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Ventricular septal defect and aortic insufficiency
Ventricular septal defect and aortic insufficiency Surgical treatment Twenty-five patients with ventricular septal defect (VSD) associated with aortic insufficiency (AI) have been seen since 1964. Of these patients, one died suddenly without operation; in 2 patients, AI developed in the late postoperative period following VSD closure; and in 3 others, AI developed shortly after VSD closure. The remaining 19 patients are discussed in detail. The VSD was subpulmonic in 13 (68 per cent) and subcristal in 6 (32 per cent). Primary suture of the VSD was undertaken in 13 patients and patch closure in 6. Seven patients had aortic valvuloplasty and 2 had aortic valve replacement. There were no surgical deaths, and the long-term follow-up shows that VSD closure alone has been sufficient to arrest progression of AI in patients with mild insufficiency, particularly in those with subpulmonic VSD. Valvuloplasty, when necessary, was more effective when done at an early age.
Ricardo J. Moreno-Cabral, M.D., Richard T. Mamiya, M.D., Frances F. Nakamura, M.D., Scott C. Brainard, M . D . , and J. Judson McNamara, M . D . , Honolulu, Hawaii
V_^ controversy exists regarding the optimal surgical treatment and timing of operation for patients with ventricular septal defect (VSD) associated with aortic insufficiency (AI). AI is reported to occur in less than five per cent of all patients with VSD in Western countries. 1 An incidence of over eight per cent is reported in Japan, probably because of an increased number of patients with subpulmonic VSD. 2 The present series represents an incidence of over seven per cent. Early operation with closure of the VSD has obviated valve replacement and has been associated with no deaths. Valvuloplasty has been more successful in the younger age group. The present report summarizes our clinical experience with this entity and discusses optimal therapy on the basis of the current understanding of the disease. Clinical material Twenty-five patients with documented AI and VSD have been seen at Queen's Medical Center from 1964 From the Department of Surgery, University of Hawaii School of Medicine and Queen's Medical Center, Honolulu, Hawaii 96813. Read at the Second Annual Meeting of The Samson Thoracic Surgical Society, Banff, Alberta, Canada, June 1-4, 1976. 358
Table I. Race of patient and type of VSD Subpulmonic
Subcristal
Oriental Polynesian Caucasian
8 4 1
5 1
Totals
13 (68%)
6 (32%)
to present. They represent 7 per cent of all patients with VSD. Of the group of 25 patients, one asymptomatic 44year-old subject died suddenly at home without operation; in 2 patients, AI developed one and 3 years following VSD closure; and 3 others, AI developed in the immediate postoperative period following VSD closure. On re-exploration in 2 of these 3 patients, 3 mm. perforations of the right and noncoronary cusps were repaired, with good results. The third patient has not required re-exploration. She had had a mid-diastolic rumble preoperatively, and a subpulmonic VSD was patched. Postoperatively, a Grade 2/6 to-and-fro murmur developed, but her aortic insufficiency is considered mild and no reoperation has been considered necessary. The remaining 19 patients in whom VSD was asso-
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VSD and aortic insufficiency
359
Fig. 1. ciated with spontaneous onset of AI and in whom surgical correction was undertaken are discussed in detail. The ages ranged from 3 to 31 years (mean 10 years); 17 were males and 2 females. Thirteen patients were Oriental or part Oriental, 2 were Polynesian, one was Caucasian, and 3 were Polynesian-Caucasian (Table I). The degree of AI was considered mild in 10 patients, moderate in 4, and severe in 5. The degree of preoperative and postoperative AI was judged clinically, usually by the same physician, on the basis of the length of
diastolic murmur, the hyperactivity of the left ventricular impulse, the pulse pressure, the electrocardiograms and roentgenograms, and, when available, angiograms with injections of dye into the aortic root. The calculated pulmonary-to-systemic flow ratios varied from minimal, detectable only by H+ curves, to 2.3:1, with a mean of 1.5:1. The VSD murmur was heard at birth in most patients, and the AI murmur was noted between 6 months and 14 years of age.
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Moreno-Cabral et al.
Table II VSD Case No. Sex 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 Mean
M M M M M M M M F M M M M M M M M M F
Age (yr.) 3 3 17 4 29 3 18 17 4 5 8 7 5 7 9 10 31 9 3
Type
Size (mm.)
Preop. BP (mm. Hg)
SP SP SCr SCr SP SP SP SP SCr SP SP SCr SP SP SCr SP SP SP SCr
15 10 NR 8 10 15 23 13 12 10 8 1 10 10 Small 15 12 10 10
120/20 100/40 90/0 100/50 126/90 100/30 120/74 95/30 100/60 110/40 112/22 102/48 118/70 90/40 106/70 140/40 110/50 100/50 100/50
10.5
Preop. degree ofAl
Qp/Qs 1.2 2:1 1.2 2:1 1.8 2:1 1.2 2.3 2:1 1.5 H+ 1.5 1.3 1.2 2.2 1.5 1.2 1.6
1 1 1 1 1 1 detected 1 1 1 1 1 1 1
Severe Moderate Moderate Mild Mild Severe Mild Severe Mild Mild Severe Moderate Mild Mild Mild Severe Moderate Moderate Mild
VSD procedure Suture Suture Suture Suture Suture Suture Suture Suture Patch Suture Suture Suture Patch Patch Suture Patch Patch Patch Suture
Latest postop.
Aortic valve procedure
Late Post op. AI
BP
ECG
CXR
None Valvuloplasty Valvuloplasty None Valvuloplasty Valvuloplasty Valvuloplasty AVR None None None None None Valvuloplasty None Valvuloplasty AVR Valvuloplasty None
Minimal Absent Mild Mild Mild Mild Absent Absent Mild Mild Moderate Severe Mild Minimal Minimal Absent Absent Moderate Mild
148/80 86/40 100/60 106/60 112/72 118/78 120/80 110/70 100/60 100/60 130/60 100/25 110/80 90/60 100/60 125/65 112/48 120/70 90/58
Normal Normal Normal Normal LVH LVH Normal LVH LVH Normal LVH BVH Normal LVH Normal Normal LVH LVH Normal
Normal Normal Normal Normal Normal LVH Normal Normal Normal Normal LVH LVH LVH LVH Normal Normal BVH BVH Normal
1.5 1
Legend: VSD, Ventricular septal defect. BP, Blood pressure. Qp/Qs, Pulmonary-to-systemic flow ratio. AI, Aortic insufficiency. ECG, Electrocardiogram. CXR, Chest x-ray. SP, Subpulmonic. SCr, Subcristal. AVR, Aortic valve replacement. LVH, Left ventricular hypertrophy. BVH, Biventricular hypertrophy. NR, Not recorded.
Follow-up was from 2 to 12 years (mean 6). An electrocardiogram, chest roentgenogram, and clinical re-evaluation by the same cardiologist were available in all patients (Table II). Operative findings The VSD was subpulmonic in 13 patients (68 per cent) and subcristal in 6 (32 per cent). The aortic valve was bicuspid in one patient. One other patient had a ruptured aneurysm of the sinus of Valsalva. The right or noncoronary cusps prolapsed through the VSD in 11 patients. One patient had a persistent left superior vena cava. Two patients had anatomic stenosis of the right ventricular outflow tract, and in 3 others a gradient, demonstrated preoperatively, was probably due to a prolapsed aortic cusp. Surgical procedures Operations were performed with cardiopulmonary bypass, moderate hypothermia (30 to 32° C ) . cardiac arrest with topical hypothermia, no coronary perfusion, and left ventricular venting. A right atriotomy was sometimes done first to rule out the presence of an atrial septal defect. A right transverse ventriculotomy was done next to expose the VSD and inspect the degree of
AI. The aortic valve was exposed through a low oblique aortotomy. Aortotomy was not used in 6 patients in whom valvuloplasty or A VR was deemed unnecessary. In these cases, the degree of residual AI was estimated by the amount of drainage from the left ventricular vent and by the diastolic pressure following VSD closure. Valvuloplasty was done in 7 patients, valve replacement in 2, and VSD closure alone in 10. Valvuloplasty is undertaken prior to VSD closure so that the degree of residual AI can be assessed at the time of VSD repair. The method of valvuloplasty most frequently used was the one described by Frater.3 A variation of this method that has been effective in our hands is shown in Fig. 1, a and b. This consists of plicating the redundant segment and suturing it through the aortic wall with mattress sutures; the imbricated segment is then sutured to its adjacent commissure with a figure-of-eight 5-0 silk suture. The 2 patients undergoing valve replacement with Starr-Edwards prostheses were in the older age group. A 31-year-old man (Case 17) with cardiac cirrhosis had a markedly elongated and prolapsed noncoronary cusp which was used to patch the VSD. In another patient (Case 8), AI was diagnosed at the age of 14 years. When the boy was 17 years old, subacute bacterial
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Follow-up (yr.) 12 9 9 9 9 8 8 7 6 6 5 5 4 4 4 3 3 2 2
VSD and aortic insufficiency
Comments 10 mm. pulmonary gradient 10 mm. pulmonary gradient
Ruptured sinus of Valsalva aneurysm
7 mm. pulmonary gradient; residual VSD Possible AVR Plan AVR; abnormal commissure
Bicuspid aortic valve VSD patched with noncoronary cusp 20 mm. pulmonary gradient; tetralogy of Fallot
6
endocarditis with acute congestive heart failure developed secondary to destruction of the aortic valve. Valve replacement and VSD closure were performed following a month of intensive medical therapy. The VSD was repaired through a right ventriculotomy in all cases; the incision was made high in the outflow tract when a subpulmonic VSD was suspected. Primary suture was used in 13 patients and a Dacron patch in 5. The prolapsing cusp was used for VSD closure in one patient as described by GonzalezLavin and Barratt-Boyes.4 A teardrop-shaped patch has proved most successful for closure of subpulmonic VSD (Fig. 2). Results VSD closure alone (9 patients) (Fig. 3). The VSD was closed by suture in 7 patients and by patch in 2. The AI improved in 3 and has remained stable in 6. One patient (Case 11) had severe AI for which he was treated by VSD suture alone. Postoperatively, the AI became moderate and has remained stable for 5 years, with a blood pressure of 130/60 mm. Hg, cardiomegaly, and a Grade 2/6 diastolic murmur. The patient eventually may require aortic valve replacement. Another boy (Case 12) had a 1 mm. VSD. The anterior commissure appeared lower than the others; valvulo-
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plasty was attempted but, because regurgitation was made worse, the valve was not repaired. He now has a blood pressure of 100/25 mm. Hg, cardiomegaly, and a Grade 3/6 diastolic murmur. Although he is asymptomatic 5 years postoperatively, he is considered a candidate for aortic valve replacement. Patient 1 had severe AI owing to prolapse of an aortic cusp into the VSD, which was corrected by VSD suture at the age of 3 years. Twelve years after the operation, the blood pressure is 140/68 mm. Hg, there is a Grade 1 protodiastolic whiff, and the chest x-ray film and electrocardiogram are normal. The long-term result in this case is excellent. Valvuloplasty (8 patients) (Fig. 4). AI was lessened in 6 patients and has remained stable in 2. The follow-up is 9 years in 3 patients, 8 years in one patient, and 4, 3, and 2 years in the remaining 3 patients. Aortic valve replacement (2 patients). Both patients are taking anticoagulants and have remained free of complications for 7 and 5 years postoperatively. There were no hospital or late deaths. Discussion Since the first successful operation for VSD and AI reported by Garamella5 in 1960, only 204 cases involving surgical treatment have been described in some detail in the English literature. The 19 cases reported here make a total of 223. This is in contrast to the high incidence of VSD and AI in Japan, where a total of 380 surgical cases had been reported through April of 1974. The increased frequency of AI associated with VSD correlates closely with a higher incidence of subpulmonic VSD, which occurred in 80 per cent of the cases of this syndrome reported by Sakakibara.6 The incidence of subpulmonic VSD in our series was 68 per cent. There is no unanimity of opinion in the literature regarding the optimal treatment and timing of operation for patients with VSD and AI.1' 7_12 One extreme is represented by Nadas,1 Somerville,7 and their colleagues, who recommend surgery only for those patients with very severe aortic regurgitation. Most authors now disagree with this view, feeling that surgery shortly after the onset of significant AI is imperative to preserve the integrity of the aortic valve.10' 12~17 Although the pathogenesis of AI in these patients is not well understood, it has been logically proposed that AI results from loss of subvalvular supporting tissues, which allows the cusp to prolapse as a result of the pressure gradient between the left ventricle and right ventricle.18 This theory is strengthened by the noted higher incidence of AI in subpulmonic VSD, in which
The Journal of Thoracic and Cardiovascular Surgery
3 6 2 Moreno-Cabral et al.
Fig. 2. FOLLOW DEGREE
OF
A . I. PREOPERATIVE
POSTOPERATIVE
% #^^_
SEVERE
2
3 4
^ ^ ^ ^ ^ ^ "
* n
i in
UP 6
7
(YEARS) 8
9
10 II 12
/
MODERATE
d
5
^HHW
i i
MILD
MINIMAL
Fig. 3. Results of VSD closure alone. the loss of subaortic fibromuscular tissue is greater than with the usually positioned subcristal VSD. From this it follows that VSD closure alone may correct, halt, or delay the progression of AI. Furthermore, if some valvular deformity has occurred, satisfactory correction with valvuloplasty is far more likely than when AI has become severe owing to extensive leaflet distortion. In one case in this series of subcristal VSD (Case 12), the commissure between the left and right cusps appeared to be situated at a lower level and valvuloplasty could not be performed; thus a defective commissure may
produce AI without cusp prolapse into the VSD. An abnormal commissure could also explain the late onset of AI one and 3 years following VSD closure in 2 other patients. This malposition of an aortic commissure has been previously suggested as an explanation for AI, particularly in patients with subcristal VSD.18 It is clear from the literature that the older the age group, the greater the need for aortic valve replacement and the higher the surgical mortality rate. Excellent results have been reported by Trusler17 with valvuloplasty, and it should be noted that all of his patients were
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VSD and aortic insufficiency
DEGREE OF A . I . PREOPERATIVE
SEVERE
6 16
MODERATE
18 3 2
MILD
5 14 7
POSTOPERATIVE
363
FOLLOW UP (YEARS) I 2 3 4 5 6 7 8 9
MINIMAL ABSENT
Fig. 4. Results of valvuloplasty. under 12 years of age. Accumulated experience seems to indicate a direct correlation between younger age and successful valvuloplasty. VSD closure alone may completely relieve AI, especially in the younger age group, as reported by Chung, 15 Dimich, 14 and others. 13, i6, i9, 20 j h e fact m a t A ] h a s b e e n lessened or has remained stable in 9 of our patients treated by VSD closure alone supports the view that this treatment may be all that is needed in many patients, especially in those with subpulmonic VSD. VSD closures by suture or patch were equally effective in this series in correcting or arresting the development of AI. We agree with Blumenthal and associates 21 that an effort should be made to identify the subpulmonic VSD and outline the abnormal aortic valve by routine use of left ventriculograms and aortic root angiograms, and we recommend VSD closure as soon as the diagnosis of AI is made. Furthermore, we believe VSD closure should be done when a subpulmonic VSD is diagnosed. VSD closure alone may be sufficient for many patients. If valvuloplasty is necessary, it too should be performed early. Further delay may result in irreparable aortic valve destruction, and aortic valve replacement will become necessary. Addendum Since the presentation of this paper, we have operated upon 2 additional patients with VSD and AI. A 5-year-old boy with a subcristal VSD and mild AI underwent patch closure of the VSD. A 4-year-old boy with a subpulmonic VSD and severe AI underwent aortic valvuloplasty and patch closure of the VSD. Another of our patients (Case 12) underwent aortic valve replacement with a No. 19 Hancock valve. The immediate results have been satisfactory in all these patients.
We are indebted to Jane Muramoto, Susie Anderson, and Alejandro Moreno for their assistance in preparing the manuscript.
1
2 3 4
REFERENCES Nadas, S. A., and Fyler, C. D.: Ventricular Septal Defect With Aortic Regurgitation, in Nadas, S. A., editor: Pediatric Cardiology, Philadelphia, 1972, W. B. Saunders Co., p. 379. Tatsuno, K., Konno, S., and Sakakibara, S.: Ventricular Septal Defect With Aortic Insufficiency, Am. Heart J. 85: 13, 1973. Frater, R. W. M.: The Prolapsing Aortic Cusp: Experimental and Clinical Observations, Ann. Thorac. Surg. 3: 63, 1967. Gonzalez-Lavin, L., and Barratt-Boyes, B. G.: Surgical Considerations in the Treatment of Ventricular Septal Defect Associated With Aortic Valvular Incompetence, J. THORAC. CARDIOVASC. SURG. 57: 422, 1969.
5 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 Transaortic Approach, Am. J. Cardiol. 5: 266, 1960. 6 Sakakibara, S.: Experiences With Congenital Anomalies of the Heart in Japan, J. THORAC. CARDIOVASC. SURG.
68: 189, 1974. 7 Somerville, J., Brandao, A., and Ross, D. N.: Aortic Regurgitation With Ventricular Septal Defect: Surgical Management and Clinical Features, Circulation 41: 317, 1970. 8 Ellis, F. H., Jr., Ongley, P. A., and Kirklin, J. W.: Ventricular Septal Defect With Aortic Valvular Incompetence: Surgical Considerations, Circulation 27: 789, 1963. 9 Sanfelippo, P. M., DuShane, J. W., McGoon, D. C , and Danielson, G. K.: Ventricular Septal Defect and Aortic
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Moreno-Cabral
Insufficiency: Surgical Considerations and Results of Operation, Ann. Thorac. Surg. 17: 213, 1974. 10 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. 11 Spencer, F. C , Doyle, E. F., Danilowicz, D. A., Bahnson, H. T., and Weldon, C. S.: Long-Term Evaluation of Aortic Valvuloplasty for Aortic Insufficiency and Ventricular Septal Defect, J. THORAC. CARDIOVASC. SURG. 65: 15,
1973.
12 Kawashima, Y., Danno, M., Shimizu, Y., Matsuda, H., Miyamoto, T., Fujita, T., Kosuka, T., and Manabe, H.: Ventricular Septal Defect and Aortic Insufficiency: Anatomic Classification and Method of Operation, Circulation 47: 1057, 1973. 13 Glasser, S. P., Cheitlin, M.D., McCarty, R. J., Haas, J. H., Hall, R. H., and Mullins, CH. E.: Thirty-two Cases of Interventricular Septal Defect and Aortic Insufficiency: Clinical Hemodynamic and Surgical Features, Am. J. Med. 53: 473, 1972. 14 Dimich, I., Steinfeld, L., Litwak, R. S., Park, S., and Silver, N.: Subpulmonic Ventricular Septal Defect Associated With Aortic Insufficiency, Am. J. Cardiol. 32: 325, 1973. 15 Chung, K. J., and Manning, J. A.: Ventricular Septal Defect Associated With Aortic Insufficiency: Medical and Surgical Management, Am. Heart J. 87: 435, 1974. 16 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. 17 Trusler, G. A., Moes, C. A. F., and Kidd, B. S. L.: Repair of Ventricular Septal Defect With Aortic Insufficiency, J. THORAC. CARDIOVASC. SURG. 66: 394, 1973.
18 Van Praagh, R., and McNamara, J. J.: Anatomic Types of Ventricular Septal Defect With Aortic Insufficiency, Am. Heart J. 75: 604, 1968. 19 Robinson, G., Fell, S. C , and Jacobson, B. E.: Ventricular Septal Defect With Aortic Insufficiency: A Method of Management, J. THORAC. CARDIOVASC. SURG. 43: 785,
The Journal of Thoracic and Cardiovascular
et al.
1962.
20 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. 21 Blumenthal, S., Kangal, J. K., Bauman, F. O., et al.: Ventricular Septal Defects With Prolapse of the Aortic Cusp (Abstr.), Circulation 28: 691, 1963.
Discussion DR. R O B E R T M. A N D E R S O N Tucson, Ariz.
I am envious of the essayists' opportunity to observe a fairly large series of cases that are rare to the rest of us. It is easy for me to agree with their hypothesis that early repair of the ventricular septal defect is prophylactic against
Surgery
progressive development of aortic insufficiency. Any ventricular septal defect that extends into and involves the support of the sinus of Valsalva allows some prolapse. The result is progressive acquired stretching of the valve's leading edge, with the development of shallower and shallower valve cusps, and, consequently, progressive aortic insufficiency. Late repair of the ventricular septal defect does not restore the normal length of the valve's leading edge. When aortic insufficiency is significant, valvuloplasty rather than aortic valve replacement is frequently desirable because of the age and small size of the patients. We have found that plication by the method described by the essayists is not an effective way in which to repair the aortic insufficiency. The stretching of the leading edge of the aortic valve fans in such a way that a tuck in one sector leaves prolapse in other areas away from the tuck. In Annals of Surgery in 1961, Kay and I reported a method of valve suspension that makes the valve totally sufficient. I want to report a 17 year follow-up of that means of valve suspension. A cable of one strand of polyester and one strand of silk strung from one commissure to the other, with the valve leaflet edge reefed to such a cable, has restored competency to the present time. The objective of this method of valvuloplasty is to place a cable between the two commissures of the prolapsed valve, stretch the cables over bolsters external to the aorta, and tighten the cable until it is the exact diameter of the aorta. The leading edge of the valve is then attached to the cable. When the valve edge is restored to the diameter of the aorta, the leaflet properly apposes the other two valve cusps. There is some concern in the literature over abnormal position of commissures contributing to aortic insufficiency. Any photographer who has a tripod knows that it takes three points to determine a plane. It is the length of the leading edge of the valve and the shallowness of the cusp that determine whether a valve is sufficient, not the position of the commissures. To squeeze a bit more information out of the essayists' experience, I would like to ask two questions: Have they been able to correlate the degree of insufficiency with measurements of the length of the leading edge and depth of the cusps? What criterion have they used to determine whether to accept the amount of insufficiency and not interfere with it, and what is their indication for valvuloplasty and valve replacement? DR. J O H N G. J A C O B S O N Loma Linda, Calif.
Our experience is not as extensive as that of Dr. MorenoCabral, but in the past 2 years we have treated 5 patients. Dr. Vyhmeister has provided this material for me to share with you. The first patient was treated at Loma Linda University and the subsequent 4 were treated during a trip to South Vietnam. Two patients had supracristal ventricular septal defects, and the others had infracristal defects. One patient had perforation of the right coronary cusp and another had perforation of the
Volume 73 Number 3 March, 1977
right sinus of Valsalva and of the pulmonary valve. Two patients had infundibular pulmonary stenosis. [Slide] This slide reinforces the associated anatomic and pathological problems: Infracristal ventricular septal defects, infundibular stenosis, and supracristal defects. Prolapsing of the aortic cusp is common in Oriental patients. Dr. George Trusler has recently expanded upon the earlier method described by Frater. One of the important points that Dr. Trusler makes is that the plication needs to be reinforced with pericardial, Dacron, or Teflon bolsters in order to prevent the plication from being dissected away from the aortic wall. Earlier attempts at valvuloplasty were associated with a high incidence of breakdown of the repair. Dr. Trusler feels that the pledget should extend beyond the plication, up on to the aortic wall, and in addition another pledget should be placed across the commissure to try to prevent breakdown of the valvuloplasty. In one of our patients with a supracristal ventricular septal defect, the defect was repaired with a patch after the valve had been plicated. Another patient had a perforation of the right coronary cusp. The valve was plicated and the perforation closed, followed by a patch closure of the ventricular septal defect. In a patient who had a small ventricular septal defect, the defect was closed by direct suture over Teflon felt pledgets. We believe that an attempt should be made to resuspend and restore competence to the valve prior to repairing the ventricular septal defect, as was mentioned in MorenoCabral's paper, so that the adequacy of this repair can be assessed when the ventricular septal defect is subsequently repaired. I would like to ask Dr. Moreno-Cabral two questions: Is an attempt now being made to repair all of the incompetent valves, and do the 10 patients in whom no repair was attempted reflect your earlier experience with this problem?
VSD and aortic insufficiency
3 65
D R . M O R E N O - C A B R A L (Closing) Dr. Anderson, we have not done any studies to try to correlate the degree of elongation of the leading edge of the aortic cusps with the degree of incompetence nor with the depth of the aortic commissures, so we do not have an answer to your first question. The amount of residual insufficiency was estimated by the amount of drainage from the left ventricular vent during bypass and by the diastolic pressure after bypass. Regarding the second question, relating to indications for valvuloplasty or aortic valve replacement, we believe that in mild aortic insufficiency, especially if the VSD is subpulmonic, a VSD closure alone may be sufficient. In patients who have moderate or severe incompetence, valvuloplasty is done first; aortic valve replacement is left for those patients with severe aortic insufficiency in whom the VSD has been closed and valvuloplasty has been ineffective. We prefer to wait until these children reach an older age. The child whom we have scheduled for aortic valve replacement is now 12 years of age. In reply to Dr. Jacobson's question, we do not repair all incompetent valves. As mentioned in the manuscript, we employ valvuloplasty in most patients who have moderate or severe aortic insufficiency. I may add that some patients, as mentioned by Dr. Anderson, have abnormal commissures. This was the case in one of our patients in whom the valve incompetence was worse following the plication procedure and the suture had to be removed. This is the patient I mentioned before, who is scheduled for aortic valve replacement. Plication may not be feasible in these instances. Patients with residual aortic insufficiency are usually asymptomatic, and heart failure may not develop for several years. Our cardiologists usually just restrict the physical activity of these patients, and we wait until they grow before replacing the aortic valve.
Ricardo J. Moreno-Cabral, M.D., Richard T. Mamiya, M.D., Frances F. Nakamura, M.D., Scott C. Brainard, M . D . , and J. Judson McNamara, M . D . , Honolulu, Hawaii
V_^ controversy exists regarding the optimal surgical treatment and timing of operation for patients with ventricular septal defect (VSD) associated with aortic insufficiency (AI). AI is reported to occur in less than five per cent of all patients with VSD in Western countries. 1 An incidence of over eight per cent is reported in Japan, probably because of an increased number of patients with subpulmonic VSD. 2 The present series represents an incidence of over seven per cent. Early operation with closure of the VSD has obviated valve replacement and has been associated with no deaths. Valvuloplasty has been more successful in the younger age group. The present report summarizes our clinical experience with this entity and discusses optimal therapy on the basis of the current understanding of the disease. Clinical material Twenty-five patients with documented AI and VSD have been seen at Queen's Medical Center from 1964 From the Department of Surgery, University of Hawaii School of Medicine and Queen's Medical Center, Honolulu, Hawaii 96813. Read at the Second Annual Meeting of The Samson Thoracic Surgical Society, Banff, Alberta, Canada, June 1-4, 1976. 358
Table I. Race of patient and type of VSD Subpulmonic
Subcristal
Oriental Polynesian Caucasian
8 4 1
5 1
Totals
13 (68%)
6 (32%)
to present. They represent 7 per cent of all patients with VSD. Of the group of 25 patients, one asymptomatic 44year-old subject died suddenly at home without operation; in 2 patients, AI developed one and 3 years following VSD closure; and 3 others, AI developed in the immediate postoperative period following VSD closure. On re-exploration in 2 of these 3 patients, 3 mm. perforations of the right and noncoronary cusps were repaired, with good results. The third patient has not required re-exploration. She had had a mid-diastolic rumble preoperatively, and a subpulmonic VSD was patched. Postoperatively, a Grade 2/6 to-and-fro murmur developed, but her aortic insufficiency is considered mild and no reoperation has been considered necessary. The remaining 19 patients in whom VSD was asso-
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VSD and aortic insufficiency
359
Fig. 1. ciated with spontaneous onset of AI and in whom surgical correction was undertaken are discussed in detail. The ages ranged from 3 to 31 years (mean 10 years); 17 were males and 2 females. Thirteen patients were Oriental or part Oriental, 2 were Polynesian, one was Caucasian, and 3 were Polynesian-Caucasian (Table I). The degree of AI was considered mild in 10 patients, moderate in 4, and severe in 5. The degree of preoperative and postoperative AI was judged clinically, usually by the same physician, on the basis of the length of
diastolic murmur, the hyperactivity of the left ventricular impulse, the pulse pressure, the electrocardiograms and roentgenograms, and, when available, angiograms with injections of dye into the aortic root. The calculated pulmonary-to-systemic flow ratios varied from minimal, detectable only by H+ curves, to 2.3:1, with a mean of 1.5:1. The VSD murmur was heard at birth in most patients, and the AI murmur was noted between 6 months and 14 years of age.
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Table II VSD Case No. Sex 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 Mean
M M M M M M M M F M M M M M M M M M F
Age (yr.) 3 3 17 4 29 3 18 17 4 5 8 7 5 7 9 10 31 9 3
Type
Size (mm.)
Preop. BP (mm. Hg)
SP SP SCr SCr SP SP SP SP SCr SP SP SCr SP SP SCr SP SP SP SCr
15 10 NR 8 10 15 23 13 12 10 8 1 10 10 Small 15 12 10 10
120/20 100/40 90/0 100/50 126/90 100/30 120/74 95/30 100/60 110/40 112/22 102/48 118/70 90/40 106/70 140/40 110/50 100/50 100/50
10.5
Preop. degree ofAl
Qp/Qs 1.2 2:1 1.2 2:1 1.8 2:1 1.2 2.3 2:1 1.5 H+ 1.5 1.3 1.2 2.2 1.5 1.2 1.6
1 1 1 1 1 1 detected 1 1 1 1 1 1 1
Severe Moderate Moderate Mild Mild Severe Mild Severe Mild Mild Severe Moderate Mild Mild Mild Severe Moderate Moderate Mild
VSD procedure Suture Suture Suture Suture Suture Suture Suture Suture Patch Suture Suture Suture Patch Patch Suture Patch Patch Patch Suture
Latest postop.
Aortic valve procedure
Late Post op. AI
BP
ECG
CXR
None Valvuloplasty Valvuloplasty None Valvuloplasty Valvuloplasty Valvuloplasty AVR None None None None None Valvuloplasty None Valvuloplasty AVR Valvuloplasty None
Minimal Absent Mild Mild Mild Mild Absent Absent Mild Mild Moderate Severe Mild Minimal Minimal Absent Absent Moderate Mild
148/80 86/40 100/60 106/60 112/72 118/78 120/80 110/70 100/60 100/60 130/60 100/25 110/80 90/60 100/60 125/65 112/48 120/70 90/58
Normal Normal Normal Normal LVH LVH Normal LVH LVH Normal LVH BVH Normal LVH Normal Normal LVH LVH Normal
Normal Normal Normal Normal Normal LVH Normal Normal Normal Normal LVH LVH LVH LVH Normal Normal BVH BVH Normal
1.5 1
Legend: VSD, Ventricular septal defect. BP, Blood pressure. Qp/Qs, Pulmonary-to-systemic flow ratio. AI, Aortic insufficiency. ECG, Electrocardiogram. CXR, Chest x-ray. SP, Subpulmonic. SCr, Subcristal. AVR, Aortic valve replacement. LVH, Left ventricular hypertrophy. BVH, Biventricular hypertrophy. NR, Not recorded.
Follow-up was from 2 to 12 years (mean 6). An electrocardiogram, chest roentgenogram, and clinical re-evaluation by the same cardiologist were available in all patients (Table II). Operative findings The VSD was subpulmonic in 13 patients (68 per cent) and subcristal in 6 (32 per cent). The aortic valve was bicuspid in one patient. One other patient had a ruptured aneurysm of the sinus of Valsalva. The right or noncoronary cusps prolapsed through the VSD in 11 patients. One patient had a persistent left superior vena cava. Two patients had anatomic stenosis of the right ventricular outflow tract, and in 3 others a gradient, demonstrated preoperatively, was probably due to a prolapsed aortic cusp. Surgical procedures Operations were performed with cardiopulmonary bypass, moderate hypothermia (30 to 32° C ) . cardiac arrest with topical hypothermia, no coronary perfusion, and left ventricular venting. A right atriotomy was sometimes done first to rule out the presence of an atrial septal defect. A right transverse ventriculotomy was done next to expose the VSD and inspect the degree of
AI. The aortic valve was exposed through a low oblique aortotomy. Aortotomy was not used in 6 patients in whom valvuloplasty or A VR was deemed unnecessary. In these cases, the degree of residual AI was estimated by the amount of drainage from the left ventricular vent and by the diastolic pressure following VSD closure. Valvuloplasty was done in 7 patients, valve replacement in 2, and VSD closure alone in 10. Valvuloplasty is undertaken prior to VSD closure so that the degree of residual AI can be assessed at the time of VSD repair. The method of valvuloplasty most frequently used was the one described by Frater.3 A variation of this method that has been effective in our hands is shown in Fig. 1, a and b. This consists of plicating the redundant segment and suturing it through the aortic wall with mattress sutures; the imbricated segment is then sutured to its adjacent commissure with a figure-of-eight 5-0 silk suture. The 2 patients undergoing valve replacement with Starr-Edwards prostheses were in the older age group. A 31-year-old man (Case 17) with cardiac cirrhosis had a markedly elongated and prolapsed noncoronary cusp which was used to patch the VSD. In another patient (Case 8), AI was diagnosed at the age of 14 years. When the boy was 17 years old, subacute bacterial
Volume 73 Number 3 March, 1977
Follow-up (yr.) 12 9 9 9 9 8 8 7 6 6 5 5 4 4 4 3 3 2 2
VSD and aortic insufficiency
Comments 10 mm. pulmonary gradient 10 mm. pulmonary gradient
Ruptured sinus of Valsalva aneurysm
7 mm. pulmonary gradient; residual VSD Possible AVR Plan AVR; abnormal commissure
Bicuspid aortic valve VSD patched with noncoronary cusp 20 mm. pulmonary gradient; tetralogy of Fallot
6
endocarditis with acute congestive heart failure developed secondary to destruction of the aortic valve. Valve replacement and VSD closure were performed following a month of intensive medical therapy. The VSD was repaired through a right ventriculotomy in all cases; the incision was made high in the outflow tract when a subpulmonic VSD was suspected. Primary suture was used in 13 patients and a Dacron patch in 5. The prolapsing cusp was used for VSD closure in one patient as described by GonzalezLavin and Barratt-Boyes.4 A teardrop-shaped patch has proved most successful for closure of subpulmonic VSD (Fig. 2). Results VSD closure alone (9 patients) (Fig. 3). The VSD was closed by suture in 7 patients and by patch in 2. The AI improved in 3 and has remained stable in 6. One patient (Case 11) had severe AI for which he was treated by VSD suture alone. Postoperatively, the AI became moderate and has remained stable for 5 years, with a blood pressure of 130/60 mm. Hg, cardiomegaly, and a Grade 2/6 diastolic murmur. The patient eventually may require aortic valve replacement. Another boy (Case 12) had a 1 mm. VSD. The anterior commissure appeared lower than the others; valvulo-
3 61
plasty was attempted but, because regurgitation was made worse, the valve was not repaired. He now has a blood pressure of 100/25 mm. Hg, cardiomegaly, and a Grade 3/6 diastolic murmur. Although he is asymptomatic 5 years postoperatively, he is considered a candidate for aortic valve replacement. Patient 1 had severe AI owing to prolapse of an aortic cusp into the VSD, which was corrected by VSD suture at the age of 3 years. Twelve years after the operation, the blood pressure is 140/68 mm. Hg, there is a Grade 1 protodiastolic whiff, and the chest x-ray film and electrocardiogram are normal. The long-term result in this case is excellent. Valvuloplasty (8 patients) (Fig. 4). AI was lessened in 6 patients and has remained stable in 2. The follow-up is 9 years in 3 patients, 8 years in one patient, and 4, 3, and 2 years in the remaining 3 patients. Aortic valve replacement (2 patients). Both patients are taking anticoagulants and have remained free of complications for 7 and 5 years postoperatively. There were no hospital or late deaths. Discussion Since the first successful operation for VSD and AI reported by Garamella5 in 1960, only 204 cases involving surgical treatment have been described in some detail in the English literature. The 19 cases reported here make a total of 223. This is in contrast to the high incidence of VSD and AI in Japan, where a total of 380 surgical cases had been reported through April of 1974. The increased frequency of AI associated with VSD correlates closely with a higher incidence of subpulmonic VSD, which occurred in 80 per cent of the cases of this syndrome reported by Sakakibara.6 The incidence of subpulmonic VSD in our series was 68 per cent. There is no unanimity of opinion in the literature regarding the optimal treatment and timing of operation for patients with VSD and AI.1' 7_12 One extreme is represented by Nadas,1 Somerville,7 and their colleagues, who recommend surgery only for those patients with very severe aortic regurgitation. Most authors now disagree with this view, feeling that surgery shortly after the onset of significant AI is imperative to preserve the integrity of the aortic valve.10' 12~17 Although the pathogenesis of AI in these patients is not well understood, it has been logically proposed that AI results from loss of subvalvular supporting tissues, which allows the cusp to prolapse as a result of the pressure gradient between the left ventricle and right ventricle.18 This theory is strengthened by the noted higher incidence of AI in subpulmonic VSD, in which
The Journal of Thoracic and Cardiovascular Surgery
3 6 2 Moreno-Cabral et al.
Fig. 2. FOLLOW DEGREE
OF
A . I. PREOPERATIVE
POSTOPERATIVE
% #^^_
SEVERE
2
3 4
^ ^ ^ ^ ^ ^ "
* n
i in
UP 6
7
(YEARS) 8
9
10 II 12
/
MODERATE
d
5
^HHW
i i
MILD
MINIMAL
Fig. 3. Results of VSD closure alone. the loss of subaortic fibromuscular tissue is greater than with the usually positioned subcristal VSD. From this it follows that VSD closure alone may correct, halt, or delay the progression of AI. Furthermore, if some valvular deformity has occurred, satisfactory correction with valvuloplasty is far more likely than when AI has become severe owing to extensive leaflet distortion. In one case in this series of subcristal VSD (Case 12), the commissure between the left and right cusps appeared to be situated at a lower level and valvuloplasty could not be performed; thus a defective commissure may
produce AI without cusp prolapse into the VSD. An abnormal commissure could also explain the late onset of AI one and 3 years following VSD closure in 2 other patients. This malposition of an aortic commissure has been previously suggested as an explanation for AI, particularly in patients with subcristal VSD.18 It is clear from the literature that the older the age group, the greater the need for aortic valve replacement and the higher the surgical mortality rate. Excellent results have been reported by Trusler17 with valvuloplasty, and it should be noted that all of his patients were
Volume 73 Number 3 March, 1977
VSD and aortic insufficiency
DEGREE OF A . I . PREOPERATIVE
SEVERE
6 16
MODERATE
18 3 2
MILD
5 14 7
POSTOPERATIVE
363
FOLLOW UP (YEARS) I 2 3 4 5 6 7 8 9
MINIMAL ABSENT
Fig. 4. Results of valvuloplasty. under 12 years of age. Accumulated experience seems to indicate a direct correlation between younger age and successful valvuloplasty. VSD closure alone may completely relieve AI, especially in the younger age group, as reported by Chung, 15 Dimich, 14 and others. 13, i6, i9, 20 j h e fact m a t A ] h a s b e e n lessened or has remained stable in 9 of our patients treated by VSD closure alone supports the view that this treatment may be all that is needed in many patients, especially in those with subpulmonic VSD. VSD closures by suture or patch were equally effective in this series in correcting or arresting the development of AI. We agree with Blumenthal and associates 21 that an effort should be made to identify the subpulmonic VSD and outline the abnormal aortic valve by routine use of left ventriculograms and aortic root angiograms, and we recommend VSD closure as soon as the diagnosis of AI is made. Furthermore, we believe VSD closure should be done when a subpulmonic VSD is diagnosed. VSD closure alone may be sufficient for many patients. If valvuloplasty is necessary, it too should be performed early. Further delay may result in irreparable aortic valve destruction, and aortic valve replacement will become necessary. Addendum Since the presentation of this paper, we have operated upon 2 additional patients with VSD and AI. A 5-year-old boy with a subcristal VSD and mild AI underwent patch closure of the VSD. A 4-year-old boy with a subpulmonic VSD and severe AI underwent aortic valvuloplasty and patch closure of the VSD. Another of our patients (Case 12) underwent aortic valve replacement with a No. 19 Hancock valve. The immediate results have been satisfactory in all these patients.
We are indebted to Jane Muramoto, Susie Anderson, and Alejandro Moreno for their assistance in preparing the manuscript.
1
2 3 4
REFERENCES Nadas, S. A., and Fyler, C. D.: Ventricular Septal Defect With Aortic Regurgitation, in Nadas, S. A., editor: Pediatric Cardiology, Philadelphia, 1972, W. B. Saunders Co., p. 379. Tatsuno, K., Konno, S., and Sakakibara, S.: Ventricular Septal Defect With Aortic Insufficiency, Am. Heart J. 85: 13, 1973. Frater, R. W. M.: The Prolapsing Aortic Cusp: Experimental and Clinical Observations, Ann. Thorac. Surg. 3: 63, 1967. Gonzalez-Lavin, L., and Barratt-Boyes, B. G.: Surgical Considerations in the Treatment of Ventricular Septal Defect Associated With Aortic Valvular Incompetence, J. THORAC. CARDIOVASC. SURG. 57: 422, 1969.
5 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 Transaortic Approach, Am. J. Cardiol. 5: 266, 1960. 6 Sakakibara, S.: Experiences With Congenital Anomalies of the Heart in Japan, J. THORAC. CARDIOVASC. SURG.
68: 189, 1974. 7 Somerville, J., Brandao, A., and Ross, D. N.: Aortic Regurgitation With Ventricular Septal Defect: Surgical Management and Clinical Features, Circulation 41: 317, 1970. 8 Ellis, F. H., Jr., Ongley, P. A., and Kirklin, J. W.: Ventricular Septal Defect With Aortic Valvular Incompetence: Surgical Considerations, Circulation 27: 789, 1963. 9 Sanfelippo, P. M., DuShane, J. W., McGoon, D. C , and Danielson, G. K.: Ventricular Septal Defect and Aortic
3 64
Moreno-Cabral
Insufficiency: Surgical Considerations and Results of Operation, Ann. Thorac. Surg. 17: 213, 1974. 10 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. 11 Spencer, F. C , Doyle, E. F., Danilowicz, D. A., Bahnson, H. T., and Weldon, C. S.: Long-Term Evaluation of Aortic Valvuloplasty for Aortic Insufficiency and Ventricular Septal Defect, J. THORAC. CARDIOVASC. SURG. 65: 15,
1973.
12 Kawashima, Y., Danno, M., Shimizu, Y., Matsuda, H., Miyamoto, T., Fujita, T., Kosuka, T., and Manabe, H.: Ventricular Septal Defect and Aortic Insufficiency: Anatomic Classification and Method of Operation, Circulation 47: 1057, 1973. 13 Glasser, S. P., Cheitlin, M.D., McCarty, R. J., Haas, J. H., Hall, R. H., and Mullins, CH. E.: Thirty-two Cases of Interventricular Septal Defect and Aortic Insufficiency: Clinical Hemodynamic and Surgical Features, Am. J. Med. 53: 473, 1972. 14 Dimich, I., Steinfeld, L., Litwak, R. S., Park, S., and Silver, N.: Subpulmonic Ventricular Septal Defect Associated With Aortic Insufficiency, Am. J. Cardiol. 32: 325, 1973. 15 Chung, K. J., and Manning, J. A.: Ventricular Septal Defect Associated With Aortic Insufficiency: Medical and Surgical Management, Am. Heart J. 87: 435, 1974. 16 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. 17 Trusler, G. A., Moes, C. A. F., and Kidd, B. S. L.: Repair of Ventricular Septal Defect With Aortic Insufficiency, J. THORAC. CARDIOVASC. SURG. 66: 394, 1973.
18 Van Praagh, R., and McNamara, J. J.: Anatomic Types of Ventricular Septal Defect With Aortic Insufficiency, Am. Heart J. 75: 604, 1968. 19 Robinson, G., Fell, S. C , and Jacobson, B. E.: Ventricular Septal Defect With Aortic Insufficiency: A Method of Management, J. THORAC. CARDIOVASC. SURG. 43: 785,
The Journal of Thoracic and Cardiovascular
et al.
1962.
20 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. 21 Blumenthal, S., Kangal, J. K., Bauman, F. O., et al.: Ventricular Septal Defects With Prolapse of the Aortic Cusp (Abstr.), Circulation 28: 691, 1963.
Discussion DR. R O B E R T M. A N D E R S O N Tucson, Ariz.
I am envious of the essayists' opportunity to observe a fairly large series of cases that are rare to the rest of us. It is easy for me to agree with their hypothesis that early repair of the ventricular septal defect is prophylactic against
Surgery
progressive development of aortic insufficiency. Any ventricular septal defect that extends into and involves the support of the sinus of Valsalva allows some prolapse. The result is progressive acquired stretching of the valve's leading edge, with the development of shallower and shallower valve cusps, and, consequently, progressive aortic insufficiency. Late repair of the ventricular septal defect does not restore the normal length of the valve's leading edge. When aortic insufficiency is significant, valvuloplasty rather than aortic valve replacement is frequently desirable because of the age and small size of the patients. We have found that plication by the method described by the essayists is not an effective way in which to repair the aortic insufficiency. The stretching of the leading edge of the aortic valve fans in such a way that a tuck in one sector leaves prolapse in other areas away from the tuck. In Annals of Surgery in 1961, Kay and I reported a method of valve suspension that makes the valve totally sufficient. I want to report a 17 year follow-up of that means of valve suspension. A cable of one strand of polyester and one strand of silk strung from one commissure to the other, with the valve leaflet edge reefed to such a cable, has restored competency to the present time. The objective of this method of valvuloplasty is to place a cable between the two commissures of the prolapsed valve, stretch the cables over bolsters external to the aorta, and tighten the cable until it is the exact diameter of the aorta. The leading edge of the valve is then attached to the cable. When the valve edge is restored to the diameter of the aorta, the leaflet properly apposes the other two valve cusps. There is some concern in the literature over abnormal position of commissures contributing to aortic insufficiency. Any photographer who has a tripod knows that it takes three points to determine a plane. It is the length of the leading edge of the valve and the shallowness of the cusp that determine whether a valve is sufficient, not the position of the commissures. To squeeze a bit more information out of the essayists' experience, I would like to ask two questions: Have they been able to correlate the degree of insufficiency with measurements of the length of the leading edge and depth of the cusps? What criterion have they used to determine whether to accept the amount of insufficiency and not interfere with it, and what is their indication for valvuloplasty and valve replacement? DR. J O H N G. J A C O B S O N Loma Linda, Calif.
Our experience is not as extensive as that of Dr. MorenoCabral, but in the past 2 years we have treated 5 patients. Dr. Vyhmeister has provided this material for me to share with you. The first patient was treated at Loma Linda University and the subsequent 4 were treated during a trip to South Vietnam. Two patients had supracristal ventricular septal defects, and the others had infracristal defects. One patient had perforation of the right coronary cusp and another had perforation of the
Volume 73 Number 3 March, 1977
right sinus of Valsalva and of the pulmonary valve. Two patients had infundibular pulmonary stenosis. [Slide] This slide reinforces the associated anatomic and pathological problems: Infracristal ventricular septal defects, infundibular stenosis, and supracristal defects. Prolapsing of the aortic cusp is common in Oriental patients. Dr. George Trusler has recently expanded upon the earlier method described by Frater. One of the important points that Dr. Trusler makes is that the plication needs to be reinforced with pericardial, Dacron, or Teflon bolsters in order to prevent the plication from being dissected away from the aortic wall. Earlier attempts at valvuloplasty were associated with a high incidence of breakdown of the repair. Dr. Trusler feels that the pledget should extend beyond the plication, up on to the aortic wall, and in addition another pledget should be placed across the commissure to try to prevent breakdown of the valvuloplasty. In one of our patients with a supracristal ventricular septal defect, the defect was repaired with a patch after the valve had been plicated. Another patient had a perforation of the right coronary cusp. The valve was plicated and the perforation closed, followed by a patch closure of the ventricular septal defect. In a patient who had a small ventricular septal defect, the defect was closed by direct suture over Teflon felt pledgets. We believe that an attempt should be made to resuspend and restore competence to the valve prior to repairing the ventricular septal defect, as was mentioned in MorenoCabral's paper, so that the adequacy of this repair can be assessed when the ventricular septal defect is subsequently repaired. I would like to ask Dr. Moreno-Cabral two questions: Is an attempt now being made to repair all of the incompetent valves, and do the 10 patients in whom no repair was attempted reflect your earlier experience with this problem?
VSD and aortic insufficiency
3 65
D R . M O R E N O - C A B R A L (Closing) Dr. Anderson, we have not done any studies to try to correlate the degree of elongation of the leading edge of the aortic cusps with the degree of incompetence nor with the depth of the aortic commissures, so we do not have an answer to your first question. The amount of residual insufficiency was estimated by the amount of drainage from the left ventricular vent during bypass and by the diastolic pressure after bypass. Regarding the second question, relating to indications for valvuloplasty or aortic valve replacement, we believe that in mild aortic insufficiency, especially if the VSD is subpulmonic, a VSD closure alone may be sufficient. In patients who have moderate or severe incompetence, valvuloplasty is done first; aortic valve replacement is left for those patients with severe aortic insufficiency in whom the VSD has been closed and valvuloplasty has been ineffective. We prefer to wait until these children reach an older age. The child whom we have scheduled for aortic valve replacement is now 12 years of age. In reply to Dr. Jacobson's question, we do not repair all incompetent valves. As mentioned in the manuscript, we employ valvuloplasty in most patients who have moderate or severe aortic insufficiency. I may add that some patients, as mentioned by Dr. Anderson, have abnormal commissures. This was the case in one of our patients in whom the valve incompetence was worse following the plication procedure and the suture had to be removed. This is the patient I mentioned before, who is scheduled for aortic valve replacement. Plication may not be feasible in these instances. Patients with residual aortic insufficiency are usually asymptomatic, and heart failure may not develop for several years. Our cardiologists usually just restrict the physical activity of these patients, and we wait until they grow before replacing the aortic valve.