![]() This is especially true for patients with Pulmonary Arterial Hypertension (PAH) in the current era of disease-specific drug therapy. Therefore, timely and accurate recognition of the underlying cause for PHT is essential for appropriate management. Pulmonary hypertension is not a diagnosis in itself it is caused by a number of differing diseases each with different treatments and prognoses. Pulmonary Hypertension (PHT) is relatively common, dangerous and under-recognised. These patients need early referral to transplantation centers. Without definitive treatment, the prognosis is very poor, and the life expectancy of these patients is much shorter than patients with pulmonary arterial hypertension. The recent discovery of the biallelic mutations of the EIF2AK4 gene as an etiology for heritable form of pulmonary veno-occlusive disease increases our understanding of the disease pathogenesis and potentially identifies a future approach to treatment. There is no effective medical therapy for these patients, and treatment with the pulmonary arterial hypertension specific medications often causes acute deterioration with pulmonary edema. Similar changes occur in the small arteries and arterioles but are less pronounced than the venous changes. ![]() This can result in total venous occlusion and subsequent recanalization. Lung biopsies demonstrate remodeling of the venules and small veins with intimal and adventitial fibrosis. Echocardiography can estimate the level of pulmonary artery pressures right heart catheterization is needed for complete hemodynamic characterization of these patients. High resolution computed tomography scans reveal ground glass opacities, interlobular septal thickening, and lymphadenopathy. Chest x-rays sometimes differ from idiopathic pulmonary arterial hypertension and may demonstrate alveolar infiltrates and pleural effusions. These patients present with nonspecific symptoms, including dyspnea, exercise intolerance, and weakness. The disease is poorly understood and difficult to diagnose it has no definitive cure to date. Pulmonary veno-occlusive disease is a rare subcategory of pulmonary arterial hypertension (WHO Group 1). It is characterized by the triad of severe pulmonary arterial hypertension, pulmonary congestion with Kerley's B lines 12,13 It is a disease of unknown cause which is manifested by progressive dyspnea and cyanosis and is usually fatal a few years after symptoms first appear. This disease was initially described in the 1930s. In addition to experimental evidence that the PAWP is not capillary pressure, there is also a disease which demonstrates this fact: pulmonary veno-occlusive disease (PVOD). There is a single outlet where CLINICAL CORRELATION Because there is no flow in the occluded segment, pressure everywhere in this segment is the same. When the artery is occluded, blood flow in the segment distal to the occlusion ceases, as is illustrated by the shaded area (Fig 1A). Figure 1A illustrates measurement of the PAWP. The difference between the PAWP and the pulmonary capillary pressure can be understood by realizing that the PAWP is actually a distorted measure of the pressure in the pulmonary veins.
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