title: Noonan syndrome associated with anomalous coronary artery and other cardiac defects.
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.
authors.affiliation: Division of Cardiovascular Surgery, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Aya
authors.initials: A
authors.lastname: Saito
authors.forename: Akihiko
authors.initials: A
authors.lastname: Sekiguchi
authors.forename: Masahide
authors.initials: M
authors.lastname: Chikada
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Tonari
mesh_terms: D000015:Abnormalities, Multiple; D003330:Coronary Vessel Anomalies / Q000175:diagnosis / Q000601:surgery*; D003937:Diagnosis, Differential; D006801:Humans; D007223:Infant; D008297:Male; D009634:Noonan Syndrome / Q000175:diagnosis / Q000601:surgery*; D011666:Pulmonary Valve Stenosis / Q000175:diagnosis / Q000601:surgery*