An 82-year-old woman presented with dyspnea on exertion and weight gain of 5 kg over the past month. Her medical history included atrial fibrillation and lung adenocarcinoma (a lung lobectomy was performed 8 years ago). Her blood pressure was 112/60 mm Hg, her heart rate was 100 beats per minute, her respiratory rate was 37 breaths per minute, her body temperature was 36.6°C, and her oxygen saturation was 100% while breathing 10 L of oxygen. A cardiac examination was clinically significant for an irregular rhythm, an increased pulmonic closure sound, and jugular venous distension. Bibasilar coarse crackles and pretibial edema were also noted. A complete blood count had results within normal limits. The serum lactate dehydrogenase level was 278 U/L (to convert to microkatal per liter, multiply by 0.0167). A chest radiographic image showed bibasilar infiltrates. An electrocardiogram was notable for atrial fibrillation, and echocardiography revealed normal left ventricular function, mild right atrial and ventricular dilatation, and severe tricuspid regurgitation (transtricuspid pressure gradient, 40 mm Hg). A contrast-enhanced computed tomography image revealed bilateral ground-glass opacities and right basilar consolidations, with no evidence of thromboembolisms (Figure, A). Para-aortic and inguinal lymphadenopathies were also noted. Presumptive diagnoses of pulmonary hypertension (PH) and right-sided heart failure were made. The patient began receiving dobutamine and furosemide, but she continuously required 2 to 10 L of oxygen, and further invasive evaluations could not be performed. An echocardiogram identified aggravating PH (transtricuspid pressure gradient, 57 mm Hg). A thorough physical examination identified scaly, crusty genital skin lesions pathologically compatible with extramammary Paget disease. The patient deteriorated and died despite treatment. An autopsy was performed (Figure, B).

一名82岁的女性在过去一个月内因劳累和体重增加5公斤而出现呼吸困难。她的病史包括房颤和肺腺癌（8年前进行了肺叶切除术）。她的血压为112/60毫米汞柱，她的心律为每分钟100次搏动，呼吸频率为每分钟37次呼吸，她的体温为36.6°C，在呼吸10升氧气时其氧饱和度为100％。心脏检查对于节律不规则，肺动脉闭锁声音增加和颈静脉扩张具有临床意义。还注意到双基底粗大裂纹和胫前水肿。全血细胞计数的结果在正常范围内。血清乳酸脱氢酶水平为278 U / L（转换为每升微katal，乘以0.0167）。胸部影像学检查显示双基底浸润。心电图表现为房颤，超声心动图显示正常的左心室功能，轻度的右心房和心室扩张以及严重的三尖瓣关闭不全（经窦房压梯度为40 mm Hg）。对比增强的计算机断层扫描图像显示双侧毛玻璃混浊和右基底巩固，没有血栓栓塞的迹象（图A）。还注意到主动脉旁和腹股沟淋巴腺病。做出肺动脉高压（PH）和右侧心力衰竭的推定诊断。患者开始接受多巴酚丁胺和速尿，但她持续需要2至10 L的氧气，因此无法进行进一步的侵入性评估。超声心动图可识别出加重的PH（穿刺压力梯度，57 mm Hg）。彻底的体格检查发现鳞状，结的生殖器皮肤病变在病理上与乳腺Paget病相容。尽管治疗，该患者恶化并死亡。进行了尸检（图B）。