards hospital and late mortality (absent in this series) and adequacy of relief of pulmonary stenosis. Each patient at follow-up was in NYHA Class I and without cardiac medication. Twenty-two (36%) of the 61 patients in this series had a normal pulmonary valve, and theoretically with adequate relief of pulmonary stenosis, a competent pulmonary valve, and an intact right ventricle, they should have nearly normal right ventricular function. Overall, 74% of the group of 61 patients did not have transannular patching. Similarly, the majority of these should be without severe pulmonary incompetence and perhaps
have a greater probability of preserved right ventricular function over the very long term. In addition, the remaining 26% who had limited transannular patching and thus resultant pulmonary insufficiency are left with a more intact right ventricle than that which is present after transventricular repair. Kawashima and colleagues," using similar surgical methods that included preservation or reconstruction of the pulmonary valve, have demonstrated a smaller right ventricular enddiastolic volume index, higher right ventricular ejection fraction during isoproterenol infusion, and fewer ventricular arrhythmias than in patients operated on by the conventional method. Additional studies, however, and late follow-up are required to assess the function of the right ventricle and to determine if differences exist between this method and the classical transventricu1ar technique of repair. These results, however, support continuation of this method for repair of the tetralogy of Fallot. REFERENCES 1. Hudspeth AS, Cordell AR, Johnston FR. Transatrial approach to total correction of tetralogy of Fallot. Circulation 1963;27:796-800. 2. Edmunds LH Jr, Saxena NC, Friedman S, Rashkind WJ, Dodd PF. Transatrial repair of tetralogy of Fallot. Surgery 1976;80:681-8. 3. March HW, Ross JK, Weirich WL, Gerbode F. The influence of the ventriculotomy site on the contraction and function of the right ventricle. Circulation 1961;24:5727. 4. Sanger PW, Robicsek F, Taylor FH, Davis SC. A method of preventing myocardial damage by using a modified ventriculotomy incision. Ann Surg 1962;155:874-82. 5. Stirling GR, Stanley PH, Lillehei CWo The effects of
9 2 4 Pacifico et al.
cardiac bypass and ventriculotomy upon right ventricular function. Surg Forum 1957;8:433-8. 6. Pacifico AD. Low flow bypass: the University of Alabama technique. In: Congenital heart surgery: current techniques and controversies, Moulton AL, ed., Pasadena, California: Appleton Davies, Inc., 1984:187-192. 7. Rowlatt UF, Rimoldi HJA, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am 1963;10:499-588.
The Journal of Thoracic and Cardiovascular Surgery
8. Pacifico AD, Kirklin JW, Blackstone EH. Surgical management of pulmonary stenosis in tetralogy of Fallot. J THORAC CARDIOVASC SURG 1977;74:382-95. 9. Kawashima Y, Matsuda H, Hirose H, Nakano S, Shirakura R, Kobayashi J. Ninety consecutive corrective operations for tetralogy of Fallot with or without minimal right ventriculotomy. J THORAC CARDIOVASC SURG 1985; 90:856-63.
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