The spectrum of pulmonary arterio-venous fistulae. Clinico-pathological correlations.

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.