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[Surgical treatment for tetralogy of Fallot with unilateral absence of a pulmonary artery (author's transl)].

Among 843 patients with Tetralogy of Fallot, 10 showed unilateral absence of a pulmonary artery. In 7 cases the cause was congenital aplasia, whereas thrombosis of one of the pulmonary arteries following systemic-pulmonary anastomosis was the cause in the remaining 3 patients. According to the reports of other authors and to our own experiences, this rare malformation is treated best with palliative operations, the technique depending on the patient's age and on the individual anatomical condition. Only in very few cases with an acceptable pathologic anatomy there will be a reasonable chance for successful repair with reconstruction of the continuity from the right ventricle to the affected lung. However, if complete repair is performed, reconstruction of the absent pulmonary artery usually will be necessary for avoidance of pulmonary hypertension of the contralateral lung.