Pulmonary arterial hypertension (PAH) is a Rare Disease that causes an increase in the pulmonary vascular resistance and an increase in blood pressure in the pulmonary circulation. The reduced blood flow through the lungs is accompanied by an insufficient supply of oxygen to the body. As a consequence, patients suffer from lethargy, shortness of breath and a drop in physical performance. If PAH is left untreated for two or three years after first diagnosis has been made, it will lead to life-threatening heart failure. Early diagnosis and commencement of proper treatment are important for the progress of the disease and the prognosis.
Pulmonary hypertension occurs when the mean arterial blood pressure in the pulmonary artery increases above 25 mmHg during rest (normal pressure: <20 mmHg).
Pulmonary hypertension means that there is an increase in pressure in the pulmonary circulation due to a narrowing of the blood vessels in the lungs. This places extra strain on the heart as it tries to continue pumping a sufficient amount of blood through the narrowed blood vessels of the lungs. These blood vessels will continue to narrow and become increasingly inelastic. If the arteries (the blood vessels that lead away from the right ventricle of the heart) are affected, then the disorder is called pulmonary arterial hypertension.
As the disease progresses, the right heart muscle mass will continue to increase in size to the point that the heart will finally lose the ability to pump enough blood into the lungs. The supply of oxygen to the body and heart will then deteriorate further. If the disease is left untreated, it will eventually lead to heart failure.
There are four types of pulmonary hypertension. Pulmonary arterial hypertension is one type and can be further divided into:
The primary symptom of the disease is overwhelming fatigue and lack of breath to the extent that breathing assistance may be necessary.
Other symptoms that may be present include:
If a patient is suspected of having pulmonary hypertension (due to poor physical performance and impaired breathing), they will undergo a comprehensive physical examination and their medical history will be analyzed. Aside from the family medical history, it is also very important to provide details of any previous drug prescriptions and possible substance abuse.
Other possible tests:
After a patient is diagnosed with pulmonary arterial hypertension, they must learn how to adapt to the new condition.
Strenuous physical activity should be avoided since the lungs cannot provide the body with the necessary oxygen requirements any longer. Breathing difficulties are a clear sign that the strenuous activity should be stopped. However, it is very important that the patient does light exercise and walks regularly in order to maintain muscle strength and to encourage circulation.
The patient's diet should be rich in micronutrients, fiber, and vitamins. However, the intake of cooking salt should be reduced.
Alcohol should only be enjoyed in small amounts.
Bodyweight needs to be inspected at regular intervals, preferably always at the same time of the day. Sudden increases in bodyweight can be a signal of a deterioration in the heart and kidneys.
Both active and passive smoking should be avoided.
If a female patient wants to have a child it is of utmost importance for her to first consult a PAH specialist given the increased risk for expectant mothers and their unborn children caused by PAH.