Pulmonary arterial hypertension (PAH) is a rare disease that affects the lungs and the arteries that carry blood from the heart. PAH can be caused by certain medicines or by other medical conditions such as connective tissue diseases, surgical complications, congenital heart defects, and autoimmune disorders.
In patients with PAH, the heart's right ventricle becomes larger to compensate for the inability of blood vessels in the lungs to expand. This can lead to reduced cardiac output and shortness of breath. This occurs because pulmonary hypertension can cause the right ventricle to enlarge.
This sign may also be caused by other conditions such as sleep apnea, asthma, and chronic obstructive pulmonary disease (COPD).
Palpitations are an abnormal awareness of your heartbeat. They may be pulsating, irregular, or skipped altogether. Palpitations are common in individuals with heart problems, but it is unusual for them to become aware of their heartbeat, called tachycardia. Palpitations are also very common in patients with PAH.
Palpitations are often a symptom of PAH. Palpitations are caused by the rapid heart rate that occurs in most patients with PAH or caused by the shortening of your diastolic relaxation time (the amount of time between when your heart muscle relaxes after systole and before it contracts again to start another systole).
Pulmonary artery stenosis or aortic valve stenosis is a narrowing of the passageway between your heart and your lungs. The result is that less blood can flow from your heart to the lungs to make up for the oxygen you need. As a result, you may have to take more medicine or may even need a heart transplant. The oxygen shortage also makes blue lips common in patients with pulmonary hypertension.
Patients with PAH will likely have a cough and may have a persistent fever. In severe cases, there may be signs of severe pulmonary hypertension such as blood clotting, difficulty breathing, and heart failure.
As the right ventricle continues to work harder to pump blood out of the lungs, it becomes overworked. This leads to high blood pressure in the pulmonary arteries, which places stress on other parts of your body.
This can lead to paleness or a bluish tint to the skin. The excess force of blood flowing out of the pulmonary arteries can also cause fatality in these arteries. This excess force makes it hard for the arteries around other organs to do their jobs such as keeping your digestive track working or keeping your brain alive.
Many patients with pulmonary hypertension will have engorged neck veins. Engorged neck veins are caused by the increased pressure in the pulmonary arteries causing fluid to get pushed into your lungs. This can lead to chest pain and shortness of breath. This fluid buildup can also lead to bacterial infections in the lungs.
To help reduce the blood pressure in the pulmonary arteries, it is common to take a diuretic. This is a type of medication that helps the body retain less fluid. Diuretics can make engorged neck veins worse because they encourage fluid retention.
A heart murmur is a sign that your heart is working harder than it should be. The right ventricle of your heart becomes enlarged (called cardiac hypertrophy). This enlargement makes the heart muscle stiffer, making it harder for the ventricle to fill with blood.
A heart murmur is common in patients with PAH. The murmur can be a "slur" sound that occurs with the ejection of blood from the right side of the heart.
The murmur may vary in pitch, ranging from a "whooshing" sound to softer sounds like a sighing or grumbling noise.
When you develop PAH, less oxygen will flow into your bloodstream. As a result, you will have lower levels of carbon dioxide in your bloodstream. Your body uses carbon dioxide as a way to measure how hard it needs to breathe. This causes your blood to become more acidic. As a result, your body will start using the calcium in your blood to balance out the acid in your blood.
The hardening of the arteries in the lungs causes the lung tissue to become less flexible, which makes it harder for air to flow in and out of your lungs. This is called pulmonary arterial stiffening or hypertrophy. After a while, this can cause a "printing" or "swelling" effect in areas around your heart and lungs. This swelling can cause a reduction in your ability to breathe.
In patients with PAH, there is a buildup of connective tissue that makes up the walls of the pulmonary arteries. This buildup, called pulmonary arterial fibrosis, occurs due to inflammation and cellular abnormality caused by PAH. This buildup causes stiffening of the lungs, which makes it harder for oxygen to flow. This causes the lungs to become more tender, which leads to an increase in drainage from the body. This leads to swelling, pain, and shortness of breath.
The amount of weakness present in the veins around your heart is called vascular insufficiency. When you have vascular insufficiency, veins connected to the heart become enlarged because of the buildup of connective tissue in your arteries. This can lead to blood clots forming in your lungs. The lung clots cause pulmonary embolisms. These blood clots can also decrease your ability to breathe.
Patients with pulmonary hypertension have reduced blood flow to their extremities. The arms, legs, and face are the most commonly affected areas in the body. The lower extremities are most often affected because blood is needed up high in your body when you have PAH. Pressure in the shoulders due to fluid buildup can result in shoulder pain. This can then lead to chest pain and shortness of breath. Over time, the fluid buildup can lead to a heart attack.
Frequent coughs and chest pains may also cause you to experience pain in the shoulders. This may be due to blood clots that are forming in your lungs.
Arrhythmias are irregular heartbeats. Congenital heart defects can cause them. Arrhythmias are either supraventricular or ventricular. Ventricular arrhythmias are more common in patients with PAH, while supraventricular arrhythmias are less frequent.
In people with PAH, ventricular fibrillation can cause sudden death because the heart isn't supplied enough blood from the lungs. In these cases, a cardiac defibrillator is used to get the heart beating again. In other cases, you may use medication to treat arrhythmias.
Pulmonary hypertension can be deadly because it causes your heart to work harder to pump the blood around your body. It also causes damage to your heart muscle, which can lead to arrhythmias or cardiac arrest. PAH is diagnosed in the very young, elderly, or people with other health conditions like high blood pressure.
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