NOONAN SYNDROME WITH DOUBLE-CHAMBERED RIGHT VENTRICLE

The association of Noonan syndrome and a double-chambered right ventri cle has not been reported previously in the medical literature. We rep ort two patients with Noonan syndrome associated with pulmonary valve stenosis and double-chambered right ventricle due to anomalous hypertr ophied muscle bundles. Pulmonary valve stenosis was operated on. Howev er, hypertrophied muscle bundles were not resected, as they caused no obstruction to blood passage. Postoperative follow-up examinations rev ealed no symptoms. In conclusion, in those with Noonan syndrome there may be anomalous muscle bundles in the right ventricular cavity. It ma y not be necessary to resect them if they do not obstruct the right ve ntricular outflow tract.