Masters Kyle, Bennett Steven
Department of Medicine, Madigan Army Medical Center, Tacoma, Washington, USA.
BMJ Case Rep. 2013 Feb 1;2013:bcr2012007752. doi: 10.1136/bcr-2012-007752.
Pulmonary veno-occlusive disease (PVOD) is a rare and challenging cause of pulmonary hypertension. Clinical presentation is non-specific, including dyspnoea, cough and fatigue. Diagnosis of PVOD is typically based on high clinical suspicion with a definitive diagnosis confirmed by histology. Our case involves a healthy 21-year-old man who developed dyspnoea on exertion at an elevated altitude during deployment to Afghanistan. His work-up included an echocardiogram, a high-resolution CT scan, V/Q scan, pulmonary function tests with diffusion capacity, and a cardiac catheterisation with vasodilator challenge. Initially diagnosed with vasodilator responsive pulmonary arterial hypertension, an oral vasodilator was given with subsequent development of non-cardiogenic pulmonary oedema, thus confirming a clinical diagnosis of PVOD. He was medically stabilised with diuretic therapy, but developed progressive right-ventricular failure. For definitive treatment, he underwent a successful bilateral lung transplant. Explanted lung histology confirmed the diagnosis of PVOD.
肺静脉闭塞病(PVOD)是一种罕见且具有挑战性的肺动脉高压病因。临床表现不具特异性,包括呼吸困难、咳嗽和乏力。PVOD的诊断通常基于高度的临床怀疑,并通过组织学确诊。我们的病例涉及一名21岁健康男性,他在被派往阿富汗期间于高海拔地区出现劳力性呼吸困难。他的检查包括超声心动图、高分辨率CT扫描、通气/灌注扫描、肺功能测试及弥散功能测定,以及心脏导管插入术和血管扩张剂激发试验。最初被诊断为血管扩张剂反应性肺动脉高压,给予口服血管扩张剂后出现非心源性肺水肿,从而确诊PVOD的临床诊断。他通过利尿剂治疗病情稳定,但出现了进行性右心室衰竭。为进行确定性治疗,他成功接受了双侧肺移植。切除肺的组织学检查证实了PVOD的诊断。