Pulmonary hypertension (PH) is a rare but potentially fatal type of high blood pressure. Rather than the more common high blood pressure that occurs throughout the entire body, PH is focused specifically in the arteries of the lungs, called pulmonary arteries. These particular arteries become damaged or get too narrow, and it becomes harder for the heart to pump blood through them. Eventually, the right ventricle (bottom chamber) of the heart, which is responsible for sending blood directly to the lungs, has to work so hard to pump efficiently, it gets worn out and weakened to the point of failure. While there is no cure for PH, there are several treatments that can make it less debilitating.
Vasodilators actually work to enlarge the blood vessels, thus making it easier for blood to pass through. Epoprostenol is one of the most commonly prescribed of these medications. Along with the potential to cause jaw pain, upset stomach, and leg cramping, the effects of epoprostenol don’t work for very long—only a few minutes. An intravenous catheter must be applied to the site and attached to a pump that can be carried in a shoulder or belt pack. Patients using epoprostenol must learn to take care of their own catheter, prepare their own medicine, and operate the pump properly. Frequent doctor visits are a necessity to make sure everything is working properly. Iloprost, on the other hand, comes in an inhalable form, and is taken through a nebulizer every three hours. Not only is it easier and less painful, the medication goes directly to the lungs, where it is needed. It may, however, cause difficulty breathing, chest pain, nausea or headaches.
Endothelin Receptor Antagonists
Endothelin is present in the walls of blood vessels and is the substance that causes them to narrow. An antagonist keeps this from happening by blocking the endothelin and reversing its effects. These medications should be strictly avoided during pregnancy. They may also cause serious damage to the liver, and so require careful monitoring of liver levels.
Sildenafil and tadalafil are also used for PH—although sildenafil (aka Viagra) has a much more common use as well. These medications help the affected blood vessels to open up and relax, so that blood can pass more easily. It may come in a liquid form, and side effects include dizziness, difficulties with vision, and stomach problems. These inhibitors can react badly with several different medications, so your physician should know any medication or supplements you take before you start a prescription.
Calcium channel-blockers are only effective in a few people with PH; they work similarly to phosphodiesterase inhibitors by relaxing vessel walls. An anticoagulant, such as warfarin, may also be prescribed to prevent blood clots from forming in vessels that have already grown smaller than they should be. Clots in the pulmonary vessels can lead to a pulmonary embolism, which is potentially fatal. A diuretic may be prescribed to help rid the body of any excess fluid, which reduces the heart’s work and prevents the lungs from developing too much fluid. Inhaling pure oxygen under a doctor’s instruction may provide additional assistance for patients suffering from PH who also have sleep apnea or live in high altitude areas.