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Pulmonary Atresia and Ventricular Septal Defect

  Case radiograph

Contributor: Alan Friedman, MD

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Pulmonary Atresia and Ventricular Septal Defect (PA/VSD) represents the most severe form of Tetralogy of Fallot. PA/VSD presents a difficult management plan, since variations in blood supply to the pulmonary arterial tree may be complex and create significant variations in the development of the pulmonary arteries.

Pulmonary atresia is strictly defined as loss of continuity between the right ventricle and the pulmonary artery. Most commonly the pulmonary valve and the proximal pulmonary trunk are involved. The relationship of right and left pulmonary arteries is of surgical importance. The pulmonary arteries are considered 'confluent' when they maintain free communication with each other. However, when the atretic process extends past the bifurcation of the main pulmonary artery (MPA) into the branches, interrupting the continuity between the LPA and RPA, 'non-confluence' is said to exist.

Clinical considerations...

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