Objective: To study the effects of long-term growth hormone (GH) treatment on left ventricular (LV) dimensions in children with Noonan’s syndrome (NS). Methods: Echocardiographic measurements of LV dimensions were performed before and during GH treatment in 27 participants (21 boys, 6 girls) in a partly controlled 3-year trial of high-dose GH treatment (0.15 IU/kg/day). Nineteen children had a congenital heart defect, 1 of them had hypertrophic obstructive cardiomyopathy. In the first 3 years, the children were assigned to 1 of 2 groups: group A with discontinuation of GH treatment in the 3rd year, or group B without GH treatment in the 1st year. After the 3rd year, 12 of the 27 children were followed up for 2 additional years to evaluate the long-term effects of GH treatment on the heart. Results: At baseline, LV internal diameters were smaller, while posterior wall thickness were thicker than normal. Over the 1st year, changes in LV dimensions were comparable between the 2 groups. No significant differences were found in LV dimensions between the situation at baseline and after 4 years of GH treatment. Conclusion: Long-term high-dose GH treatment does not have clinically significant adverse effects on LV dimensions in children with NS.

1.
Noonan JA: Hypertelorism with Turner phenotype: A new syndrome with associated congenital heart disease. Am J Dis Child 1968;116:373–380.
2.
Allanson JE: Noonan syndrome. J Med Genet 1987;24:9–13.
3.
Sharland M, Burch M, McKenna WM, Patton MA: A clinical study of Noonan syndrome. Arch Dis Child 1992;67:178–183.
4.
Ranke MB, Heidemann P, Knupfer C, Enders H, Schmaltz AA, Bierich JR: Noonan syndrome: Growth and clinical manifestations in 144 cases. Eur J Pediatr 1988;148:220–227.
5.
Municchi G, Pasquino AM, Pucarelli I, Cianfarani S, Passeri F: Growth hormone treatment in Noonan syndrome: Report of four cases who reached final height. Horm Res 1995;44:164–167.
6.
Ahmed ML, Foot AB, Edge JA, Lamkin VA, Savage MO, Dunger DB: Noonan’s syndrome: Abnormalities of the growth hormone/IGF-I axis and the response to treatment with human biosynthetic growth hormone. Acta Pediatr Scand 1991;80:446–450.
7.
Thomas BC, Stanhope R: Long-term treatment with growth hormone in Noonan’s syndrome. Acta Pediatr 1993;82:853–855.
8.
De Schepper J, Otten BJ, Francois I, Bourguignon JP, Craen M, van der Burgt I, Massa GG: Growth hormone therapy in pre-pubertal children with Noonan syndrome: First year growth response and comparison with Turner syndrome. Acta Paediatr 1997;86:943–946.
9.
Romano AA, Blethen SL, Dana K, Noto RA: Growth hormone treatment in Noonan syndrome: The National Cooperative Growth Study experience. J Pediatr 1996;128:S18–S21.
10.
Otten BJ, Noordam C: Short stature in Noonan syndrome: Demography and response to growth hormone treatment in KIGS; in Ranke MB, Wilton P (eds): Growth Hormone Therapy in KIGS –10 Years’ Experience. Heidelberg, Barth, 1999, pp 269–280.
11.
Burch M, Sharland M, Shinebourne E, Smith G, Patton M, McKenna W: Cardiologic abnormalities in Noonan syndrome: Phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol 1993;22:1189–1192.
12.
Marino B, Digilio MC, Toscano A, Giannotti A, Dallapiccola B: Congenital heart diseases in children with Noonan syndrome: An expanded cardiac spectrum with high prevalence of atrioventricular canal. J Pediatr 1999;135:703–706.
13.
Sacca L, Cittadini A, Fazio S: Growth hormone and the heart. Endocr Rev 1994;15:555–573.
14.
Osterziel KJ, Strohm O, Schuler J, Friedrich M, Hanlein D, Willenbrock R, Anker SD, Poole Wilson PA, Ranke MB, Dietz R: Randomised, double-blind, placebo-controlled trial of human recombinant growth hormone in patients with chronic heart failure du to dilated cardiomyopathy. Lancet 1998;351:1233–1237.
15.
Cotterill AM, McKenna WJ, Brady AF, Sharland M, Elsawi M, Yamada M, Camacho Hubner C, Kelnar CJ, Dunger DB, Patton MA, Savage MO: The short-term effects of growth hormone therapy on height velocity and cardiac ventricular wall thickness in children with Noonan’s syndrome. J Clin Endocrinol Metab 1996;81:2291–2297.
16.
van der Burgt I, Berends E, Lommen E, van Beersum S, Hamel B, Mariman E: Clinical and molecular studies in a large Dutch family with Noonan syndrome. Am J Genet 1994;53:187–191.
17.
Sobotka-Plojhar MA: Anthracycline cardiotoxicity in children an echocardiographic study; thesis VU University, Amsterdam, 1991, pp 97–100.
18.
Kiraly P; Kapusta L, van Lier H, Otten Hofman A, Daniels O: Natural history of congenital aortic valvar stenosis: An echo and Doppler cardiographic study. Cardiol Young 1997;7:188–193.
19.
Fredriks AM, van Buuren S, Burgmeyer RJF, Meulmeester JF, Beuker RJ, Brugman E, Roede MJ, Verloove-Vanhorick SP, Wit JM: Continuing positive secular growth change in the Netherlands 1955–1997. Pediatr Res 2000;47:316–323.
20.
Lampit M, Lorber A, Vilkas DL, Nave T, Hochberg Z: GH dependence and GH withdrawal syndrome in GH treatment on short normal children: Evidence from growth and cardiac output. Eur J Endocrinol 1998;138:401–407.
21.
Barton JS, Cullen S, Hindmarsh PC, Brook CG, Preece MA: Growth hormone treatment in idiopathic short stature: A preliminary analysis of cardiovascular effects. Acta Paediatr Suppl 1992;383:35–38.
22.
Daubeney PE, McCaughey ES, Chase C, Walker JM, Slavik Z, Betts PR, Webber SA: Cardiac effects of growth hormone in short normal children: Results after four years of treatment. Arch Dis Child 1995;72:337–339.
23.
Crepaz R, Pitscheider W, Radetti G, Paganini C, Gentili L, Morini G, Braito E, Mengarda G: Cardiovascular effects of high-dose growth hormone treatment in growth hormone-deficient children. Pediatr Cardiol 1995;16:223–227.
24.
Bengtsson BA, Brummer RJ, Eden S, Bosaeus I, Lindstedt G: Body composition in acromegaly: The effect of treatment. Clin Endocrinol (Oxf) 1989;31:481–490.
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