Tetralogy of Fallot with absent pulmonary valve
This is an uncommon variation of tetralogy of Fallot with a ring-like and usually stenotic malformation (rather than absence) of the pulmonary valve, with failure of the development of valve cusps. The central pulmonary arteries are usually hugely dilated or aneurysmal. The pulmonary artery dilatation may extend beyond what is expected from severe pulmonary regurgitation to several generations of pulmonary arteries causing tracheobronchial compression; this often results in either hyperexpansion from air trapping or collapse of lobes, or even an entire lung. The ductus arteriosus is generally absent. The intracardiac anatomy generally presents with the same characteristics of tetralogy of Fallot (see chapter “Tetralogy of Fallot”) except that the right ventricular outflow tract is not excessively restrictive.
KeywordsPulmonary Artery Pulmonary Artery Aneurysm Central Pulmonary Artery Absent Pulmonary Valve Severe Pulmonary Regurgitation
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