What is the best approach to educate a patient with pulmonary hypertension (PH), normal ejection fraction (EF), and normal diastolic function, who is oxygen dependent at 2 liters per minute (LPM), on the cause and treatment of their condition?

From the Guidelines

The best approach to educate a patient with pulmonary hypertension, normal ejection fraction, and normal diastolic function who requires oxygen at 2 LPM is to focus on clear explanation of their condition and comprehensive treatment options, as recommended by the 2019 Chest guideline and expert panel report 1. Begin by explaining that pulmonary hypertension involves high blood pressure in the lung arteries, making the right side of the heart work harder, despite their heart's normal pumping ability.

Key Points to Discuss with the Patient:

• Treatment typically includes oxygen therapy at 2 LPM as prescribed, which helps reduce strain on the heart and improves symptoms.
• Medication options may include phosphodiesterase-5 inhibitors like sildenafil or tadalafil, endothelin receptor antagonists such as bosentan or ambrisentan, and prostacyclin pathway agents like selexipag.
• Lifestyle modifications are crucial, including a low-sodium diet, moderate physical activity as tolerated, smoking cessation, limiting alcohol, maintaining vaccination status, and avoiding high altitudes, as suggested by the 2019 Chest guideline and expert panel report 1.
• Regular follow-up appointments every 3-6 months are essential to monitor disease progression and medication effectiveness.
• Providing patients with education and information about their condition and management options is a key component of best practice care, as highlighted in a 2020 systematic review 1.
• The importance of expert referral centers in the management of PH patients has been highlighted in both the diagnostic and treatment algorithms, as stated in the 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension 1. This comprehensive approach addresses the physiological basis of their condition while providing practical management strategies, ultimately aiming to improve morbidity, mortality, and quality of life outcomes.

From the FDA Drug Label

The effects of epoprostenol on mean pulmonary arterial pressure (PAPm) were variable and minor Chronic continuous infusions of epoprostenol in patients with idiopathic or heritable PAH were studied in 2 prospective, open, randomized trials of 8 and 12 weeks’ duration comparing epoprostenol plus conventional therapy to conventional therapy alone. Increases in CI, SV, and arterial oxygen saturation and decreases in PAPm, mean right atrial pressure (RAPm), TPR, and systemic vascular resistance (SVR) were observed in patients who received epoprostenol chronically compared to those who did not.

To educate a patient with pulmonary hypertension (PH), normal ejection fraction (EF), and normal diastolic function, who is oxygen dependent at 2 liters per minute (LPM), on the cause and treatment of their condition, consider the following key points:

• Pulmonary hypertension is a condition characterized by high blood pressure in the arteries of the lungs, which can lead to symptoms such as shortness of breath and fatigue.
• Epoprostenol is a medication that can help improve symptoms and exercise capacity in patients with PH.
• The patient's oxygen dependence is likely related to their PH, and treatment with epoprostenol may help improve their oxygen saturation and reduce their need for supplemental oxygen.
• Treatment goals should focus on improving symptoms, exercise capacity, and quality of life, while also slowing disease progression.
• It is essential to work with a healthcare team to develop a personalized treatment plan and monitor the patient's condition regularly 2.

Key takeaways for patient education:

• Understanding the condition and its symptoms
• The importance of adherence to treatment and follow-up appointments
• Lifestyle modifications to manage symptoms and improve quality of life
• The role of epoprostenol in treating PH and its potential benefits and risks 2.

From the Research

Patient Education on Pulmonary Hypertension

To educate a patient with pulmonary hypertension (PH), normal ejection fraction (EF), and normal diastolic function, who is oxygen dependent at 2 liters per minute (LPM), on the cause and treatment of their condition, consider the following points:

• PH is a condition characterized by high blood pressure in the arteries of the lungs, which can lead to right heart failure and other complications 3.
• The patient's condition is likely classified as pulmonary hypertension due to left-sided heart disease or another underlying condition, given their normal EF and diastolic function 3, 4.
• The patient's oxygen dependence at 2 LPM indicates the need for ongoing oxygen therapy to manage their condition.

Diagnosis and Treatment

When discussing diagnosis and treatment with the patient, consider the following:

• Routine tests such as electrocardiography, chest radiography, and pulmonary function tests may be used to diagnose PH 3.
• Transthoracic echocardiography can estimate the probability of PH, while right-sided heart catheterization is essential for accurate diagnosis and classification 3.
• Treatment for PH depends on the underlying cause, but may include targeted therapies such as phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists 3, 5.
• For patients with PH due to left-sided heart disease, management primarily involves treatment of the underlying condition 3.

Management and Prognosis

When discussing management and prognosis with the patient, consider the following:

• PH is a significant contributor to the pathophysiology of heart failure with preserved ejection fraction (HFpEF) and may be an important target for therapy 4, 6.
• The development of PH in patients with HFpEF is considered a high-risk phenotype in need of targeted therapies 7.
• Hemodynamic markers such as transpulmonary gradient, pulmonary vascular resistance, and diastolic pulmonary gradient are associated with mortality and cardiac hospitalizations in patients with PH-HFpEF 7.