Ann Thorac Surg 1997;64:1146-1149
© 1997 The Society of Thoracic Surgeons
Original Article: Cardiovascular
Surgical Strategy for Doubly Committed Subarterial Ventricular Septal Defect With Aortic Cusp Prolapse
Hiroyoshi Komai, MD,
Yasuaki Naito, MD,
Keiichi Fujiwara, MD,
Yasuzo Noguchi, MD,
Yoshiharu Nishimura, MD,
Shigeru Uemura, MD
Departments of Thoracic and Cardiovascular Surgery and Pediatrics, Wakayama Medical College, Wakayama, Japan
Accepted for publication April 3, 1997.
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Abstract
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Background. Many surgeons recommend early repair of doubly committed subarterial ventricular septal defect regardless of the clinical symptoms. We reviewed our patients of this anomaly with aortic cusp prolapse to justify this strategy.
Methods. We reviewed the preoperative and postoperative records of 27 patients with doubly committed subarterial ventricular septal defect and aortic cusp prolapse. The patients' ages ranged from 2 months to 11 years (median, 4.6 years).
Results. During the preoperative observation period, aortic regurgitation (AR) developed in 65% of the patients. In the 8 patients without AR before the operation, AR did not develop after the operation, whereas AR persisted in 12 (63%) of the 19 patients with preoperative AR. To identify the risk factors for persistent AR after the operation we analyzed the data for the patients with preoperative AR in the persistent AR group (n = 12) and eliminated AR group (n = 7) and found a longer period from the onset of AR to the operation in the persistent AR group (32.1 ± 10.1 versus 5.6 ± 1.9 months; p = 0.014). During the follow-up period 10 of the 17 patients with mild AR before the operation showed persistent AR in the postoperative period, but it did not progress.
Conclusions. We conclude that early surgical repair with a minimum observation period is essential for prevention of residual AR. Even if a tiny AR is detected preoperatively, the patient should be surgically treated immediately.
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Introduction
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Although a doubly committed subarterial ventricular septal defect (VSD) is relatively uncommon in Western countries, the incidence of this anomaly is approximately 30% among surgical patients with ventricular septal defects in Asian countries [1]. This type of VSD is often associated with aortic cusp prolapse and aortic regurgitation (AR). The prolapsed coronary cusp tends to lead to the established deformity and is reported to possibly be associated with rupture of the sinus of Valsalva in adulthood, which is also very common in most Asian countries [2]. In Japan, there is a widely accepted consensus as to the management of patients with this type of defect, and thus most of the patients undergo operation during the first decade of life even if there have been no clinical symptoms. Many surgeons recommended that patients with doubly committed subarterial VSD should be operated on as soon as the diagnosis is established [3, 4]. We reviewed the records of our patients with doubly committed subarterial VSD and aortic cusp prolapse in an attempt to justify this surgical strategy.
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Patients and Methods
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Twenty-seven patients with doubly committed subarterial VSD and aortic cusp prolapse (all in the right cusp) in the period of 1985 to 1995, who had been followed up after the corrective operation for 1 year or more, were enrolled in this study. The patients' age and body weight at the time of the operation ranged from 2 months to 11 years (median, 4.6 years) and from 4.9 to 33.0 kg (median, 15.0 kg), respectively. Their preoperative and postoperative clinical records, echocardiograms, and cardiac catheterization results were reviewed retrospectively.
They routinely underwent echocardiography performed by our pediatric cardiologists once every 6 months before the operation, once every month in the early postoperative period, and once or twice each year in the late postoperative period. All patients underwent preoperative cardiac catheterization study. Their mean pulmonary arterial pressure was 22.0 ± 16.3 mm Hg, and the pulmonary-to-systemic flow ratio was 1.44 ± 0.47. The aortic cusp prolapse and AR were diagnosed either by two-dimensional and color Doppler echocardiography or by retrograde aortography. Aortic regurgitation was graded with color Doppler echocardiography as mild for the regurgitation jet just beneath the aortic valve and moderate for the regurgitation jet beyond the anterior cusp of the mitral valve but not reaching the apex. (We had no patient with severe AR in this group.)
The operations were all performed by the same surgical team using our established routine method of patch closure of the VSD through the pulmonary arteriotomy under extracorporeal circulation with moderate hypothermia and cold crystalloid cardioplegic arrest. We performed the aortic valvoplasty (redundant cusp plication) when the preoperative AR was evaluated as moderate, and we did this in 2 children in this group simultaneously with the VSD closure. The rest of the patients underwent patch closure even if they had had mild AR.
The results are presented as mean ± standard error of the mean. Mann-Whitney U test was used to assess the differences between the patient groups. Values were considered to be statistically significant when the p value was less than 0.05.
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Results
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Preoperative Observation Period
Four patients already had AR when they were referred to our hospital. Among the other 23 patients, AR developed in 15 (65%) during the preoperative observation period. In all patients with AR the grade was mild except in 2, 1 of whom had already had mild AR at his initial visit and the other of whom did not. In both children the grade progressed to moderate during the observation period. We performed aortic valvoplasty for these patients, but mild AR remained after the operation. In 1 patient the grade of AR is still progressing in the moderate range after the operation.
Relation of Preoperative Aortic Regurgitation and Persistent Aortic Regurgitation
In 8 patients without AR before the operation, AR did not develop after the operation. On the other hand, 12 (63%) of 19 patients with preoperative AR had postoperative AR, and in only 7 patients did the AR disappear.
Factors Affecting Persistent Aortic Regurgitation
To identify the risk factors for persistent AR after the operation we analyzed the data for the patients with preoperative AR in the persistent AR group (n = 12) and eliminated AR group (n = 7). Between these two groups there were no significant differences in the age at the operation, the age at the first diagnosis of the AR, the body weight at the operation, the mean pulmonary arterial pressure, the pulmonary-to-systemic blood flow ratio, the left-to-right shunt in the preoperative catheter study, and the size of the VSD (Table 1
). The period from the first diagnosis of the AR to the operation (the waiting period), however, was significantly longer in the persistent AR group (32.1 ± 10.1 months) than that in the eliminated AR group (5.6 ± 1.9 months; p = 0.014) (Fig 1).
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Fig 1. . The period from the first diagnosis of aortic regurgitation (AR) until the operation (the waiting period) was significantly longer in the persistent AR group (32.1 ± 10.1 months) than in the eliminated AR group (5.6 ± 1.9 months; p = 0.014).
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The retrograde aortographic data obtained before the operation were available for the assessment of the flexibility of the cusp in 11 patients. The prolapsed cusp was fixed and did not change the shape of the protruded portion in both the systolic and diastolic phase in 4 of the 6 patients in the persistent AR group (Fig 2A), whereas in the eliminated AR group the cusp was flexible in 4 of the 5 patients (Fig 2B).
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Fig 2. . Retrograde aortography before the operation. In the group with persistent aortic regurgitation the prolapsed cusp was fixed and did not change the shape of the protruded portion in both the systolic and diastolic phases in 4 of the 6 patients (A), whereas in the group with eliminated aortic regurgitation the cusp was flexible in 4 of the 5 patients (B). The arrow indicates the site of prolapse.
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Postoperative Follow-up
There was no early or late death and no severe complication in the 27 patients. The follow-up period of AR after the operation ranged from 1.0 to 7.7 years (median, 3.0 years). Figure 3 illustrates the grade of AR during each phase of the period. In 15 patients without AR in the early postoperative period, AR did not develop thereafter. Ten patients with mild AR after the operation continue to show regurgitation in the late period, but it did not progress to moderate or severe. One of the 2 patients who had had moderate AR before the operation continues to show mild regurgitation, and the AR progressed to moderate 5 years after the operation. He had shown AR at his initial visit to our outpatient clinic, but he and his family members had refused to consent to the operation for 4 years. In the other patient with moderate AR it persisted but it did not progress in the late period.
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Comment
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With the recent trend toward earlier operation for VSD, we seldom encounter a patient with VSD with massive AR, but in some patients with this anomaly severe AR develops from the very early period of life. Once the aortic cusp deformity occurs to a certain level surgeons find difficulty in repairing it completely and sometimes an artificial valve replacement is required before adolescence. Because we surgeons in Japan have experienced many patients with this anomaly we have come to schedule such patients for operation earlier than one would consider is warranted by the clinical symptoms. But in our hospital as a regional medical center we could not always plan the operation ideally because of the limited capacity of the hospital. In our basic strategy during study period, once the cardiologist found the prolapse we carefully followed up the patient, mainly by echocardiography, and if the prolapse was large or progressive we considered surgical repair. When the patient presented heart failure, pulmonary hypertension, or AR, we performed a catheter study, followed by the operation as soon as possible. Fortunately, we have encountered no patient with severe AR in the interval we have applied this strategy.
During the observation period before the operation AR develops in quite a high percentage of the patients, which indicates that this disease is progressive, as many reports have suggested [5–7]. Previous reports point out that the AR develops at a mean patient age of 6 years or older. We can detect trivial AR at the median age of 3 years by color Doppler echocardiography. The youngest patient in whom AR developed was 1.5 years old, which is consistent with other reports demonstrating that AR can develop quite early [6, 7]. One must not consider the pulmonary-to-systemic flow ratio as an indicator of when to operate because severe deformity of the cusp often closes the defect and decrease the left-to-right shunt.
No patient without AR had development of AR in the early or late postoperative follow-up period. The AR is caused by anatomic and hemodynamic factors [8], and we believe that the patch closure of the VSD is adequate to eliminate the progression of both factors as long as the cusp deformity is not severe. We particularly focused on the patients with persistent AR and attempted to ascertain the factor(s) most affecting persistent AR in the VSD patients with preoperative AR, because even mild AR retains the potential for progression [3, 9] and for the development of infective endocarditis [9, 10] in the long life after the operation. The age at operation and the shunt flow across the defect did not differ in the groups with and without persistent AR. The main point from this study we would stress is the importance of the duration of the "waiting period." Although the grade of preoperative AR is the same (actually, in most of the patients the grade was even trivial rather than mild), the patients with postoperative persistent AR had waiting periods of as long as 3 years. The waiting period was longer than 10 months in all the patients with persistent AR except 1, whereas all the patients with eliminated AR underwent the operation within a year. The long duration of AR could cause morphologic changes on the cusp and decrease the flexibility of the cusp, as was shown in the aortography conducted by others [11] and ourselves. Clearly, the best way to avoid AR is to operate on the patient as soon as the diagnosis is established, as many surgeons have suggested [3, 4]. And if the patient with even a tiny AR is referred to a surgical team we strongly recommend that one proceed with the operation as soon as possible.
There are many reports on the long-term results of aortic valvoplasty for VSD with AR. The results are excellent, but there are still some patients who retain persistent AR and even show progression of AR to the extent that valve replacement is required [11–13]. In our group 1 patient retained mild AR after the closure of the VSD with valvoplasty and had development of moderate AR in the late phase. This patient had a long waiting period, which might have affected the progress in the late postoperative period. The patient has few clinical symptoms at present, but he should be followed up very carefully.
We conclude that the most prudent strategy for the patient with VSD and aortic cusp prolapse is early closure of the VSD before the onset of AR. Because of these data we now think we should put the patient on the operating list earlier than before. Currently the results of VSD closure in infancy are excellent with minimal mortality, and thus we know of no valid reason to wait until AR develops.
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Footnotes
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Address reprint requests to Dr Komai, Department of Thoracic and Cardiovascular Surgery, Wakayama Medical College, 27, 7-bancho, Wakayama, 640 Japan.
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References
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Abstract
Introduction
