An Autopsy Case of Pulmonary Veno-Occlusive Disease Complicated with Chronic Obstructive Pulmonary Disease and Severe Pulmonary Hypertension.

Chronic obstructive pulmonary disease (COPD) is a chronic inflammatory lung disease with obstructed airflow and frequently causes secondary mild-moderate pulmonary hypertension (PH). However, a low proportion (1%-5%) of COPD patients develop severe therapy-resistant PH, and it is crucial to determine whether the patient has another disease capable of causing severe PH, including pulmonary arterial hypertension.Here, we describe a case of a 71-year-old male with COPD complicated by severe PH and right heart failure. He had a history of heavy smoking and developed progressive hypoxemia on exertion. He had severe airflow limitation (forced expiratory volume % in one second, FEV 1.0% = 42.8%) with a markedly reduced diffusing capacity of the lung (predicted diffusion capacity of carbon monoxide, %DLCO = 29%), and high-resolution computed tomography (CT) demonstrated significant lung parenchymal abnormalities such as diffuse interlobular septal thickening, ground-glass opacities, and enlarged mediastinal lymph nodes. He was diagnosed with group 3 PH caused by COPD but resistant to the treatment of COPD, diuretics, and oxygen therapy. Pathohistological analysis of autopsy specimens revealed the coexistence of interstitial fibrosis and partial occlusion of the small intrapulmonary veins, which led to a conclusive diagnosis of pulmonary veno-occlusive disease (PVOD).Because of its rarity and similarity with idiopathic pulmonary arterial hypertension, PVOD is difficult to diagnose antemortem and has a poor prognosis. High-resolution CT findings (septal thickening, ground glass, and enlarged lymph nodes) and severely reduced DLCO should be carefully evaluated for the early detection and treatment of PVOD in COPD patients with severe PH.

Application of pressure-derived myocardial fractional flow reserve in chronic hemodialysis patients.

BACKGROUND: Although fractional flow reserve (FFR) measurements during coronary angiography are performed in routine clinical practice, few studies have evaluated FFR measurements in dialysis patients. METHODS: We retrospectively studied 42 hemodialysis patients with suspected or known coronary artery disease (CAD) who underwent stress myocardial perfusion imaging and coronary angiography with FFR measurements for 61 coronary lesions. The cut-off value for FFR to detect myocardial ischemia was determined by receiver operating characteristic (ROC) curve analysis. RESULTS: There were 61 coronary vessels measured by FFR. The FFR range was 0.34-0.93 with a mean of 0.74±0.13. The ROC curve analysis revealed that the best cut-off value of FFR for detecting myocardial ischemia was 0.76 (p<0.0001), with 70% sensitivity, 86% specificity, and 76% accuracy for myocardial ischemia. Compared with patients who had positive myocardial ischemia and an FFR≤0.76, those who had negative myocardial ischemia despite an FFR≤0.76 had less left ventricular (LV) mass index, whereas patients who had positive myocardial ischemia despite an FFR>0.76 had greater LV mass indexor serum calcium-phosphorus product. CONCLUSIONS: The cut-off value of FFR for myocardial ischemia in chronic hemodialysis patients is similar to that in other CAD patients. However, caution is necessary when FFR measurements are applied to dialysis patients with significantly increased LV mass index or serum calcium-phosphorus product.