McConnell M E, Pacifico A D, Johnson W H, Mroczek E
Department of Pediatrics, Pathology, and Surgery, University of Alabama in Birmingham.
Pediatr Cardiol. 1994 Mar-Apr;15(2):95-9. doi: 10.1007/BF00817617.
Stenosis of individual pulmonary veins is a rare condition associated with a very high mortality. The present report is the first known case of progressive pulmonary venous obstruction associated with the tetralogy of Fallot. This clinical and anatomical study suggests that initial dynamic and possibly reversible occlusion occurs at the junction of the pulmonary vein and left atrium. Anatomical lesions in pulmonary veins then develop, possibly secondary to functional stenosis. The usually fatal outcome of these cases is due to the development of fixed pulmonary venous occlusion and possibly pulmonary interstitial fibrosis. The rapid progression of pulmonary venous stenosis strongly suggests that discrete veno-atrial stenosis should be surgically corrected as early as possible to prevent the irreversible diffuse pulmonary venous obstruction associated with an invariably fatal prognosis.
个别肺静脉狭窄是一种罕见疾病，死亡率极高。本报告是首例已知的与法洛四联症相关的进行性肺静脉梗阻病例。这项临床和解剖学研究表明，最初的动态且可能可逆的阻塞发生在肺静脉与左心房的交界处。随后肺静脉会出现解剖学病变，可能继发于功能性狭窄。这些病例通常致命的结局是由于固定性肺静脉阻塞以及可能的肺间质纤维化的发展。肺静脉狭窄的快速进展强烈提示，应尽早对离散性腔静脉-心房狭窄进行手术矫正，以防止出现与必然致命预后相关的不可逆弥漫性肺静脉梗阻。
