Slovis B S, Chazova I, Loyd J E, Meyrick B O
Division of Pulmonary and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA.
Eur Respir J. 1998 Jul;12(1):240-4. doi: 10.1183/09031936.98.12010240.
A 24 yr old white female presented with dyspnoea, orthopnoea, paroxysmal nocturnal dyspnoea, cough and fatigue. Transthoracic echocardiography revealed a sinus venosus atrial septal defect (ASD). Right heart catheterization confirmed severe pulmonary hypertension (80/37 mmHg). A chest radiograph showed enlarged pulmonary arteries with peripheral pruning. Surgical repair of the ASD and lung biopsy were performed. Two days later, she developed right heart failure and was treated with inhaled nitric oxide and then a calcium channel blocker. She failed to improve and was readmitted three months later with severe right heart failure and progressive dyspnoea. While waiting for lung transplantation, she developed haematochezia and died. Light microscopy of lung biopsy and autopsy tissue revealed the structural changes of pulmonary hypertension and focal increases in congested pulmonary capillaries consistent with the diagnosis of pulmonary capillary haemangiomatosis. Quantitative analysis demonstrated that the pathological changes were rapidly progressive.
一名24岁的白人女性出现呼吸困难、端坐呼吸、阵发性夜间呼吸困难、咳嗽和疲劳症状。经胸超声心动图显示存在静脉窦型房间隔缺损（ASD）。右心导管检查证实存在严重肺动脉高压（80/37 mmHg）。胸部X线片显示肺动脉增粗伴外周血管纹理稀疏。进行了房间隔缺损手术修复及肺活检。两天后，她出现右心衰竭，接受了吸入一氧化氮治疗，随后使用了钙通道阻滞剂。病情未改善，三个月后因严重右心衰竭和进行性呼吸困难再次入院。在等待肺移植期间，她出现便血并死亡。肺活检及尸检组织的光镜检查显示肺动脉高压的结构改变以及肺毛细血管充血灶性增加，符合肺毛细血管血管瘤病的诊断。定量分析表明病理改变进展迅速。
