Respiratory Failure Unit, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Ther Adv Respir Dis. 2013 Apr;7(2):119-23. doi: 10.1177/1753465812468042. Epub 2012 Dec 12.
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). Misdiagnosis of the disease is common since PVOD presents with clinical and radiographic features mimicking idiopathic pulmonary arterial hypertension or even PH due to interstitial lung disease. Vasodilators may not be efficacious in PVOD and may in fact worsen hemodynamic status with the development of pulmonary edema. Lung transplantation represents the best treatment option. In the present report we describe the challenging diagnosis of PVOD in a patient with PH referred to our department. Final diagnosis was established by surgical lung biopsy. The patient was offered sequential combination therapy under close monitoring and maintained remarkable clinical stabilization while being on the waiting list for lung transplantation.
肺静脉闭塞病（PVOD）是肺动脉高压（PH）的一种罕见病因。由于 PVOD 具有类似于特发性肺动脉高压甚至间质性肺疾病所致 PH 的临床和影像学特征，因此误诊较为常见。血管扩张剂在 PVOD 中可能无效，实际上可能会因肺水肿的发展而使血流动力学状态恶化。肺移植是最佳的治疗选择。在本报告中，我们描述了一位因 PH 就诊于我科的患者的 PVOD 诊断面临的挑战。最终诊断通过肺活检确立。该患者接受了序贯联合治疗，并在密切监测下得到了显著的临床稳定，同时等待肺移植。
