Pulmonary hypertension (PH) is a life threatening, progressive disease in which there is elevated blood pressure in the arteries supplying the lungs. Pulmonary hypertension shows an imbalance between endothelial derived vasodilators and vasoconstrictors that causes pulmonary vasoconstriction and vascular proliferation. This causes disruption in the blood flow from heart to the lungs increasing the workload on heart, eventually leading to weakness in the heart muscles. Mean pulmonary arterial pressure above 25 mm Hg is considered as pulmonary arterial hypertension.
Pulmonary hypertension can be idiopathic (no known cause), known as primary pulmonary hypertension as well as secondary, which results due to other diseases. The different causes of secondary pulmonary hypertension include autoimmune diseases such as rheumatoid arthritis and SLE, connective tissue diseases including scleroderma, CREST syndrome, liver diseases, and congenital heart diseases such as atrial and ventricular septal defects, HIV infection and schistosomiasis. Other causes include drugs such as diet pills (fenfluramine and dexfenfluramine) and recreational drugs, chronic obstructive pulmonary disease (COPD), emphysema, pulmonary fibrosis (interstitial lung diseases), sleep apnea, pulmonary emboli, mitral valve disease, chronic systemic elevated blood pressure, polycythemia vera, thrombocythemia, metabolic disorders such as thyroid diseases and glycogen storage disease, systemic disorders including sarcoidosis and vasculitis, and kidney diseases.
What are the Symptoms of Pulmonary Hypertension?
The symptoms of pulmonary hypertension arise from increased pressure in the pulmonary arteries, leading to constriction of the blood vessels. This in turn creates excess stress to heart muscles ultimately leading to weakness of heart muscles with time. The common symptoms of secondary pulmonary hypertension are fatigue, difficulty breathing and dizziness. Other symptoms include rapid breathing, exertional shortness of breath (dyspnea) as well as dyspnea on rest, chest pain or pressure, cyanosis (bluish discoloration of skin and lips), anxiety, lower extremity swelling, tachycardia, palpitations, distention of jugular vein, ascites (fluid collection in the abdomen), clubbing of fingers, non-productive cough, and exercise induced nausea and vomiting. In addition, severe pulmonary hypertension may also lead to heart failure, blood clots, bleeding and arrhythmias.
The diagnosis of pulmonary hypertension is difficult to make as the symptoms of pulmonary hypertension often overlap those of other heart and lung diseases. Therefore, it is not diagnosed until it becomes very severe. In addition to blood tests, certain imaging tests are also done including right heart catheterization, echocardiogram (heart ultrasound), pulmonary function tests, chest X-ray, computed tomography, electrocardiogram, stress test and nuclear medicine lung scan. The treatment plan is based on the results of these tests as these tests also help in finding out the cause of disease.
What Is The Treatment For Pulmonary Hypertension?
Unfortunately, there is no definitive treatment for pulmonary hypertension. The aim of the treatment is to slow progression of disease by improving quality of life of the patient with various therapies. Phosphodiesterase-5 inhibitors (PDE 5) inhibitors cause vasodilatation of the pulmonary blood vessels by facilitating cGMP and NO. PDE 5 inhibitors used are sildenafil (Revatio) and tadalafil (Adcirca) in oral form. Prostacyclin Analogue reduces blood pressure by relaxation of pulmonary blood vessels. The prostacyclin used is treprostinil, which can be taken orally (Orenitram), inhaled (Tyvaso), IV (Remodulin), and subcutaneously. Endothelin Receptor Antagonists (ERAs) prevent vasoconstriction. They are usually taken orally and the various ERAs used are ambrisentan (Letairis), macitentan (Opsumit) and bosentan (Tracleer). Selective IP Receptor Agonist helps in relaxing blood vessels in the lungs by activating prostacyclin receptor. The example is selexipag (Uptravi) taken orally. Soluble Guanylate Cyclase (sGC) relaxes pulmonary blood vessels by increasing interaction of sGC with NO. Example includes riociguat (Adempas). Other treatment options include calcium channel blockers, anticoagulants in the form of warfarin (Coumadin). Digoxin is used for better heart pumping, diuretics for extra fluid removal from the body and artificial oxygen in cases of hypoxemia.
In severe cases of PH, along with medications surgeries such as atrial septostomy, lung transplant or heart-lung transplant may be recommended. All the above treatments help prolong survival rate of pulmonary hypertension patients.
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