In single ventricle patients, after the Fontan procedure, the blood has to flow passively through the lungs without a pump. Since normal functioning of a Fontan circuit depends on the lungs being in good health, we will perform pulmonary (lung) function tests.
The health of the lungs will be assessed using spirometry which measures airflow by measuring how much air you inhale and how quickly the air is exhaled. During these tests you may be asked to perform a forced inhalation or exhalation after a deep breath, breathe normally and quietly or inhale a medicine to see how it changes your test results.
Plastic bronchitis is a rare but serious lung complication that may develop following the Fontan operation. In plastic bronchitis, the lung is unable to clear the lymphatic secretions and large, rubbery, cheese like mucus cast forms within the airways which can obstruct the airflow
within the airways. In this condition, patients can have severe respiratory symptoms including breathing difficulty, low oxygen saturation and coughing episodes during which the mucus casts are coughed out. The Single Ventricle Survivorship team includes a pulmonologist who will evaluate, manage and follow patients with plastic bronchitis. With the newer treatment options, some patients with plastic bronchitis may improve without the need for cardiac transplantation.