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Pulmonary Hypertension

Pulmonary hypertension (PH) is a disorder in which blood vessels in the lungs become narrow, impeding blood flow. As a result, blood pressure in the lungs rises above normal. This forces the right side of the heart to work harder than it should to pump blood out through the lungs.

Pulmonary hypertension often worsens over time. Without treatment, it can advance to the point where the right side of the heart fails. All children with PH should undergo a thorough evaluation from a physician and team with expertise in PH. The evaluation should include a search for the underlying cause of PH to determine the best treatment options.

Treatments can ease symptoms and slow the disease's progression, but there is currently no cure for PH. However, a great deal of research is under way, and the long-term prognosis is considerably better than it was even five years ago.

Causes of Pulmonary Hypertension

A number of conditions can cause or contribute to pulmonary hypertension. They include:

Birth defects of the heart
Genetic predisposition
Lung disease, such as bronchopulmonary dysplasia (mostly in premature infants)
Abnormal lung development, such as congenital diaphragmatic hernia
Abnormalities in the left side of the heart, such as mitral valve disease
Autoimmune diseases, such as lupus and scleroderma
Sleep apnea and other breathing disorders
Blood diseases, including clotting disorders and sickle cell anemia
Liver disease
Certain drugs or toxins
HIV

Sometimes we can't find a cause. This is called idiopathic pulmonary hypertension.

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Signs and Symptoms
Diagnosis
Treatment

Symptoms of pulmonary hypertension are often minor at first but can gradually worsen over time. Other conditions can cause similar symptoms, which makes early detection challenging. If your child experiences any sign of pulmonary hypertension, consult his or her doctor right away. The most common are:

Shortness of breath during everyday activities such as walking or climbing stairs
Excessive fatigue
Dizziness
Fainting spells
Rapid heartbeats
Chest pain or pressure
Bluish color to lips and skin

The first steps to diagnosing PH are to collect a medical history and perform a complete physical exam. Additional testing may be required to help determine whether your child has PH and what may be causing it, including:

Doppler echocardiogram — This test uses sound waves to examine the heart's anatomy and function, and the estimated pressures on the right side of the heart. This is our most commonly used screening tool to detect PH.
Right heart catheterization — If an echocardiogram suggests pulmonary hypertension, the next step is right heart catheterization. This is the gold standard for diagnosing PH but is more invasive than the echocardiogram. During the test, a doctor threads a thin, flexible tube from blood vessels in the groin up to the right side of the heart. This allows us to measure blood pressure in the heart and lungs and can even find leaks between the heart's right and left sides.

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The first step in treatment is usually to identify the underlying cause of PH and try to correct it. Other treatment options may include supplemental oxygen and medications that are taken orally, inhaled or delivered by continuous infusion under the skin or into a vein. The medications we currently use include:

Endothelin receptor antagonists (ERAs)— These oral medications block endothelin, a substance in the body that constricts blood vessels. As the vessels relax, blood pressure in the pulmonary artery lowers, heart function improves and ultimately your child can be more active. ERAs may also slow the disease's progression. These medications include bosentan (Tracleer), ambrisentan (Letaris) and macitentan (Opsumit).

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