Maladie veino-occlusive et hémangiomatose capillaire pulmonaire

摘要: The new classification of pulmonary hypertension proposed in the joint European Society Cardiology (ESC) and Respiratory (ERS) guidelines, combines veno-occlusive disease (PVOD) capillary hemangiomatosis (PCH) from separate categories into a single subcategory within arterial (PAH) because specific similarities their diagnosis, prognosis, management. These diseases are characterized histologically by predominant involvement small veins capillaries (PCH). Their precise prevalence is not known, but they thought to account for 5 10% forms PAH initially considered idiopathic. They cannot be distinguished idiopathic clinical or hemodynamic presentation. Only pathology examination can confirm lung biopsies high-risk procedures recommended. A less invasive approach combining chest CT (centrilobular ground-glass opacities, septal lines, mediastinal adenopathy), blood gases (resting hypoxemia), function tests [collapse carbon monoxide diffusion (DLCO)] bronchoalveolar lavage (occult intra-alveolar hemorrhage) makes it possible screen patients at risk PVOD HCP thus avoid biopsy. have poor prognosis developing severe edema after initiation treatment PAH. In view limited response transplantation remains choice HCP. with most disease, prudent use continuous intravenous epoprostenol, serve as bridge-therapy while awaiting transplant.