Cardiopulmonary rehabilitation in a patient with Noonan syndrome

Noonan syndrome, an autosomal dominant disease occurring with an incidence of 1 in 1,000 to 1 in 2,500 live births, is characterized by its particular cardiovascular abnormalities, including pulmonic valve stenosis, pulmonary artery stenosis, and, mon rarely septal defects and coarctation of the aor ta. The case of a 20-year-old man admitted for inpatient cardiopulmonary re habilitation after pulmonic valve repair, left pulmonary artery angioplasty , and pectus excavatum repair is presented. His endurance was markedly decr eased, thus limiting his ability to perform activities of daily living and reducing his exercise tolerance. With participation in a comprehensive card iopulmonary rehabilitation program, he experienced marked improvement with independence in his activities of daily living and an increase in his metab olic equivalent levels from to 2.8 to 5.4. After inpatient rehabilitation, he underwent left pulmonary stent placement before being discharged home. S ubsequent outpatient cardiopulmonary rehabilitation has continued to improv e significantly his overall exercise tolerance. Given that Noonan syndrome is viewed as the most common syndrome associated with congenital heart dise ase after Down syndrome, physiatrists must be familiar with its presentatio n, its associated abnormalities, and the treatment approach to optimize the patient's cardiopulmonary, musculoskeletal, and psychological status.