CARDIOVASCULAR-ABNORMALITIES IN NOONAN SYNDROME - THE CLINICAL FINDINGS AND TREATMENTS

The clinical findings and treatment of cardiovascular abnormalities in 33 patients with Noonan syndrome are reviewed. Major cardiovascular a bnormalities were pulmonary valvular stenosis in 17 patients (51.1%). hypertrophic cardiomyopathy (HCM) in 11 (33.3%), and atrial septal def ect in 9 (27.3%). Dysplastic pulmonary valve was seen in 6 (35.3%). Ba lloon pulmonary valvuloplasty was performed for 4 patients with dyspla stic pulmonary valves. Two patients were successfully treated. Surgica l treatment was performed in 13 patients, 11 alive and 2 died. Two pat ients with HCM and arrhythmia died suddenly. In conclusion, balloon pu lmonary valvuloplasty should be the initial palliation for dysplastic pulmonary valve in Noonan syndrome, and HCM is the risk factor for sud den death in Noonan syndrome.