Severe pulmonary hypertension: What to know

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Doctors define pulmonary hypertension as an elevated blood pressure in the pulmonary arteries. Typically, a doctor will diagnose pulmonary hypertension when a person’s average systolic arterial pressure reaches 35 mmHg or higher. The systolic pressure is the pressure in your arteries when your heart beats.

Other clinical symptoms may appear too.

Pulmonary hypertension is a progressive disorder. Without prompt intervention, it can gradually worsen and become severe.

Severe pulmonary hypertension occurs when the mean resting blood pressure in the pulmonary arteries is above 70 mmHg. Severe pulmonary hypertension is rare but may occur in people with chronic medical disorders, such as chronic obstructive pulmonary disease (COPD).

Learn more about COPD and high blood pressure.

Severe pulmonary hypertension has associations with increased vascular resistance, a condition that can result in right-sided heart failure. Reduced blood supply and the heart working harder are likely the reasons for the heart failure.

Consequently, individuals with severe pulmonary hypertension may present with any of the following symptoms:

• cyanosis, or blue skin and lips, but in darker skin tones, it may present as a gray or white discoloration
• chest pressure or pain
• dizziness or fainting spells
• recurrent cough
• rapid or irregular heartbeat
• feeling fatigued easily
• severe difficulty in breathing, even on minimal exertion or at rest
• peripheral swelling in the ankles, legs, and abdomen.

Severe pulmonary hypertension is life threatening, so a person with these symptoms should seek urgent medical attention.

Learn more about symptoms, causes, and diagnosis of PAH.

Several factors, such as genetics, environmental toxins, and underlying chronic conditions, can predispose someone to severe pulmonary hypertension.

The World Health Organisation (WHO) classifies the causes of severe pulmonary hypertension into different groups, including:

Group 1: Pulmonary arterial hypertension (PAH)

Causes include:

• unknown cause (idiopathic)
• hereditary or genetic factors
• using certain psychoactive drugs (drugs that affect mental processes) or exposure to toxins
• congenital heart diseases, which are heart problems at birth
• chronic medical conditions, such as HIV, chronic liver disease (cirrhosis), and connective tissue disorders

Group 2: Pulmonary hypertension due to left-sided cardiac disease

Causes are:

• left-sided valvular heart valve disease, such as aortic or mitral valve disease
• left ventricular failure
• other congenital cardiomyopathies

Group 3: Pulmonary hypertension due to lung disease

Examples include:

• chronic obstructive pulmonary disease (COPD)
• interstitial lung disease, which causes scarring in the lung’s air sacs
• obstructive sleep apnea
• prolonged exposure to high altitudes

Group 4: Pulmonary hypertension due to chronic blood clots

Group 4 pulmonary hypertension can only occur as a result of a condition called chronic thromboembolic pulmonary hypertension (CTEPH). Chronic blood clots in the lungs cause CTEPH.

Group 5: Pulmonary hypertension due to other health conditions

Causes include:

• blood disorders, such as myeloproliferative disorders
• inflammatory conditions such as sarcoidosis and vasculitis
• metabolic disorders, such as glycogen storage diseases.
• chronic kidney disease
• mechanical obstruction from tumors compressing the pulmonary arteries

Learn more about the five classes of pulmonary hypertension.

Doctors typically diagnose pulmonary hypertension using a combination of clinical findings and laboratory investigations. During the medical history, the doctor will ask questions about the presenting symptoms and conduct a physical examination.

The doctor will typically perform a general and chest examination to identify signs of pulmonary hypertension.

Doctors will conduct the following laboratory tests to diagnose pulmonary hypertension:

• Cardiac catheterization: This procedure helps to measure the blood pressure in the pulmonary arteries. A doctor will diagnose severe pulmonary hypertension if a person’s blood pressure exceeds 70 mmHg.
• Echocardiography: Doctors use it to evaluate the structure and functions of the heart chambers.

Other supportive tests include:

• blood tests
• chest X-ray
• electrocardiogram (ECG or EKG)

Learn more about diagnosing pulmonary hypertension.

The treatment of severe pulmonary hypertension requires a combination of different types of treatments.

According to the Pulmonary Hypertension Association, therapeutic interventions include:

• Intravenous medications: Epoprostenol is a vasodilator that relaxes the pulmonary blood vessels and improves blood flow. It is typically the first-line medication for treating severe pulmonary hypertension. Doctors may also use ambrisentan, bosentan, macitentan, riociguat.
• Oxygen therapy and ventilation support.
• Lung transplant, especially for those with severe lung damage.
• Interventional cardiac surgeries
• Supportive care and other medications, such as diuretics, to reduce fluid retention in the lungs.

Treatment for pulmonary hypertension depends on which WHO group the person falls into and the level of their pulmonary blood pressure. For example, someone in WHO Group 1 will undertake a different treatment course than someone from Group 2.

Learn more about treating pulmonary hypertension.

Below are some commonly asked questions about severe pulmonary hypertension:

What are the stages of pulmonary hypertension?

The four stages of pulmonary hypertension are:

• Class I: No symptoms during rest or when performing activities.
• Class II: Presence of symptoms, such as fatigue or shortness of breath, during routine activities, except at rest.
• Class III: Symptomatic and experiences fatigue, shortness of breath, and chest pain. People may also experience limitations in performing everyday activities.
• Class IV: Symptoms occur at rest and during any physical activity.

What is the life expectancy for severe pulmonary hypertension?

The life expectancy of severe pulmonary hypertension varies for each person. The following factors can have an impact on someone’s life expectancy:

• what WHO group the pulmonary hypertension falls into — for example, someone in group 1 with idiopathic pulmonary hypertension is likely to have a poor outlook
• the available treatments
• the class of symptoms a person has

How common is severe pulmonary hypertension?

Generally, pulmonary hypertension is rare. However, severe pulmonary hypertension tends to be more prevalent in people with COPD.

Severe pulmonary hypertension is a clinical condition in which the resting blood pressure in the pulmonary arteries is above 70mmHg. Individuals with severe pulmonary hypertension may notice profound shortness of breath, cyanosis, chest pain, intense fatigue, and severe dizziness.

Genetic factors, chronic medical conditions, and exposure to environmental toxins are possible causes of severe pulmonary hypertension.

Cardiac catheterization and echocardiography are essential tests for diagnosing severe pulmonary arterial hypertension.

Severe pulmonary hypertension treatment includes pulmonary vasodilators, oxygen and ventilation support, and lung transplantation.

Learn more about hypertension in our dedicated hub.