What is primary pulmonary hypertension? As mentioned, primary pulmonary hypertension is a very rare condition wherein there is a gradual narrowing of the arteries in the lungs that eventually leads to high blood pressure against those arteries. Symptoms of primary pulmonary hypertension develop slowly over time. The early symptoms of primary pulmonary hypertension develop slowly like an individual might be suffering from primary pulmonary hypertension but it could not be identified for years. This is because the symptoms take time to develop and progress to the stage where a doctor can correctly identify it.
The most common symptom of pulmonary hypertension is the accumulation of calcium deposits within the arteries of the lung. These calcium deposits prevent the free flow of blood to the heart. The accumulation of calcium within the arteries of the lung causes the decreased blood flow to the heart muscle causing a decrease in its ability to pump blood to the rest of the body. The condition may also cause the heart muscle to become atrophied or softened.
The condition called primary pulmonary hypertension develops when there are inadequate numbers of heart valves or heart muscles present within the pulmonary system. Primary pulmonary hypertension occurs when the number of left ventricular outgrowths exceeds the number of right ventricular outgrowths. This condition is known to be congenital. This means that the condition develops in a person before he or she attains puberty. The most common congenital condition is Idiopathic Pulmonary Arterial Hypertosis or IPAH. IPAH develops when there are inadequate numbers or absence of heart valves.
The most common symptoms associated with IPAH are chest pain, shortness of breath, breathlessness and coughing. If there is sufficient growth of left ventricular outgrowths in lungs there will be an increase in blood pressure. The usual pathophysiological mechanism involved in secondary pulmonary hypertension is increased blood flow through the pulmonary artery, which leads to increased blood flow in heart muscles, which weakens them. In fact heart muscles contract because of the increase blood flow. So, in case of primary pulmonary hypertension the blood flow in heart muscles leads to increase heart failure.
Secondary pulmonary hypertension may include genetic factors, age, smoking, chronic obstructive pulmonary disease (COPD), diabetes mellitus and inflammation of lungs. It may include other risk factors like blood clot, allergic reaction to dust, smoking, high cholesterol, obesity, sedentary lifestyle and alcohol abuse. Other than these factors, the medications and therapies commonly used for treating IPAH also lead to this condition. Treatments like diuretics, antiplatelet, anti-inflammatory drugs, antioxidants, beta-blockers, aspirin and steroidal drugs are used for controlling the symptoms of IPAH.
The symptoms get worse over time and it becomes very difficult to control it. If someone has been treated successfully for IPAH then there is no need to monitor the progress of this disease during the entire duration of treatment. If the symptoms get worse over time then it becomes necessary to check with the doctor at regular intervals, even after the completion of treatment. The most important point to note is that patients with IPAH must not slow down their lifestyle or try and stop taking all the medicines, until the doctors tell them that the disease progresses no further.
It is very important for people diagnosed with IPAH to avoid smoking and keep themselves fit and active. Patients of IPAH may need to modify their lifestyle to prevent worsening of the condition. IPAH is not curable and cannot be cured overnight. If a person has been told that they have primary pulmonary hypertension and they are given the all clear by the doctor but still they want to take some more medicines, then they can do so under medical supervision.
Primary pulmonary hypertension is a dangerous condition because the increased pressures of the blood in arteries lead to increasing risk of heart attacks and stroke. The increased blood pressure in arteries leads to thickening of walls of arteries and restricted blood supply to the brain. If the arteries are blocked or narrowed, the chances of stroke are high. A stroke can lead to permanent vision loss and can even be fatal.