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Ann Thorac Surg 2005;79:47-51
© 2005 The Society of Thoracic Surgeons
a Department of Cardiovascular Surgery, Pediatric Cardiac Surgery Unit, University of Padova Medical School, Padova, Italy
b Department of Pediatrics, University of Padova Medical School, Padova, Italy
c Department of Anaesthesia, University of Padova Medical School, Padova, Italy
Accepted for publication February 3, 2004.
* Address reprint requests to Dr Vida, Department of Cardiovascular Surgery, Pediatric Cardiac Surgery Unit, University of Padova Medical School, Via Giustiniani, 2-35128 Padova, Italy
vladimirovida{at}interfree.it
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Abstract |
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METHODS: Since 1989, 11 of the 38 patients who survived aortic balloon dilation (28.9%) have undergone surgical treatment for residual aortic valve dysfunction. Median time from aortic balloon dilation to surgical intervention was 7 months (range 1 to 56 months). Residual aortic stenosis was the predominant problem in 8 patients and aortic regurgitation was predominant in 2 patients.
RESULTS: Aortic valvuloplasty was possible in 5 children; pulmonary autograft replacement of the aortic valve was performed in 6 children. Two children underwent a Ross-Konno procedure because of annulus hypoplasia and severe left ventricular outflow tract obstruction. Two early deaths occurred after a Ross-Konno procedure, both with findings of severe left ventricular fibroelastosis at the pathologic examination. Median follow-up time was 5 years (range 1 month to 11.9 years). No late deaths occurred. One patient with moderate-severe aortic valve regurgitation after aortic valvuloplasty underwent a successful Ross operation. All 9 patients are asymptomatic and are in good clinical condition.
CONCLUSIONS: We are convinced that the best aortic valve in the pediatric age group is the native one, provided it can function acceptably. However, in cases where conservative surgical treatment fails to yield a functional aortic valve, replacement of the valve is indicated, and the best aortic valve substitute in infants is the pulmonary autograft because of its potential for growth.
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Introduction |
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TopAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Aortic valve balloon dilation has become the procedure of choice in many centers for the treatment of critically ill infants with severe aortic valve disease [2]. However, reports of surgical results in infants indicate that repair of the aortic valve can be performed with low risk and with 85% freedom from reintervention at 5 years [3].
Some have suggested that these infants should be treated only by surgery, because it allows more precise plasty of the valve and may prolong the interval before a valve replacement must be attempted [3]. We present a study of our recent experience with infants who were treated surgically after aortic valve balloon dilation for severe aortic valve stenosis in the first 3 months of life.
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Material and Methods |
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TopAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Median age at the balloon dilation procedure was 17 days (range 1 to 80 days). There was one death (2.6%) from ventricular fibrillation related to the procedure and one serious complication (2.6%), a fistula between the aorta and the right atrium that was closed surgically 2 days later. No complications were related to the transcarotid approach, and a right carotid Doppler at discharge was normal in all patients. One patient died 3 months after the procedure with findings of severe endocardial fibroelastosis at the postmortem examination.
Between January 1990 and April 2001, 11 of the 38 patients who survived aortic balloon dilation (28.9%) underwent surgical treatment for residual aortic valve disfunction. The median time from aortic balloon dilation to surgical intervention was 7 months (range, 1 to 56 months). The median age at surgical treatment was 7 months (range, 2 to 59 months). Four children presented with congestive heart failure, the remaining 7 were asymptomatic and underwent elective valve repair. The electrocardiogram showed sinus rhythm in all. Mean cardiothoracic ratio was 0.57, ranging from 0.51 to 0.68.
All of the children were evaluated preoperatively by means of two-dimensional echocardiography and color flow Doppler. Aortic stenosis was the primary residual problem in 8 patients. The transaortic gradient was between 50 and 70 mm Hg (median, 56 mm Hg). Aortic regurgitation was the predominant problem in 3 patients (moderate in 2 and severe in 1). Left ventricular hypertrophy was present in all. Preoperatively, 4 patients had severe left ventricular dysfunction, and signs of left ventricular fibroelastosis were present in 2 patients. Associated cardiac lesions that were repaired at the same operation included subaortic discrete fibrous membrane (3 patients), mitral valve dysplasia with predominant stenosis (2 patients), and aortic arch hypoplasia (1 patient). Two patients had undergone cardiac surgery previously. Patient 6 (Table 1) had coarctation repair and patient 11 (Table 1) had aorto-right atrial fistula closure.
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Surgical Technique
All patients underwent intraoperative transesophageal or epicardial (patients < 6 kg body weight) echocardiography [6]. Particular attention was paid to the aortic valve morphology and the mechanism of valve dysfunction.
After aorto-bicaval cardiopulmonary bypass with moderate systemic hypothermia was established, aortic cross-clamping and antegrade/retrograde blood cardioplegia was employed in all. The aortic valve was exposed through a transverse aortotomy and carefully inspected. The aortic valve was bicuspid in 8 patients and tricuspid in the other 3 patients. Valve dysfunction was because of combined anatomic factors such as dysplastic and thickened leaflets (6 patients), leaflet fusion at the commissures (5 patients), annulus hypoplasia (2 patients), torn leaflet after balloon valvuloplasty (2 patients), fibrous subaortic stenosis causing aortic valve leaflet distortion (1 patient), and pseudocommissure distorting the valve leaflet (1 patient). Figure 1 shows the surgical findings for these patients. Details of the surgical techniques that were used in each patient are summarized in Table 1 and shown in Figure 2.
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Results |
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TopAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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The final decision whether to perform an aortic valvuloplasty or a Ross operation was made after direct inspection of the aortic valve to evaluate its potential for repair. When the valve was judged severely dysplastic, we did not attempt repair but performed a Ross operation. In our series, no Ross procedures were performed after attempted conservative surgery on the aortic valve.
Follow-Up
A complete clinical and echocardiographic follow-up was obtained in all 9 survivors. No late deaths occurred. One patient with residual moderate-severe aortic valve regurgitation after surgical aortic valvuloplasty underwent a successful Ross operation 6 years after the initial surgical repair (patient 1, Table 1). Median follow-up time was 5 years, ranging from 1 month to 11.9 years. All patients are asymptomatic and in good clinical condition. Eight patients have mild aortic valve regurgitation and 1 patient, who underwent a Ross operation, has moderate regurgitation. No patient has residual stenosis of the aortic valve (gradient < 15 mm Hg in all). No right ventricular outflow tract obstruction was detected in the patients who underwent a Ross procedure, and none had more than mild homograft regurgitation.
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Comment |
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TopAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Recent reports in the literature show that in newborns and infants with critical aortic stenosis, the aortic valve can be exposed by conventional cardiopulmonary bypass and that precise commissurotomy, shaving of thickened leaflets, excision of obstructive myxomatous nodularities, and mobilization of leaflets can be performed with a very low surgical risk and 85% freedom from reoperation at 5 years [3, 8–10].
In our experience with the complementary use of balloon dilation and surgical valvuloplasty in the management of infantile critical aortic stenosis, nearly all of the patients could be successfully managed by balloon dilation alone, with low mortality and morbidity. In the patients that did not respond as favorably to balloon dilation, subsequent surgical intervention was successful in 80%, and valve repair was done in nearly half of the patients.
We believe this cooperative approach between interventional cardiologists and cardiac surgeons provided excellent results in this group of very difficult patients. Further, our experience demonstrates that primary balloon dilation does not reduce the likelihood of subsequent successful surgical repair or replacement of the valve.
In some cases, surgical treatment fails to adequately improve valve function, and aortic valve replacement is indicated. The selection of the most appropriate substitute in infants with irreparable aortic valve lesions remains controversial. Prosthetic valve replacement in infants requires an extensive aortoventriculoplasty (Konno procedure) to insert even the smallest size of prosthetic valve available (16 mm). The mechanical prosthesis imposes the risk of thromboembolism and the need for anticoagulation, which is particularly cumbersome in infants and children. Repeated prosthetic valve replacement will be needed during childhood because of somatic growth [11].
The Ross procedure has been suggested recently as the treatment of choice in infants with critical aortic stenosis, particularly when it is associated with other left-sided heart anomalies. Although we, and others, [12] prefer this approach in infants with unsuitable anatomy for valve-sparing surgery, it is clearly not a "curative" operation. Some pulmonary autograft valves deteriorate and must be replaced. Further, the homograft in the pulmonary position does not grow and in infants, inevitably becomes stenotic and must be replaced [13–16].
Because there is no long-lasting solution for aortic valve replacement in infants, conservative surgical aortic valve treatment has enjoyed a revival recently [17]. Undoubtedly, the best aortic valve in children is the natural one, provided the valve functions adequately. For this reason, pediatric cardiac surgeons must concentrate their efforts on preserving the native valve, whenever possible.
Careful intraoperative epicardial or transesophageal two-dimensional echocardiography and Doppler were performed in all of our patients. The preoperative evaluation led to a better understanding of the valve morphology and pathology and helped us to plan the most appropriate surgical reconstruction. The postoperative echocardiogram confirmed the results of the surgical reconstruction.
Any type of treatment for aortic stenosis in newborns and infants is palliative, and future aortic valve replacement is inevitable. In light of the actual results with aortic valve replacement, reconstructive surgery must be the primary objective for any valve with the aim of postponing aortic valve replacement until adolescence or adulthood.
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Acknowledgments |
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References |
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  P. Rehnstrom, T. Malm, P. Jogi, E. Fernlund, P. Winberg, J. Johansson, and S. Johansson Outcome of Surgical Commissurotomy for Aortic Valve Stenosis in Early Infancy Ann. Thorac. Surg., August 1, 2007; 84(2): 594 - 598. [Abstract] [Full Text] [PDF]
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