LYCOS RETRIEVER 
Primary Pulmonary Hypertension: Heart
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Individuals with Primary Pulmonary Hypertension can be treated with calcium channel blocking drugs given by mouth. By relaxing the smooth muscle in the walls of the heart and blood vessels, these calcium channel blockers improve the ability of the heart to pump blood. In addition, a vasodilator is helping some severely ill individuals who are unresponsive to treatment with calcium channel blockers. These medicines enable the vessels in the lungs to expand and allow the blood to move through them with less resistance. The primary drugs in this category are prostacyclin, which is given as a continuous intravenous infusion, and treprostinil, which is given as a continuous infusion under the skin. Protstacyclin seems to improve Primary Pulmonary Hypertension and permit more physical activity.
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Pulmonary hypertension (PHT) is an elevation in pulmonary arterial pressure that can be the result of diverse diseases. Primary pulmonary hypertension (PPH) is a term that describes this condition when no cause for the PHT can be found. In secondary PHT, a coexisting disease or stimulus has been identified that presumably explains the PHT. Recently, a new classification was proposed at a World Health Organization–sponsored symposium. It uses terminology that better characterizes our current understanding of the biological mechanisms involved in the various types of PHT.1 In this nomenclature, pulmonary arterial hypertension (PAH) refers to a disease spectrum with a common pathological picture and shared pathobiological processes that include PPH and PHT which cannot be distinguished from PPH. PAH can occur in association with collagen vascular disease or congenital heart disease, or it may be triggered by an exogenous stimulus such as an anorexigen or the HIV virus.
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Primary pulmonary hypertension (PPH) is a progressive disorder characterized by increased pulmonary arterial pressure and pathologic changes in precapillary pulmonary arteries. In the U.S. National Institutes of Health registry, PPH is defined as mean pulmonary artery pressure (MPAP) greater than 25 mm Hg at rest (30 mm Hg with exertion) in the absence of heart disease, chronic thromboembolic disease, underlying pulmonary disorder, or secondary causes [1]. An increase in pulmonary vascular resistance (PVR) and subsequent compensatory right ventricular hypertrophy leads to an increase in pulmonary pressure that often results in increased right ventricular afterload and failure. The disorder progresses, leading to right heart failure and death a median of 2.8 years after diagnosis [2, 3].
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Primary pulmonary hypertension is generally considered to be a genetic disorder characterized by high blood pressure in the respiratory system. People who suffer from PPH generally have a difficult time exercising or experience shortness of breath during simple to moderate activity. In extreme cases it can lead to heart failure and death.
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Primary pulmonary hypertension occurs when no cause is found. Pulmonary hypertension may be associated with other conditions such as severe sleep apnoea, emphysema, pulmonary emboli, some congenital heart defects, left heart failure and autoimmune disorders.
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Pulmonary venous hypertension is a common disorder associated with pulmonary hypertension. Pulmonary venous hypertension is usually caused by dysfunction of the left side of the heart (e.g. mitral valve or left ventricle).
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