Pulmonary Hypertension

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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