PERCUTANEOUS DILATION OF THE PULMONIC VAL VE IN A PATIENT WITH VALVULAR PULMONIC STENOSIS AND VON RECKLINGHAUSEN NEUROFIBROMATOSIS

Many congenital heart defects have been reported in patients with neur ofibromatosis, including hypertrophic cardiomyopathy, valvular pulmoni c stenosis, atrial septal defect, valvular aortic stenosis, ventricula r septal defect, and coarctation of the aorta. Valvular pulmonic steno sis may be among the most common cardiac defects associated with neuro fibromatosis and is often characterized by valvular dysplasia in this setting. A case of severe valvular pulmonic stenosis without valve dys plasia in a patient with von Recklinghausen neurofibromatosis is repor ted. The gradient between pressure peaks in the right ventricle and pu lmonary artery was 215 mmHg before treatment and 20 mmHg after percuta neous dilatation of the pulmonic valve with a 25-mm balloon. This case demonstrates the efficacy of percutaneous valvular dilatation in this clinical setting.