Pharmacologic Treatment of Pulmonary Hypertension

Nnenna L. Iheagwara, PharmD, BCPS; Tyan F. Thomas, PharmD, BCPS


US Pharmacist 

In This Article

Treatment Options

Nonpharmacologic management includes measures to improve or prevent worsening functional status. This includes restricting fluid intake to <1.5 liters daily and sodium intake to <2,400 mg daily and participating in cardiopulmonary rehabilitation.[6,7,10,11]

Pharmacologic treatment encompasses primary and advanced therapy (Figure 1).[12] Primary therapy for PH, also referred to as standard or supportive therapy, is guided by identifying and treating the underlying cause. It may be utilized in all PH groups, providing symptomatic relief for WHO functional class II, III, and IV ( Table 3 ).[9] Advanced therapy focuses on preventing disease progression by treating underlying cardiopulmonary disorders. Patients refractory to treatment may require lung and/or heart transplantation.

Figure 1.

PAH Evidence-Based Treatment Algorithma
APAH: associated pulmonary arterial hypertension; ERA: endothelin receptor antagonist; HPAH: heritable pulmonary arterial hypertension; inh: inhaled; IPAH: idiopathic pulmonary arterial hypertension; IV: intravenous; NA: not applicable; PAH: pulmonary arterial hypertension; PDE-5 I: phosphodiesterase type 5 inhibitor; SC: subcutaneous; WHO: World Health Organization.
a Strength of Recommendation is based on the quality of evidence and the net benefit of therapy as it related to IPAH, HPAH, PAH associated with sclero-derma, or anorexigen use as more readily studied. Recommendations: A: strong; B: moderate; C: weak; E/A: strong based on expert opinion; E/B: moderate based on expert opinion; E/C: weak based on expert opinion.
b Iloprost IV, oral beraprost, and oral sitaxsentan are not available in the U.S.
c Treprostinil inhalation has been FDA approved in PAH adults with NYHA class 3 symptoms since original publication. Strength of Recommendation: B. Source: Reference 12.

Supportive therapy includes oral anticoagulants, diuretics, oxygen, and digoxin. Anticoagulation with warfarin may be useful in patients with or at risk for venous thromboembolism PH (Group 4) and for PAH (Group 1) patients with idiopathic, hereditary, and drug-induced or anorexigen-induced PAH, portal hypertension, scleroderma, or congenital heart disease. The target international normalized ratio range is 1.5 to 3.0 depending on the indication.[6–10] Evidence for anticoagulant use stems from observational studies where survival benefit was noted.[6] For fluid overload and hepatic congestion, loop diuretics are recommended and adjusted to response. Supplemental oxygen of 1 to 4 L/min is utilized to maintain oxygen saturations above 90% at all times and prevent vasoconstriction due to hypoxemia. Oxygen is widely used in PH due to pulmonary diseases and/or hypoxia (Group 3). Lastly, the role of digoxin is not clearly defined in Group 1 PAH. Digoxin may be useful in Group 3 patients with chronic obstructive pulmonary disease (COPD) and biventricular heart failure.[6–10]

Advanced therapy is not indicated or well studied in PH Groups 2 to 5; therefore, these patients should receive treatment for underlying cardiac, pulmonary, or vascular conditions when possible. Group 1 PAH is primarily idiopathic in nature and therefore lacks an underlying cause.[13] The subsequent sections will focus on Group 1 PAH treatment in adults where advanced therapy has been developed to prevent or slow progression when used with primary therapy.


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