Variant of tetralogy of fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

Abstract

Absent pulmonary valve leaflets and anomalous origin of a pulmonary artery from the ascending aorta have been reported as separate lesions associated with tetralogy of Fallot but not in combination. The clinical, radiologic and pathologic findings are presented in three patients with this previously undescribed combination. A search of the literature for similar cases revealed 233 patients with absent pulmonary valve leaflets and 56 patients with anomalous origin of a pulmonary artery from the ascending aorta; these cases are reviewed. Although 17 patients with absent pulmonary valve leaflets and tetralogy of Fallot also had absence of the proximal portion of the left pulmonary artery, none had an anomalously arising pulmonary artery from the ascending aorta. In the reviewed cases, anomalous origin of the right pulmonary artery from the ascending aorta was more frequent than that of the left pulmonary artery (44 compared with 12 cases, respectively), but the latter was more often associated with tetralogy of Fallot.