How to choose the best medicine for pulmonary hypertension

Choosing the best medication for pulmonary hypertension (PH) depends on several factors, including the underlying cause of PH, its severity, and the patient’s overall health. Treatment for PH is highly individualized, and the decision is typically guided by a PH specialist. Here are key considerations that influence the choice of medication:

1. Type and Cause of PH
   • Pulmonary Arterial Hypertension (PAH) (Group 1): Usually treated with specialized PAH-targeted therapies like endothelin receptor antagonists (ERAs), phosphodiesterase-5 (PDE5) inhibitors, soluble guanylate cyclase (sGC) stimulators, and prostacyclin analogs.
   • PH due to Left Heart Disease (Group 2): Focus is generally on treating the underlying heart condition rather than specific PH medications, as PH therapies are not proven effective for this group.
   • PH due to Lung Disease or Hypoxia (Group 3): Treatment targets the lung disease or oxygen therapy rather than direct PH medications.
   • Chronic Thromboembolic PH (CTEPH) (Group 4): Treatment may include anticoagulation, surgery (pulmonary thromboendarterectomy), or medications like riociguat if surgery is not an option.
     
2. Severity of the Disease
   • Early Stages (Functional Class I-II): Patients with mild symptoms might start with medications like PDE5 inhibitors (e.g., sildenafil or tadalafil) or ERAs (e.g., ambrisentan or bosentan).
   • Moderate to Severe PH (Functional Class III-IV): For more advanced cases, combinations of medications (dual or triple therapy) may be used. Options often include prostacyclin analogs (e.g., epoprostenol, treprostinil) which can be given intravenously, subcutaneously, or inhaled for more immediate effects.
     
3. Response to Acute Vasodilator Testing
   • During right heart catheterization, patients with certain types of PH may undergo vasoreactivity testing. If they respond positively (a rare case), they might benefit from calcium channel blockers (e.g., amlodipine). However, this approach is only for a small subset of patients.
     
4. Risk of Side Effects and Comorbidities
   • Each PH medication has potential side effects (e.g., liver toxicity with ERAs, bleeding risk with anticoagulants) that need to be considered in light of a patient’s other medical conditions. A PH specialist will carefully match medications to minimize risks.
     
5. Combination Therapy
   • In moderate to severe cases, combination therapy is often used to target multiple pathways involved in PH progression. The choice of combinations (e.g., an ERA with a PDE5 inhibitor) depends on the patient’s specific needs and the stage of the disease.
     
6. Lifestyle and Accessibility
   • Some therapies require regular infusions or injections, which might not be suitable for all patients. Oral medications are more convenient but may be less effective in advanced cases. Decisions around these factors often balance effectiveness, convenience, and quality of life.

Follow-Up and Monitoring

After starting a medication, regular follow-up appointments and testing (such as echocardiograms or walk tests) help determine if adjustments are needed. The goal is to find a regimen that improves symptoms, slows disease progression, and is sustainable for the patient.

A personalized approach in collaboration with a PH specialist is key to finding the most effective treatment strategy for each patient.