Pulmonary Hypertension (PH)

Pulmonary hypertension (PH), or pulmonary HTN, is a condition that causes high blood pressure in the arteries in your lungs and can affect the right side of your heart.

Normal pulmonary artery pressure is 8-20 mm Hg when resting. If the pulmonary artery pressure is greater than 20 mm Hg at rest, it’s classified as PH. Pulmonary arterial hypertension (PAH), formerly called primary pulmonary hypertension, is a progressive form of PH that occurs when blood vessels in the lungs narrow, harden, or become blocked. If you have PH, you may experience symptoms such as shortness of breath, dizziness, fatigue, chest pain, or a racing heart. Left untreated, PH can lead to right heart failure and death.

PH can develop slowly and not show any signs or symptoms. When a patient has severe pulmonary hypertension, symptoms are often present at rest and during physical activity. Your doctor will have to run a variety of tests to diagnose PH. Once a diagnosis is confirmed, your doctor will classify the severity of the condition and the type of PH to help determine the treatment. Treatment may include medications or surgery to help improve symptoms and slow the progression of the disease.

Types of pulmonary hypertension and causes

Depending on the cause, PH is classified into five groups, and our heart experts treat them all.

Group 1: Pulmonary arterial hypertension (PAH)

PAH disease, also known as primary pulmonary hypertension (PPH), occurs when the arterial walls in the lungs tighten and stiffen. As a result, the right side of your heart enlarges and struggles to pump blood through the lungs.

Group 2: Pulmonary venous hypertension (PVH) due to left heart disease

This condition often results from diseases of the left side of the heart, such as structural heart disease or heart valve disease, and failure of the left heart chamber or left ventricle. This causes “back pressure” of fluid from the left side of the heart to the blood vessels of the lungs.

Group 3: Pulmonary hypertension related to lung disease or hypoxia

This condition is caused by chronic lung disease or hypoxia. Causes can include COPD/emphysema, pulmonary fibrosis, sleep apnea, and living in a high-altitude area for too long.

Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH)

This rare form of PH occurs when blood clots in the lung (pulmonary emboli) block blood flow within the lungs’ arteries. Learn more about CTEPH.

Group 5: Pulmonary hypertension due to multifactorial or miscellaneous causes

This classification occurs when PH is associated with other conditions in a way that is often not completely understood. These conditions may include metabolic disorders, anemia, spleen removal, and sarcoidosis. 

Pulmonary hypertension symptoms

Symptoms for PH typically develop slowly over time. Patients may not experience any symptoms for months or years until the disease progresses.

Pulmonary hypertension symptoms can include:

  • Shortness of breath while at rest or during physical exercise.
  • Fatigue.
  • Palpitations.
  • Swelling in the ankles or legs.
  • Fatigue.
  • Dizziness or fainting.
  • Chest pressure or pain.
  • Lips and skin turning a blue color (cyanosis).

Pulmonary hypertension program at AHN: why choose us?

It’s not uncommon for people with PH to be misdiagnosed with asthma, bronchitis, left-sided congestive heart failure, or another lung ailment. An incorrect diagnosis and treatment plan increases your risk for right heart failure and can be life-threatening. Our experts have the training, experience, and tools to provide quick, accurate PH care. We offer:

Depth of expertise

You receive care from some of the most esteemed PH specialists in the country. Our accomplishments include:

Advanced care for complex PH

Our CTEPH program is internationally renowned for exceptional patient results and skilled care. We are the only medical center in the region offering highly complex surgical procedures and interventional therapies for CTEPH.

Home monitoring

We’re pioneering the use of the CardioMEMS™ implantable device to remotely monitor pulmonary artery pressure and heart function as you go about daily life. Daily readings sent from the convenience of your home alert our team to problems before symptoms start, allowing us to quickly adjust treatments.

Individualized treatment

Our physicians use a collaborative team approach to tailor an aggressive treatment plan that halts disease progression and adds quality to your life. When appropriate, you may choose to participate in clinical trials for new therapies via the Cardiovascular Research Institute.

Dedicated team of experts

Our board-certified physicians in cardiology, pulmonary medicine, rheumatology, cardiothoracic surgery, and palliative care work with PH specialty nurses, nutritionists, social workers, and geneticists to provide you with all-inclusive care. A dedicated nurse navigator guides you throughout the diagnosis and treatment process.

Partnerships with referring physicians

We work closely with your referring physician to ensure seamless continuity of care, from diagnosis to treatment and disease management. We’re always available to answer questions from you or your primary physician.

Pulmonary hypertension diagnosis

An accurate diagnosis is critical in ensuring that you receive the best treatment for your type of PH. Our team uses sophisticated imaging technology to precisely diagnose your condition. Our diagnostic tests include:

Echocardiogram

This noninvasive ultrasound procedure uses sound waves to create pictures of your heart structure, including valves, walls, and blood vessels.

Electrocardiogram (EKG/ECG)

This test records your heart’s electrical activity and can show whether your right ventricle is enlarged or strained.

Right heart catheterization

This procedure measures pressure inside your pulmonary arteries. Your physician performs tests via a thin, flexible tube called a catheter threaded through a blood vessel into the right side of your heart and lungs.

Cardiac magnetic resonance imaging (MRI)

This noninvasive procedure uses electromagnetic radio waves to create detailed 3D still images and real-time videos of your heart and circulatory system.

Pulmonary hypertension treatments

Our physicians offer the most advanced PH treatments available, including drug therapies still in clinical trial phase. Your treatment depends on the PH type and may include:

  • Specialized medicines for pulmonary hypertension, which may include pulmonary blood vessel dilators, warfarin, digoxin, high-dose calcium blockers, and diuretics.
  • Oxygen therapy.
  • Treatments for structural heart disease and heart valve disease.

PH risk factors, prevention, and maintenance

Risk factors of pulmonary hypertension include:

  • Family history of the condition.
  • Obesity.
  • Living in high altitude.
  • Use of drugs such as diet drugs, some cancer agents, and illegal drugs such as amphetamines and cocaine.
  • Congenital heart disease.
  • Blood-clotting disorders.
  • Some autoimmune/connective tissue disorders such as scleroderma and lupus.

There is currently no cure available for people living with PH; however, it can be managed. Lifestyle modifications and medications can help decrease symptoms of PH and slow down the progression of the condition. If left untreated, PH can become life-threatening within a few years.

Contact us

Call (412) DOCTORS (412) 362-8677 or request an appointment to learn more about AHN cardiovascular services.

CardioMEMS is a trademark of Abbott and is used with permission.

*For more information on the Pulmonary Hypertension Association Center for Comprehensive Care accreditation, visit phassociation.org/phcarecenters/accredited-centers