Diagnosis
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History,
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Physical examination can show abnormal heart sounds, such as a split second heart sounds, neck vein enlargement, leg swelling, enlarged liver and spleen, and shortness of breath in advanced cases. Breath sounds may be normal.
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Chest x-ray may show right ventricular enlargement.
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Cat scan of the chest,
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EKG,
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Echocardiography
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Nuclear scan of the lung
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Cardiac catheterization- right heart and pulmonary artery pressure can be directly measured by this
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Pulmonary arteriogram
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Pulmonary function tests
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Genetic testing
Treatment
There is no known cure. The goal of treatment is to control symptoms. If cause of high blood pressure is known- treat the cause.
Treatment needs to be done in highly specialized centers.
Drugs used:
- Vasodilators-
- Epoprostenol- it is a short acting drug, given as continuous IV injection. Side effects include diarrhea, leg cramps and pain at injection site.
- Iloprost- also a vasodilator which can be inhaled via vaporizer. Side effects include chest pain, headache and shortness of breathe
- Endothelin receptor antagonist- Endothelin is a substance which causes blood vessels to constrict. Endothelin receptor antagonist blocks this action
- Sildenafil- causes pulmonary blood vessels to dilate
- Calcium channel blockers- these relax the muscles of the blood vessels, leading to blood vessel dilatation
- Diltiazem, nefidipine, and amlodipine
- Ambrisentan- stops narrowing of blood vessels. Side effects include serious liver damage
Anticoagulants are used in these patients to prevent clot formation. Diuretics decrease the work of the heart by eliminating excess fluid. Supplemental oxygen is used when necessary.
Lifestyle changes that help these patients include plenty of rest, moderate exercise, avoid smoking, avoid pregnancy, and maintain healthy weight and diet.
Complications
Right heart failure, blood clots, arrthymias, bleedings which may lead to hemoptysis.
Prognosis
Prognosis is very poor and can progress to heart failure and death. |
