What is the treatment for severe pulmonary hypertension confirmed on Right Heart Catheterization (RHC)?

From the Guidelines

Vasomodulator therapy should be offered to all patients with severe pulmonary hypertension confirmed on Right Heart Catheterization (RHC) and mean pulmonary arterial pressure >35 mmHg and pulmonary vascular resistance >3 Wood units. The treatment approach for severe pulmonary hypertension confirmed on RHC requires a comprehensive strategy, with vasomodulator therapy as the primary treatment option 1.

Key Treatment Considerations

• Vasomodulator therapy includes endothelin-receptor antagonists, phosphodiesterase type-5 inhibitors, and prostacycline analogs, which can be used in monotherapy or combination therapy 1.
• The goal of treatment is to achieve an adequate haemodynamic response, defined as mean pulmonary arterial pressure <35 mmHg and pulmonary vascular resistance <5 Wood units, or mean pulmonary arterial pressure of 35 to 45 mmHg and pulmonary vascular resistance <3 Wood units 1.
• Treatment strategies may include the use of oral medications such as endothelin-receptor antagonists and phosphodiesterase type-5 inhibitors, as well as parenteral prostacycline analogs 1.

Additional Supportive Measures

• Oxygen therapy to maintain saturation above 90% may be necessary to support patients with severe pulmonary hypertension 1.
• Diuretics, such as furosemide, may be used to manage right heart failure symptoms 1.
• Anticoagulation with warfarin may be considered in selected patients, with a target INR of 2-3 1.
• Treatment of underlying causes, such as liver disease, is also crucial in managing severe pulmonary hypertension 1.

Monitoring and Follow-up

• Regular follow-up with a pulmonary hypertension specialist is essential for monitoring treatment response and adjusting therapy as needed 1.
• Right heart catheterization is a critical tool for assessing treatment response and guiding therapy 1.

From the FDA Drug Label

Acute intravenous infusions of epoprostenol for up to 15 minutes in patients with idiopathic or heritable PAH or PAH associated with scleroderma spectrum of diseases (PAH/SSD) produce dose-related increases in cardiac index (CI) and stroke volume (SV) and dose-related decreases in pulmonary vascular resistance (PVR), total pulmonary resistance (TPR), and mean systemic arterial pressure (SAPm).

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.

The treatment for severe pulmonary hypertension confirmed on Right Heart Catheterization (RHC) is epoprostenol (IV), which can be administered by continuous intravenous infusion via a central venous catheter using an ambulatory infusion pump 2.

• The dosage of epoprostenol should be initiated at 2 ng/kg/min and increased in increments of 2 ng/kg/min every 15 minutes or longer until dose-limiting pharmacologic effects are elicited or until a tolerance limit to the drug is established 2.
• Key benefits of epoprostenol treatment include:
  • Increases in cardiac index (CI) and stroke volume (SV)
  • Decreases in pulmonary vascular resistance (PVR), total pulmonary resistance (TPR), and mean systemic arterial pressure (SAPm)
  • Improvements in exercise capacity and survival in NYHA functional Class III and Class IV patients with idiopathic or heritable PAH 2 2.

From the Research

Treatment for Severe Pulmonary Hypertension

The treatment for severe pulmonary hypertension confirmed on Right Heart Catheterization (RHC) typically involves a combination of medications that target multiple biological pathways.

• The primary goal of treatment is to improve symptoms, exercise capacity, and quality of life, as well as to slow disease progression and improve survival rates 3, 4, 5.
• Current treatment options for pulmonary arterial hypertension (PAH) include:
  • Endothelin receptor antagonists (e.g., bosentan, ambrisentan) 6
  • Phosphodiesterase type 5 inhibitors (e.g., sildenafil, tadalafil) 3, 4
  • Soluble guanylate cyclase stimulators (e.g., riociguat) 4, 5
  • Prostacyclin pathway agonists (e.g., epoprostenol, treprostinil) 3, 4
• Combination therapy, which targets multiple biological pathways, has been shown to be more effective than monotherapy in improving outcomes for patients with PAH 3, 4.
• In addition to medical therapy, other treatment options may be considered, such as:
  • Surgical pulmonary endarterectomy for patients with chronic thromboembolic pulmonary hypertension (CTEPH) 4, 5
  • Balloon pulmonary angioplasty for patients with CTEPH 5
• Hospital and intensive care unit management of decompensated pulmonary hypertension and right ventricular failure requires prompt recognition of the underlying cause and institution of treatment with a thorough understanding of the elements necessary to optimize preload, cardiac contractility, enhance systemic arterial perfusion, and reduce right ventricular afterload 7.