From the Guidelines
The most effective treatment for pulmonary hypertension involves a combination of medications, including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogs, and soluble guanylate cyclase stimulators, with the specific treatment selection depending on the type of pulmonary hypertension, disease severity, and patient-specific factors. According to the most recent and highest quality study 1, phosphodiesterase-5 inhibitors like sildenafil (20mg three times per day orally) and tadalafil (40mg daily orally) are often first-line treatments, as they relax pulmonary blood vessels by increasing nitric oxide effects. Endothelin receptor antagonists such as ambrisentan (5-10mg daily orally), bosentan (62.5-125mg, twice per day orally), and macitentan (10mg daily orally) block the vasoconstricting effects of endothelin. Prostacyclin analogs including epoprostenol (1-12ng/kg/min continuous intravenous infusion via central venous catheter), iloprost (inhaled 6-9 times per day), and treprostinil (subcutaneous, IV, or inhaled) promote vasodilation and inhibit platelet aggregation. Soluble guanylate cyclase stimulators like riociguat (0.5-2.5mg three times per day orally) enhance nitric oxide signaling. Some key points to consider when treating pulmonary hypertension include:
- Regular monitoring of liver function, blood counts, and clinical response is essential, as some medications require special monitoring programs due to potential side effects 1.
- Supportive measures including oxygen therapy, diuretics for fluid overload, and anticoagulation may also be necessary components of comprehensive treatment.
- Combination therapy is often needed for optimal management, and treatment selection should be individualized based on the type of pulmonary hypertension (WHO groups 1-5), disease severity, and patient-specific factors 1.
From the FDA Drug Label
Tadalafil tablets are also marketed as ADCIRCA for the treatment of pulmonary arterial hypertension. Tadalafil tablets can cause your blood pressure to drop suddenly to an unsafe level if it is taken with certain other medicines... Do not take tadalafil tablets if you take medicines called guanylate cyclase stimulators which include: Riociguat (Adempas®) a medicine that treats pulmonary arterial hypertension and chronicthromboembolic pulmonary hypertension.
Meds for pulmonary hypertension:
- Tadalafil (PO) is used to treat pulmonary arterial hypertension.
- It is marketed as ADCIRCA for this condition.
- However, it should not be taken with other medicines that treat pulmonary hypertension, such as riociguat (Adempas®) 2, 2.
From the Research
Medications for Pulmonary Hypertension
- Bosentan, a dual endothelin-receptor antagonist, has been shown to improve exercise capacity and hemodynamics in patients with pulmonary hypertension 3, 4.
- Sildenafil, a phosphodiesterase inhibitor, has been used in combination with bosentan, but its effectiveness in this combination is unclear, with one study showing no benefit over placebo for 12-week change from baseline in 6MWD 5.
- Transitioning from the combination of bosentan and sildenafil to alternative therapy, such as macitentan, has been shown to be safe and result in clinical stability 6.
- Other medications, such as epoprostenol, treprostinil, and iloprost, have also been used to treat pulmonary arterial hypertension, with varying degrees of success 7.
Treatment Options
- Endothelin-receptor antagonists, such as bosentan and macitentan, are oral therapies that can improve exercise capacity and hemodynamics in patients with pulmonary hypertension 3, 4, 6.
- Phosphodiesterase inhibitors, such as sildenafil, can improve exercise capacity and quality of life in patients with pulmonary hypertension, but their effectiveness in combination with other medications is unclear 5, 7.
- Prostacyclin analogues, such as epoprostenol and treprostinil, are effective in treating moderate to severe pulmonary arterial hypertension, but require intravenous or subcutaneous administration 7.
Considerations for Treatment
- The choice of medication should be based on the severity of the disease, the patient's symptoms and functional class, and the presence of any comorbidities 7.
- Combination therapy may be necessary for some patients, but the effectiveness and safety of different combinations are not well established 5, 6.
- Regular monitoring of the patient's condition and adjustment of the treatment plan as needed are crucial for optimal management of pulmonary hypertension 6, 7.