Pulmonary Arterial Hypertension life expectancy

What is pulmonary arterial hypertension?

Pulmonary arterial hypertension (PAH) is a progressive disease of the lung vascular system, primarily affecting small pulmonary arterioles. A combination of endothelial dysfunction and increased contractility of small pulmonary arteries (PAs), proliferation and remodeling of endothelial and smooth muscle cells, and in situ thrombosis leads to progressive narrowing of the blood vessels. This results in a progressive resistance to blood flow and an increase in PA pressures.

Pulmonary hypertension is a type of high blood pressure that affects the arteries in your lungs and the right side of your heart.

In one form of pulmonary hypertension, tiny arteries in your lungs, called pulmonary arterioles, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, and raises pressure within your lungs' arteries. As the pressure builds, your heart's lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and fail.

Some forms of pulmonary hypertension are serious conditions that become progressively worse and are sometimes fatal. Although some forms of pulmonary hypertension aren't curable, treatment can help lessen symptoms and improve your quality of life.

Five types of PH exist. One common type is called pulmonary arterial hypertension (PAH). It’s a severe disease that progresses rapidly and usually causes right heart failure. In many cases, PAH is fatal and leads to death.

Symptoms

The signs and symptoms of pulmonary hypertension in its early stages might not be noticeable for months or even years. As the disease progresses, symptoms become worse.

Pulmonary hypertension symptoms include:

Shortness of breath (dyspnea), initially while exercising and eventually while at rest

Fatigue

Dizziness or fainting spells (syncope)

Chest pressure or pain

Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites)

Bluish color to your lips and skin (cyanosis)

Racing pulse or heart palpitations

Causes

Your heart has two upper chambers (atria) and two lower chambers (ventricles). Each time blood passes through your heart, the lower right chamber (right ventricle) pumps blood to your lungs through a large blood vessel (pulmonary artery).

In your lungs, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then flows through blood vessels in your lungs (pulmonary arteries, capillaries and veins) to the left side of your heart. Ordinarily, the blood flows easily through the vessels in your lungs, so blood pressure is usually much lower in your lungs.

With pulmonary hypertension, the rise in blood pressure is caused by changes in the cells that line your pulmonary arteries. These changes can cause the walls of the arteries to become stiff and thick, and extra tissue may form. The blood vessels may also become inflamed and tight.

These changes in the pulmonary arteries can reduce or block blood flow through the blood vessels. This makes it harder for blood to flow, raising the blood pressure in the pulmonary arteries.

Diagnosis

Cardiac catheterization

Cardiac catheterization is the criterion standard test to definitively confirm any form of PAH. It is essential in the workup of all patients suspected of IPAH. Excluding left-sided heart disease, including diastolic dysfunction, is especially important in these patients because of major treatment implications. Catheterization is also performed to determine pulmonary vasoreactivity, which can be prognostic and figures in the initiation and titration of high-dose calcium channel blocker (CCB) therapy.

Laboratory studies

Antinuclear antibody

Thyroid function

B-type natriuretic peptide

Imaging studies

Radiography

Echocardiography

Computed tomography (CT), magnetic resonance imaging (MRI), and lung scanning

Pulmonary angiography

Electrocardiography

Electrocardiographic results are often abnormal in patients with PAH, revealing right atrial enlargement, right axis deviation, right ventricular hypertrophy, and characteristic ST depression and T-wave inversions in the anterior leads. Sometimes, an incomplete RBBB may be seen (usually in patients with atrial septal defects). However, some patients with IPAH have few or no abnormal electrocardiographic findings.

Histology

Several histologic subtypes are associated with pulmonary arteriopathy in IPAH, one of which involves in situ thrombosis.

Exercise testing

In patients with IPAH, values for peak exercise oxygen consumption, oxygen pulse, and ventilator equivalents (ratio of expired volume to carbon dioxide output [ie, wasted ventilation fraction] at the anaerobic threshold) during exercise are abnormal to varying degrees.

Commonly, a 6-minute walk test is performed in the office as a crude measurement of exercise capacity.

What is (IPAH) Idiopathic Pulmonary Arterial Hypertension?

Idiopathic Pulmonary Arterial Hypertension (IPAH) is a rare progressive disease characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs.

Pulmonary Hypertension Prognosis

"Pulmonary hypertension is progressive and it's non-curable," says pulmonary hypertension expert Samuel A. Allen, DO, director of the Pulmonary Hypertension Center at Beaumont Hospital in Troy, Mich.

However, he adds, with the correct treatment, people living with pulmonary hypertension can improve their quality of life and slow the progression of the disease.

If you have symptoms, such as shortness of breath that occurs without strenuous activity, be proactive about seeking a diagnosis and getting treated by health professionals with expertise in managing the condition.

Treatment goals

PAH is a type of high blood pressure. It affects your pulmonary arteries and the right side of your heart. Your pulmonary arteries carry blood from your heart to your lungs where fresh oxygen is pumped into your blood. If you have PAH, it’s tricky for these arteries to carry enough oxygen and blood to your body. Over time, PAH can get worse. It can lead to death if your organs don’t receive enough oxygen. The goal of PAH drugs is to stop further damage to your pulmonary arteries.

PAH may cause symptoms. These can include shortness of breath, dizziness, fainting, and chest pain. Drugs for PAH can also help relieve these symptoms.