Hospital General de México Dr. Eduardo Liceaga, Clínica de Hipertensión Pulmonar, Servicio de Neumología, Ciudad de México, México.
Hospital General de México Dr. Eduardo Liceaga, Clínica de Hipertensión Pulmonar, Servicio de Neumología, Ciudad de México, México.
Curr Probl Cardiol. 2022 Apr;47(4):100856. doi: 10.1016/j.cpcardiol.2021.100856. Epub 2021 Apr 15.
The combination of pulmonary fibrosis and emphysema (CPFE) has been recently defined as a syndrome, it is radiologically recognized and is characterized by the simultaneous coexistence of emphysema of superior pulmonary location and fibrosis predominantly in lower lobes.
We present three patients with CPFE, who underwent right cardiac catheterization for pulmonary hemodynamic assessment, finding mean pulmonary artery pressure (mPAP) between 37-52 mm Hg (mean 45 mm Hg), who received treatment with specific vasodilators for pulmonary arterial hypertension (PAH).
The three patients had higher mPAP than expected for Group III (Pulmonary hypertension due to lung disease and/or hypoxia) of the classification of pulmonary hypertension (PH) by the World Health Organization (WHO), in whom the use of Sildenafil was justified by the presence of progressive dyspnea, and no symptoms suggestive of infectious exacerbation associated with right ventricular failure.
肺纤维化和肺气肿的合并（CPFE）最近被定义为一种综合征，它在影像学上被识别，其特征是同时存在上肺部位的肺气肿和主要在下肺的纤维化。
我们介绍了三例 CPFE 患者，他们接受了右心导管检查以评估肺血流动力学，发现平均肺动脉压（mPAP）在 37-52mmHg 之间（平均 45mmHg），他们接受了肺动脉高压（PAH）特异性血管扩张剂治疗。
这三例患者的 mPAP 高于世界卫生组织（WHO）肺动脉高压分类中 III 组（肺疾病和/或缺氧引起的肺动脉高压）的预期值，其中 Sildenafil 的使用是合理的，因为存在进行性呼吸困难，并且没有提示与右心衰竭相关的感染加重的症状。
