Gula G, Nakvi A, Radley-Smith R, Yacoub M
Thorac Cardiovasc Surg. 1981 Feb;29(1):51-4. doi: 10.1055/s-2007-1023441.
Four male patients with pulmonary arterio-venous fistulae are reported. Familiar occurrence was noticed in 2 brothers. Physiological effects, clinical pictures and treatment of these lesions depend on the size and number of the fistulae. A 4-day-old baby presented with severe cyanosis and cardiomegaly. Cardiac catheterization and angiography demonstrated a large arterio-venous fistula affecting the whole of the right upper lobe and a small ventricular septal defect resulting in a high output cardiac failure. The patient underwent emergency lobectomy with good early and long-term results. The other 3 patients (mean age of 7.6 years) developed progressive cyanosis and polycythemia. Cardiac catheterization showed normal intracardiac pressures and peripheral arterial desaturation. Pulmonary angiogram demonstrated multiple fistulae in both lungs. Surgery was not performed because of the diffuse nature of the lesions. The patients reported in this paper illustrate the wide spectrum of clinico-pathological conditions of this anomaly.
本文报道了4例患有肺动静脉瘘的男性患者。在2名兄弟中发现有家族发病情况。这些病变的生理影响、临床表现及治疗取决于瘘的大小和数量。一名4天大的婴儿出现严重青紫和心脏扩大。心导管检查和血管造影显示一个大的动静脉瘘累及整个右上叶，并有一个小的室间隔缺损，导致高输出量心力衰竭。该患者接受了急诊肺叶切除术，早期和长期效果良好。另外3例患者（平均年龄7.6岁）出现进行性青紫和红细胞增多症。心导管检查显示心腔内压力正常，外周动脉血氧饱和度降低。肺血管造影显示双肺有多个瘘。由于病变弥漫，未进行手术。本文报道的患者说明了这种异常的临床病理情况范围很广。
