Absent Pulmonary Valve
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Absent Pulmonary Valve and the Adult Patient

Patients lacking a pulmonary valve will have persistent pulmonary insufficiency (leakage). While some patients remain symptom-free, problems with right heart function and anatomy may develop over time.

For example, dilatation (enlargement) of the right ventricle can lead to arrhythmias, decreased right ventricular function and other symptoms. Eventually, these patients may require valve replacement or repair operations.

Absent pulmonary valve often causes significant dilatation of the pulmonary arteries, which persists into adulthood. However, if there is no bronchial obstruction, this abnormality may cause no symptoms and require no treatment. In cases where obstruction does occur, the pulmonary arteries may be reduced in size surgically.

Some patients will continue to experience respiratory difficulties after surgery. This may be due to the lingering effects of the compression of bronchi (air passages) in the lungs by the enlarged pulmonary arteries before the repair was made.

Those absent pulmonary valve patients who have undergone valve replacements should be monitored for pulmonary valve function. Most surgically implanted valves will last 5-10 years before they develop severe loss of valve function usually a mix of obstruction and insufficiency. When their function becomes impaired for any of these reasons, replacement becomes necessary, especially if symptomatic.

Adult patients with absent pulmonary valve should take steps to avoid the development of lung disease as this will aggravate the symptoms associated with pulmonary valve inefficiency. This means that these patients should avoid tobacco products and should pursue prompt treatment of sleep apnea.