Cystic fibrosis is an inherited disease affecting many organs. The lungs are affected by secretions that get trapped in the airways, leading to gradual deterioration of lung function, and repeated, chronic infection. In some cases lung transplantation is the treatment of choice. These children should be evaluated at a center specializing in cystic fibrosis, which can refer patients for pediatric lung transplantation when indicated. The Cardiac Center, together with several other divisions at Children's Hospital has a team of physicians specializing in the management of the pulmonary manifestations of cystic fibrosis.
Pulmonary hypertension is high blood pressure in the arteries leading to the lungs.
Another cause in children is longstanding, severe congenital heart disease. A pediatrician suspecting this problem should refer a child to a specialty service in pediatric pulmonary hypertension, such as the one at Children's Hospital. Severe cases are cosmetically undesirable, and children sometimes encounter social and emotional problems in school as they get older. Some studies indicate that severe pectus excavatum can compromise heart and lung function during exercise. Concened parents should have a consultation with one of our surgeons.