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特发性肺高压患者出现不常见病因:部分性肺静脉异位连接致肺动脉高压。

An unexpected and unusual cause of pulmonary hypertension in a patient with hypersensitivity pneumonitis: a partial anomalous pulmonary venous connection causing pulmonary artery hypertension.

机构信息

Department of Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry.

Department of Radiodiagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry.

出版信息

Monaldi Arch Chest Dis. 2023 Mar 3;94(1). doi: 10.4081/monaldi.2023.2497.

Abstract

Partial anomalous pulmonary venous connection (PAPVC) occurs when any pulmonary vein, but not all, drains directly into the right atrium or its venous tributaries. PAPVC can very rarely present as an individual cause of pulmonary artery hypertension. Here we are presenting the case of a 41-year-old farmer with a history of exertional dyspnea for the past 3 years, which increased over 6 months. Chest high-resolution computed tomography was suggestive of non-fibrotic hypersensitivity pneumonitis. Hence, the patient was started on systemic steroids, with which the patient's oxygen saturation improved. On 2D echocardiography, the right ventricle systolic pressure was 48 + right atrial pressure. Right heart catheterization showed a mean pulmonary artery pressure of 73 mmHg and pulmonary vascular resistance of 8.7. On further evaluation, a computed tomography pulmonary angiogram was done, which surprisingly revealed the left superior pulmonary vein draining into the left brachiocephalic vein.

摘要

部分肺静脉异常连接(PAPVC)是指任何一条肺静脉,但并非全部,直接流入右心房或其属支静脉。PAPVC 非常罕见地可作为肺动脉高压的一个独立病因。在此我们报告一例 41 岁农民,3 年来劳力性呼吸困难,近 6 个月加重。胸部高分辨率 CT 提示非纤维化型高敏性肺炎。因此,给予患者全身类固醇治疗,氧饱和度有所改善。二维超声心动图显示右心室收缩压为 48+右心房压。右心导管检查显示平均肺动脉压为 73mmHg,肺血管阻力为 8.7。进一步评估时,行 CT 肺动脉造影,结果令人惊讶地显示左肺上静脉直接流入左头臂静脉。

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