Review Article

Pulmonary Arterial Hypertension in Adults: Novel Drugs and Catheter Ablation Techniques Show Promise? Systematic Review on Pharmacotherapy and Interventional Strategies

Table 4

Patients, etiology, end points, treatment effects, and adverse reactions of the US Food and Drug Administration approved soluble guanylate cyclase stimulators in the pivotal Phase III randomized controlled trials for treatment of pulmonary arterial hypertension in adults.

PATENT-1 [34]CHEST-1 [35]

Patients (no.)443261
DrugRIOCIGUAT
Dosing/route0.5–2.5 mg tid/os
Follow-up (months)34
Etiology (%)*
 IPAH63
 CTD25
 CHD8
 Portopulmonary3
 Anorexigen use1
 CTEPH
  Inoperable70
  Postoperative30
Functional class
 NYHA/WHO I32
 NYHA/WHO II4032
 NYHA/WHO III5662
 NYHA/WHO IV15
Primary end point6MWD6MWD
Treatment effects
6MWD (m) 3039
 HemodynamicsImprovedImproved
 Clinical worseningReducedNo change
 Adverse reactions Headache, peripheral edema, hypotension, dizziness, and syncope

PATENT: Pulmonary Arterial hyperTENsion Soluble Guanylate-Cyclase-Stimulator Trial; CHEST: CHronic thromboEmbolic Pulmonary Hypertension Soluble Guanylate-Cyclase-Stimulator Trial; tid: three times daily; os: oral; sum of percentage may not be 100% for rounding to the nearest unit; 0.5 is rounded to the upper unit; IPAH: idiopathic pulmonary arterial hypertension; CTD: connective tissue disease; CHD: congenital heart disease (congenital systemic-to-pulmonary shunts); CTPEH: chronic thromboembolic pulmonary hypertension; NYHA: New York Heart Association; WHO: World Health Organization; 6MWD: 6 min walk distance; : mean (or median) change from baseline.