What are PPH & PAH ?
Primary pulmonary hypertension (PPH) and Pulmonary Arterial Hypertension (PAH) are rare and often fatal diseases that results in the progressive narrowing of the blood vessels of the lungs which as a result causes high blood pressure in these blood vessels and eventually leads to heart failure. The lungs contain millions of tiny blood vessels called capillaries. These capillaries are extremely small, just wide enough for blood cells to move through. The capillaries are lined with endothelial cells that prevent blood from leaking out of the vessel. The outside of the blood vessels are muscle type cells that expand and contract as blood moves through from the heart into the lungs. PAH & PPH causes the cell lining of the lung’s blood vessels to weaken and allow leakage. The leaking of blood out of the vessels causes the muscles that surround the blood vessels to constrict. This continuous constriction increases the pressure within the blood vessels and cuts off blood flow. The constrictio
