Pérez Núñez Marta, Alonso Charterina Sergio, Pérez-Olivares Carmen, Revilla Ostolaza Yolanda, Morales Ruiz Rafael, Enguita Valls Ana Belén, Tenorio Jair Antonio, Gallego Zazo Natalia, De Pablo Gafas Alicia, Lapunzina Pablo, Rodríguez Chaverri Adriana, Escribano Subías Pilar
Servicio de Radiología, Unidad Multidisciplinar de Hipertensión Pulmonar, Hospital Universitario Doce de Octubre, 28041 Madrid, Spain.
Centro de Referencia Nacional de Hipertensión Pulmonar Compleja, Spain and ERN-Lung-Pulmonary Hypertension) Referal Center, 28041 Madrid, Spain.
Diagnostics (Basel). 2021 Jan 19;11(1):141. doi: 10.3390/diagnostics11010141.
Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the absence of histological or genetic confirmation, the diagnosis is complex, requiring a clinical suspicion. Multidetector computed tomography (MDCT) is an essential part of the non-invasive diagnostic tools of PVOD. We retrospectively reviewed the MDCT findings from a consecutive series of 25 patients diagnosed with PVOD, 9 with the sporadic form and 16 with the hereditary form of the disease. The presence and extent of typical findings of the diagnostic triad were assessed in all patients (ground glass parenchymal involvement, septal lines, and lymphadenopathy). In our series, 92% of patients showed at least two of the radiological findings described as typical of the disease. All patients presented at least one typical radiological characteristic. The incidence of radiological findings considered typical is very high, however was not associated with greater hemodynamic severity nor to the development of acute lung edema. No significant differences were found between the two groups. A poorly expressive MDCT does not exclude the disease.
肺静脉闭塞病（PVOD）是一种非常罕见的肺动脉高压形式，临床病程凶险，对特定血管扩张剂治疗反应不佳，生存率低。明确诊断对于指导治疗和评估肺移植至关重要。然而，在缺乏组织学或基因确诊的情况下，诊断较为复杂，需要临床怀疑。多排螺旋计算机断层扫描（MDCT）是PVOD无创诊断工具的重要组成部分。我们回顾性分析了连续25例诊断为PVOD患者的MDCT表现，其中9例为散发性，16例为遗传性。评估了所有患者诊断三联征典型表现的存在情况和范围（磨玻璃样实质受累、间隔线和淋巴结病）。在我们的系列研究中，92%的患者至少表现出两种被描述为该病典型的影像学表现。所有患者至少呈现一种典型的影像学特征。被认为典型的影像学表现发生率非常高，但与更高的血流动力学严重程度或急性肺水肿的发生无关。两组之间未发现显著差异。MDCT表现不典型并不能排除该病。
