Sheikhzadeh A, Paydar M H, Ghabussi P, Hashemian M, Yazdanyar A, Hakim H S
Herz. 1983 Jun;8(3):179-86.
This report describes characteristic features and clinical presentation of six young patients (ranging in age from five to 25 years) with various congenital forms of pulmonary arteriovenous fistulas as well as one 21-year old patient with traumatic intrapulmonary arteriovenous fistula. The former six patients were cyanotic, had clubbing of the digits and exertional dyspnea; two, additionally, had telangiectasia. All patients underwent cardiac catheterization and pulmonary angiography. None had pulmonary hypertension. Angiographically, four had diffuse arteriovenous fistulas in both lungs (Figures 1 and 3 to 5) and two had arteriovenous fistulas localized in one pulmonary segment. Four patients were additionally studied with radionuclide angiography and pulmonary perfusion scintigraphy. In three of these, substantial right-to-left shunt in the region of the lungs was detected; the diagnosis could not be established in only one of the latter with a small traumatic intrapulmonary arteriovenous shunt of less than 10%. While noninvasive methods provide adjunctive diagnostic support, pulmonary angiography is still required to assess, with certainty, the nature and localization of the lesion. In cyanotic patients with no evidence of cardiac disease, differential diagnostic consideration should be given to pulmonary arteriovenous fistulas.
本报告描述了6例患有各种先天性肺动静脉瘘的年轻患者（年龄从5岁至25岁不等）以及1例21岁创伤性肺内动静脉瘘患者的特征和临床表现。前6例患者发绀、有杵状指和劳力性呼吸困难；其中2例还患有毛细血管扩张症。所有患者均接受了心导管检查和肺血管造影。均无肺动脉高压。血管造影显示，4例患者双肺有弥漫性动静脉瘘（图1和图3至图5），2例患者动静脉瘘局限于一个肺段。另外4例患者还接受了放射性核素血管造影和肺灌注闪烁扫描。其中3例检测到肺部区域有大量右向左分流；仅1例创伤性肺内动静脉分流小于10%的患者无法确诊。虽然非侵入性方法提供辅助诊断支持，但仍需要肺血管造影来确定病变的性质和位置。对于无心脏病证据的发绀患者，应鉴别诊断是否为肺动静脉瘘。
