Hernandez R J, Stern A M, Rosenthal A
AJR Am J Roentgenol. 1980 Jan;134(1):75-80. doi: 10.2214/ajr.134.1.75.
This report describes the course and prognosis of four patients with Noonan syndrome and associated pulmonary lymphangiectasia. All patients had (1) superior deviation of the QRS axis in the frontal plane of the electrocardiogram; (2) moderate to severe valvular pulmonary stenosis, with an additional atrial septal defect secundum type present in two and a small ventricular septal defect in one; and (3) bilateral prominence of the pulmonary interstitial markings (dilated lymphatics), with or without accompanying pleural effusions on the chest radiographs. Surgical repair of the cardiac lesion may cause undue morbidity because of the interruption of the engorged pleural or mediastinal lymphatics.
本报告描述了4例努南综合征合并肺淋巴管扩张患者的病程及预后。所有患者均有：（1）心电图额面QRS轴向上偏移；（2）中度至重度瓣膜性肺动脉狭窄，2例合并继发孔型房间隔缺损，1例合并小型室间隔缺损；（3）胸部X线片显示双侧肺间质纹理增粗（淋巴管扩张），伴或不伴胸腔积液。由于充血的胸膜或纵隔淋巴管中断，心脏病变的手术修复可能会导致过度的发病率。
