How to Manage Pulmonary Hypertension in the United States 2025

Early and Specialized Diagnosis Is Crucial

The cornerstone of effective pulmonary hypertension management is early and accurate diagnosis by specialized physicians. In the United States, newly diagnosed PH patients—especially those with PAH—should be promptly referred to pulmonary hypertension specialists or accredited PH care centers. These centers, such as those recognized by the Pulmonary Hypertension Association and Mayo Clinic Centers of Comprehensive Care, offer expert knowledge, advanced diagnostic testing, and access to the latest treatment protocols.

Diagnosis requires right heart catheterization (RHC) to measure pulmonary artery pressure and confirm PH, defined as a mean pulmonary artery pressure (mPAP) above 20 mmHg. Classification into distinct PH groups guides therapy. Given vague symptoms like shortness of breath and fatigue, diagnosis may be delayed (often over two years), emphasizing the need for increased awareness and expert referral.

Personalized Risk Stratification Guides Treatment

Effective PH management demands comprehensive risk stratification at diagnosis and during follow-up. Tools such as the REVEAL 2.0 risk calculator and European Society of Cardiology/European Respiratory Society (ESC/ERS) models assess disease severity and right ventricular (RV) function. Since right heart failure is the primary cause of death in PAH, treatments aim to preserve RV function and avoid failure.

Risk evaluation enables clinicians to customize treatment intensity depending on whether patients are low, intermediate, or high risk for mortality. Continuous risk reassessment encourages therapeutic changes to attain or maintain a low-risk status, which correlates with better survival and quality of life.

Targeted FDA-Approved Medications Form the Treatment Backbone

Pharmacologic therapy for PAH addresses three disrupted lung physiological pathways:

• Endothelin Pathway — Overactive in PAH, causing vasoconstriction.
• Nitric Oxide (NO) Pathway — Deficient, reducing natural vasodilation.
• Prostacyclin Pathway — Decreased, contributing to vasoconstriction and vascular remodeling.

FDA-approved medications modulating these pathways include:

• Endothelin Receptor Antagonists (ERAs): bosentan, ambrisentan, macitentan
• Phosphodiesterase-5 Inhibitors (PDE5i): sildenafil, tadalafil
• Soluble Guanylate Cyclase (sGC) Stimulator: riociguat
• Prostacyclin Analogues: epoprostenol, treprostinil, iloprost
• Prostacyclin Receptor Agonist: selexipag
• Activin Signaling Inhibitor: sotatercept (recently FDA approved, notably effective for patients who do not achieve low-risk status on standard dual combination therapy)

Sotatercept introduces a novel mechanism by directly inhibiting activin signaling and rebalancing growth factor pathways involved in vascular remodeling.

Early, Aggressive Combination Therapy Is Recommended

Current guidelines advise starting treatment with an aggressive strategy:

• First-line therapy: Dual oral combination of a PDE5 inhibitor and an ERA for all PAH patients.
• High-risk patients: Addition of parenteral prostacyclin analogues, administered intravenously or subcutaneously, to intensify treatment.
• Patients not reaching low-risk status: Consider adding sotatercept to further decrease pulmonary vascular resistance and improve clinical outcomes.

This method is supported by expert consensus as a powerful approach to slow disease progression and enhance quality of life.

Regular Monitoring and Follow-Up Are Essential

Patients require frequent follow-up visits involving comprehensive evaluations such as:

• Echocardiograms to assess heart function
• Six-minute walk tests to measure exercise capacity
• Right heart catheterizations periodically to track pulmonary artery pressures and cardiac output
• Cardiopulmonary exercise testing (CPET) at some centers for detailed exercise assessment

Close observation of medication adherence, side effects, and symptom changes allows prompt treatment plan adjustments. Specialist nurses and care coordinators often aid patients with medication management and symptom monitoring.

Supportive Care and Lifestyle Measures Complement Therapy

In addition to medications, PH management involves supportive strategies to optimize overall health:

• Fluid control: Low-sodium diets and diuretics (including spironolactone) to prevent fluid retention and right heart strain.
• Oxygen therapy: Supplemental oxygen per American Thoracic Society guidelines when appropriate.
• Management of comorbidities: Addressing connective tissue diseases, iron deficiency anemia, and cardiac risk factors such as systemic hypertension and arrhythmias.
• Lifestyle adjustments: Smoking cessation, participation in supervised moderate exercise or pulmonary rehabilitation, maintaining a heart-healthy diet (low in saturated fats, trans fats, and salt), and avoiding environmental stressors like high altitudes or hot tubs.

Psychosocial Support and Multidisciplinary Care Improve Quality of Life

Living with PH can present emotional challenges. Incorporating psychosocial support through mental health providers, social workers, and patient support networks—such as the American Lung Association’s Living with PAH Support Community and Better Breathers Club—assists patients and caregivers in managing anxiety, depression, and disease burden.

Multidisciplinary teams including pulmonologists, cardiologists, pharmacists, nurses, and dietitians collaborate to deliver comprehensive, patient-centered care.

Special Considerations: Pregnancy and Advanced Interventions

Pregnancy is a significant risk factor for women with PAH, often resulting in life-threatening complications. Women of childbearing age should receive thorough counseling on contraception and alternative family planning options such as surrogacy or adoption along with their healthcare team before attempting pregnancy.

For other PH forms, including chronic thromboembolic pulmonary hypertension (CTEPH), specialized surgical and interventional options are available, such as balloon pulmonary angioplasty and surgical thromboendarterectomy, performed at expert centers like Mayo Clinic.

In end-stage disease, lung or heart-lung transplantation may be considered but is reserved for selected individuals meeting strict eligibility criteria.

Challenges and Ongoing Needs in 2025

Despite progress, several obstacles hinder optimal PH management:

• Delays in diagnosis due to nonspecific symptoms and limited awareness.
• Lack of head-to-head clinical trials comparing medications to determine best treatment sequencing.
• Uncertainty regarding optimal timing for introducing newer agents like sotatercept.
• High costs and limited availability of specialized medications and care centers.
• Complex management of patients with multiple comorbidities including overlapping cardiac and pulmonary conditions.

Ongoing studies and clinical trials seek to address these gaps and develop more personalized, mechanism-targeted therapies.

In summary, managing pulmonary hypertension in the United States in 2025 entails early specialist diagnosis, risk-adapted combination drug therapies targeting multiple pathogenic pathways, regular monitoring, and comprehensive supportive care. Accreditation of treatment centers and multidisciplinary collaboration contribute to improved outcomes. While challenges such as diagnostic delays and treatment expenses remain, ongoing innovations and a holistic care approach continue to enhance the standard of care for PH patients.

Sources

• American Lung Association - Treating and Managing PAH
• PMC - Treatment of pulmonary hypertension after seven world symposia (2025)
• Mayo Clinic Pulmonary Hypertension Clinic Overview

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