Pulmonary Arterial Hypertension (PAH) is a very serious, rare and often fatal lung condition. It causes high pressure in the pulmonary artery, which is the large artery connecting the lungs and heart. The increased pressure leads to right heart enlargement which, if left untreated, will cause right heart failure and eventual death.
There are three types of Pulmonary Arterial Hypertension. Pulmonary Arterial Hypertension is the most common. The causes of PAH include soft tissue disease, like Scleroderma, and Pulmonary Embolisms, and blood clots in the lungs. IPAH, (Idiopathic Pulmonary Arterial Hypertension) was formerly known as PPH (Primary Pulmonary Hypertension). IPAH is much less common than PAH, and much more difficult to diagnose, as it is often misdiagnosed as asthma or not diagnosed until the later, severe stages. Popular Pulmonary Hypertension treatments include Sildenafil (Viagra) and Bosentan (Tracleer). FPAH (Familiar Pulmonary Arterial Hypertension) is similar to IPAH in that it is often not diagnosed until the later, severe stage, but the cause is genetic. FPAH is the least common form of Pulmonary Hypertension and is found in parent-child genetics, but not grandparent-grandchild. If patients possess the gene to develop this condition, it is a marker, but does not necessarily mean they will develop the disease. FPAH was unknown until recently and was formerly diagnosed as PPH (Primary Pulmonary Hypertension) because the cause was unknown. Genetic research has only recently provided a cause naming this form as FPAH. The most severe Pulmonary Hypertension symptoms experienced by our members are Shortness of Breath and Heart Palpitations.
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