- Email: [email protected]
Ventricular septal defect with associated aortic valve insufficiency
J
THoRAc CARDIOVASC SURG
82: 182-189, 1981
Ventricular septal defect with associated aortic valve insufficiency Progression of insufficiency and operative results in young children Thirty-two children with both ventricular septal defect (VSD) and aortic insufficiency (AI) were evaluated for progression in degree of AI as well as effectiveness of operation in reducing or eliminating regurgitation. At the time of initial study, AI was mild in 21, moderate in eight, and severe in three patients. Twenty-one patients were followed medically over a 2 year to 19 year period, during which the degree of AI progressed in five. One death occurred during an episode of endocarditis. A total of 24 patients underwent operation at ages ranging from I year to 23 years. Postoperative evaluations continued from a minimum of I year to 24 years in 19 patients, with residual AI noted in 63% of these. Children less than 5 years of age, even with little valvular involvement, exhibited minimal benefit from attempted correction compared with those in the latter part of the first decade of life (p < 0.05). Closure of the VSD with associated valvuloplasty resulted in a higher prevalence of elimination or improvement of AI compared with VSD closure alone (p < 0.01). As AI can progress slowly without compromising the child's development, attempted surgical repair can safely be deferred in children less than 5 years of age with minimal valvular involvement unless careful medical management fails to prevent cardiac decompensation.
Peter P. Karpawich, M.D., Desmond F. Duff, M.D., Charles E. Mullins, M.D., Denton A. Cooley, M.D., and Dan G. McNamara, M.D., Houston, Texas
T
he indications for and timing of operation for repair of ventricular septal defect (VSD) with associated aortic insufficiency (AI) remain unsettled. The prevalence of AI among patients with a VSD is reported to range from 2.5% to 7% in this country and 8.2% in Japan.":" The reported progression of AP-4, 8-12 has led to the recommendation for early closure of the VSD with or without repair of the aortic valve." 4. 6, 9. 12-16 Al-
From The Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas Supported in part by Grant HL-07l90 from the National Institutes of Health, U.S. Public Health Service, and USPH Grant RR-OOI88 from the General Clinical Research Branch, National Institutes of Health. Received for publication Nov. 14, 1980. Accepted for publication Feb. II, 1981. Address for reprints: Peter P. Karpawich, M.D., Section ofCardiology, Texas Children's Hospital, 6621 Fannin, Houston, Texas 77030.
182
though such a recommendation can be applied to patients with severe insufficiency, there are very few studies which deal with operation in young child with only slight valvular incompetence and the long-term effects of attempted early repair. The purpose of this study was to assess the long-term effectiveness of operation for the correction of VSD and AI. This study emphasizes the natural progression of insufficiency, surgical techniques of VSD closure and aortic valve repair, and age at the time of operation. Methods Population. From 1952 to 1978, a total of 17,176 patients were seen at Texas Children's Hospital for evaluation of congenital heart defects. At initial examination, an isolated VSD was considered to be the only cardiac lesion in 1,692 (9.8%) of this population, of whom 32, or 1.9%, exhibited clinical evidence of AI at a median of 32 months after the murmur of VSD was detected (median age 4 months). This group included 23 male and nine female patients. Patients with a his-
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tory of previous rheumatic fever or infective endocarditis were not included, unless those lesions arose after the onset of insufficiency. The patients were assigned to three groups on the basis of their therapeutic management. The first group consisted of six patients who never underwent operation. They were evaluated over a 5 to 17 year period (median 6.5 years). These patients constituted the nonsurgical group. Median age at the time of initial evaluation was 3 years. The second group of 16 patients was also treated medically first, during a 2 to 19 year period (median 5 years), to monitor each patient's course and possible progression of insufficiency. They later underwent operation at a median age of 7 years. Postoperative evaluations were then continued among 15 of these patients from 1 to 24 years to evaluate surgical effectiveness in correction of AI. One child was lost to follow-up soon after operation and was not included in any of the postoperative groups. Owing to the chronological order of surgical intervention during their course of management, these patients were referred to as the late surgical group. The third group consisted of 10 patients, who were referred from other institutions specifically for surgical correction of VSD and AI. Since these patients originated from beyond our geographical area and medical information was often inadequate to evaluate any preoperative progression in degree of AI, these children were not included in the natural history of AI. One child was lost to follow-up after catheterization and prior to surgical intervention. The remaining nine children all underwent operation within a year of our initial examination and did contribute to our evaluation of surgical effectiveness in improving or eliminating insufficiency. However, time of clinical onset of insufficiency as well as preoperative catheterization data were known in each patient. To separate them from those patients we had evaluated prior to operation, we assigned these patients to the referred surgical group. Median age at operation was 6 years. Postoperative evaluations were then continued over a 2 to 15 year period. Hemodynamic data. Initial preoperative catheterization data were available on all 32 patients. Patient age at documentation of insufficiency, shunt size as determined by the Qp/Qs ratio, left ventricular enddiastolic pressure, and pulse pressure were analyzed from available catheterization data. Position of the VSD and aortic cusp involvement were determined by both left ventricular and aortic root angiograms in all cases and subsequently confirmed in
VSD with aortic insufficiency
18 3
19 (59%) and 14 (44%) cases, respectively, at the time of operation or autopsy. Degree of AI was quantitated by the method of Lehman, Boyle, and Debbas'"; severe (4+), rapid reflux with uniform and maximal opacification of the left ventricular chamber; mild (l ,2+), minor degrees of reflux; and moderate (3+), an intermediary position between the two extreme degrees of regurgitation. Surgical technique. Initial surgical technique utilized total cardiopulmonary bypass with identification of the VSD either through a right ventriculotomy or a right atrial and tricuspid valve approach. Closure of the VSD was either by direct suture (eight patients) or use of a Dacron patch (16 patients). Aortic valve repair was performed in 13 patients. A transverse aortotomy was made across the base of the ascending aorta to expose the prolapsed leaflet. Valvuloplasty was performed by reapproximating and resuspending the prolapsed valve cusp at the commissural margins to the adjoining valve cusps. Excess leaflet tissue was plicated with sutures from the commissures through the aortic wall. In five patients the plication was reinforced with pledget-supported mattress sutures from outside to inside the aorta. The aortotomy and right ventriculotomy were closed with continuous sutures. Two patients, one of whom had a bicuspid aortic valve, received aortic valve replacement. To evaluate the effectiveness of operation for correction of the insufficiency, comparisons were made among techniques of VSD repair and association of AI repair, patient ages at time of operation, locations of the VSD, and degrees of AI. Both the t test for independent means and chi square analyses were used to evaluate statistical significance of results. Significance was defined as exceeding the 95% confidence level (p < 0.05). Results Hemodynamic data. Cardiac catheterization data at the time of initial evaluation for suspected AI are listed in Table I. The degree of AI was found to be mild in 21, moderate in eight, and severe in three patients. Although the left ventricular end-diastolic pressure and Qp/Qs did not differ significantly among the three degrees of insufficiency, pulse pressure did correlate (p < 0.05) with the angiographic estimation of valve reflux as a percentage of systolic pressure. Position of the VSD was infracristal in 17 (53%) and supracristal in 10 (31 %) cases. Position could not be determined accurately from available data in five cases (16%). Aortic valve prolapse involved the right coronary cusp in 16 (50%), noncoronary cusp in nine
The Journal of Thoracic and Cardiovascular Surgery
184 Karpawich et al.
Table I. Catheterization data at initial Al evaluation Pressure (mm Hg)
I
Case No.
Age (yr)
Aorta
Left ventricle
I 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
3 10 2 1.5 5 12 3 11 6 14 10 1.5 1 5 3 17 12 9 5 5 5 [5.0]"
90/40 120/80 105/60
130/0,10 105/0,10
100/65
105/0,9
130/80 105/70 125/75 110/75 100/40 100/50 125/65 100/60 95/65 130/80 100/60 130/70 125/75 108/55 100/70 [105/65]
130/0,20 105/0,10 100/0,14 120/0,5 95/0,5 90/0,5 125/0,10 100/0,10
22 23 24 25 26
9 3 5 3 1 7 5 7 [5.0]
130/70 100/47 85/35 130/43 120/50 95/60 90/30 110/40 [105/44]
3 12 4 [4.0]
120/35 170/80 100/40 [120/40]
Qp:Q.
Mild
NA
NA
NA
NA
95/0,13
130/0,18 100/0,16 128/0,5 125/0,5 100/0,13 [105/0,10]
2.8: I 1.7: I 1.9: 1 1.8: I 1.3: 1 1.2: 1 1:1 2.4: I 1.5: I 1.6: I 1.5: I 1.8: I 1.4: 1 1.3: I 1.5: 1 1.7: 1 1.4: I 1.8: 1 [1.5:1]
Pulse pressure <50% systolic
No Yes Yes Yes Yes Yes Yes Yes
No
Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes
Moderate
27
28 29
130/0,10 95/0,10 85/0,13
100/0,8 90/0,20 [95/0,10]
1:[ 2.3: 1 1.4: 1 1.2: 1 1.4: 1 2.8: 1 1.5: I 2: I [1.4: 1]
Yes
No No No No
Yes
No No
Severe
30 31 32
120/0,21 115/0,7 100/0,10 [115/0,10]
1:1
No No No
[1 : I]
Legend: AI, Aortic insufficiency. NA, Not available.
• Brackets indicate median value.
(28%), and both the right and noncoronary cusps in two (6%) patients. Cusp involvement could not be determined in five (16%) patients. Natural progression of AI. As shown in Fig. 1, only one of the nonsurgical patients has exhibited angiographic progression of insufficiency over a 6.5 year median follow-up period; all have remained asymptomatic. The other four children are currently being followed for evidence of deterioration in cardiac status. The one child with an initially severe degree of AI
remained stable for 15 years prior to a fatal episode of infective endocarditis. The remaining data in Fig. I illustrate the progression of AI among those patients whom we were able to follow prior to surgical intervention. Six patients initially exhibited mild AI and no symptoms. During a median of 6.5 years the degree of insufficiency progressed in three of these patients. Only the one who had severe AI demonstrated any clinical change, i.e., onset of exercise intolerance at 23 years of age. There was no
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VSD with aortic insufficiency
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N • 6 FOLLOWED 5 - 17 Y
N • 16 FOLLOWE 0 2 - 19 Y PRE-OPERA TI VE
MODERATE
MILD
SEVERE
DEGREE AI
INITIAL FOLLOW-UP
,/: ), e: 3.5 Y
6.5 Y
MODERATE
MILD
6.5 Y
14Y 1--1
1
SEVERE
SEVERE
MILD
INITIAL PRE-OP
,/:
l5Y 1 - - 1 (OIED)
Fig. 1. Progression in degree of aortic insufficiency (AI) among the 16 patients followed prior to operation (' 'late surgical" group) and the six patients who have not undergone any operation ("nonsurgical" group). Number of patients at each level of valvular incompetence followed over the listed median time intervals. N • 7 I 9 (77%)
N • 12 I 16 (75%)
MODERATE
MILD DEGREE AI
PRE
POST
4 Y
4
NONE
22 Y ~ED)
1
7/1\
MODERATE MILD
PRE POST POSH PRE
2.5 Y
SEVERE
SEVERE
-,
3- 1
\2
\
\ 2
'I (AVRI
POST
5 Y 2
\,
llAVR)
MILD PRE POST
SEVERE PRE POST
4
/:
2
1 \1
6- 1
\3
Fig. 2. Operative results among the 16 patients followed prior to operation ("late surgical" group) and nine of the patients referred for operation ("referred surgical" group). Preoperative and postoperative degrees of aortic insufficiency (AI) with number of patients at each level of valvular incompetence followed over the listed median postoperative period. AVR. Aortic valve replacement. * Patient required repeat operation.
progression in degree of insufficiency in one patient who had infective endocarditis during this period. One of the patients with initially moderate AI showed progression to severe AI; all, however, continued to be asymptomatic, although two exhibited compensated heart failure when treated with digitalis and diuretics. The one patient with an initially severe degree of AI remained asymptomatic for 14 years, having no abnormalities on a treadmill exercise test performed at 16 years of age. During a repeat test performed 2 years
later, however, ST-segment changes were noted on the electrocardiogram. Postoperative results. A total of 24 patients underwent operation, between the ages of 1 and 23 years (median 6.5 years); 12 of these were 6 years or younger. Among the nine children referred specifically for operation, there was little previous medical data available with which to judge the antecedent clinical course and subsequent timing for surgical intervention. The 16 patients of the late surgical group, however, had all been evaluated in this institution several times prior
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Table II. Operative results
Procedure Patch/valvuloplasty
Age at operation (yr)
3 3 6 6 9 5 6 12 6 5 10
Postoperative
Site ofVSD
Degree of Al
Al
VSD
II II II II I II I II NA II II
Moderate Moderate Mild Severe Mild Moderate Moderate Severe Mild Mild Moderate
Yes* No No No No Yes*t Yest Yest No No NA
Yes* No No No* Yes Yes* No No Yes No NA
II NA II II II
Mild Moderate Mild Mild Moderate
Yes* Yes* Yes No Yes
Yes* Yes* No No Yes
I I II II
Mild Moderate Mild Mild
Yes* Yes* No Yes
No* Yes* No No
3
II II
Mild Moderate
No Yes*
Yes Yes*
4 2
Severe (B) Severe
No No
No No
[6.0]* Patch only
I
7 12 14 5 [7.0] Primary closure
4 7 II
10
I
FlU (yr)
7 0.75 4 5 4 4 3 2 3 I
[3.5] 10 I
3 3 2 [3.0] 15 24 I
[9]
[9.0] Primary closure/valvuloplasty
3 II
Primary closure/A VR
[7.0] 23 18 [20.5]
[3]
[ I]
Legend: VSD. Ventricular septal defect. AI. Aortic insufficiency. FlU. Follow-up. VSD: I. supracristal; II. infracristal. AVR, Aortic valve replacement. NA, Not available. B, Bicuspid valve. * Catheterization confirmation. t Improvement in degree of residual AI. Brackets indicate median value.
*
to operation. Among this latter group, correction was recommended in five patients (four with moderate and one with severe AI) on the basis of cardiac dysfunction as determined by increasing electrocardiographic hypertrophy or strain. Four of these children also exhibited increasing cardiomegaly by chest radiograph, three of whom had no evidence of increase in shunt size at preoperative catheterization. The remaining 11 of these 16 patients showed no clinical change at the time of repair when compared with initial evaluation. Degree of AI. The postoperative results in degree of insufficiency among the two surgical groups are presented in Fig. 2. Long-term postoperative data were available in 12 of the 16 patients initially from this center (median age at operation 7 years; median preoperative Qp/Qs 1.5: 1). Only three (25%) patients, not having an initial valve replacement, exhibited no residual AI. There was persistence of valvular regurgita-
tion in eight (66%) patients, with progression in degree of AI in four of these children. Two of these patients, as described, had exhibited evidence of cardiac decompensation preoperatively, demonstrated by increasing cardiomegaly and ventricular hypertrophy or strain. Because of a deterioration in clinical status during the postoperative period, all four patients required a second operation, during which two died. Only one of the survivors did not have a valve replacement and she continues to have severe residual AI 22 years later. Among the 10 patients referred specifically for surgical repair, long-term postoperative data were available in seven (median age at operation 6.5 years; median preoperative Qp/Qs 1.7: 1). Among the six patients with initially mild AI, there was progression of insufficiency after a median 12.5 years in two following ineffective patch repair of the VSD in one and primary closure in the other child (median age at operation 3
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~
f----
SEVERE MOOERATE MILD NONE
1-4YEARS
1-9YEARS
N • 4 I 19
N • 9 I 19
NOT IMPROVEO PRE
t
POST
IMPROVEO PRE
POST
<,
NOT IMPROVEO PRE
POST
/' ----.0
'----
10 - II YEARS N • 6 I 19
IMPROVlO POST
PRE
~
NOT IMPROVlO PRE
POST
/
t
---.0 ----.0
IMPROVlO PRE
POST
~ ~ \
t • DIED
Fig. 3. Separation of patients according to age at operation with preoperative and postoperative degrees of aortic insufficiency (AI). Each dot represents one patient. Dashed line indicates valve replacement. Improvement is based on elimination or improvement in degree of AI.t Patient died. years). Both of these children later required valve replacement. The one patient with an initially severe degree of prolapse did improve after operation. Surgical technique. As presented in Table II, excluding valve replacement as an effective method of correcting insufficiency, the majority of our patients exhibited the most successful results from patch closure of the VSD associated with plication of the prolapsed valve cusp. Although residual AI was found in four (40%) of these 10 children after a median of 3.5 years, the degree of AI was improved in three. The other listed techniques performed at comparable patient ages, degrees of AI, and after sufficient postoperative intervals were associated with a significantly higher incidence (p < 0.05) of residual insufficiency. Closure of the VSD alone did not prove to be effective in preventing the progression of insufficiency. Of the nine patients in whom aortic valvuloplasty was not performed, seven (77%) had residual AI, with postoperative progression in degree of insufficiency noted in three of these patients after a median 10 year follow-up period. Age at the time of operation. To evaluate the effect of age at the time of operation as a contributory factor toward a successful outcome, all patients were compared by means of preoperative and postoperative estimates of AI. As presented in Fig. 3, there was a statistical difference (p < 0.05) among the age groupings and successful resolution of AI. There were four children less than 5 years of age at the time of operation. Although the degree of AI was only mild or moderate, operation resulted in improvement in only one; in the remainder the degree of insufficiency progressed. In comparison, among our patients between the ages of 5 and 9 years of age,
who exhibited all three degrees of valvular incompetency, six (66%) showed improvement. Likewise, children over 10 years of age also manifested favorable outcomes.
Discussion Aortic insufficiency (AI) is generally considered to be a progressive lesion. 4. 8. 9. II. 14 In the pediatric age range, this may occur at a variable and slow rate,": 3. 5. 14. 18 even without a deterioration in clinical status. In this study, only five of our 21 children with AI manifested progression in the degree of insufficiency over a median of 5 years. Only one of these, with onset of AI in late adolescence, became symptomatic. His course is compatible with the theory": 5 that rapid progression may be more prevalent in late-onset regurgitation. Such a variable and slow progression in valvular incompetence has caused much controversy as to the proper timing and type of operation to correct or at least arrest the progression of insufficiency. This present study attempts to add to knowledge in this field by reviewing the effects of different surgical techniques performed on a population of young children, the majority of whom had only minimal valvular involvement, after postoperative periods extending to 24 years. The success rate in eliminating or minimizing the degree of valvular incompetence varies. Some authors have reported that VSD closure alone is sufficient in supracristal lesions," 6. 8. 9. 16 whereas others have found the need for associated valvular repair. 14. 15. 19 Among our four patients with supracristal lesions not having valve replacements, residual AI persisted in three children after a median follow-up period of 9.5 years. The AI either improved or was eliminated in the two children who had VSD closure plus valvuloplasty
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Karpawich et al.
Thoracic and Cardiovascular Surgery
(median age 7.5 years). The degree of AI did not change in those patients with VSD closure alone (median age 5.5 years). Unfortunately, the small number of patients does not permit statistical differences between the surgical methods. Other reports describe patients with supracristal lesions comparable to ours in age and type of operation. 4.6 Their postoperative data show similar findings, which would favor the theory that insufficiency may not be a totally acquired lesion, but rather involves an inherent defect in valvular embryogenesis.v 14. 17 Correction of regurgitation associated with an infracristal lesion is a technically demanding operation. Surgical procedures on the aortic valve have included variations in cusp plication ': 5. 6. 9. 1:1. 15 as well as partial or total valve replacement. 2. 5. 6. 12. 14. 19 Neither of these techniques has yielded totally satisfactory results. Eight of our 15 patients (53%) with infracristal lesions manifested residual AI after a median postoperative period of 2.5 years. Three of these patients, whom we were able to follow longer (median 7 years), showed progression in degree of AI. From our data, both closure of the VSD with and without repair of the aortic valve proved to be equally effective in completely eliminating insufficiency in children with infracristallesions. However, there was a significant difference (p < 0.025) in improvement in degree of AI in those patients who underwent an associated valvuloplasty. Likewise, statistical analysis of the entire postoperative patient population, combining both supracristal and infracristal lesions, reveals a significant difference (p < 0.01) in success rate of eliminating or improving the degree of valvular insufficiency when repair of the prolapsed valve cusp was performed at the time of VSD closure. Ten of our 12 patients (83%) who underwent an associated valvuloplasty were improved. This result compares with other reports which describe slightly different methods of valvuloplasty. 6. 1:1. 15 In our experience, patients with and without plication reinforcement with pledgets manifested equally successful results regardless of age, location of the VSD, or preoperative degree of AI. Two of our children, both with residual VSDs, continued to have unimproved AI. Persistent lack of valvular support and hemodynamic contributions from the VSD may have prevented satisfactory surgical results in these patients. The majority of our patients demonstrated no evidence of cardiac dysfunction prior to operation. Among the four children with electrocardiographic and radiographic evidence of ventricular hypertrophy and dilatation, repair was ineffective in two. One of these chil-
dren had onset of AI during adolescence which, as previously discussed, may be associated with rapid progression of valvular deformity. The other child demonstrated an increase in shunt size (Qp/Qs 1.2: 1 to 2.8: 1) during the 7 year preoperative period. Although the small number of patients does not permit statistical evaluation, operation performed prior to evidence of cardiac decompensation may result in a more favorable surgical outcome. The final variable that we examined to determine effectiveness of surgical intervention was patient age at the time of operation. Compared to the numerous reports of operation performed on older children and adolescents, very few studies have included young children, less than 5 years of age. Our current review includes four such children, whom we have been able to evaluate for a median 8.5 years after operation. Only one of these younger children exhibited any definite benefit from early operation; the remaining three all manifested an increase in the degree of valvular incompetence. As opposed to this, children in the latter part of the first decade of life exhibited a far greater success rate (p < 0.05) in total repair or improvement in valvular regurgitation. There was no significant difference in the preoperative degree of AI or surgical techniques in the older children as compared with the younger children. The older children actually exhibited more preoperative valvular involvement. Since surgical techniques have varied over the past 25 years, future data may yield different outcomes from those presented in this review. Nevertheless, several recommendations can be advocated. Surgical corrections of VSD and AI is a feasible procedure in children. However, as the degree of AI can progress slowly without compromising the child's development, there is time for regular and careful medical supervision with operation being reserved for those patients who exhibit a change in cardiac status prior to any overt clinical deterioration. The ineffective surgical outcome seen in younger children, especially with minimal valvular involvement, would preclude early operative intervention unless medical management fails to prevent cardiac decompensation. Because of potentially inherent defects in the aortic valve itself, apart from any hemodynamic contributions, repair of the prolapsed aortic valve cusps can contribute to a more effective surgical outcome. REFERENCES Nadas A, Thilenius 0, Lafarge C, Hauck A: Ventricular septal defect with aortic regurgitation. Medical and pathologic aspects. Circulation 29:862-873, 1964 2 Gonzalez-Lavin L, Barratt-Boyes B: Surgical consid-
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3
4
5
6
7
8
9
10
erations in the treatment of ventricular septal defect associated with aortic valvular incompetence. J THoRAc CARDIOVASC SURG 57:422-430, 1969 Glasser S, Cheitlin M, McCarty R, Haas J, Hall R, Mullins C: Thirty-two cases of intraventricular septal defect and aortic insufficiency: Clinical, hemodynamic and surgical features. Am J Med 53:473-480, 1972 Chung K, Manning J: Ventricular septal defect associated with aortic insufficiency. Medical and surgical management. Am Heart J 87:435-438, 1974 Keane J, Plauth W, Nadas A: Ventricular septal defect with aortic regurgitation. Circulation 56:Suppl 1:72-77, 1977 Moreno-Cabral R, Mamiya R, Nakamura F, Brainard S, McNamara J: Ventricular septal defect aortic insufficiency: Surgical treatment. J THoRAc CARDIOVASC SURG 73:358-365, 1977 Corone P, Dayton F, Gaudeau S, Guerin F, Vernant P, Duncam H, Rumeau-Rouquette C, Gaudeul P: Natural history of ventricular septal defect. A study involving 790 cases. Circulation 55:908-915, 1977 Van Praagh R, McNamara J: Anatomic types of ventricular septal defect with aortic insufficiency. Diagnostic and surgical consideration. Am Heart J 75:604-619, 1968 Kawashima Y, Danno M, Shimizu Y, Matsuda H, Miyamoto T, Fujita T, Kozuka T, Manabe H: Ventricular septal defect associated with aortic insufficiency. Anatomic classification and method of operation. Circulation 47:1057-1064, 1973 Spencer F, Bahnson H, Neill C: The treatment of aortic regurgitation associated with a ventricular septal defect. J THORAC CARDIOVASC SURG 43:222-233, 1962
II Dimich I, Steinfeld L, Litwak R, Park S, Silvers N: Subpulmonic ventricular septal defect associated with aortic insufficiency. Am J Cardiol 32:325-328, 1973 12 Plass R, Munster W, Ivanov S, Sturner V: Angiographic diagnosis of aortic insufficiency in cases of ventricular septal defect associated with partial prolapse of the aortic valve. Cardiology 58:257-272, 1973 13 Spencer F, Doyle E, Danilowicz D, Bahnson H, Weldon C: Long-term evaluation of aortic valvuloplasty for aortic insufficiency and ventricular septal defect. J THoRAc CARDIOVASC SURG 65:15-31, 1973 14 Somerville J, Brando A, Ross D: Aortic regurgitation with ventricular septal defect. Surgical management and clinical features. Circulation 41:317-330, 1979 15 Trusler G, Moes C, Kidd B: Repair of ventricular septal defect with aortic insufficiency. J THoRAc CARDIOVASC SURG 66:394-403, 1973 16 Robinson G, Fell S, Jacobson B: Ventricular septal defect with aortic insufficiency. A method of management. J THoRAc CARDIOVASC SURG 43:785-791, 1962 17 Lehman J, Boyle J, Debbas J: Quantitation of aortic valvular insufficiency by catheter thoracic angiography. Radiology 79:361-369, 1962 18 Halloran K, Talner N, Browne M: A study of ventricular septal defect associated with aortic insufficiency. Am Heart J 69:320-326, 1965 19 Ellis F, Ongley P, Kirklin J: Ventricular septal defect with aortic valve incompetence. Surgical considerations. Circulation 27:789-795, 1963
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Ventricular septal defect with associated aortic valve insufficiency Progression of insufficiency and operative results in young children Thirty-two children with both ventricular septal defect (VSD) and aortic insufficiency (AI) were evaluated for progression in degree of AI as well as effectiveness of operation in reducing or eliminating regurgitation. At the time of initial study, AI was mild in 21, moderate in eight, and severe in three patients. Twenty-one patients were followed medically over a 2 year to 19 year period, during which the degree of AI progressed in five. One death occurred during an episode of endocarditis. A total of 24 patients underwent operation at ages ranging from I year to 23 years. Postoperative evaluations continued from a minimum of I year to 24 years in 19 patients, with residual AI noted in 63% of these. Children less than 5 years of age, even with little valvular involvement, exhibited minimal benefit from attempted correction compared with those in the latter part of the first decade of life (p < 0.05). Closure of the VSD with associated valvuloplasty resulted in a higher prevalence of elimination or improvement of AI compared with VSD closure alone (p < 0.01). As AI can progress slowly without compromising the child's development, attempted surgical repair can safely be deferred in children less than 5 years of age with minimal valvular involvement unless careful medical management fails to prevent cardiac decompensation.
Peter P. Karpawich, M.D., Desmond F. Duff, M.D., Charles E. Mullins, M.D., Denton A. Cooley, M.D., and Dan G. McNamara, M.D., Houston, Texas
T
he indications for and timing of operation for repair of ventricular septal defect (VSD) with associated aortic insufficiency (AI) remain unsettled. The prevalence of AI among patients with a VSD is reported to range from 2.5% to 7% in this country and 8.2% in Japan.":" The reported progression of AP-4, 8-12 has led to the recommendation for early closure of the VSD with or without repair of the aortic valve." 4. 6, 9. 12-16 Al-
From The Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas Supported in part by Grant HL-07l90 from the National Institutes of Health, U.S. Public Health Service, and USPH Grant RR-OOI88 from the General Clinical Research Branch, National Institutes of Health. Received for publication Nov. 14, 1980. Accepted for publication Feb. II, 1981. Address for reprints: Peter P. Karpawich, M.D., Section ofCardiology, Texas Children's Hospital, 6621 Fannin, Houston, Texas 77030.
182
though such a recommendation can be applied to patients with severe insufficiency, there are very few studies which deal with operation in young child with only slight valvular incompetence and the long-term effects of attempted early repair. The purpose of this study was to assess the long-term effectiveness of operation for the correction of VSD and AI. This study emphasizes the natural progression of insufficiency, surgical techniques of VSD closure and aortic valve repair, and age at the time of operation. Methods Population. From 1952 to 1978, a total of 17,176 patients were seen at Texas Children's Hospital for evaluation of congenital heart defects. At initial examination, an isolated VSD was considered to be the only cardiac lesion in 1,692 (9.8%) of this population, of whom 32, or 1.9%, exhibited clinical evidence of AI at a median of 32 months after the murmur of VSD was detected (median age 4 months). This group included 23 male and nine female patients. Patients with a his-
0022-5223/81/080182+08$00.80/0 © 1981 The C. V. Mosby Co.
Volume 82 Number2 August, 1981
tory of previous rheumatic fever or infective endocarditis were not included, unless those lesions arose after the onset of insufficiency. The patients were assigned to three groups on the basis of their therapeutic management. The first group consisted of six patients who never underwent operation. They were evaluated over a 5 to 17 year period (median 6.5 years). These patients constituted the nonsurgical group. Median age at the time of initial evaluation was 3 years. The second group of 16 patients was also treated medically first, during a 2 to 19 year period (median 5 years), to monitor each patient's course and possible progression of insufficiency. They later underwent operation at a median age of 7 years. Postoperative evaluations were then continued among 15 of these patients from 1 to 24 years to evaluate surgical effectiveness in correction of AI. One child was lost to follow-up soon after operation and was not included in any of the postoperative groups. Owing to the chronological order of surgical intervention during their course of management, these patients were referred to as the late surgical group. The third group consisted of 10 patients, who were referred from other institutions specifically for surgical correction of VSD and AI. Since these patients originated from beyond our geographical area and medical information was often inadequate to evaluate any preoperative progression in degree of AI, these children were not included in the natural history of AI. One child was lost to follow-up after catheterization and prior to surgical intervention. The remaining nine children all underwent operation within a year of our initial examination and did contribute to our evaluation of surgical effectiveness in improving or eliminating insufficiency. However, time of clinical onset of insufficiency as well as preoperative catheterization data were known in each patient. To separate them from those patients we had evaluated prior to operation, we assigned these patients to the referred surgical group. Median age at operation was 6 years. Postoperative evaluations were then continued over a 2 to 15 year period. Hemodynamic data. Initial preoperative catheterization data were available on all 32 patients. Patient age at documentation of insufficiency, shunt size as determined by the Qp/Qs ratio, left ventricular enddiastolic pressure, and pulse pressure were analyzed from available catheterization data. Position of the VSD and aortic cusp involvement were determined by both left ventricular and aortic root angiograms in all cases and subsequently confirmed in
VSD with aortic insufficiency
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19 (59%) and 14 (44%) cases, respectively, at the time of operation or autopsy. Degree of AI was quantitated by the method of Lehman, Boyle, and Debbas'"; severe (4+), rapid reflux with uniform and maximal opacification of the left ventricular chamber; mild (l ,2+), minor degrees of reflux; and moderate (3+), an intermediary position between the two extreme degrees of regurgitation. Surgical technique. Initial surgical technique utilized total cardiopulmonary bypass with identification of the VSD either through a right ventriculotomy or a right atrial and tricuspid valve approach. Closure of the VSD was either by direct suture (eight patients) or use of a Dacron patch (16 patients). Aortic valve repair was performed in 13 patients. A transverse aortotomy was made across the base of the ascending aorta to expose the prolapsed leaflet. Valvuloplasty was performed by reapproximating and resuspending the prolapsed valve cusp at the commissural margins to the adjoining valve cusps. Excess leaflet tissue was plicated with sutures from the commissures through the aortic wall. In five patients the plication was reinforced with pledget-supported mattress sutures from outside to inside the aorta. The aortotomy and right ventriculotomy were closed with continuous sutures. Two patients, one of whom had a bicuspid aortic valve, received aortic valve replacement. To evaluate the effectiveness of operation for correction of the insufficiency, comparisons were made among techniques of VSD repair and association of AI repair, patient ages at time of operation, locations of the VSD, and degrees of AI. Both the t test for independent means and chi square analyses were used to evaluate statistical significance of results. Significance was defined as exceeding the 95% confidence level (p < 0.05). Results Hemodynamic data. Cardiac catheterization data at the time of initial evaluation for suspected AI are listed in Table I. The degree of AI was found to be mild in 21, moderate in eight, and severe in three patients. Although the left ventricular end-diastolic pressure and Qp/Qs did not differ significantly among the three degrees of insufficiency, pulse pressure did correlate (p < 0.05) with the angiographic estimation of valve reflux as a percentage of systolic pressure. Position of the VSD was infracristal in 17 (53%) and supracristal in 10 (31 %) cases. Position could not be determined accurately from available data in five cases (16%). Aortic valve prolapse involved the right coronary cusp in 16 (50%), noncoronary cusp in nine
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Table I. Catheterization data at initial Al evaluation Pressure (mm Hg)
I
Case No.
Age (yr)
Aorta
Left ventricle
I 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
3 10 2 1.5 5 12 3 11 6 14 10 1.5 1 5 3 17 12 9 5 5 5 [5.0]"
90/40 120/80 105/60
130/0,10 105/0,10
100/65
105/0,9
130/80 105/70 125/75 110/75 100/40 100/50 125/65 100/60 95/65 130/80 100/60 130/70 125/75 108/55 100/70 [105/65]
130/0,20 105/0,10 100/0,14 120/0,5 95/0,5 90/0,5 125/0,10 100/0,10
22 23 24 25 26
9 3 5 3 1 7 5 7 [5.0]
130/70 100/47 85/35 130/43 120/50 95/60 90/30 110/40 [105/44]
3 12 4 [4.0]
120/35 170/80 100/40 [120/40]
Qp:Q.
Mild
NA
NA
NA
NA
95/0,13
130/0,18 100/0,16 128/0,5 125/0,5 100/0,13 [105/0,10]
2.8: I 1.7: I 1.9: 1 1.8: I 1.3: 1 1.2: 1 1:1 2.4: I 1.5: I 1.6: I 1.5: I 1.8: I 1.4: 1 1.3: I 1.5: 1 1.7: 1 1.4: I 1.8: 1 [1.5:1]
Pulse pressure <50% systolic
No Yes Yes Yes Yes Yes Yes Yes
No
Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes
Moderate
27
28 29
130/0,10 95/0,10 85/0,13
100/0,8 90/0,20 [95/0,10]
1:[ 2.3: 1 1.4: 1 1.2: 1 1.4: 1 2.8: 1 1.5: I 2: I [1.4: 1]
Yes
No No No No
Yes
No No
Severe
30 31 32
120/0,21 115/0,7 100/0,10 [115/0,10]
1:1
No No No
[1 : I]
Legend: AI, Aortic insufficiency. NA, Not available.
• Brackets indicate median value.
(28%), and both the right and noncoronary cusps in two (6%) patients. Cusp involvement could not be determined in five (16%) patients. Natural progression of AI. As shown in Fig. 1, only one of the nonsurgical patients has exhibited angiographic progression of insufficiency over a 6.5 year median follow-up period; all have remained asymptomatic. The other four children are currently being followed for evidence of deterioration in cardiac status. The one child with an initially severe degree of AI
remained stable for 15 years prior to a fatal episode of infective endocarditis. The remaining data in Fig. I illustrate the progression of AI among those patients whom we were able to follow prior to surgical intervention. Six patients initially exhibited mild AI and no symptoms. During a median of 6.5 years the degree of insufficiency progressed in three of these patients. Only the one who had severe AI demonstrated any clinical change, i.e., onset of exercise intolerance at 23 years of age. There was no
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N • 6 FOLLOWED 5 - 17 Y
N • 16 FOLLOWE 0 2 - 19 Y PRE-OPERA TI VE
MODERATE
MILD
SEVERE
DEGREE AI
INITIAL FOLLOW-UP
,/: ), e: 3.5 Y
6.5 Y
MODERATE
MILD
6.5 Y
14Y 1--1
1
SEVERE
SEVERE
MILD
INITIAL PRE-OP
,/:
l5Y 1 - - 1 (OIED)
Fig. 1. Progression in degree of aortic insufficiency (AI) among the 16 patients followed prior to operation (' 'late surgical" group) and the six patients who have not undergone any operation ("nonsurgical" group). Number of patients at each level of valvular incompetence followed over the listed median time intervals. N • 7 I 9 (77%)
N • 12 I 16 (75%)
MODERATE
MILD DEGREE AI
PRE
POST
4 Y
4
NONE
22 Y ~ED)
1
7/1\
MODERATE MILD
PRE POST POSH PRE
2.5 Y
SEVERE
SEVERE
-,
3- 1
\2
\
\ 2
'I (AVRI
POST
5 Y 2
\,
llAVR)
MILD PRE POST
SEVERE PRE POST
4
/:
2
1 \1
6- 1
\3
Fig. 2. Operative results among the 16 patients followed prior to operation ("late surgical" group) and nine of the patients referred for operation ("referred surgical" group). Preoperative and postoperative degrees of aortic insufficiency (AI) with number of patients at each level of valvular incompetence followed over the listed median postoperative period. AVR. Aortic valve replacement. * Patient required repeat operation.
progression in degree of insufficiency in one patient who had infective endocarditis during this period. One of the patients with initially moderate AI showed progression to severe AI; all, however, continued to be asymptomatic, although two exhibited compensated heart failure when treated with digitalis and diuretics. The one patient with an initially severe degree of AI remained asymptomatic for 14 years, having no abnormalities on a treadmill exercise test performed at 16 years of age. During a repeat test performed 2 years
later, however, ST-segment changes were noted on the electrocardiogram. Postoperative results. A total of 24 patients underwent operation, between the ages of 1 and 23 years (median 6.5 years); 12 of these were 6 years or younger. Among the nine children referred specifically for operation, there was little previous medical data available with which to judge the antecedent clinical course and subsequent timing for surgical intervention. The 16 patients of the late surgical group, however, had all been evaluated in this institution several times prior
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Table II. Operative results
Procedure Patch/valvuloplasty
Age at operation (yr)
3 3 6 6 9 5 6 12 6 5 10
Postoperative
Site ofVSD
Degree of Al
Al
VSD
II II II II I II I II NA II II
Moderate Moderate Mild Severe Mild Moderate Moderate Severe Mild Mild Moderate
Yes* No No No No Yes*t Yest Yest No No NA
Yes* No No No* Yes Yes* No No Yes No NA
II NA II II II
Mild Moderate Mild Mild Moderate
Yes* Yes* Yes No Yes
Yes* Yes* No No Yes
I I II II
Mild Moderate Mild Mild
Yes* Yes* No Yes
No* Yes* No No
3
II II
Mild Moderate
No Yes*
Yes Yes*
4 2
Severe (B) Severe
No No
No No
[6.0]* Patch only
I
7 12 14 5 [7.0] Primary closure
4 7 II
10
I
FlU (yr)
7 0.75 4 5 4 4 3 2 3 I
[3.5] 10 I
3 3 2 [3.0] 15 24 I
[9]
[9.0] Primary closure/valvuloplasty
3 II
Primary closure/A VR
[7.0] 23 18 [20.5]
[3]
[ I]
Legend: VSD. Ventricular septal defect. AI. Aortic insufficiency. FlU. Follow-up. VSD: I. supracristal; II. infracristal. AVR, Aortic valve replacement. NA, Not available. B, Bicuspid valve. * Catheterization confirmation. t Improvement in degree of residual AI. Brackets indicate median value.
*
to operation. Among this latter group, correction was recommended in five patients (four with moderate and one with severe AI) on the basis of cardiac dysfunction as determined by increasing electrocardiographic hypertrophy or strain. Four of these children also exhibited increasing cardiomegaly by chest radiograph, three of whom had no evidence of increase in shunt size at preoperative catheterization. The remaining 11 of these 16 patients showed no clinical change at the time of repair when compared with initial evaluation. Degree of AI. The postoperative results in degree of insufficiency among the two surgical groups are presented in Fig. 2. Long-term postoperative data were available in 12 of the 16 patients initially from this center (median age at operation 7 years; median preoperative Qp/Qs 1.5: 1). Only three (25%) patients, not having an initial valve replacement, exhibited no residual AI. There was persistence of valvular regurgita-
tion in eight (66%) patients, with progression in degree of AI in four of these children. Two of these patients, as described, had exhibited evidence of cardiac decompensation preoperatively, demonstrated by increasing cardiomegaly and ventricular hypertrophy or strain. Because of a deterioration in clinical status during the postoperative period, all four patients required a second operation, during which two died. Only one of the survivors did not have a valve replacement and she continues to have severe residual AI 22 years later. Among the 10 patients referred specifically for surgical repair, long-term postoperative data were available in seven (median age at operation 6.5 years; median preoperative Qp/Qs 1.7: 1). Among the six patients with initially mild AI, there was progression of insufficiency after a median 12.5 years in two following ineffective patch repair of the VSD in one and primary closure in the other child (median age at operation 3
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~
f----
SEVERE MOOERATE MILD NONE
1-4YEARS
1-9YEARS
N • 4 I 19
N • 9 I 19
NOT IMPROVEO PRE
t
POST
IMPROVEO PRE
POST
<,
NOT IMPROVEO PRE
POST
/' ----.0
'----
10 - II YEARS N • 6 I 19
IMPROVlO POST
PRE
~
NOT IMPROVlO PRE
POST
/
t
---.0 ----.0
IMPROVlO PRE
POST
~ ~ \
t • DIED
Fig. 3. Separation of patients according to age at operation with preoperative and postoperative degrees of aortic insufficiency (AI). Each dot represents one patient. Dashed line indicates valve replacement. Improvement is based on elimination or improvement in degree of AI.t Patient died. years). Both of these children later required valve replacement. The one patient with an initially severe degree of prolapse did improve after operation. Surgical technique. As presented in Table II, excluding valve replacement as an effective method of correcting insufficiency, the majority of our patients exhibited the most successful results from patch closure of the VSD associated with plication of the prolapsed valve cusp. Although residual AI was found in four (40%) of these 10 children after a median of 3.5 years, the degree of AI was improved in three. The other listed techniques performed at comparable patient ages, degrees of AI, and after sufficient postoperative intervals were associated with a significantly higher incidence (p < 0.05) of residual insufficiency. Closure of the VSD alone did not prove to be effective in preventing the progression of insufficiency. Of the nine patients in whom aortic valvuloplasty was not performed, seven (77%) had residual AI, with postoperative progression in degree of insufficiency noted in three of these patients after a median 10 year follow-up period. Age at the time of operation. To evaluate the effect of age at the time of operation as a contributory factor toward a successful outcome, all patients were compared by means of preoperative and postoperative estimates of AI. As presented in Fig. 3, there was a statistical difference (p < 0.05) among the age groupings and successful resolution of AI. There were four children less than 5 years of age at the time of operation. Although the degree of AI was only mild or moderate, operation resulted in improvement in only one; in the remainder the degree of insufficiency progressed. In comparison, among our patients between the ages of 5 and 9 years of age,
who exhibited all three degrees of valvular incompetency, six (66%) showed improvement. Likewise, children over 10 years of age also manifested favorable outcomes.
Discussion Aortic insufficiency (AI) is generally considered to be a progressive lesion. 4. 8. 9. II. 14 In the pediatric age range, this may occur at a variable and slow rate,": 3. 5. 14. 18 even without a deterioration in clinical status. In this study, only five of our 21 children with AI manifested progression in the degree of insufficiency over a median of 5 years. Only one of these, with onset of AI in late adolescence, became symptomatic. His course is compatible with the theory": 5 that rapid progression may be more prevalent in late-onset regurgitation. Such a variable and slow progression in valvular incompetence has caused much controversy as to the proper timing and type of operation to correct or at least arrest the progression of insufficiency. This present study attempts to add to knowledge in this field by reviewing the effects of different surgical techniques performed on a population of young children, the majority of whom had only minimal valvular involvement, after postoperative periods extending to 24 years. The success rate in eliminating or minimizing the degree of valvular incompetence varies. Some authors have reported that VSD closure alone is sufficient in supracristal lesions," 6. 8. 9. 16 whereas others have found the need for associated valvular repair. 14. 15. 19 Among our four patients with supracristal lesions not having valve replacements, residual AI persisted in three children after a median follow-up period of 9.5 years. The AI either improved or was eliminated in the two children who had VSD closure plus valvuloplasty
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(median age 7.5 years). The degree of AI did not change in those patients with VSD closure alone (median age 5.5 years). Unfortunately, the small number of patients does not permit statistical differences between the surgical methods. Other reports describe patients with supracristal lesions comparable to ours in age and type of operation. 4.6 Their postoperative data show similar findings, which would favor the theory that insufficiency may not be a totally acquired lesion, but rather involves an inherent defect in valvular embryogenesis.v 14. 17 Correction of regurgitation associated with an infracristal lesion is a technically demanding operation. Surgical procedures on the aortic valve have included variations in cusp plication ': 5. 6. 9. 1:1. 15 as well as partial or total valve replacement. 2. 5. 6. 12. 14. 19 Neither of these techniques has yielded totally satisfactory results. Eight of our 15 patients (53%) with infracristal lesions manifested residual AI after a median postoperative period of 2.5 years. Three of these patients, whom we were able to follow longer (median 7 years), showed progression in degree of AI. From our data, both closure of the VSD with and without repair of the aortic valve proved to be equally effective in completely eliminating insufficiency in children with infracristallesions. However, there was a significant difference (p < 0.025) in improvement in degree of AI in those patients who underwent an associated valvuloplasty. Likewise, statistical analysis of the entire postoperative patient population, combining both supracristal and infracristal lesions, reveals a significant difference (p < 0.01) in success rate of eliminating or improving the degree of valvular insufficiency when repair of the prolapsed valve cusp was performed at the time of VSD closure. Ten of our 12 patients (83%) who underwent an associated valvuloplasty were improved. This result compares with other reports which describe slightly different methods of valvuloplasty. 6. 1:1. 15 In our experience, patients with and without plication reinforcement with pledgets manifested equally successful results regardless of age, location of the VSD, or preoperative degree of AI. Two of our children, both with residual VSDs, continued to have unimproved AI. Persistent lack of valvular support and hemodynamic contributions from the VSD may have prevented satisfactory surgical results in these patients. The majority of our patients demonstrated no evidence of cardiac dysfunction prior to operation. Among the four children with electrocardiographic and radiographic evidence of ventricular hypertrophy and dilatation, repair was ineffective in two. One of these chil-
dren had onset of AI during adolescence which, as previously discussed, may be associated with rapid progression of valvular deformity. The other child demonstrated an increase in shunt size (Qp/Qs 1.2: 1 to 2.8: 1) during the 7 year preoperative period. Although the small number of patients does not permit statistical evaluation, operation performed prior to evidence of cardiac decompensation may result in a more favorable surgical outcome. The final variable that we examined to determine effectiveness of surgical intervention was patient age at the time of operation. Compared to the numerous reports of operation performed on older children and adolescents, very few studies have included young children, less than 5 years of age. Our current review includes four such children, whom we have been able to evaluate for a median 8.5 years after operation. Only one of these younger children exhibited any definite benefit from early operation; the remaining three all manifested an increase in the degree of valvular incompetence. As opposed to this, children in the latter part of the first decade of life exhibited a far greater success rate (p < 0.05) in total repair or improvement in valvular regurgitation. There was no significant difference in the preoperative degree of AI or surgical techniques in the older children as compared with the younger children. The older children actually exhibited more preoperative valvular involvement. Since surgical techniques have varied over the past 25 years, future data may yield different outcomes from those presented in this review. Nevertheless, several recommendations can be advocated. Surgical corrections of VSD and AI is a feasible procedure in children. However, as the degree of AI can progress slowly without compromising the child's development, there is time for regular and careful medical supervision with operation being reserved for those patients who exhibit a change in cardiac status prior to any overt clinical deterioration. The ineffective surgical outcome seen in younger children, especially with minimal valvular involvement, would preclude early operative intervention unless medical management fails to prevent cardiac decompensation. Because of potentially inherent defects in the aortic valve itself, apart from any hemodynamic contributions, repair of the prolapsed aortic valve cusps can contribute to a more effective surgical outcome. REFERENCES Nadas A, Thilenius 0, Lafarge C, Hauck A: Ventricular septal defect with aortic regurgitation. Medical and pathologic aspects. Circulation 29:862-873, 1964 2 Gonzalez-Lavin L, Barratt-Boyes B: Surgical consid-
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3
4
5
6
7
8
9
10
erations in the treatment of ventricular septal defect associated with aortic valvular incompetence. J THoRAc CARDIOVASC SURG 57:422-430, 1969 Glasser S, Cheitlin M, McCarty R, Haas J, Hall R, Mullins C: Thirty-two cases of intraventricular septal defect and aortic insufficiency: Clinical, hemodynamic and surgical features. Am J Med 53:473-480, 1972 Chung K, Manning J: Ventricular septal defect associated with aortic insufficiency. Medical and surgical management. Am Heart J 87:435-438, 1974 Keane J, Plauth W, Nadas A: Ventricular septal defect with aortic regurgitation. Circulation 56:Suppl 1:72-77, 1977 Moreno-Cabral R, Mamiya R, Nakamura F, Brainard S, McNamara J: Ventricular septal defect aortic insufficiency: Surgical treatment. J THoRAc CARDIOVASC SURG 73:358-365, 1977 Corone P, Dayton F, Gaudeau S, Guerin F, Vernant P, Duncam H, Rumeau-Rouquette C, Gaudeul P: Natural history of ventricular septal defect. A study involving 790 cases. Circulation 55:908-915, 1977 Van Praagh R, McNamara J: Anatomic types of ventricular septal defect with aortic insufficiency. Diagnostic and surgical consideration. Am Heart J 75:604-619, 1968 Kawashima Y, Danno M, Shimizu Y, Matsuda H, Miyamoto T, Fujita T, Kozuka T, Manabe H: Ventricular septal defect associated with aortic insufficiency. Anatomic classification and method of operation. Circulation 47:1057-1064, 1973 Spencer F, Bahnson H, Neill C: The treatment of aortic regurgitation associated with a ventricular septal defect. J THORAC CARDIOVASC SURG 43:222-233, 1962
II Dimich I, Steinfeld L, Litwak R, Park S, Silvers N: Subpulmonic ventricular septal defect associated with aortic insufficiency. Am J Cardiol 32:325-328, 1973 12 Plass R, Munster W, Ivanov S, Sturner V: Angiographic diagnosis of aortic insufficiency in cases of ventricular septal defect associated with partial prolapse of the aortic valve. Cardiology 58:257-272, 1973 13 Spencer F, Doyle E, Danilowicz D, Bahnson H, Weldon C: Long-term evaluation of aortic valvuloplasty for aortic insufficiency and ventricular septal defect. J THoRAc CARDIOVASC SURG 65:15-31, 1973 14 Somerville J, Brando A, Ross D: Aortic regurgitation with ventricular septal defect. Surgical management and clinical features. Circulation 41:317-330, 1979 15 Trusler G, Moes C, Kidd B: Repair of ventricular septal defect with aortic insufficiency. J THoRAc CARDIOVASC SURG 66:394-403, 1973 16 Robinson G, Fell S, Jacobson B: Ventricular septal defect with aortic insufficiency. A method of management. J THoRAc CARDIOVASC SURG 43:785-791, 1962 17 Lehman J, Boyle J, Debbas J: Quantitation of aortic valvular insufficiency by catheter thoracic angiography. Radiology 79:361-369, 1962 18 Halloran K, Talner N, Browne M: A study of ventricular septal defect associated with aortic insufficiency. Am Heart J 69:320-326, 1965 19 Ellis F, Ongley P, Kirklin J: Ventricular septal defect with aortic valve incompetence. Surgical considerations. Circulation 27:789-795, 1963