What are the treatment options for Pulmonary Hypertension (PHTN)?

From the Guidelines

Pulmonary hypertension treatment should prioritize vasodilator medications, including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogs, and soluble guanylate cyclase stimulators, as the cornerstone of therapy for pulmonary arterial hypertension (Group 1). The treatment approach should be multifaceted and tailored to the specific type and severity of the condition. Initial management includes treating underlying causes, supplemental oxygen therapy for hypoxemia, diuretics for fluid overload, and anticoagulation with warfarin for patients with idiopathic pulmonary arterial hypertension.

Key Medications

• Phosphodiesterase-5 inhibitors: sildenafil 20mg every 8 h or tadalafil 40mg once daily 1
• Endothelin receptor antagonists: bosentan 125mg twice daily, ambrisentan 5 or 10mg once daily, or macitentan 10mg once daily 1
• Prostacyclin analogs: epoprostenol IV 2 ng/kg/min, treprostinil subcutaneous/IV/inhaled, or iloprost inhaled 2.5-5mg 6-9 times daily 1
• Soluble guanylate cyclase stimulators: riociguat 0.5-1.0mg every 8 h 1

Treatment Approach

For Group 2-5 pulmonary hypertension, treatment focuses primarily on the underlying condition. Patients should be monitored regularly with echocardiography, 6-minute walk tests, and right heart catheterization when necessary. In severe cases refractory to medical therapy, lung transplantation or atrial septostomy may be considered. It is essential to note that advanced vasoactive agents approved only for the management of pulmonary arterial hypertension should not be routinely offered to patients with pulmonary hypertension resulting from left heart disease or hypoxemic lung diseases (Group II or III pulmonary hypertension) 1.

Monitoring and Follow-up

Regular monitoring and follow-up are crucial to assess the effectiveness of treatment and adjust the therapeutic plan as needed. The 6-minute walk test is a valuable tool to evaluate exercise capacity, and a minimally important difference of 33m is considered clinically significant 1. By prioritizing vasodilator medications and adopting a multifaceted treatment approach, patients with pulmonary hypertension can experience improved exercise capacity, quality of life, and potentially slowed disease progression.

From the FDA Drug Label

Treprostinil is indicated for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise. Epoprostenol for injection is indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity.

Pulmonary Hypertension Treatment:

• Treprostinil (SQ) and epoprostenol (IV) are indicated for the treatment of pulmonary arterial hypertension (PAH) to improve exercise capacity and diminish symptoms associated with exercise 2, 3.
• The goal of treatment is to establish a dose at which PAH symptoms are improved, while minimizing excessive pharmacologic effects of the medication 2.
• Dosage adjustments should be made to achieve this goal, with increments of 1.25 ng/kg/min per week for the first four weeks of treatment and then 2.5 ng/kg/min per week for the remaining duration of infusion, depending on clinical response 2.

From the Research

Treatment Options for Pulmonary Hypertension

• The treatment of pulmonary arterial hypertension has advanced substantially over the past 20 years, with five different classes of drugs now available, including endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and prostacyclin receptor agonists 4.
• For patients with chronic thromboembolic pulmonary hypertension, surgical pulmonary endarterectomy is the treatment of choice, while riociguat, a stimulator of soluble guanylate cyclase, has proven efficacious for inoperable patients 4, 5.
• Targeted treatments, such as phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists, are licensed for patients with pulmonary arterial hypertension 4, 5, 6.
• Management of pulmonary hypertension resulting from left-sided heart disease primarily involves treatment of the underlying condition 5.

Classes of Drugs Used to Treat Pulmonary Hypertension

• Endothelin receptor antagonists, such as bosentan, have been shown to improve exercise capacity and haemodynamics in patients with pulmonary hypertension 7.
• Phosphodiesterase-5 inhibitors, such as sildenafil, have been approved for the treatment of pulmonary arterial hypertension 8, 6.
• Soluble guanylate cyclase stimulators, such as riociguat, have been shown to be efficacious in patients with chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension 4, 5.
• Prostacyclin analogues and prostacyclin receptor agonists have been approved for the treatment of pulmonary arterial hypertension 4, 8, 6.

Surgical and Interventional Approaches

• Surgical pulmonary endarterectomy is the treatment of choice for eligible patients with chronic thromboembolic pulmonary hypertension 4, 5.
• Interventional approaches, such as balloon pulmonary angioplasty, may become a treatment option for patients with chronic thromboembolic pulmonary hypertension 4.