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Pulmonary hypertension is defined as a mean PAP of at least 20 mm Hg (3300 Pa) at rest, and PAH is defined as precapillary pulmonary hypertension (i.e. mean PAP ≥ 20 mm Hg with pulmonary arterial occlusion pressure [PAOP] ≤ 15 mm Hg and pulmonary vascular resistance [PVR] > 3 Wood Units). [58]
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. [2] The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure .
Chronic thromboembolic pulmonary hypertension (CTEPH) is a long-term disease caused by a blockage in the blood vessels that deliver blood from the heart to the lungs (the pulmonary arterial tree). These blockages cause increased resistance to flow in the pulmonary arterial tree which in turn leads to rise in pressure in these arteries ...
Eisenmenger syndrome or Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) causes pulmonary hypertension [1] [2] and eventual reversal of the shunt into a cyanotic right-to-left shunt.
Pulmonary capillary hemangiomatosis (PCH) is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance. [1] It is a rare cause of pulmonary hypertension, and occurs predominantly in young adults.
The pathophysiology of pulmonary heart disease (cor pulmonale) has always indicated that an increase in right ventricular afterload causes RV failure (pulmonary vasoconstriction, anatomic disruption/pulmonary vascular bed and increased blood viscosity are usually involved [1]), however most of the time, the right ventricle adjusts to an overload in chronic pressure.
Surgery is indicated in patients with pulmonary artery emboli that are surgically accessible. Thrombi are usually the cause of recurrent/chronic pulmonary emboli and therefore of chronic thromboembolic pulmonary hypertension (CTEPH). [2] PTE is the only definitive treatment option available for CTEPH. [3]
Pulmonary vein stenosis can be congenital or acquired. [6]A rare abnormality that accounts for 0.4% of congenital heart diseases, congenital pulmonary vein stenosis results from the common right or left pulmonary vein failing to integrate into the left atrium (LA) during the vessel's embryonic development, obliterating the pulmonary veins partially or completely on one or both sides.