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Long-term evaluation of aortic valvuloplasty for aortic insufficiency and ventricular septal defect
Long-term evaluation of aortic valvuloplasty for aortic insufficiency and ventricular septal defect Frank C. Spencer, M.D., Eugenie F. Doyle, M.D. (by invitation), Delores A. Danilowicz, M.D. (by invitation), Henry T. Bahnson, M.D., and Clarence S. Weldon, M.D., New York, N.Y., Pittsburgh, Pa., and St. Louis, Mo.
A
t present there is a great diversity of opinion regarding the effectiveness of aortic valvuloplasty for aortic insufficiency associated with a ventricular septal defect. Some physicians employ valvuloplasty in most patients, whereas, at the opposite extreme, others routinely treat such patients with a prosthetic valve, usually a homograft. The great discrepancy in results reported by different groups in the past decade partly explains this wide variation. The first articles concerning operation for aortic insufficiency with ventricular septal defect were by Garamella 1 and by Starr- in 1960; each of them reported that his patient survived. In 1962, two of us' (F. C. S. and H. T. B.) reported experiences with a method of cusp plication in 3 patients, 2 of whom survived. However, results obtained with plication by others in the next 4 years were discouraging. In 1963 Ellis" described experiences with 19 patients, From the Departments of Surgery and Pediatrics, New York University Medical Center. 550 First Avenue. New York, N.Y. 10016, the Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pa., and the Department of Surgery, Washington University School of Medicine, SI. Louis, Mo. 63110. Read at the Fifty-second Annual Meeting of The American Association for Thoracic Surgery, Los Angeles, Calif., May I, 2, and 3, 1972.
of whom 13 had some type of cusp reconstruction and 5 had cusp replacement. Of the 13 with cusp reconstruction, 10 still had moderate-to-severe insufficiency. In 1965, Plauth and colleagues" described operative experiences with 6 patients, I of whom died. In 2 the ventricular septal defect was simply closed, while in 3 valvuloplasty was done; only I of these 3 had a good result. Their review of reports by others revealed a total of 17 patients treated by cusp plication, only 6 of whom had a satisfactory result. With these discouraging results, a natural pessimism arose about the feasibility of valvuloplasty, and interest increased in aortic valve replacement with a homograft. In 1969, Gonzalez-Lavin and BarrattBoyes" reported upon 7 patients in whom the prolapsed cusp was used to close the ventricular septal defect, after which an aortic homograft was inserted. Similarly, in 1970, Somerville and associates' described experiences with 20 patients; in 6 the aortic valve was repaired, while in 14 a prosthetic valve was inserted. Of the 6 patients with valvuloplasty, done by plication in 3 and by addition of fascia lata or pericardium in 3, only I obtained a competent valve.
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Fig. 1. A , Illustration demonstrating the technique of cusp plication. The free edge of the elong ated cusp is shortened to appose the normal cusp. B, Dr awing showing the plicated cusp with its mobility preser ved. Separate repair of the abnormal comm issure is then carried out . (The drawings were adapted from the original work of Leon Schlossberg. ")
Only a few publications concerning valvuloplasty have appeared in the past 7 years. In 1967 Frater," in reporting 1 case, described a method of evaluating competence of the reconstructed valve by temporarily suturing the midpoints of the three aortic cusps togeth er. S His patient obtained an excellent result. Ten months later there was only a faint diastolic murmur with a blood pressure of 100/70 mm. Hg. In 1969 a series of 29 patients was described by Hallidie-Smith and associates," 27 of whom were operated upon. There were 23 survivors; 8 of these had no repair of the aortic valve, 3 had prostheses inserted, and 12 had plication. There was a discouragingly high frequency of significant insufficiency in those patients treated with plication. Quite recently, October, 1971 , Treasure and assoelates ':' reported experiences with 15 patients treated at Walter Reed General Hospital. In 9 of the 15 with only minimal insufficiency, only the ventricular septal defect was repaired. A valvuloplasty was performed in 2 of the 4 who had severe insufficiency. The condition of I of these 2 was improved, but the other has significant residual insufficiency. In the discussion of this report, both Weldon and Trusler described successful valvuloplasty with 3 and 6 patients, respectively.
Our report describes experiences with valvuloplasty in 18 patients and adds a recent evaluation of the 2 surviving patients originally reported in 1962. 3 The conclusions differ rather sharply from those in several reports in recent years, for prosthetic replacement was not necessary in any patient , all patients are in improved condition, and the majority now have no significant insufficiency. Methods
To report on a significant group of patients, experiences at three different universities-New York University, the University of Pittsburgh, and Washington Universityhave been combined. The three authors were all at Johns Hopkins University when the original experiences with cusp plication were described in 1961 . All patients were operated upon with cardiopulmonary bypass. Aortic valvuloplasty was first performed by an appropriate combination of cusp plication and suturing of abnormal commissures (Fig. 1). A new method of cusp plication developed by one of the authors (c. S. W.) was used in all 4 patients operated upon at Washington University (Fig. 2). The aorta was then closed, and the ventricular septal defect was repaired through a right ventriculotomy. By closing the aorta first and view-
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Posterior cusp
Right cusp
CD
®
® Fig. 2. Cont'd. D, A plicating suture draws up the excess to the commissural margin . No tissue is excised. E, The leaflet is now normally proportioned and accurately suspended.
attached with circumferential sutures, but a few were repaired by direct suture (Fig. 3). All patients have been evaluated recently for cardiac function and residual aortic insufficiency. Fig. 2. A, The right cusp is redundant. The distance from commissure to corpus arantii is unequal to that of the opposing cusp edge. B, The corpora arantii are sutured together. C, The distance from corpus arantii to commissural margin is adjusted to equal that of the opposing cusp.
ing the ventricular surface of the reconstructed valve through the septal defect, it was possible to assess the effectiveness of aortic valvuloplasty. This has consistently been a reliable method of assessment of . competence. Most septal defects were closed with a Dacron or Teflon prosthetic patch,
Results The data are given in Tables IA through IV. Results from New York University are shown in Tables IA and IB, from the University of Pittsburgh in Tables IIA and lIB, and from Washington University in Tables IlIA and IIIB. Over-all experiences are summarized in Table IV. The most common abnormality was elongation and prolapse of the right coronary cusp (Fig. 4). Additional abnormalities, however, were frequent. Often all three cusps were different in size and shape. Occasional-
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c.
Fig. 3. A, Ventricular septal defect. B, Separation of the aorta from the heart, producing a ventricular septal defect. C, Direct suture produces a downward displacement of the annulus and destruction of commissural support. D, Precise patch closure preserves annular level and commissural support.
ly, some prolapse of the posterior cusp was present as well. In several patients, commissural abnormalities were similarly recognized, including widening of the commissure, abnormal insertion of the cusp at a level below the other two cusps, or localized adhesions near the commissure which limited mobility of the diseased cusp. A few additional abnormalities were recognized. In a 35-year-old patient (Case 5, Table IA), calcification and contraction of the right cusp produced insufficiency. In another patient (Case 4, Table IIA), a small aneurysm of the sinus of Valsalva was present as well. Patient 3 (Table IIA) had subvalvular aortic stenosis with adhesions limiting mobility of the cusps, but he did not have prolapse of a cusp. Seventeen of the 18 patients survived
operation, and all are in improved condition (Table IV). Ten of the 17 have no significant insufficiency, while 7 have some insufficiency but are in significantly improved condition, with absence of symptoms, a decrease in heart size, and little or no restriction of physical activity (Fig. 5). Among the 18 patients operated upon, 1 had two valve cusps replaced with fascia lata (Case 2, Table IIA). A competent valve was obtained, but progressive insufficiency appeared within a few months, requiring reoperation. Unfortunately, the patient died, the only death in the entire series. With the recent experiences with valves made from fascia lata, it seems likely that the recurrent insufficiency was due to progressive contraction of the fascia lata. In only 1 other patient (Case 1, Table IIA) was any prosthetic
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Fig. 4. A, Operative photograph showing repair of the aortic cusp in a patient operated upon at New York University (Case 2, Table I). Plication of the cusp and suture of the commissure can be seen. B, View of the ventricular septal defect in the same patient. The large right coronary cusp, although elongated, was repaired so that aortic insufficiency is no longer present. C, Operative photograph of the ventricular septal defect in another patient (Case 3, Table I). The prolapsed right coronary cusp can be clearly seen. D, Operative view showing elongated, prolapsing right coronary cusp (Case 4, Table I).
material employed: A strip of pericardium was used to lengthen one cusp. There was some residual insufficiency, but the patient's condition has remained satisfactory in the ensuing 8 years (Case I, Table lIB). The ventricular septal defect was supracristal in 6, subcristal in 7, and not stated in the operative report in 5 cases. Commissural abnormalities were noted in several patients, but there was no distinct correlation with
the location of the ventricular septal defect. In 1968, Van Praagh,!' in a careful analysis of I I autopsied patients, divided them into two groups depending upon whether the ventricular septal defect was above or below the crista supraventricularis. Those with septal defects above the crista, a subpulmonary location, usually had simple prolapse of the right coronary cusp; those with infracristal defects had more extensive malformation of
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Table IA. Aortic insufficiency: Operative findings and results at New York University Case No. (initials, age in years) 1
I
Date of operation
Aortic valve pathology
A ortic valve repair
VSD pathology (size; location)
VSD repair
Initial result
Comment
(E. G.,* 8)
1963
RC prolapse
Plication of RC
Supracristal
Patch
No AI Good result
2 (P. N., 9)
1967
RC prolapse
15-18 mm. plication of RC; suture of RCLC and RCPC commissures
2 ern.
Patch
No AI Good result
(J. C., 2)
1968
RC prolapse; RC-LC commissure adhesions
7 rnm. plication of RC; RC-LC commissure sutured
15 mrn.; supracristal
Sutured
No AI Good result
4 (R. C., 9)
Jan., 1970
RC prolapse; Plication of all 3 cusps ab- RC; suture of normal; RC-PC RC-LC and adhesions RC-PC commissures
15 mm.; subcristal
Patch
No AI Good result
RC-PC commissure sutured
12 mm.; subcristal
Patch
Slight AI
3
5 (A. W., 35)
April, 1971 Calcification of RC
6
Good result
(M. D., 7)
July, 1971
RC prolapse; Plication of all 3 cusps dif- RC; RC-PC ferent; RC-PC sutured commissure abnormal
10 mm.; subcrista I
Patch
No AI Good result
7 (D. C., 4)
July, 1971
RC prolapse; RC-PC commissure abnormal
Small, subcristal
Patch
No AI Good result
16 mm. plication; RC-PC and RC-LC sutured
Legend: RC. Right cusp. PC. Posterior (noncoronary) cusp. LC. Left cusp. VSD, Ventricular septal defect. AI, Aortic insufficiency. 'Operated upon at Brooke Army Hospital, San Antonio, Texas.
the aortic valve, including commissural deformities, and often separate abnormalities in the outflow tract of the right ventricle. In the preparation of this report, a recent evaluation was made of all 17 surviving patients as well as of the 2 patients originally described in 1962. As shown in Table IV, 7 patients are now less than 1 year since operation, 7 are between I and 4 years, and 5 are between 5 and 12 years following operation. All surviving patients are in sig-
nificantly improved condition, even though 7 of them have some aortic insufficiency. Curiously enough, there has been a significant variation in the frequency of residual insufficiency among the three institutions. A similar operative technique was employed at New York University and the University of Pittsburgh, but at Washington University the method of plication by longitudinal suture (Fig. 3) was used. A concern often expressed for patients
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Table lB. Postoperative aortic insufficiency: Long-term evaluation at New York University Case No. (initials) 1 (E. G.) 2
(P. N.)
3 (1. C.)
4 (R. C.)
Time postop.; date last seen
1*
2* (I. F.)
Angio-
gram
Comment
8 yr.; Aug., 1971
None
? 1 of 6
120/88 Normal
Good result
5 yr.; April, 1972
None
1 of 6
124/76 Slight RV enlargement
Good result
3~
None
None
100/60 Normal
5 yr.; April, 1972
None
1 of 6
104/76 -
Good result
None
None
110/70 -
Good result
None
1 of 6
100/68 Normal
Good result
None
3 of 6
100/52 -
Moderate AI; decrease in heart size
12 yr.; None; active? 1 of 6 March, 1972 skier
120/70 -
Good result
12 yr.; March, 1972
120/75 -
Good result; minimal AI unchanged in 10 yr.
yr.; March, 1972
5 (A. W.) 1 yr.; April, 1972 6 (M. D.) 6 mo.; Ian., 1972 7 (D. C.) 8 mo.; March, 1972 (C. A.)
Diastolic Blood murmur pressure Symptoms (grade) (mm. Hg) Heart size
None
1 of 6
Jan., 1970, no AI
Good result
Legend: RV,Right ventricle. For other abbreviations, see Table IA.
'Previously reported upon,"
with some insufficiency after operation is that the insufficiency will gradually become more severe in subsequent years, similar to that with rheumatic aortic insufficiency, and eventually will necessitate replacement of the valve with a prosthesis. Fortunately, to date this has not been true. Following operation some increase in aortic insufficiency has been seen in a few patients in the first few months, but after this time no further change has occurred in periods of observation extending up to 7 or more years. The presence of slight (Grade 1 of 6) diastolic murmurs, associated with no measurable hemodynamic abnormalities, has thus far been innocuous. Several such patients have been observed for several years with no demonstrable change
(Table IB). The absence of change may be similar to the findings reported by others" 10 in which patients with mild aortic insufficiency and a ventricular septal defect have been simply treated by closure of the ventricular septal defect. The residual aortic insufficiency has shown no significant change in the ensuing years. Discussion Clinical characteristics. Beginning with the classic paper by Scott and colleagues> in 1958, the clinical syndrome of ventricular septal defect with aortic insufficiency from a prolapsed cusp has been described in detail. Only 22 patients had been reported upon at the time of the paper by Scott. Subsequently,
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Table IIA. Aortic insufficiency: Operative findings and results at the University of Pittsburgh Case No. (initials, age in years)
1
Date of operation
Aortic valve pathology
Aortic valve repair
VSD pathology (size; location)
VSD repair
Initial result
Comment
(A. M., 8)
June, 1964
BP 120/40 mm. Plication of RC Small Hg; prolapsed and PC; strip right leaflet of pericardium attached
Sutured
ImMore AI 1 proved; year later residual AI
2 (R. P., 4Y2)
Aug., 1969
Prolapsed RC and PC
Patched
ReopFailure of eration fascia lata after 4 cusps mo. for progressive AIdied
Replacement of 6 rnm.; supra2 cusps with fascia lata cristal
.
3 (W. R., 9)
4 (S. H., 12)
Dec., 1970
2/4 AI; cusps Excision of normal; subval- subaortic vular AS with stenosis adhesions
Small
Sutured
Mild AI Subvalvular AS corrected
March, 1971 * RC and PC cusps prolapsed; aneurysm of right sinus of Valsalva
Excision of aneurysm; plication of RC and PC
12 rnm.; Sutured subcristal
No AI
(A. M., 12)
July, 1971
3/4 AI; large prolapsing RC
Plication of RC and PC; suture of RC-PC commissure
12 mm.; Patched subcristal
Mild AI Good result
6 (R. H., 9)
Nov., 1971
2/ 4 AI; prolapse Plication of
Supracrista1
Sutured
Mild AI
Mild AI
Plication of SupraRC and LC; cristal body and free edge
Sutured
Mild AI
Mild AI
Good result
5
of RC; LC abnormal 7 (C. G., 9)
Dec., 1971
4/4 AI
RC and LC
Legend: BP, Blood pressure. AS, Aortic stenosis. For other abbreviations, see Table IA. 'Operation by Dr. Crile Crisler.
in 1964, Nadas'" described a group of 34 patients, the largest single series which had been reported upon. These 34 patients were from a group of 750 patients with ventricular septal defects seen at the Boston Children's Hospital over a period of 14 years, a frequency near 5 per cent. Subsequent
reports emphasizing different features of the clinical syndrome have been by Keck,14 Plauth," Somerville,7 and their co-workers. Two broad groups have been recognized. In one there is trivial aortic insufficiency with a ventricular septal defect, and the clinical features are those of a large ven-
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Table lIB. Postoperative aortic insufficiency: Long-term evaluation at the University of Pittsburgh
Case No. (initials) I (A.
M.)
2 (R. P.) 3 (W. R.)
..
(S. H.)
Time postop.; date last seen
s
yr.; 1972
Symptoms
None
Blood Diastolic pressure III urm ur (mm. Hg) (grade)
3 of 6
Heart size tct ratio)
Angio-
gram
Comment
110/60
0.49
No progression of AI during last 7 years; doing well
0.52
Much improved; unusual cause of AI
Died at reoperation 4 mo. postop. 15 rno.: Feb., 1972
Active in sports
2 of 6
110/60
.. mo.: July, 1971
None
None
110/70
6 mo.; Jan., 1972
None
3 of 6
100/50
5 mo.; April, 1972
None
2 of 6
Good result
5 (A. M.)
6 (R. H.) 7 (C. G.}
2 mo.; Feb., 1972
Present
130/80
0.49
Decrease in heart size; CT 0.56 before operation
0.5
Residual AI but improved
0.49
Decrease in heart size; CT 0.55 before operation
Legend: CT, Cardiothoracic ratio.
tricular septal defect. In the other group, however, the major hemodynamic burden is that of aortic insufficiency. Earlier, before the syndrome was well defined, some patients were erroneously subjected to a thoracotomy with the mistaken diagnosis of patent ductus arteriosus. The left-to-right shunt in such patients is usually small, often with a pulmonary blood flow less than twice normal, probably because the prolapsed aortic cusp partly occludes the ventricular septal defect. Pulmonary hypertension is accordingly rare. In most patients a diastolic murmur appears after the second year of life but before the tenth year. The left ventricle enlarges rapidly within 2 to 3 years in about 20 per cent of the patients, while in the majority it grows more slowly. Once aortic insufficiency appears, the clinical course is very similar to that of rheumatic aortic insufficiency. The diagnosis, once suspected, can be easily con-
firmed by cardiac catheterization and aortography. Technique of aortic valvuloplasty. Several guidelines in the technique of valvuloplasty appear useful. As a general principle, it is clear from the variation in pathology among different patients, including different abnormalities in the cusps and the commissures, that a single uniform method of repair cannot be applied to all patients. What combination of techniques should be used must be decided at operation when the aortic valve is examined. Similarly, it is clear that no technique has yet been uniformly successful for all patients. A small percentage of patients may have such extensive cusp deformities that repair cannot be done, but this is probably no more than 10 to 15 per cent of all patients seen. Shortening of the free margin of the elongated right coronary cusp. As elongation and prolapse of the right coronary cusp is
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Table IlIA. Aortic insufficiency: Operative findings and results at Washington University Case No. (initials, age in years)
Date oj operation
Aortic valve pathology
Aortic valve repair
VSD pathology (size; location)
VSD repair
Initial result
Comment
Patched
No AI
Good result
1
Nov., 1968
RC prolapse Shortening of free edge of RC
2 (B. L., 6)
April, 1969
RC prolapse Double short- Infracristal; ening of free small edge of RC
Patched
No AI
Good result
3 (S. S., 4Y2)
April, 1970
RC prolapse Double short- Infracristal; ening of free small edge of RC
Patched
No AI
Good result
4 (M. L., 2)
Feb., 1972
RC prolapse Double short- Supracristal; Patched ening of free large edge of RC
No AI
Good result
(Y. T.,
4~)
Small
For legend, see Table IA.
Table IIIB. Postoperative aortic insufficiency: Long-term evaluation at Washington University Blood pressure (mm. Hg)
Time postop.; date last seen
Symp-
toms
Diastolic murmur (grade)
2Y2 yr.; March, 1971
None
None
105/80
Cath. 1 yr.; trace of AI
Good result
(B. L.) 3
2+ yr.; June, 1971
None
None
100/70
No AI
Good result
(S. S.)
19 mo.; Nov., 1971
None
None
104/70
No AI (aortogram, March, 1971)
Good result
2 mo.; April, 1972
None
2 of 6
Case No. (initials) 1 (Y. T.)
2
4
(M. L.)
Heart size
Angiogram
Comment
Moderate AI recurred
For legend, see Table IA.
the most frequent abnormality, shortening of the free margin to prevent prolapse is the essential feature in repair (Fig. 6). In some patients this cusp has enlarged to an astonishing degree, probably analogous to the continued enlargement of a longstanding inguinal hernia. Accordingly, the degree of shortening necessary is surprising, as much as 15 to 18 mm. in some patients. The left and posterior cusps are often abnormal in shape and contour, but significant elonga-
tion and prolapse is rare-almost unknown with the left cusp. Plication has been done in several ways, the most familiar of which is simply folding the leaflet to form a pleat and securing it with sutures (Fig. 1). The method recently developed by one of the authors (C. S. W.), which involves insertion of a continuous longitudinal suture along the free margin and then tying it, may be simpler and more certain (Fig. 2). The question arises as to whether shortening of
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the cusp margin is sufficient or whether the shortening should also encompass part of the body of the leaflet. The effectiveness of the longitudinal suture technique (Fig. 2), if confirmed by more experience, indicates that shortening the body of the cusp may not be necessary. Regardless of the method of plication used, the plicated segment should probably remain mobile rather than attached to a fixed area, such as the aortic wall or a commissure. A familiar technique illustrated in several reports that described frequent valvuloplasty failures shows the plicated segment anchored with sutures through the aortic wall. G A plicated segment anchored to a fixed point probably has a greater likelihood of dehiscence than one which remains mobile. A separate consideration is that a short double plication near each commissure of the right cusp seems preferable to a single large plication near the center of the cusp. A large central plication has been observed to prolapse into the ventricle whenever blood flow was restored to the aorta. Plication near the commissures, where cusp mobility is less, has much less tendency to prolapse. Suturing of commissures. As emphasized by Van Praagh," abnormal commissures are common, especially with infracristal septal defects. Different abnormalities which have been seen include a widened commissure, an abnormally low cusp attachment, or commissural adhesions restricting mobility. Correction of such abnormal commissures is perhaps the second most important part of valvuloplasty, a separate consideration from plication. Partly obliterating the commissure by suturing the cusp margins and reinforcing the sutures with a small Dacron pledget seems adequate. Suturing of two commissures may be necessary but can be done to a limited extent to avoid production of aortic stenosis. This has been satisfactorily accomplished in several patients (Table IA). In the unusual 35-year-old patient with calcification and retraction of the right coronary cusp (Case 5, Table IA), simple suture of one commissure was sufficient.
Table IV. Summary of data No. of cases University Washington Pittsburgh New York Johns Hopkins* Total Length of postop. observation 6 mo. 6-12 mo. l~
y~
2-3 yr. 3-4 yr. 5-12 yr. Position of VSD Supracristal Subcristal Not stated Previously reported
4 7 7 2
20
No. with significant residual Al 1 5 1
o 7
5 2 3 3 1 5 6 7 5 2
Legend: There has been one death-in a patient who had replacement of two cusps with fascia lata. All 19 of the survivors are in improved condition. There has been no late (after I year) progression of aortic insufficiency (AI). VSD, Ventricular septal defect.
'These cases were reported previously.
Evaluation of effectiveness of aortic repair. Several reports of aortic valvuloplasty did not describe the operative method by which the effectiveness of operative repair was evaluated. It is possible that valvuloplasty failure detected later may have resulted from inaccurate assessment at operation. Temporarily approximating the corpora arantii of the three cusps with a suture, as described by Frater," is one method. A method used by the senior author, somewhat more cumbersome, is to measure both the distance between the commissures and the length of the free margin of the leaflet and then to calculate the ratio between the commissural distance and the length of the free margin. A similar ratio is then determined for the other two cusps, after which the elongated cusp is shortened to an appropriate degree. However, both of these methods are subjective and should be supplemented by inspection of the functioning
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Fig. 5. Pre- (A ) and postoper ative (A' ) angiograms showing only trivial postoperative aortic insufficiency (Case I, Table III). Pre- (8) and postoperative (8 ') aortogra ms showing massive preoperative aortic insufficiency and the corrected aortic insufficie ncy (Case 2, Table III) .
valve through the ventricular septal defect. In all cases described in this report, the ventricular surface of the aortic valve was inspected through the ventricular septal defect after completion of the valvuloplasty. It was particularly impressive to the senior author that, despite careful attention to all known guidelines, significant insufficiency remained after the initial repair in 2 of the last 5 patients. When this was detected,
the aorta was reopened and additional plication was done. This corrected the remaining insufficiency, changing what probably would have been limited improvement to an excellent result. Without accurate operative assessment , it is difficult to discern whether inadequate operative repair or subsequent dehiscence is the cause of residual insufficiency. Some of the pessimism regarding the durability
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Fig. S. Cont'd, Pre- (C) and postoperative (C') aortograms showing total correction of severe aortic insufficiency (Case 3, Table III).
of valvuloplasty may have erroneously evolved from late recognition of insufficiency which was never corrected at operation. Repair of the ventricular septal defect. Much variation has been reported in the technique of closure of the ventricular septal defect. Actually, we ourselves are not in complete agreement on this point. Different methods include suture repair through the aorta; closure with a patch through the aorta; direct suture closure through a ventriculotomy, either in the horizontal or transverse axis; or closure with a patch. The last method, closure with a patch, is preferred by two of the authors, who fear that sutures may distort the aortic annulus (Fig . 3). A different viewpoint is that sutures properly placed may reattach the aortic annulus and more properly support the aortic cusps. Which of these two hypotheses is correct requires further data. In patients with minimal aortic insufficiency, the proper approach is not certain. Treasure'? reported that simply repairing the ventricular septal defect may be adequate, as the existing insufficiency often does not increase. It is possible that the prolpased cusp may be supported by repair of the septal leaflet, but this has not been pre-
dieted with any certainty. More likely, the value of early repair is to prevent further elongation and prolapse. With the knowledge that valvuloplasty can be effectively done in most patients, a question for future study is whether patients with minimal insufficiency should have a concomitant valvuloplasty at the time of closure of the ventricular septal defect. Why has cusp plication failed so frequently in the past? Considerable thought has naturally been given to the serious question of why most patients described in this report obtained a good-to-excellent result with valvuloplasty while several reports from others have described such poor results that valvuloplasty has been almost abandoned in favor of homograft valve replacement. As this condition is unusual, it seemed likely for some years that variation in pathology with a rare lesion would prevent the experiences of different groups from being comparable. For this reason, experiences at three different universities were combined in this report to minimize the likelihood that a chance variation in pathology would permit a single surgeon to encounter a small number of patients with abnormalities particularly favorable
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Fig. 6. Operative view of the repaired aortic valve (C ase 4, Table I) showing cusp plication and suture of both commissures.
for repair. With experiences now extending over 10 years, encompassing 20 patients in three different universities, the likelihood of such chance variation seems small. The reasons for valvuloplasty failure arc partly conjectural, and suggestions presented here must be verified by future experience. However, several points described in the section on technique seem applicable. First is the necessity for variation in technique of plication, including both location and extent; in some patients, a segment as large as 15 to 18 mm. must be plicated. The recently developed technique of longitudinal plication of the free margin of the leaflet with a continuous suture (Fig. 3) may well represent an important advance . The concept of keeping the plicated segment mobile also seems significant. Second, the importance of correcting commissural abnormalities separately may be important, especially with infracristal defects." Third, perhaps most important is the use of a reliable operative method of evaluating effectiveness of valvuloplasty . If repair is done completely through the aorta, this simply cannot be done. A fourth suggestion, again hypothetical, is that the unfavorable experience published by several groups between 1961 and 1966
generated pessimism about the possibility of repair of these abnormal cusps and discouraged further investigation. In this regard, there undoubtedly are a few patients in whom valve deformities are so extensive that no method of valvuloplasty can be successful and prosthetic replacement is required . However, this must represent only a small percentage of the total group of patients seen. Finally, there has been unwarranted pessimism about the durability of an initially successful valvuloplasty. Recurrences evident within a few days of operation may be due to inadequate operative correction or dehiscence of an initially effective plication. In at least 2 patients in this report there was considerably more aortic insufficiency within 3 months after operation than was present at the time of leaving the hospital. However, this period of the first few months after operation represents the only time in which increasing insufficiency has occurred. Beyond this point, residual insufficiency has remained stable. With the period of postoperative observation described, as long as 10 to 12 years, it seems clear that progressive insufficiency is unusual. Admittedly, continued fibrosis in an area of turbulent blood flow could produce further contraction of an abnormal cusp, but this must be rare. In Table IB are shown 4 patients with Grade 1 of 6 diastolic murmurs who have been observed for periods between 5 and 12 years with no change whatever.
Summary I. Combined experiences from three separate universities, involving 18 patients who were treated by valvuloplasty for aortic insufficiency associated with a ventricular septal defect, are described in this report. In addition, recent evaluation of 2 patients previously reported upon in 1962 is included, making a total of 20 patients. 2. The only operative death in this series followed replacement of the abnormal aortic cusp with fascia lata , resulting in recurrent progressi ve insufficiency within a short time. All of the 19 patients are in significantly impro ved condition . Significant aortic in-
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January, 1973
sufficiency remains in 7 of these, but improvement is shown by a decrease in heart size, absence of symptoms, and little limitation of physical activity. 3. In all patients, the abnormal aortic cusps were first repaired through an aortotomy, and then the ventricular septal defect was closed through a right ventriculotomy. Postponing closure of the ventricular septal defect until the aortotomy had been sutured permitted the surgeon to evaluate the effectiveness of valvuloplasty by inspecting the aortic valve through the ventricular septal defect. This was useful, for residual insufficiency was found in some patients and was corrected by reopening the aorta and performing further valvuloplasty. 4. Methods of valvuloplasty varied but essentially included shortening of the elongated right coronary cusp, either by plication or by longitudinal suture of the free edge of the cusp (Figs. 1 and 2). Closure of the ventricular septal defect also varied but was usually done with a prosthetic patch (Fig. 3). 5. A particularly significant point was the absence of progression of aortic insufficiency beyond the first few months after operation. As periods of observation in 12 of the patients are now between 1 and 12 years, the likelihood of increasing insufficiency in those with slight diastolic murmurs seems remote. 6. The fact that aortic valve replacement with a prosthesis was not necessary in any of the 20 patients indicates that most patients may be treated by different forms of valvuloplasty. Some patients remain with significant regurgitation, however, indicating that further study is needed. REFERENCES Garamella, J. J., Cruz, A. B., Jr., Heupel, W. H., Dahl, J. C., Jensen, N. K., and Berman, R.: Ventricular Septal Defect With Aortic Insufficiency: Successful Surgical Correction of Both Defects by the Transaortic Approach, Am. J. Cardiol. 5: 266, 1960. 2 Starr, A, Menashe, V., and Dotter, D.: Surgical Correction of Aortic Insufficiency Associated With Ventricular Septal Defect, Surg. Gynecol. Obstet. 111: 71, 1960. 3 Spencer, F. C., Bahnson, H. T., and Neill,
4
5
6
7
8 9
10
11
12
13
14
C. A: The Treatment of Aortic Regurgitation Associated With a Ventricular Septal Defect, J. THoRAc. CARDIOVASC. SURG. 43: 222, 1962. Ellis, F. H., Jr., Ongley, P. A., and Kirklin, J. W.: Ventricular Septal Defect With Aortic Valvular Incompetence: Surgical Considerations, Circulation 27: 789, 1963. Plauth, W. H., Jr., Braunwald, E., Rockoff, S. D., Mason, D. T., and Morrow, A. G.: Ventricular Septal Defect and Aortic Regurgitation: Clinical, Hemodynamic and Surgical Considerations, Am. J. Med. 39: 552, 1965. Gonzalez-Lavin, L., and Barratt-Boyes, B. G.: Surgical Considerations in the Treatment of Ventricular Septal Defect Associated With Aortic Valvular Incompetence, 1. THORAC. CARDIOVASC. SURG. 57: 422, 1969. Somerville, J., Brandao, A., and Ross, D. N.: Aortic Regurgitation With Ventricular Septal Defect: Surgical Management and Clinical Features, Circulation 41: 317, 1970. Frater, R. W. M.: The Prolapsing Aortic Cusp: Experimental and Clinical Observations, Ann. Thorac. Surg. 3: 63, 1967. Hallidie-Smith, K. A, Olsen, E. G. J., Oakley, C. M., Goodwin, J. F., and Cleland, W. P.: Ventricular Septal Defect and Aortic Regurgitation, Thorax 24: 257, 1969. Treasure, R. L., Hopeman, A. R., Jahnke, E. J., Green, D. C., and Czarnecki, S. W.: Ventricular Septal Defect With Aortic Insufficiency, Ann. Thorac. Surg. 12: 411, 1971. Van Praagh, R., and McNamara, J. J.: Anatomic Types of Ventricular Septal Defect With Aortic Insufficiency, Am. Heart J. 75: 604, 1968. Scott, R. C., McGuire, J., Kaplan, S., Fowler, N. 0., Green, R. S., Gordon, L. Z., Shabetai, R., and Davolos, D. D.: The Syndrome of Ventricular Septal Defect With Aortic Insufficiency, Am. J. Cardiol. 2: 530, 1958. Nadas, AS., Thilenius, O. G., LaFarge, C. G., and Hauck, A J.: Ventricular Septal Defect With Aortic Regurgitation: Medical and Pathologic Aspects, Circulation 29: 862, 1964. Keck, E. W.O., Ongley, P. A, Kincaid, O. W., and Swan, H. J. C.: Ventricular Septal Defect With Aortic Insufficiency: Clinical and Hemodynamic Study of 18 Proved Cases, Circulation 27: 203, 1963.
Discussion DR. DAVID A. MURPHY Montreal, Quebec, Canada
As the abstract states, the techniques of valvuloplasty will vary as to the type of pathology you find. We were very familiar with Dr. Spencer and Dr. Bahnson's earlier published works on this subject. We recently had a case in Montreal in which,
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before surgery, we thought we were going to plicate the aortic valve, as described so nicely here. However, when we saw the valve, we found, as Van Praagh described, a commissure that was lower than usual, counterclockwise rotation of the aortic root, and noncoronary and right coronary cusps that reminded me of my father's old wool bathing suit as he got out of the water. They were sagging down into the lumen of the left ventricle. We were unable to do the plication as Dr. Spencer has suggested, since the area of the valve adjacent to the affected commissure was very thin. Since the child was only 6 years old, we really didn't think the stitches would hold. Therefore, I extend the aortotomy down between the affected commissure carefully. The redundant free margins of the affected leaflets were then brought upward and through the split in the commissure outside the aortic lumen. They were held fast in their new position by horizontal mattress stitches which were used to close the split commissure and aortotomy. The everted edge and excess valve tissue on the outside of the aorta were then closed with a continuous stitch. It is a little premature to say how successful this has been. However, at postoperative catheterization, there was only a whiff of regurgitation. The pulse pressure has returned to normal, and the patient has no diastolic murmur. This method is offered as an alternative treatment for this variable lesion. DR. CLARENCES. WELDON St. LOllis, Mo.
I apologize for discussing a paper of which I am an author, but there are several points about this malformation which I think are important. This malformation is unusual in that there is aortic insufficiency in conjunction with an excess of aortic valve tissue. I think the aortic insufficiency occurs because the aortic leaflet has lost its support. In the infracristal variety, the VSD appears to be a separation of the aorta from the heart in the region of the right cusp of the aortic valve. It is not a simple defect in the membranous portion of the interventricular septum. The annulus, therefore, loses its support. Secondarily, as the valve herniates through the defect, the commissural support is lost because the free margin of the valve elongates. The surgeon must repair both of these support mechanisms. It is very important to repair the defect with a patch because there is a danger of pulling the valve down to the septum and making the aortic valve insufficiency worse. We use a cloth patch which provides some rigidity in the area to support the annulus of the aortic valve. The commissural support can be reestablished, as was illustrated, simply by adjusting the length of the free margins so that they equal the coapting free edges of adjacent leaflets.
Our results in the infracristal repair were all very good. The supracristal variety, in which there is a very large VSD, is quite a different thing. When we tried to apply the same operation to this condition (although we could easily adjust the free margins of the cusp) we were unable to restore the support of the annulus by placing a patch in the very large supracristal defect. Our result is not perfect in the case of supracristal defect. I think one should have some reservations about cutting out this good valve tissue in a child and replacing it with a prosthesis or with some tissue which is likely to be less functional for a long period of time than the normal aortic valve tissue. DR. LESTER R. BRYANT Lexington, Ky.
I would like to give an indirect endorsement of the plea for aortic valvuloplasty by Dr. Spencer and his associates. Our experience with 3 patients who had this combination of congenital defects includes 2 adults, in contrast to the majority of patients who appear to be in the pediatric age group. All 3 of our patients were managed by valve replacement, and it seems in retrospect that this may have been a somewhat misguided approach. Two patients have developed serious paravalvular leaks, 1 of whom was reoperated upon just 2 months ago. The opportunity for reoperation allowed us to observe what is probably another anatomic feature that is found in at least some patients with this defect. This feature was probably responsible for the paravalvular leak in our 2 patients. I refer to the very deep insertion of the aortic cusps in this anomaly. In the patient whom we reoperated upon, the vertical distance of the cusp attachments to the aortic wall had been nearly twice that seen in normal patients. In placing the valve sutures originally, we had followed the attachment of the valve cusps up to the commissures, so that the vertical depth of the sutures was too great for accomodation by the single plane of the valve suture ring. As the sutures were tied down, undue tension was placed on the aortic wall, causing early paravalvular leak. I would point out that if one is required to do a valve replacement in this condition, he should take into account the abnormal insertion of the cusps and use adequate buttressing to bridge the defect between the commissures. DR. ROBERT W. M. FRATER Bronx, New York
I would like to add my support to Dr. Spencer's contention by telling about the 3 patients whom I treated between 1965 and 1966. They now have been followed for 5 or more years.
Volume 65 Number 1 January, 1973
As Dr. Weldon warned, the 1 patient who had a supracristal VSD does have some residual insufficiency. However, that insufficiency has remained constant over the 5 years from a period of a few months after operation, when it reached its maximum extent. These 3 patients are all continuing to do well and all confirm the worth of this procedure. DR. FRANK C. SPENCER (Closing) I appreciate the comments of the different discussers. With the limits of time, I will not comment specifically on their remarks but rather will make two or three general observations. This is an uncommon lesion. We see only about 1 such patient a year; hence, it is difficult for most groups to acquire significant experience. With the widespread pessimism about the likelihood of correction without valve replacement, cardiologists have become increasingly reluctant to refer patients for operation, so that operative experience with valvuloplasty has remained small. For some time I felt our group had seen a group of patients who were in unusually favorable
Aortic valvuloplasty
31
condition for surgical correction. However, with the pooling of data from three different institutions, representing 20 patients from widely separated geographical areas, it is very unlikely that a chance variation exists. Almost clearly, the data are truly representative. Hence, our most significant conclusion is the concept that effective valvuloplasty can be done in the majority of patients by one of a variety of techniques. With this concept, operation is indicated at an early stage of the disease, soon after insufficiency is recognized, to prevent progressive enlargement of the left ventricle and increasing deformity of the right cusp. At present, if valve replacement is considered likely, the opposite approach is taken. Operation is deferred as long as possible, resulting in massive enlargement of the left ventricle and extensive deformity of the aortic valve. Consequently, a valvuloplasty is much more difficult. Hopefully, with the data presented here today, a much more favorable view of early valvuloplasty will develop.
A
t present there is a great diversity of opinion regarding the effectiveness of aortic valvuloplasty for aortic insufficiency associated with a ventricular septal defect. Some physicians employ valvuloplasty in most patients, whereas, at the opposite extreme, others routinely treat such patients with a prosthetic valve, usually a homograft. The great discrepancy in results reported by different groups in the past decade partly explains this wide variation. The first articles concerning operation for aortic insufficiency with ventricular septal defect were by Garamella 1 and by Starr- in 1960; each of them reported that his patient survived. In 1962, two of us' (F. C. S. and H. T. B.) reported experiences with a method of cusp plication in 3 patients, 2 of whom survived. However, results obtained with plication by others in the next 4 years were discouraging. In 1963 Ellis" described experiences with 19 patients, From the Departments of Surgery and Pediatrics, New York University Medical Center. 550 First Avenue. New York, N.Y. 10016, the Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pa., and the Department of Surgery, Washington University School of Medicine, SI. Louis, Mo. 63110. Read at the Fifty-second Annual Meeting of The American Association for Thoracic Surgery, Los Angeles, Calif., May I, 2, and 3, 1972.
of whom 13 had some type of cusp reconstruction and 5 had cusp replacement. Of the 13 with cusp reconstruction, 10 still had moderate-to-severe insufficiency. In 1965, Plauth and colleagues" described operative experiences with 6 patients, I of whom died. In 2 the ventricular septal defect was simply closed, while in 3 valvuloplasty was done; only I of these 3 had a good result. Their review of reports by others revealed a total of 17 patients treated by cusp plication, only 6 of whom had a satisfactory result. With these discouraging results, a natural pessimism arose about the feasibility of valvuloplasty, and interest increased in aortic valve replacement with a homograft. In 1969, Gonzalez-Lavin and BarrattBoyes" reported upon 7 patients in whom the prolapsed cusp was used to close the ventricular septal defect, after which an aortic homograft was inserted. Similarly, in 1970, Somerville and associates' described experiences with 20 patients; in 6 the aortic valve was repaired, while in 14 a prosthetic valve was inserted. Of the 6 patients with valvuloplasty, done by plication in 3 and by addition of fascia lata or pericardium in 3, only I obtained a competent valve.
15
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Fig. 1. A , Illustration demonstrating the technique of cusp plication. The free edge of the elong ated cusp is shortened to appose the normal cusp. B, Dr awing showing the plicated cusp with its mobility preser ved. Separate repair of the abnormal comm issure is then carried out . (The drawings were adapted from the original work of Leon Schlossberg. ")
Only a few publications concerning valvuloplasty have appeared in the past 7 years. In 1967 Frater," in reporting 1 case, described a method of evaluating competence of the reconstructed valve by temporarily suturing the midpoints of the three aortic cusps togeth er. S His patient obtained an excellent result. Ten months later there was only a faint diastolic murmur with a blood pressure of 100/70 mm. Hg. In 1969 a series of 29 patients was described by Hallidie-Smith and associates," 27 of whom were operated upon. There were 23 survivors; 8 of these had no repair of the aortic valve, 3 had prostheses inserted, and 12 had plication. There was a discouragingly high frequency of significant insufficiency in those patients treated with plication. Quite recently, October, 1971 , Treasure and assoelates ':' reported experiences with 15 patients treated at Walter Reed General Hospital. In 9 of the 15 with only minimal insufficiency, only the ventricular septal defect was repaired. A valvuloplasty was performed in 2 of the 4 who had severe insufficiency. The condition of I of these 2 was improved, but the other has significant residual insufficiency. In the discussion of this report, both Weldon and Trusler described successful valvuloplasty with 3 and 6 patients, respectively.
Our report describes experiences with valvuloplasty in 18 patients and adds a recent evaluation of the 2 surviving patients originally reported in 1962. 3 The conclusions differ rather sharply from those in several reports in recent years, for prosthetic replacement was not necessary in any patient , all patients are in improved condition, and the majority now have no significant insufficiency. Methods
To report on a significant group of patients, experiences at three different universities-New York University, the University of Pittsburgh, and Washington Universityhave been combined. The three authors were all at Johns Hopkins University when the original experiences with cusp plication were described in 1961 . All patients were operated upon with cardiopulmonary bypass. Aortic valvuloplasty was first performed by an appropriate combination of cusp plication and suturing of abnormal commissures (Fig. 1). A new method of cusp plication developed by one of the authors (c. S. W.) was used in all 4 patients operated upon at Washington University (Fig. 2). The aorta was then closed, and the ventricular septal defect was repaired through a right ventriculotomy. By closing the aorta first and view-
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Posterior cusp
Right cusp
CD
®
® Fig. 2. Cont'd. D, A plicating suture draws up the excess to the commissural margin . No tissue is excised. E, The leaflet is now normally proportioned and accurately suspended.
attached with circumferential sutures, but a few were repaired by direct suture (Fig. 3). All patients have been evaluated recently for cardiac function and residual aortic insufficiency. Fig. 2. A, The right cusp is redundant. The distance from commissure to corpus arantii is unequal to that of the opposing cusp edge. B, The corpora arantii are sutured together. C, The distance from corpus arantii to commissural margin is adjusted to equal that of the opposing cusp.
ing the ventricular surface of the reconstructed valve through the septal defect, it was possible to assess the effectiveness of aortic valvuloplasty. This has consistently been a reliable method of assessment of . competence. Most septal defects were closed with a Dacron or Teflon prosthetic patch,
Results The data are given in Tables IA through IV. Results from New York University are shown in Tables IA and IB, from the University of Pittsburgh in Tables IIA and lIB, and from Washington University in Tables IlIA and IIIB. Over-all experiences are summarized in Table IV. The most common abnormality was elongation and prolapse of the right coronary cusp (Fig. 4). Additional abnormalities, however, were frequent. Often all three cusps were different in size and shape. Occasional-
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c.
Fig. 3. A, Ventricular septal defect. B, Separation of the aorta from the heart, producing a ventricular septal defect. C, Direct suture produces a downward displacement of the annulus and destruction of commissural support. D, Precise patch closure preserves annular level and commissural support.
ly, some prolapse of the posterior cusp was present as well. In several patients, commissural abnormalities were similarly recognized, including widening of the commissure, abnormal insertion of the cusp at a level below the other two cusps, or localized adhesions near the commissure which limited mobility of the diseased cusp. A few additional abnormalities were recognized. In a 35-year-old patient (Case 5, Table IA), calcification and contraction of the right cusp produced insufficiency. In another patient (Case 4, Table IIA), a small aneurysm of the sinus of Valsalva was present as well. Patient 3 (Table IIA) had subvalvular aortic stenosis with adhesions limiting mobility of the cusps, but he did not have prolapse of a cusp. Seventeen of the 18 patients survived
operation, and all are in improved condition (Table IV). Ten of the 17 have no significant insufficiency, while 7 have some insufficiency but are in significantly improved condition, with absence of symptoms, a decrease in heart size, and little or no restriction of physical activity (Fig. 5). Among the 18 patients operated upon, 1 had two valve cusps replaced with fascia lata (Case 2, Table IIA). A competent valve was obtained, but progressive insufficiency appeared within a few months, requiring reoperation. Unfortunately, the patient died, the only death in the entire series. With the recent experiences with valves made from fascia lata, it seems likely that the recurrent insufficiency was due to progressive contraction of the fascia lata. In only 1 other patient (Case 1, Table IIA) was any prosthetic
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Fig. 4. A, Operative photograph showing repair of the aortic cusp in a patient operated upon at New York University (Case 2, Table I). Plication of the cusp and suture of the commissure can be seen. B, View of the ventricular septal defect in the same patient. The large right coronary cusp, although elongated, was repaired so that aortic insufficiency is no longer present. C, Operative photograph of the ventricular septal defect in another patient (Case 3, Table I). The prolapsed right coronary cusp can be clearly seen. D, Operative view showing elongated, prolapsing right coronary cusp (Case 4, Table I).
material employed: A strip of pericardium was used to lengthen one cusp. There was some residual insufficiency, but the patient's condition has remained satisfactory in the ensuing 8 years (Case I, Table lIB). The ventricular septal defect was supracristal in 6, subcristal in 7, and not stated in the operative report in 5 cases. Commissural abnormalities were noted in several patients, but there was no distinct correlation with
the location of the ventricular septal defect. In 1968, Van Praagh,!' in a careful analysis of I I autopsied patients, divided them into two groups depending upon whether the ventricular septal defect was above or below the crista supraventricularis. Those with septal defects above the crista, a subpulmonary location, usually had simple prolapse of the right coronary cusp; those with infracristal defects had more extensive malformation of
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Table IA. Aortic insufficiency: Operative findings and results at New York University Case No. (initials, age in years) 1
I
Date of operation
Aortic valve pathology
A ortic valve repair
VSD pathology (size; location)
VSD repair
Initial result
Comment
(E. G.,* 8)
1963
RC prolapse
Plication of RC
Supracristal
Patch
No AI Good result
2 (P. N., 9)
1967
RC prolapse
15-18 mm. plication of RC; suture of RCLC and RCPC commissures
2 ern.
Patch
No AI Good result
(J. C., 2)
1968
RC prolapse; RC-LC commissure adhesions
7 rnm. plication of RC; RC-LC commissure sutured
15 mrn.; supracristal
Sutured
No AI Good result
4 (R. C., 9)
Jan., 1970
RC prolapse; Plication of all 3 cusps ab- RC; suture of normal; RC-PC RC-LC and adhesions RC-PC commissures
15 mm.; subcristal
Patch
No AI Good result
RC-PC commissure sutured
12 mm.; subcristal
Patch
Slight AI
3
5 (A. W., 35)
April, 1971 Calcification of RC
6
Good result
(M. D., 7)
July, 1971
RC prolapse; Plication of all 3 cusps dif- RC; RC-PC ferent; RC-PC sutured commissure abnormal
10 mm.; subcrista I
Patch
No AI Good result
7 (D. C., 4)
July, 1971
RC prolapse; RC-PC commissure abnormal
Small, subcristal
Patch
No AI Good result
16 mm. plication; RC-PC and RC-LC sutured
Legend: RC. Right cusp. PC. Posterior (noncoronary) cusp. LC. Left cusp. VSD, Ventricular septal defect. AI, Aortic insufficiency. 'Operated upon at Brooke Army Hospital, San Antonio, Texas.
the aortic valve, including commissural deformities, and often separate abnormalities in the outflow tract of the right ventricle. In the preparation of this report, a recent evaluation was made of all 17 surviving patients as well as of the 2 patients originally described in 1962. As shown in Table IV, 7 patients are now less than 1 year since operation, 7 are between I and 4 years, and 5 are between 5 and 12 years following operation. All surviving patients are in sig-
nificantly improved condition, even though 7 of them have some aortic insufficiency. Curiously enough, there has been a significant variation in the frequency of residual insufficiency among the three institutions. A similar operative technique was employed at New York University and the University of Pittsburgh, but at Washington University the method of plication by longitudinal suture (Fig. 3) was used. A concern often expressed for patients
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Table lB. Postoperative aortic insufficiency: Long-term evaluation at New York University Case No. (initials) 1 (E. G.) 2
(P. N.)
3 (1. C.)
4 (R. C.)
Time postop.; date last seen
1*
2* (I. F.)
Angio-
gram
Comment
8 yr.; Aug., 1971
None
? 1 of 6
120/88 Normal
Good result
5 yr.; April, 1972
None
1 of 6
124/76 Slight RV enlargement
Good result
3~
None
None
100/60 Normal
5 yr.; April, 1972
None
1 of 6
104/76 -
Good result
None
None
110/70 -
Good result
None
1 of 6
100/68 Normal
Good result
None
3 of 6
100/52 -
Moderate AI; decrease in heart size
12 yr.; None; active? 1 of 6 March, 1972 skier
120/70 -
Good result
12 yr.; March, 1972
120/75 -
Good result; minimal AI unchanged in 10 yr.
yr.; March, 1972
5 (A. W.) 1 yr.; April, 1972 6 (M. D.) 6 mo.; Ian., 1972 7 (D. C.) 8 mo.; March, 1972 (C. A.)
Diastolic Blood murmur pressure Symptoms (grade) (mm. Hg) Heart size
None
1 of 6
Jan., 1970, no AI
Good result
Legend: RV,Right ventricle. For other abbreviations, see Table IA.
'Previously reported upon,"
with some insufficiency after operation is that the insufficiency will gradually become more severe in subsequent years, similar to that with rheumatic aortic insufficiency, and eventually will necessitate replacement of the valve with a prosthesis. Fortunately, to date this has not been true. Following operation some increase in aortic insufficiency has been seen in a few patients in the first few months, but after this time no further change has occurred in periods of observation extending up to 7 or more years. The presence of slight (Grade 1 of 6) diastolic murmurs, associated with no measurable hemodynamic abnormalities, has thus far been innocuous. Several such patients have been observed for several years with no demonstrable change
(Table IB). The absence of change may be similar to the findings reported by others" 10 in which patients with mild aortic insufficiency and a ventricular septal defect have been simply treated by closure of the ventricular septal defect. The residual aortic insufficiency has shown no significant change in the ensuing years. Discussion Clinical characteristics. Beginning with the classic paper by Scott and colleagues> in 1958, the clinical syndrome of ventricular septal defect with aortic insufficiency from a prolapsed cusp has been described in detail. Only 22 patients had been reported upon at the time of the paper by Scott. Subsequently,
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Table IIA. Aortic insufficiency: Operative findings and results at the University of Pittsburgh Case No. (initials, age in years)
1
Date of operation
Aortic valve pathology
Aortic valve repair
VSD pathology (size; location)
VSD repair
Initial result
Comment
(A. M., 8)
June, 1964
BP 120/40 mm. Plication of RC Small Hg; prolapsed and PC; strip right leaflet of pericardium attached
Sutured
ImMore AI 1 proved; year later residual AI
2 (R. P., 4Y2)
Aug., 1969
Prolapsed RC and PC
Patched
ReopFailure of eration fascia lata after 4 cusps mo. for progressive AIdied
Replacement of 6 rnm.; supra2 cusps with fascia lata cristal
.
3 (W. R., 9)
4 (S. H., 12)
Dec., 1970
2/4 AI; cusps Excision of normal; subval- subaortic vular AS with stenosis adhesions
Small
Sutured
Mild AI Subvalvular AS corrected
March, 1971 * RC and PC cusps prolapsed; aneurysm of right sinus of Valsalva
Excision of aneurysm; plication of RC and PC
12 rnm.; Sutured subcristal
No AI
(A. M., 12)
July, 1971
3/4 AI; large prolapsing RC
Plication of RC and PC; suture of RC-PC commissure
12 mm.; Patched subcristal
Mild AI Good result
6 (R. H., 9)
Nov., 1971
2/ 4 AI; prolapse Plication of
Supracrista1
Sutured
Mild AI
Mild AI
Plication of SupraRC and LC; cristal body and free edge
Sutured
Mild AI
Mild AI
Good result
5
of RC; LC abnormal 7 (C. G., 9)
Dec., 1971
4/4 AI
RC and LC
Legend: BP, Blood pressure. AS, Aortic stenosis. For other abbreviations, see Table IA. 'Operation by Dr. Crile Crisler.
in 1964, Nadas'" described a group of 34 patients, the largest single series which had been reported upon. These 34 patients were from a group of 750 patients with ventricular septal defects seen at the Boston Children's Hospital over a period of 14 years, a frequency near 5 per cent. Subsequent
reports emphasizing different features of the clinical syndrome have been by Keck,14 Plauth," Somerville,7 and their co-workers. Two broad groups have been recognized. In one there is trivial aortic insufficiency with a ventricular septal defect, and the clinical features are those of a large ven-
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Table lIB. Postoperative aortic insufficiency: Long-term evaluation at the University of Pittsburgh
Case No. (initials) I (A.
M.)
2 (R. P.) 3 (W. R.)
..
(S. H.)
Time postop.; date last seen
s
yr.; 1972
Symptoms
None
Blood Diastolic pressure III urm ur (mm. Hg) (grade)
3 of 6
Heart size tct ratio)
Angio-
gram
Comment
110/60
0.49
No progression of AI during last 7 years; doing well
0.52
Much improved; unusual cause of AI
Died at reoperation 4 mo. postop. 15 rno.: Feb., 1972
Active in sports
2 of 6
110/60
.. mo.: July, 1971
None
None
110/70
6 mo.; Jan., 1972
None
3 of 6
100/50
5 mo.; April, 1972
None
2 of 6
Good result
5 (A. M.)
6 (R. H.) 7 (C. G.}
2 mo.; Feb., 1972
Present
130/80
0.49
Decrease in heart size; CT 0.56 before operation
0.5
Residual AI but improved
0.49
Decrease in heart size; CT 0.55 before operation
Legend: CT, Cardiothoracic ratio.
tricular septal defect. In the other group, however, the major hemodynamic burden is that of aortic insufficiency. Earlier, before the syndrome was well defined, some patients were erroneously subjected to a thoracotomy with the mistaken diagnosis of patent ductus arteriosus. The left-to-right shunt in such patients is usually small, often with a pulmonary blood flow less than twice normal, probably because the prolapsed aortic cusp partly occludes the ventricular septal defect. Pulmonary hypertension is accordingly rare. In most patients a diastolic murmur appears after the second year of life but before the tenth year. The left ventricle enlarges rapidly within 2 to 3 years in about 20 per cent of the patients, while in the majority it grows more slowly. Once aortic insufficiency appears, the clinical course is very similar to that of rheumatic aortic insufficiency. The diagnosis, once suspected, can be easily con-
firmed by cardiac catheterization and aortography. Technique of aortic valvuloplasty. Several guidelines in the technique of valvuloplasty appear useful. As a general principle, it is clear from the variation in pathology among different patients, including different abnormalities in the cusps and the commissures, that a single uniform method of repair cannot be applied to all patients. What combination of techniques should be used must be decided at operation when the aortic valve is examined. Similarly, it is clear that no technique has yet been uniformly successful for all patients. A small percentage of patients may have such extensive cusp deformities that repair cannot be done, but this is probably no more than 10 to 15 per cent of all patients seen. Shortening of the free margin of the elongated right coronary cusp. As elongation and prolapse of the right coronary cusp is
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Table IlIA. Aortic insufficiency: Operative findings and results at Washington University Case No. (initials, age in years)
Date oj operation
Aortic valve pathology
Aortic valve repair
VSD pathology (size; location)
VSD repair
Initial result
Comment
Patched
No AI
Good result
1
Nov., 1968
RC prolapse Shortening of free edge of RC
2 (B. L., 6)
April, 1969
RC prolapse Double short- Infracristal; ening of free small edge of RC
Patched
No AI
Good result
3 (S. S., 4Y2)
April, 1970
RC prolapse Double short- Infracristal; ening of free small edge of RC
Patched
No AI
Good result
4 (M. L., 2)
Feb., 1972
RC prolapse Double short- Supracristal; Patched ening of free large edge of RC
No AI
Good result
(Y. T.,
4~)
Small
For legend, see Table IA.
Table IIIB. Postoperative aortic insufficiency: Long-term evaluation at Washington University Blood pressure (mm. Hg)
Time postop.; date last seen
Symp-
toms
Diastolic murmur (grade)
2Y2 yr.; March, 1971
None
None
105/80
Cath. 1 yr.; trace of AI
Good result
(B. L.) 3
2+ yr.; June, 1971
None
None
100/70
No AI
Good result
(S. S.)
19 mo.; Nov., 1971
None
None
104/70
No AI (aortogram, March, 1971)
Good result
2 mo.; April, 1972
None
2 of 6
Case No. (initials) 1 (Y. T.)
2
4
(M. L.)
Heart size
Angiogram
Comment
Moderate AI recurred
For legend, see Table IA.
the most frequent abnormality, shortening of the free margin to prevent prolapse is the essential feature in repair (Fig. 6). In some patients this cusp has enlarged to an astonishing degree, probably analogous to the continued enlargement of a longstanding inguinal hernia. Accordingly, the degree of shortening necessary is surprising, as much as 15 to 18 mm. in some patients. The left and posterior cusps are often abnormal in shape and contour, but significant elonga-
tion and prolapse is rare-almost unknown with the left cusp. Plication has been done in several ways, the most familiar of which is simply folding the leaflet to form a pleat and securing it with sutures (Fig. 1). The method recently developed by one of the authors (C. S. W.), which involves insertion of a continuous longitudinal suture along the free margin and then tying it, may be simpler and more certain (Fig. 2). The question arises as to whether shortening of
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the cusp margin is sufficient or whether the shortening should also encompass part of the body of the leaflet. The effectiveness of the longitudinal suture technique (Fig. 2), if confirmed by more experience, indicates that shortening the body of the cusp may not be necessary. Regardless of the method of plication used, the plicated segment should probably remain mobile rather than attached to a fixed area, such as the aortic wall or a commissure. A familiar technique illustrated in several reports that described frequent valvuloplasty failures shows the plicated segment anchored with sutures through the aortic wall. G A plicated segment anchored to a fixed point probably has a greater likelihood of dehiscence than one which remains mobile. A separate consideration is that a short double plication near each commissure of the right cusp seems preferable to a single large plication near the center of the cusp. A large central plication has been observed to prolapse into the ventricle whenever blood flow was restored to the aorta. Plication near the commissures, where cusp mobility is less, has much less tendency to prolapse. Suturing of commissures. As emphasized by Van Praagh," abnormal commissures are common, especially with infracristal septal defects. Different abnormalities which have been seen include a widened commissure, an abnormally low cusp attachment, or commissural adhesions restricting mobility. Correction of such abnormal commissures is perhaps the second most important part of valvuloplasty, a separate consideration from plication. Partly obliterating the commissure by suturing the cusp margins and reinforcing the sutures with a small Dacron pledget seems adequate. Suturing of two commissures may be necessary but can be done to a limited extent to avoid production of aortic stenosis. This has been satisfactorily accomplished in several patients (Table IA). In the unusual 35-year-old patient with calcification and retraction of the right coronary cusp (Case 5, Table IA), simple suture of one commissure was sufficient.
Table IV. Summary of data No. of cases University Washington Pittsburgh New York Johns Hopkins* Total Length of postop. observation 6 mo. 6-12 mo. l~
y~
2-3 yr. 3-4 yr. 5-12 yr. Position of VSD Supracristal Subcristal Not stated Previously reported
4 7 7 2
20
No. with significant residual Al 1 5 1
o 7
5 2 3 3 1 5 6 7 5 2
Legend: There has been one death-in a patient who had replacement of two cusps with fascia lata. All 19 of the survivors are in improved condition. There has been no late (after I year) progression of aortic insufficiency (AI). VSD, Ventricular septal defect.
'These cases were reported previously.
Evaluation of effectiveness of aortic repair. Several reports of aortic valvuloplasty did not describe the operative method by which the effectiveness of operative repair was evaluated. It is possible that valvuloplasty failure detected later may have resulted from inaccurate assessment at operation. Temporarily approximating the corpora arantii of the three cusps with a suture, as described by Frater," is one method. A method used by the senior author, somewhat more cumbersome, is to measure both the distance between the commissures and the length of the free margin of the leaflet and then to calculate the ratio between the commissural distance and the length of the free margin. A similar ratio is then determined for the other two cusps, after which the elongated cusp is shortened to an appropriate degree. However, both of these methods are subjective and should be supplemented by inspection of the functioning
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The Journa l of Thorac ic a nd Cardiovas cular Surgery
Fig. 5. Pre- (A ) and postoper ative (A' ) angiograms showing only trivial postoperative aortic insufficiency (Case I, Table III). Pre- (8) and postoperative (8 ') aortogra ms showing massive preoperative aortic insufficiency and the corrected aortic insufficie ncy (Case 2, Table III) .
valve through the ventricular septal defect. In all cases described in this report, the ventricular surface of the aortic valve was inspected through the ventricular septal defect after completion of the valvuloplasty. It was particularly impressive to the senior author that, despite careful attention to all known guidelines, significant insufficiency remained after the initial repair in 2 of the last 5 patients. When this was detected,
the aorta was reopened and additional plication was done. This corrected the remaining insufficiency, changing what probably would have been limited improvement to an excellent result. Without accurate operative assessment , it is difficult to discern whether inadequate operative repair or subsequent dehiscence is the cause of residual insufficiency. Some of the pessimism regarding the durability
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Fig. S. Cont'd, Pre- (C) and postoperative (C') aortograms showing total correction of severe aortic insufficiency (Case 3, Table III).
of valvuloplasty may have erroneously evolved from late recognition of insufficiency which was never corrected at operation. Repair of the ventricular septal defect. Much variation has been reported in the technique of closure of the ventricular septal defect. Actually, we ourselves are not in complete agreement on this point. Different methods include suture repair through the aorta; closure with a patch through the aorta; direct suture closure through a ventriculotomy, either in the horizontal or transverse axis; or closure with a patch. The last method, closure with a patch, is preferred by two of the authors, who fear that sutures may distort the aortic annulus (Fig . 3). A different viewpoint is that sutures properly placed may reattach the aortic annulus and more properly support the aortic cusps. Which of these two hypotheses is correct requires further data. In patients with minimal aortic insufficiency, the proper approach is not certain. Treasure'? reported that simply repairing the ventricular septal defect may be adequate, as the existing insufficiency often does not increase. It is possible that the prolpased cusp may be supported by repair of the septal leaflet, but this has not been pre-
dieted with any certainty. More likely, the value of early repair is to prevent further elongation and prolapse. With the knowledge that valvuloplasty can be effectively done in most patients, a question for future study is whether patients with minimal insufficiency should have a concomitant valvuloplasty at the time of closure of the ventricular septal defect. Why has cusp plication failed so frequently in the past? Considerable thought has naturally been given to the serious question of why most patients described in this report obtained a good-to-excellent result with valvuloplasty while several reports from others have described such poor results that valvuloplasty has been almost abandoned in favor of homograft valve replacement. As this condition is unusual, it seemed likely for some years that variation in pathology with a rare lesion would prevent the experiences of different groups from being comparable. For this reason, experiences at three different universities were combined in this report to minimize the likelihood that a chance variation in pathology would permit a single surgeon to encounter a small number of patients with abnormalities particularly favorable
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Fig. 6. Operative view of the repaired aortic valve (C ase 4, Table I) showing cusp plication and suture of both commissures.
for repair. With experiences now extending over 10 years, encompassing 20 patients in three different universities, the likelihood of such chance variation seems small. The reasons for valvuloplasty failure arc partly conjectural, and suggestions presented here must be verified by future experience. However, several points described in the section on technique seem applicable. First is the necessity for variation in technique of plication, including both location and extent; in some patients, a segment as large as 15 to 18 mm. must be plicated. The recently developed technique of longitudinal plication of the free margin of the leaflet with a continuous suture (Fig. 3) may well represent an important advance . The concept of keeping the plicated segment mobile also seems significant. Second, the importance of correcting commissural abnormalities separately may be important, especially with infracristal defects." Third, perhaps most important is the use of a reliable operative method of evaluating effectiveness of valvuloplasty . If repair is done completely through the aorta, this simply cannot be done. A fourth suggestion, again hypothetical, is that the unfavorable experience published by several groups between 1961 and 1966
generated pessimism about the possibility of repair of these abnormal cusps and discouraged further investigation. In this regard, there undoubtedly are a few patients in whom valve deformities are so extensive that no method of valvuloplasty can be successful and prosthetic replacement is required . However, this must represent only a small percentage of the total group of patients seen. Finally, there has been unwarranted pessimism about the durability of an initially successful valvuloplasty. Recurrences evident within a few days of operation may be due to inadequate operative correction or dehiscence of an initially effective plication. In at least 2 patients in this report there was considerably more aortic insufficiency within 3 months after operation than was present at the time of leaving the hospital. However, this period of the first few months after operation represents the only time in which increasing insufficiency has occurred. Beyond this point, residual insufficiency has remained stable. With the period of postoperative observation described, as long as 10 to 12 years, it seems clear that progressive insufficiency is unusual. Admittedly, continued fibrosis in an area of turbulent blood flow could produce further contraction of an abnormal cusp, but this must be rare. In Table IB are shown 4 patients with Grade 1 of 6 diastolic murmurs who have been observed for periods between 5 and 12 years with no change whatever.
Summary I. Combined experiences from three separate universities, involving 18 patients who were treated by valvuloplasty for aortic insufficiency associated with a ventricular septal defect, are described in this report. In addition, recent evaluation of 2 patients previously reported upon in 1962 is included, making a total of 20 patients. 2. The only operative death in this series followed replacement of the abnormal aortic cusp with fascia lata , resulting in recurrent progressi ve insufficiency within a short time. All of the 19 patients are in significantly impro ved condition . Significant aortic in-
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sufficiency remains in 7 of these, but improvement is shown by a decrease in heart size, absence of symptoms, and little limitation of physical activity. 3. In all patients, the abnormal aortic cusps were first repaired through an aortotomy, and then the ventricular septal defect was closed through a right ventriculotomy. Postponing closure of the ventricular septal defect until the aortotomy had been sutured permitted the surgeon to evaluate the effectiveness of valvuloplasty by inspecting the aortic valve through the ventricular septal defect. This was useful, for residual insufficiency was found in some patients and was corrected by reopening the aorta and performing further valvuloplasty. 4. Methods of valvuloplasty varied but essentially included shortening of the elongated right coronary cusp, either by plication or by longitudinal suture of the free edge of the cusp (Figs. 1 and 2). Closure of the ventricular septal defect also varied but was usually done with a prosthetic patch (Fig. 3). 5. A particularly significant point was the absence of progression of aortic insufficiency beyond the first few months after operation. As periods of observation in 12 of the patients are now between 1 and 12 years, the likelihood of increasing insufficiency in those with slight diastolic murmurs seems remote. 6. The fact that aortic valve replacement with a prosthesis was not necessary in any of the 20 patients indicates that most patients may be treated by different forms of valvuloplasty. Some patients remain with significant regurgitation, however, indicating that further study is needed. REFERENCES Garamella, J. J., Cruz, A. B., Jr., Heupel, W. H., Dahl, J. C., Jensen, N. K., and Berman, R.: Ventricular Septal Defect With Aortic Insufficiency: Successful Surgical Correction of Both Defects by the Transaortic Approach, Am. J. Cardiol. 5: 266, 1960. 2 Starr, A, Menashe, V., and Dotter, D.: Surgical Correction of Aortic Insufficiency Associated With Ventricular Septal Defect, Surg. Gynecol. Obstet. 111: 71, 1960. 3 Spencer, F. C., Bahnson, H. T., and Neill,
4
5
6
7
8 9
10
11
12
13
14
C. A: The Treatment of Aortic Regurgitation Associated With a Ventricular Septal Defect, J. THoRAc. CARDIOVASC. SURG. 43: 222, 1962. Ellis, F. H., Jr., Ongley, P. A., and Kirklin, J. W.: Ventricular Septal Defect With Aortic Valvular Incompetence: Surgical Considerations, Circulation 27: 789, 1963. Plauth, W. H., Jr., Braunwald, E., Rockoff, S. D., Mason, D. T., and Morrow, A. G.: Ventricular Septal Defect and Aortic Regurgitation: Clinical, Hemodynamic and Surgical Considerations, Am. J. Med. 39: 552, 1965. Gonzalez-Lavin, L., and Barratt-Boyes, B. G.: Surgical Considerations in the Treatment of Ventricular Septal Defect Associated With Aortic Valvular Incompetence, 1. THORAC. CARDIOVASC. SURG. 57: 422, 1969. Somerville, J., Brandao, A., and Ross, D. N.: Aortic Regurgitation With Ventricular Septal Defect: Surgical Management and Clinical Features, Circulation 41: 317, 1970. Frater, R. W. M.: The Prolapsing Aortic Cusp: Experimental and Clinical Observations, Ann. Thorac. Surg. 3: 63, 1967. Hallidie-Smith, K. A, Olsen, E. G. J., Oakley, C. M., Goodwin, J. F., and Cleland, W. P.: Ventricular Septal Defect and Aortic Regurgitation, Thorax 24: 257, 1969. Treasure, R. L., Hopeman, A. R., Jahnke, E. J., Green, D. C., and Czarnecki, S. W.: Ventricular Septal Defect With Aortic Insufficiency, Ann. Thorac. Surg. 12: 411, 1971. Van Praagh, R., and McNamara, J. J.: Anatomic Types of Ventricular Septal Defect With Aortic Insufficiency, Am. Heart J. 75: 604, 1968. Scott, R. C., McGuire, J., Kaplan, S., Fowler, N. 0., Green, R. S., Gordon, L. Z., Shabetai, R., and Davolos, D. D.: The Syndrome of Ventricular Septal Defect With Aortic Insufficiency, Am. J. Cardiol. 2: 530, 1958. Nadas, AS., Thilenius, O. G., LaFarge, C. G., and Hauck, A J.: Ventricular Septal Defect With Aortic Regurgitation: Medical and Pathologic Aspects, Circulation 29: 862, 1964. Keck, E. W.O., Ongley, P. A, Kincaid, O. W., and Swan, H. J. C.: Ventricular Septal Defect With Aortic Insufficiency: Clinical and Hemodynamic Study of 18 Proved Cases, Circulation 27: 203, 1963.
Discussion DR. DAVID A. MURPHY Montreal, Quebec, Canada
As the abstract states, the techniques of valvuloplasty will vary as to the type of pathology you find. We were very familiar with Dr. Spencer and Dr. Bahnson's earlier published works on this subject. We recently had a case in Montreal in which,
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before surgery, we thought we were going to plicate the aortic valve, as described so nicely here. However, when we saw the valve, we found, as Van Praagh described, a commissure that was lower than usual, counterclockwise rotation of the aortic root, and noncoronary and right coronary cusps that reminded me of my father's old wool bathing suit as he got out of the water. They were sagging down into the lumen of the left ventricle. We were unable to do the plication as Dr. Spencer has suggested, since the area of the valve adjacent to the affected commissure was very thin. Since the child was only 6 years old, we really didn't think the stitches would hold. Therefore, I extend the aortotomy down between the affected commissure carefully. The redundant free margins of the affected leaflets were then brought upward and through the split in the commissure outside the aortic lumen. They were held fast in their new position by horizontal mattress stitches which were used to close the split commissure and aortotomy. The everted edge and excess valve tissue on the outside of the aorta were then closed with a continuous stitch. It is a little premature to say how successful this has been. However, at postoperative catheterization, there was only a whiff of regurgitation. The pulse pressure has returned to normal, and the patient has no diastolic murmur. This method is offered as an alternative treatment for this variable lesion. DR. CLARENCES. WELDON St. LOllis, Mo.
I apologize for discussing a paper of which I am an author, but there are several points about this malformation which I think are important. This malformation is unusual in that there is aortic insufficiency in conjunction with an excess of aortic valve tissue. I think the aortic insufficiency occurs because the aortic leaflet has lost its support. In the infracristal variety, the VSD appears to be a separation of the aorta from the heart in the region of the right cusp of the aortic valve. It is not a simple defect in the membranous portion of the interventricular septum. The annulus, therefore, loses its support. Secondarily, as the valve herniates through the defect, the commissural support is lost because the free margin of the valve elongates. The surgeon must repair both of these support mechanisms. It is very important to repair the defect with a patch because there is a danger of pulling the valve down to the septum and making the aortic valve insufficiency worse. We use a cloth patch which provides some rigidity in the area to support the annulus of the aortic valve. The commissural support can be reestablished, as was illustrated, simply by adjusting the length of the free margins so that they equal the coapting free edges of adjacent leaflets.
Our results in the infracristal repair were all very good. The supracristal variety, in which there is a very large VSD, is quite a different thing. When we tried to apply the same operation to this condition (although we could easily adjust the free margins of the cusp) we were unable to restore the support of the annulus by placing a patch in the very large supracristal defect. Our result is not perfect in the case of supracristal defect. I think one should have some reservations about cutting out this good valve tissue in a child and replacing it with a prosthesis or with some tissue which is likely to be less functional for a long period of time than the normal aortic valve tissue. DR. LESTER R. BRYANT Lexington, Ky.
I would like to give an indirect endorsement of the plea for aortic valvuloplasty by Dr. Spencer and his associates. Our experience with 3 patients who had this combination of congenital defects includes 2 adults, in contrast to the majority of patients who appear to be in the pediatric age group. All 3 of our patients were managed by valve replacement, and it seems in retrospect that this may have been a somewhat misguided approach. Two patients have developed serious paravalvular leaks, 1 of whom was reoperated upon just 2 months ago. The opportunity for reoperation allowed us to observe what is probably another anatomic feature that is found in at least some patients with this defect. This feature was probably responsible for the paravalvular leak in our 2 patients. I refer to the very deep insertion of the aortic cusps in this anomaly. In the patient whom we reoperated upon, the vertical distance of the cusp attachments to the aortic wall had been nearly twice that seen in normal patients. In placing the valve sutures originally, we had followed the attachment of the valve cusps up to the commissures, so that the vertical depth of the sutures was too great for accomodation by the single plane of the valve suture ring. As the sutures were tied down, undue tension was placed on the aortic wall, causing early paravalvular leak. I would point out that if one is required to do a valve replacement in this condition, he should take into account the abnormal insertion of the cusps and use adequate buttressing to bridge the defect between the commissures. DR. ROBERT W. M. FRATER Bronx, New York
I would like to add my support to Dr. Spencer's contention by telling about the 3 patients whom I treated between 1965 and 1966. They now have been followed for 5 or more years.
Volume 65 Number 1 January, 1973
As Dr. Weldon warned, the 1 patient who had a supracristal VSD does have some residual insufficiency. However, that insufficiency has remained constant over the 5 years from a period of a few months after operation, when it reached its maximum extent. These 3 patients are all continuing to do well and all confirm the worth of this procedure. DR. FRANK C. SPENCER (Closing) I appreciate the comments of the different discussers. With the limits of time, I will not comment specifically on their remarks but rather will make two or three general observations. This is an uncommon lesion. We see only about 1 such patient a year; hence, it is difficult for most groups to acquire significant experience. With the widespread pessimism about the likelihood of correction without valve replacement, cardiologists have become increasingly reluctant to refer patients for operation, so that operative experience with valvuloplasty has remained small. For some time I felt our group had seen a group of patients who were in unusually favorable
Aortic valvuloplasty
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condition for surgical correction. However, with the pooling of data from three different institutions, representing 20 patients from widely separated geographical areas, it is very unlikely that a chance variation exists. Almost clearly, the data are truly representative. Hence, our most significant conclusion is the concept that effective valvuloplasty can be done in the majority of patients by one of a variety of techniques. With this concept, operation is indicated at an early stage of the disease, soon after insufficiency is recognized, to prevent progressive enlargement of the left ventricle and increasing deformity of the right cusp. At present, if valve replacement is considered likely, the opposite approach is taken. Operation is deferred as long as possible, resulting in massive enlargement of the left ventricle and extensive deformity of the aortic valve. Consequently, a valvuloplasty is much more difficult. Hopefully, with the data presented here today, a much more favorable view of early valvuloplasty will develop.