Abstract
This case-report describes an 11-month-old infant, who had been diagnosed with Noonan syndrome with a variety of associated anomalies. Multiple cardiac anomalies were present, consisting of dysplastic pulmonary valve, symmetric biventricular hypertrophy, atrial septal defect and right ventricular outflow tract (RVOT) obstruction, in which the pressure gradient measured 73 mmHg, and anomalous coronary artery. Systolic anterior motion of the mitral valve (SAM) was present, without remarkable clinical significance. RVOT transannular repair with non-cusped xenograft along with resection of hypertrophied right ventricular outflow myocardium. Left ventricular outflow tract (LVOT), which had no clinical sign of obstruction, was left untouched, expecting the RVOT repair also effectively release LVOT dynamic obstruction. The postoperative echocardiography revealed residual SAM without significant pressure gradient through LVOT.
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Masura J, Burch M, Deanfield JE, Sullivan ID. Five-year follow-up after balloon pulmonary valvuloplasty. J Am Coll Cardiol 1993; 21: 132–6.
Burch M, Sharland M, Shinebourne E, Smith G, Patton M, Mckenna W. Cardiac abnormalities in Noonan syndrome: Phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol 1993; 22: 1189–92.
Vancini M, Roberts KD, Silove ED, Singh SP. Surgical treatment of congenital pulmonary stenosis due to dysplastic leaflets and small valve anulus. J Thorac Cardiovasc Surg 1980; 79: 46–8.
Marantz PM, Huhta JC, Mullins CE, Murphy DJ Jr, Nihill MR, Ludomirsky A, et al. Results of balloon valvuloplasty in typical and dysplastic pulmonary valve stenosis: Doppler echocardiographic follow-up. J Am Coll Cardiol 1988; 12: 476–9.
Shimizu A, Oku Y, Matsuo K, Hashiba K. Hyper-trophic cardiomyopathy progressing to a dilated cardiomyopathy-like feature in Noonan’s syndrome. Am Heart J 1992; 123: 814–6.
Nomura Y, Yanagi S, Kono Y, Kamimura J, Nishi J, Yoshinaga M, et al. Bilateral coronary artery dilatation in a child with Noonan syndrome. Jpn Circ J 2000; 64: 481–3.
Wong CK, Cheng CH, Lau CP, Leung WH. Congenital coronary artery anomalies in Noonan’s syndrome. Am Heart J 1990; 119: 396–400.
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Saito, A., Sekiguchi, A., Chikada, M. et al. Noonan syndrome associated with anomalous coronary artery and other cardiac defects. Jpn J Thorac Caridovasc Surg 52, 18–20 (2004). https://doi.org/10.1007/s11748-004-0055-6
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DOI: https://doi.org/10.1007/s11748-004-0055-6