Treatment

Test Your Knowledge of Pulmonary Arterial Hypertension: Initiating Treatment

AUTHOR:
Aaron Waxman, MD, PhD
Executive Director, Center for Pulmonary Heart Disease
Director, Pulmonary Vascular Disease Program
Brigham and Women’s Hospital
Boston, Massachusetts

CITATION:
Waxman A. Test your knowledge of pulmonary arterial hypertension: initiating treatment. Consultant360. Published online July 1, 2021.

 

A 32-year-old woman presented with progressive exertional intolerance. Her shortness of breath started about 1 year prior to presentation but has became increasingly limiting over the past 6 months. She was having shortness of breath climbing a flight of stairs. When carrying something up the stairs, such as groceries or laundry, she would often have to stop partway to catch her breath.

The patient had Raynaud disease, but no other significant medical history.

When she was referred to the pulmonary hypertension program, she had undergone an echocardiography scan that raised concern for pulmonary hypertension. The echocardiogram demonstrated a dilated right atrium and right ventricle. The estimated right ventricular systolic pressure was about 75 mm Hg.

She was not taking any medications but had been treated in the past with albuterol for shortness of breath, which was believed to be related to asthma. She reported no benefit from albuterol. However, pulmonary function testing did not confirm a diagnosis of asthma.

Based on the echocardiogram, she underwent right heart catheterization that was consistent with moderate pulmonary arterial hypertension. There was no clinically relevant response to acute pulmonary vasodilator testing. Once the diagnosis was made, chronic thromboembolic pulmonary hypertension was ruled out via appropriate imaging.

The patient’s presentation was consistent with a World Health Organization (WHO) functional classification 3a.

 

 

Answer and discussion on next page.

    Correct answer: A. Tadalafil and ambrisentan, 10 mg, daily

    The standard of care is upfront combination therapy with a phosphodiesterase 5 (PDE5) inhibitor, specifically tadalafil and ambrisentan, 10 mg, daily. This standard is based on the AMBITION study, which found that tadalafil and ambrisentan combination therapy had significantly reduced the risk of clinical-failure events among patients with pulmonary arterial hypertension.

    It is important to note that a right heart catheterization is required for diagnosing pulmonary hypertension, and then an assessment of the patient’s risk or functional class is needed.

    References: