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 04/25/2024

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Therapy for Pulmonary Hypertension
 
Basic Facts
Pulmonary hypertension is persistent high blood pressure in the pulmonary arteries, which carry blood from the heart to the lungs, or in the pulmonary veins, which carry blood from the lungs to the heart.
Because there is no cure for pulmonary hypertension, the goal of therapy is to reduce high pulmonary blood pressure, as well as to treat the symptoms and the underlying cause of the condition.
Medications used to treat pulmonary hypertension include vasodilators, anticoagulants, and diuretics. For the most severe cases, lung transplant may be required.
The goal of medical treatment for pulmonary hypertension is to relieve symptoms and prolong life. This is done first by treating the underlying causes of the condition. Next, medications such as calcium channel blockers, anticoagulants, diuretics, and digitalis are prescribed to lower blood pressure, reduce clot formation, dilate, or widen, the blood vessels, and, in the case of accompanying congestive heart failure, reduce excess fluid accumulation.

  • Calcium channel blockers to slow the heartbeat and dilate (widen) the arteries;
  • Diuretics to reduce excess fluid accumulation, especially if the patient also has congestive heart failure;
  • Anticoagulants to reduce clot formation when the pulmonary hypertension is caused by a multiple pulmonary emboli (blood clots) that lodge in the pulmonary artery and obstructs blood flow. Anticoagulants also relieve symptoms in people with pulmonary hypertension who do not have a pulmonary embolism;
  • Digitalis to strengthen the force of heart contractions and improve circulation to the lungs;
  • Pulmonary artery dilators to reduce blood pressure by relaxing artery walls, as well as helping to prevent clots from forming;
  • Edothelin receptor antagonists to prevent constriction and thickening of blood vessel walls; and
  • Sildenafil (Revatio) to relax the arteries in the lungs allowing blood to flow more freely through them.
PRE-TREATMENT GUIDELINES

Because of possible interactions with other drugs, the patient should report any other medications that he or she is taking to a physician prior to undergoing medication treatment for pulmonary hypertension. Pregnant women should discuss these treatment methods with their physician because of possible harm to the fetus.

WHAT TO EXPECT

Calcium channel blockers. These medications usually take effect within 20 minutes to 1 hour. Calcium channel blockers usually act for 12 to 24 hours.

Common side effects may include:
  • Headache;
  • Constipation;
  • Drowsiness;
  • Nausea;
  • Fatigue;
  • Flushing;
  • Sudden weight gain; and
  • Swelling of the legs, feet, or ankles.
The patient should report any of the following serious side effects to a physician immediately:
  • Shortness of breath; and
  • Very slow and/or irregular heartbeat.
Diuretics. These medications usually take effect within 2 hours. Diuretics usually act for 6 to 12 hours.

Common side effects may include:
  • Dizziness;
  • Thirst;
  • Dry mouth;
  • Constipation;
  • Muscle cramps;
  • Weakness; and
  • Heart palpitations.
The patient should report any of the following serious side effects to a physician immediately:
  • Hives;
  • Skin rash;
  • Mouth and throat swelling;
  • Lightheadedness;
  • Difficulty breathing;
  • Unusual bruising or bleeding;
  • Muscle pain; and
  • Weakness.
Anticoagulants. These medications usually take effect in 36 to 48 hours. Anticoagulants usually act for 24 to 96 hours.

Common side effects may include:
  • Nausea;
  • Skin rash;
  • Weight loss;
  • Diarrhea; and
  • Cramping.
The patient should report any of the following serious side effects to a physician immediately:
  • Difficulty breathing;
  • Hives;
  • Swelling;
  • Nosebleeds;
  • Blood in urine;
  • Bluish toes;
  • Excessive bleeding anywhere;
  • Very heavy menstrual period;
  • Black vomit; and
  • Bruising.
Digitalis. Digitalis usually takes effect in 30 minutes to 2 hours. Digitalis usually acts from 3 to 4 days.

Common side effects may include:
  • Fatigue;
  • Weakness;
  • Nausea;
  • Agitation;
  • Erectile dysfunction; and
  • Male breast enlargement.
The patient should report any of the following serious side effects to a physician immediately:
  • Dizziness;
  • Palpitations;
  • Sweating;
  • Shortness of breath;
  • Fainting;
  • Hallucinations;
  • Mental confusion;
  • Agitation;
  • Extreme weakness;
  • Extreme fatigue;
  • Nausea; and
  • Vision disturbances (blurred vision, seeing double, seeing spots).
Pulmonary arterial dilators. These medications include epoprostenol (Flolan), iloprost (Ventavis), and treprostinil (Remodulin).

Common side effects of pulmonary arterial dilators include:
  • Anxiety;
  • Bone and muscle pain;
  • Chills;
  • Confusion;
  • Infection at catheter site;
  • Jaw pain;
  • Nosebleeds;
  • Pain at injection site; and
  • Palpitations (awareness of one's heartbeat).
Patients who experience any of the following serious side effects should call their physician immediately:
  • Diarrhea;
  • Headache;
  • Fever;
  • Dizziness or fainting;
  • Nausea or vomiting; or
  • Flushing.
Patients who are taking epoprostenol should carry a card or wear an identification bracelet indicating so.

Endothelin receptor antagonists. These medications include bosentan (Tracleer) and ambrisentan (Letairis). Common side effects of endothelin receptor antagonists include:
  • Nasal congestion;
  • Headache;
  • Flushing;
  • Lower extremity (legs and feet) swelling;
  • Liver damage; and
  • Lowered sperm count.
Because of the risk of liver damage and to minimize the risk to fetuses, ambrisentan can only be prescribed through the Letairis Education and Access Program (LEAP). Bosentan can also interact with cholesterol-lowering medications and decrease their effectiveness. Patients should tell their physician if they are taking lipid-lowering medications. In addition, bosentan may decrease the effectiveness of hormonal contraceptives, such as birth control pills. Because it could potentially damage the fetus women who are pregnant or may become pregnant should not take bosentan. Ambrisentan can also interact with cyclosporine A, a medication given to prevent transplant rejection, anti-fungal medications such as ketoconazole, and medications for acid reflux.

Sildenafil (Revatio). This drug works by indirectly increasing nitric oxide levels which causes blood vessels to expand (dilate). Common side effects of sildenafil include:
  • Headache;
  • Heartburn;
  • Flushing;
  • Stomach upset;
  • Dizziness;
  • Temporary muscle aches;
  • Nose bleeds;
  • Difficulty sleeping; and
  • Rash.
Patients who take medications such as nitrates or alpha blockers should not take sildenafil because it can cause a sudden drop in blood pressure.

POST-TREATMENT GUIDELINES

These drugs require medical supervision. The patient should not stop taking these medications with first consulting a physician.

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