Division of Pediatric Intensive Care, University Children's Hospital Berne, Inselspital, Berne, Switzerland.
Department of Pediatrics, Pediatric Critical Care University of California, San Francisco, California.
Pediatr Pulmonol. 2021 Mar;56(3):649-655. doi: 10.1002/ppul.24848. Epub 2020 Jun 7.
Pulmonary vein stenosis is a rare and poorly understood condition causing obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive, increasingly severe dyspnea, hemoptysis, respiratory difficulty, recurrent respiratory tract infections/pneumonia, cyanosis, and subcostal retractions. On chest radiograph, the most frequent finding is increased interstitial, ground-glass and/or reticular opacity. Transthoracic echocardiography with pulsed Doppler delineates the stenosis, magnetic resonance imaging and multislice computerized tomography are used for further evaluation. Interventional cardiac catherization, surgical techniques, and medical therapies have been used with varying success as treatment options.
肺静脉狭窄是一种罕见且尚未被充分了解的病症，会导致肺静脉和血液从肺部流向左心房的通道受阻。这会导致肺静脉压升高和肺水肿、肺动脉高压，可能导致心力衰竭和死亡。该疾病的临床症状包括生长发育迟缓、呼吸困难逐渐加重、咯血、呼吸困难、反复呼吸道感染/肺炎、发绀和肋间隙凹陷。胸部 X 线摄影最常见的表现是间质、磨玻璃样和/或网状混浊增加。经胸超声心动图伴脉冲多普勒可描绘狭窄，磁共振成像和多层计算机断层扫描用于进一步评估。介入性心脏导管术、手术技术和药物治疗已被用作治疗选择，其成功率各不相同。
