Pulmonary Hypertension

What Is Pulmonary Hypertension?

Pulmonary hypertension (PH) is high blood pressure in the blood vessels in the lungs. PH can affect both men and women of all ages and races.

When the pressures in the lungs are high, it causes the right side of the heart to have to work harder.

Although it is a relatively uncommon disease, it's important to make an early and accurate diagnosis. If untreated, this condition can lead to right heart failure and increased risk of death.

Pulmonary hypertension causes

Many different factors can cause PH.

Some causes include:

• Liver disease
• Certain heart diseases
• Thromboembolic disease
• Genetics
• Rheumatic disorders
• Lung conditions
• Low-oxygen conditions

Types of pulmonary hypertension

There are many types of PH.

• Pulmonary arterial hypertension (PAH)

  Idiopathic Familial Scleroderma-associated

  HIV-associated Congenital heart disease-associated

• Pulmonary hypertension due to lung diseases
  • Emphysema or COPD
  • Pulmonary fibrosis
  • Sleep apnea
• Pulmonary hypertension due to left heart disease
  • Diastolic heart failure (heart failure with preserved ejection faction)
  • Left heart dysfunction
  • Systolic heart failure
• Pulmonary hypertension due to chronic blood clots (CTEPH)
• Pulmonary hypertension due to other causes
• PH due to sarcoidosis
• PH due to sickle cell disease

Treatment options vary depending on the type of PH.

Make an appointment today

Starting pulmonary hypertension treatment early is important in improving overall quality of life.

To make an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:

• 1-877-PH4-UPMC, toll-free
• PHprogram@upmc.edu

Pulmonary Hypertension Symptoms and Diagnosis

Pulmonary hypertension symptoms

PH symptoms can include:

Shortness of breath with exertion Lightheadedness Fatigue Cough

Chest pain Passing out Inability to lie flat without being short of breath Swelling of the ankles or abdomen

Pulmonary hypertension diagnosis

Making the diagnosis of PH begins with reviewing symptoms and doing tests.

Common tests for PH include:

Transthoracic echocardiogram is an ultrasound of the heart.

It is a good test for:

• Evaluating the overall structure and pump-function of the heart.
• Identifying increased pressures on the right side (pulmonary side) of the heart.

If this test is positive or if there is a high suspicion of PH, often the PH specialist will perform a right heart catheterization.

Right heart catheterization is the gold standard for diagnosing pulmonary hypertension. Doctors use a catheter to measure the pressure in the heart and lungs.

In this procedure, the patient has local numbing medicine.

Then, the PH specialist will:

• Insert the catheter through the large vein in the neck or groin area.
• Pass the catheter through the right side of the heart and into the pulmonary artery.
• Measure the pressures and withdraw the catheter.

If the doctor suspects left heart disease, sometimes a patient may have a left heart catheterization at the same time. This examines the arteries of the heart for blockages and measures pressures on the left side of the heart.

Other tests for diagnosing PH include:

Blood tests Six-minute walk test Pulmonary function testing Chest x-ray

CT scan of chest Ventilation/perfusion scan Cardiac MRI

Make an appointment for PH symptoms and treatment

To make an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:

• 1-877-PH4-UPMC, toll-free
• PHprogram@upmc.edu

Pulmonary Hypertension Treatments

Although there is no cure to date, there are several medical and surgical treatments for PH.

Pulmonary hypertension treatment depends on the type and severity of disease.

For pulmonary arterial hypertension, there are currently 12 FDA-approved medicines on the market.

And, for those who do not respond to medical therapy, surgical treatments for pulmonary arterial hypertension include:

• Atrial septostomy
• Lung transplantation

Follow up care for PH

While it can take weeks to months to notice a significant improvement with medical therapy, it is important to follow up closely with your PH specialist to make sure that there is improvement.

Patients will follow up within three months of their diagnosis.

If they are doing well, they will follow up every six to 12 months for continued monitoring of their disease.

Follow up can involve repeat testing including:

• The six-minute walk test
• Echocardiogram
• Heart catheterization

Make an appointment for pulmonary hypertension treatment today

Starting PH treatment early is important in improving overall quality of life.

To schedule an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:

• 1-877-PH4-UPMC, toll-free
• PHprogram@upmc.edu