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Pulmonary Hypertension (PH) is an often neglected, yet serious condition that affects blood pressure in the lungs. PH means high blood pressure in the lung arteries. Normally, these arteries (pulmonary arteries) are low pressure, but in PH, they become narrowed/blocked and since the right side of the heart carries blood from the heart to the lungs, the pressure on the right side of the heart increases as the heart needs to work harder to pump blood through the arteries. This condition is detected in an echocardiogram (echo) of the right side of the chest.
Classical symptoms of pulmonary hypertension we often observe in patients include tiredness while walking, shortness of breath, and sometimes heart palpitations. This breathlessness can be so severe that individuals may struggle to take even one or two steps. In advanced cases, signs of right-sided failure such as swelling of the legs (edema), the impact of the liver, and kidney failure may be seen. There are various causes of pulmonary artery hypertension. When a blood vessel is affected, whether through inflammation, injury, or other pathological processes, it can play a significant role in the development of pulmonary artery hypertension. This is known as primary pulmonary hypertension (Group 1 PAH). It can also occur due to defects in the heart (holes in the heart), left heart failure which may cause back pressure affecting the right heart, and chronic lung disease (like Chronic Obstructive Pulmonary Disease) increasing pressure on the heart. Blood clots in the lungs or legs that travel to the lung artery are also a risk factor for pulmonary artery hypertension. In addition, specific diseases such as sarcoidosis can produce these symptoms that may indicate the presence of pulmonary hypertension.
There is a slight genetic component, but most cases are sporadic. Another important cause of primary pulmonary hypertension is HIV, as people with HIV have a higher chance of developing this condition. Thus, it is crucial to identify the etiology to formulate an effective treatment plan. Primary pulmonary hypertension commonly affects adults between the ages of 20 and 50 years, but it can occur in other age groups as well. The typical prevalence of this condition is one in a million and other associated conditions should be evaluated to check for a person’s risk of developing PH. While there’s no cure for pulmonary hypertension, treatment can help manage symptoms and improve quality of life. The specific treatment approach depends on the underlying cause behind high blood pressure in your lungs. If a problem with your left ventricle is the cause, medications or procedures will focus on treating the condition and improving your left heart function. Similarly, the treatment for pulmonary hypertension due to lung diseases like COPD might involve medications, oxygen therapy, or pulmonary rehabilitation programmes.
If it is a blood clot, blood thinners will be used to prevent further clotting and dissolve existing clots. However, the emphasis on treatment is for the specific type called primary pulmonary hypertension (Group 1 PAH). For this specific type, there are exciting new treatment options that offer significant hope to patients dealing with this condition.
Typically, doctors will combine two or three medications from different categories to achieve the best results. Therapies such as Ambrisentan and prostaglandin agonists are proven to be highly effective as life expectancy is increasing and patients are responding well to these new therapies. Prostaglandin agonists are available in tablet form. Other therapies are sildenafil and tadalafil which help dilate the lung arteries and calcium channel blockers. Endothelin receptor antagonists (ERAs) like Bosentan and Ambrisentan block the action of endothelin, a molecule that constricts blood vessels. Revocoglumab (Revociglo) is a separate medication used specifically for treating blood clots in the lungs (pulmonary embolism) — a significant cause of PH. Since pulmonary hypertension is a complex condition, it requires specialised treatment, and not all medical centres are equipped to handle it. Proper management should be carried out over a longer period if patients respond well to therapy.
(The author is a Bengaluru-based HOD & consultant in interventional cardiology.)