Abstract
This study evaluates the course of supravalvular aortic stenosis (SVAS)-associated right ventricular outflow tract (RVOT) obstruction and the results of surgery in children. We reviewed the medical records of 24 patients diagnosed with SVAS at initial echocardiographic examination or during the following period of RVOT obstruction. Very mild SVAS was defined as a transvalvular Doppler peak systolic instantanous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 3.1 ± 2.9 years (range, 7 days to 12.7 years), and 18 of the patients (72%) were male. Fifteen patients had Williams’ syndrome. Seventeen patients (71%) were followed for a mean of 5.2 ± 3.8 years (range, 7 months to 13.5 years). Among 17 patients with complete follow-up records, 1 (6%) had very mild, 5 (29%) mild, 3 (18%) moderate, and 3 (18%) severe aortic stenosis at initial echocardiographic examination. In a newborn patient with mild pulmonary valvular stenosis. SVAS became evident after 2 months and progressed rapidly. Supravalvular aortic stenosis was very mild in 4 patients (23%), mild in 3 (18%), moderate in 3 (18%), and severe in 7 (41%) at last echocardiographic examination. Of 17 patients who were followed, 11 (65%) had RVOT obstruction at initial echocardiographic examination. RVOT obstruction disappeared in 5 patients, regressed in 1 patient, and appeared in 1 patient over the follow-up period. Four patients underwent operation. It appears reasonable that patients with very mild and mild stenosis should be followed medically every 1 or 2 years and patients with moderate stenosis once a year. Newborns with SVAS should be followed for rapid progression of SVAS. In some patients, RVOT obstruction may disappear, and SVAS may develop in others with RVOT obstruction. Patients with RVOT obstruction (at the valvular, supravalvular, or peripheral pulmonary arterial level) should be evaluated carefully for development of SVAS at follow-up.
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Baumgartner H, Kratzer H, Helmreich G, Kühn P (1988) Quantification of aortic regurgitation by color coded cross-sectional Doppler echocardiography. Eur Heart J 9:380–387
Baumgartner H, Stefenelli T, Nisderberger J, Schima H, Maurer G (1999) Overestimation of catheter gradients in patients with aortic stenosis: a predictable manifestation of pressure recovery. J Am Coll Cardiol 33:1655–1661
Beuren AJ, Apnz J, Harmjanz D (1962) Supravalvular aortic stenosis in association with mental retardation and a certain facial appearance. Circulation 26:1235–1240
Bruno ER, Thuer O, Cordoba R, Alday LE (2003) Cardiovascular findings, and clinical course, in patients with Williams’ syndrome. Cardiol J 13:532–536
Cheatham JP (1990) Pulmonary stenosis. In: Garson A, Bricker JT, McNamara DG (eds) The Science and Practice of Pediatric Cardiology. Lea & Febiger, Philadelphia, pp 1382–1420
Giddins NG, Finley JP, Nanton MA, Roy DL (1989) The natural course of supravalvar aortic stenosis and peripheral pulmonary artery stenosis in Williams’ syndrome. Br Heart J 62:315–319
Karamlou T, Shen I, Alsoufia B, et al. (2005) The influence of valve physiology on outcome following aortic valvotomy for congenital bicuspid valve in children: 30 year results from a single institution. Eur J Cardiothorac Surg 27:81–85
Keane JF, Fellows KE, LaFarge CG, Nadas AS, Bernhard WF (1976) The surgical management of discrete and diffuse supravalvar aortic stenosis. Circulation 54:112–117
Kim YM, Yoo SJ, Choi JY, et al. (1999) Natural course of Supravalvular aortic stenosis and peripheral pulmonary arterial stenosis in Williams’ syndrome. Cardiol Young 9:37–4l
Kiraly P, Kapusta L, van Lier H, et al. (1997) Natural history of congenital aortic valvar stenosis: an echo and Doppler cardiographic study. Cardiol Young 7:188–193
Kitchiner D, Jackson M, Walsh K, Peart I, Arnold R (1996) Prognosis of supravalve aortic stenosis in 81 patients in Liverpool (1960–1993). Heart 75:396–402
Latson LA (1990) Aortic stenosis: valvular, supravalvular, and fibromuscular subvalvular. In: Garson A, Bricker JT, McNamara DG (eds) The Science and Practice of Pediatric Cardiology Lea & Febiger, Philadelphia, pp 1134–1352
Levine RA, Jimoh A, Cape EG, et al. (1989) Pressure recovery distal to a stenosis: potential cause of gradient “overestimation” by Doppler echocardiography. J Am Coll Cardiol 63:809–813
Lofland GK, McCrindle BW, Williams WG, et al. (2001) Critical aortic stenosis in the neonate: a multiinstitutional study of management, outcomes, and risk factors. J Thorac Cardiocvasc Sug 12:10–27
Mack G, Silberbach M (2000) Aortic and pulmonary stenosis. Pediatr Rev 2l:79–85
Miyatake K, Okamoto M, Kinoshita N, et al. (1984) Clinical applications of a new type of real-time two-dimensional Doppler flow imaging system. Am J Cardiol 54:857–868
Omoto KR, Yokote Y, Takamoto S, et al. (1984) The development of real-time two-dimensional Doppler echocardiography and its clinical significance in acquired valvular diseases. Jpn Heart J 25:325–340
Perry GJ, Helmcke F, Nanda NC, Byard C, Soto B (1987) Evaluation of aortic insufficiency by Doppler color flow mapping, J Am Coll Cardiol 9:952–959
Samanek M, Voriskova M (1999) Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study. Pediatr Cardiol 20:411–417
Shub C, Tajik AJ, Holmes DR, et al. (1990) Doppler echocardiography in aortic stenosis: feasibility and clinical impact. Int J Cardiol 28:57–66
Simpson IA, Valdes-Cruz LM, Yoganathan AP, et al. (1989) Spatial velocity distribution and acceleration in serial subvalve tunnel and valvular obstructions: an in vitro study using Doppler color flow mapping. J Am Coll Cardiol 13:241–248
Stamm C, Friehs I, Ho SY, et al. (2001) Congenital supravalvar aortic stenosis: a simple lesion? Eur J Cardiothorac Surg 9:195–202
Tohyama K, Satomi G, Momma K (1997) Aortic valve prolapse and aortic regurgitation associated with subpulmonic ventricular septal defect. Am J Cardiol 79:1285–1289
Valdes-Cruz LM, Jones M, Scagnelli S, et al. (1985) Prediction of gradients in fibrous subaortic stenosis by continuous wave two-dimensional Doppler echocardiography: animal studies. J Am Coll Cardiol 5:1363–1367
Valdes-Cruz LM, Yoganathan AP, Tamura T, et al. (1986) Studies in vitro of the relationship between ultrasound and laser Doppler veloeimetry and applicability to the simplified Bernoulli relationship. Circulation 73:300–308
Wessel A, Pankau R, Kececioğlu D, Ruschewski W, Bursch JH (1994) Three decades of follow-up of aortic and pulmonary vascular lesions in the Williams–Beuren syndrome. Am J Genet 52:297–301
Williams JC, Barratt-Boyes BG, Lowe JB (1961) Supravalvular aortic stenosis. Ciculation 24:1311–1318
Wren C, Oslizlok P, Bull C (1990) Natural history of supravalvular aortic stenosis and pulmonary artery stenosis. J Am Coll Cardiol 15:1625–1630
Yoganathan AP, Valdes-Cruz LM, Schmidt-Dohna J, et al. (1987) Continuous-wave Doppler velocities and gradients across fixed tunnel obstructions: studies in vitro and in vitro. Circulation 76:657–666
Zalzstein E, Moes CA, Musewe NN, Freedom RM (1993) Spectrum of cardiovascular anomalies in Williams–Beuren syndrome. Pediatr Cardiol 14:219–223
Zhang Y, Nitter-Hauge S, Ihlen H, et al. (1986) Noninvasive evaluation of aortic regurgitation by Doppler echocardiography. Br Heart J 55:32–38
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This work was supported by Research Fund of Istanbul University grant UDP-612/02082005.
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Eroglu, A.G., Babaoglu, K., Oztunc, F. et al. Echocardiographic Follow-Up of Children with Supravalvular Aortic Stenosis. Pediatr Cardiol 27, 707–712 (2006). https://doi.org/10.1007/s00246-006-1320-5
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DOI: https://doi.org/10.1007/s00246-006-1320-5