Presbitero P, Pedretti E, Orzan F, Malara D, Villani M, Ferrazzi P, Crupi G, Parenzan L
Int J Cardiol. 1984 Nov;6(5):587-98. doi: 10.1016/0167-5273(84)90004-4.
We report four cases of so-called absent pulmonary valve syndrome associated with absence or anomalous origin of the left pulmonary artery. The fate of the patients with this condition appears to be affected mainly by the occurrence of pulmonary complications (three of our patients died of pulmonary causes, two of them after surgery). The proper timing of corrective surgery is still uncertain. Preoperative investigations should strive to obtain a clear-cut identification of the pulmonary arteries, particularly the left one or, in its absence, of the anomalous vascular supply to the lung. Right ventriculography, as employed by us, is insufficient. Pulmonary angiography, aortography and pulmonary vein "wedge" angiography may be needed.
我们报告了4例所谓的肺动脉瓣缺如综合征，伴有左肺动脉缺如或起源异常。患有这种疾病的患者的预后似乎主要受肺部并发症的影响（我们的3例患者死于肺部疾病，其中2例在手术后死亡）。矫正手术的最佳时机仍不确定。术前检查应努力明确肺动脉，特别是左肺动脉的情况，或者在左肺动脉缺如时明确肺的异常血管供应。我们采用的右心室造影是不够的。可能需要进行肺动脉造影、主动脉造影和肺静脉“楔入”造影。
