Percutaneous aortic valvoplasty in congenital aortic valvar stenosis

Objective: To evaluate immediate and midterm results with percutaneous aortic valvoplasty.Material and Methods: We reviewed the records of 141 patients undergoing percutaneous aortic valvopasty over a period of 13 years.Results: The patients were aged from 2 months to 40 years, with a mean of 10.9 ± 9.9 years. Of the total, 90 (63%) were male. The initial systolic peak-to-peak gradient decreased from 163 ± 52 mmHg to 32 ± 18 mmHg (p > 0.01) after valvoplasty in all 141 patients, while the proportional reduction ranged from 0 to 100%, with a mean of 72 ± 27%. The index of the size of the balloon to the diameter of the valvar orifice was 0.88 ± 0.19 in 128 patients. The follow-up ranged from 6 to 168 months, with a mean 51 ± 48 months in 70 patients. A significant difference was found in those failing after dilation when the initial evaluation was compared to the final evaluation of patients with follow-up. In those failing, the number of patients rose from 12 (17%) to 21 (30%) (p > 0.01). In contrast, in those in whom we achieved success, there was not such a great difference between the initial and final evaluation: 58 (83%) versus 49 (70%) (p > 0.1). The actuarial freedom curve of patients not needing new percutaneous aortic valvoplasty or surgery, by 182 months, was at 87% and 82% respectively.Conclusion: We have reviewed the largest series of patients in Latin-America reported thus far after undergoing percutaneous aortic valvoplasty, concentrating on mid term follow-up and limitations. New prospective and multicentric studies are needed from our region.

Long-term results after valvotomy for congenital aortic valvar stenosis in children

As interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10–16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a rangefrom 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15–49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.

Non-invasive determination of the systolic peak-to-peak gradient in children with aortic stenosis: validation of a mathematical model

Doppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient=6.02+1.49*(mean gradient)−0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y=0.67 × + 11.11, r=0.87, SEE=6 mm Hg, p=0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y=0.66 × + 14.44, r=0.84, SEE=9 mm Hg, p=O.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.

Natural history of congenital aortic valvar stenosis: an echo and Doppler cardiographic study

AbstratThe availability of echo Doppler cardiography enables monitoring of the natural course of congenital aortic valvar stenosis more adequately than before. Between 1986 and 1993, 129 children with such stenosis were examined echocardiographically, 83 of them over a prolonged period with repeated studies of at least one per year. The pressure gradient between the left ventricle and ascending aorta, as well as the left ventricular wall thickness, were measured. When the pressure gradient detected increased to more than 60 mmHg, the patients were treated by balloon valvoplasty or surgical valvotomy. The last examination prior to intervention was taken as the final measurement.We found a significant increase (≥10 mmHg) in the measured pressure gradient in 40 of the 83 patients (48%). The onset of severe stenosis was not observed at a specific age, rather the stenosis was found suddenly to increase in severity at any age. The gradients measured in children younger than 2 years of age, however, increased twice as rapidly as those measured in the older children. A high gradient was not always accompanied by evidence of left ventricular hypertrophy.Based on our findings, we suggest that, since a sudden increase in severity of aortic valvar stenosis might be expected, the pressure gradient should be measured once every 6 months in children younger than 2 years of age and once a year after that age

Unicommissural aortic valve in neonates and its association with other congenital heart disease

The morphology of the aortic valve was studied in 1,022 heart specimens belonging to the collection of the Institute of Pathological Anatomy, University of Padua. Twenty specimens were found to have a unicommissural aortic valve, characterized by the presence of a single leaflet with only one functional commissure; however, the presence of two raphes enabled the recognition of a basically three-sinus arrangement. Age and gender were known in 19 cases: 14 male and five female, mean age nine days. In 19 cases, the unicommissural valve was dysplastic and resulted in a severe congenital aortic valvar stenosis. Only in two hearts was the unicommissural nature of the aortic valve an isolated finding; among the remaining 18, left ventricular fibroelastosis in 11, malformation of the mitral valve in 11, hypoplasia of the left ventricle in eight, ventricular septal defect in four, mitral atresia in three, and subaortic fibrous diaphragm in one. Furthermore, seven (35%) of these 20 unicommissural aortic valves were associated with coarctation of the aorta. Statistical analysis shows that this association is not a random event. Our findings support the hypothesis that the unicommissural aortic valve originates from the early fusion of the three mesenchymal valvar cushions or leaflet primordia. Although the present data do not exclude the possibility that reduction of the blood flow through the aorta during fetal life may play a role in the formation of the unicommissural aortic valve, they rather point in the direction that another etiologic factor, such as an anomalous migration of neural crest cells, may be responsible for the fusion of the valvar cushions.