Pulmonary hypertension (PH) is a disease in which small arteries responsible for carrying blood through the lungs become narrow, clogged or stiff. As the flow of blood is hampered, the pressure increases within the arteries, forcing the heart’s lower right chamber, or right ventricle, to work harder to pump blood through the lungs. Over time, this strain on the right ventricle causes the heart’s muscle to weaken and fail.

How is pulmonary hypertension diagnosed? Learn more about it here. 
A rare and deadly condition, PH affects people of all ages, races, and ethnicities, but it is more common among young adults and women. Early signs of PH can be shortness of breath, difficulties in completing daily tasks such as climbing stairs, fatigue, and dizziness or fainting spells. Other, or later signs, can include a bluish cast to the lips and skin, chest pain, swelling in the ankles or legs, and heart palpitations. There is currently no cure for pulmonary hypertension.  This progressive disease gets worse with time and, if left untreated, patients with PH may die within few years.

What happens after you are diagnosed with pulmonary hypertension? Here’s what you should expect:

Although PH cannot be cured, treatments exist that can delay the disease progression. PH patients face a higher risk of heart failure and diminished overall health, but medication and lifestyle alterations can help improve both life quality and expectancy. Early diagnosis is considered critical for increasing life expectancy among pulmonary hypertension patients.

To diagnose PH, physicians usually begin by conducting a complete medical history, physical examination, symptom evaluation, and identification of abnormal heart sounds. They search for an enlarged jugular vein in the neck, seek to find fluid retention in the abdomen, legs or ankles, examine nail beds for bluish tint, and identify any underlying signs or causes of PH.  

A number of tests will also be conducted, and likely will include blood tests, an echo cardiogram, a chest X-ray, an electrocardiogram, and — if deemed necessary — right heart catheterization, which is necessary to both confirm suspected PH and to determine its severity.  

Pulmonary hypertension (PH) is a disease diagnosed when arteries responsible for carrying blood become, thick, stiff and clogged. Normally, the arteries transport blood from the heart to the lungs, but in PH the vessel flow is hampered leading to abnormally high blood pressure in the pulmonary arteries. The disease results in excessive effort by the right heart ventricle to pump blood to the body, which causes it to expand and weaken.

In severe cases, pulmonary hypertension can lead to the complete inability of the heart to pump blood to the lungs, right heart failure or death. All of these factors contribute to life expectancy among PH patients, without treatment, of 2.8 years on average. Fortunately, novel PH therapies are working to improve outcome.

The rare condition affects people of all ages, races and ethnicity, but it is more common among young adults and women. First signs of PH can be shortness of breath, difficulties to complete daily tasks like climbing stairs, fatigue and dizziness.

​Other symptoms associated with PH include fatigue, fainting, irregular heartbeat, racing pulse, progressive shortness of breath, difficulty breathing at rest, swollen ankles, abdomen or legs; bluish lips and skin; and chest pain. Most symptoms are usually only present in advanced stages of the disease.​
Pulmonary Hypertension Life Expectancy: What To Expect After a PH Diagnosis. There is currently no cure for pulmonary hypertension. A progressive disease, it gets worse with time and if left untreated, patients with PH may die within few years.

Despite no cure for PH, treatments exist that can delay the disease progression. PH patients face a higher risk of heart failure and a decrease in overall health, but medication and lifestyle alterations can help increase life expectancy.

Though PH patients have 68% probability of surviving after the first year of diagnosis, and the chances decrease to 34% four years later, according Ohio State University Lung Center, early diagnosis is critical in increasing the pulmonary hypertension life expectancy.

First, to confirm the diagnosis, physicians usually conduct a complete medical history, physical examination, symptom evaluation and identification of abnormal heart sounds. They search for an engorgement of the jugular vein in the neck, seek to find fluid retention in the abdomen, legs or ankles, examine nail beds for bluish tint, and identify any underlying signs or causes of PH.

Additionall, other tests are completed that include blood tests, doppler echocardiogram, chest X-ray, a 6-minute walk test, pulmonary function tests, polysomnogram or overnight oximetry, right heart catheterization, ventilation perfusion scan (V/Q scan), pulmonary angiogram and chest CT scan to support the diagnosis.

Sometimes, conditions that contribute to the development of pulmonary hypertension cannot be fully understood; even when those factors influence PH prognosis and life expectancy.

Factors That Influence the Pulmonary Hypertension Life Expectancy. There are different types of pulmonary hypertension. including idiopathic pulmonary hypertension and associated pulmonary hypertension. Idiopathic pulmonary hypertension is diagnosed when the cause for the disease is unknown. Associated pulmonary hypertension is typically related to other factors such as lung disease, heart disease, hypoxia (low oxygen in the blood), clots, parasites, certain blood disorders, cirrhosis, AIDS, connective tissue diseases, weight reducing drugs and certain toxins.

Depending on the type of pulmonary hypertension, it is extremely important to carefully follow the associated disease so that it does not decrease overall health and lead to accelerated PH progression.

A study, “Factors that influence the outcome of primary pulmonary hypertension,” highlights that factors contributing to life expectancy in primary pulmonary hypertension have not been well defined.

In the study, researchers analyzed 34 PH cases and concluded that age at disease onset, symptoms, or sex of the patient could not predict pulmonary hypertension life expectancy. The study also demonstrated that while radio graphic evidence of cardiac enlargement and evidence of right heart strain on electrocardiogram are predictable factors of survival, “primary pulmonary hypertension is a heterogeneous condition in which life expectancy varies widely.”
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