Novel Therapeutic Strategies for Reducing Right Heart Failure Associated Mortality in Fibrotic Lung Diseases

Table 1

Effects of pharmacologic therapies in patients with right ventricular dysfunction.

Medication	Route of administration	Mechanism of action	Right ventricular effect	Common side effects
Therapies targeting pulmonary hypertension
Nifedipine and amlodipine	Oral	Calcium channel blockers	Reduce afterload	Headache, dizziness, and extremity edema
Bosentan, ambrisentan, and macitentan	Oral	Endothelin receptor antagonists	Reduce afterload	Headache, dizziness, and arrhythmias
Epoprostenol	IV	Prostacyclin analog	Reduces afterload	Nausea, vomiting, dizziness, and arrhythmias
Iloprost	Inhaled	Prostacyclin analog	Reduces afterload	Nausea, vomiting, headache, and diarrhea
Treprostinil	SC/IV/inhaled	Prostacyclin analog	Reduces afterload	Nausea, headache, cough, and dizziness
Sildenafil and tadalafil	Oral	Phosphodiesterase type 5 inhibitors	Reduce afterload	Nausea, vomiting, headache, and tritanopia
Riociguat	Oral	Soluble guanylate cyclase stimulator	Reduces hypertrophy	Headache, dizziness, gastritis, hypotension, and diarrhea
Imatinib	Oral	Tyrosine kinase inhibitor	Improves function	Nausea, vomiting, edema, diarrhea, rash, and pancytopenia
Fasudil	IV	Rho-kinase inhibitor	Reduces hypertrophy	Nausea, renal dysfunction, fever, and thrombocytopenia
Nitric oxide	Inhaled	Pulmonary vasodilator	Improves function	Hypotension and methemoglobinemia
Therapies targeting the right ventricle (RV)
Carvedilol and bisoprolol	Oral	β-adrenergic receptor blockers	Decrease myocardial fibrosis	Dizziness, fatigue, diarrhea, and hyperglycemia
Ranolazine and trimetazidine	Oral	Modulators of metabolism	Decrease remodeling	Nausea, headache, dizziness, constipation, edema, and dyspnea
Ramipril	Oral	ACE inhibitor	Decreases myocardial fibrosis	Nausea, vomiting, cough, headache, and dizziness
Protandim	Oral	Antioxidant	Decreases myocardial fibrosis	Nausea, vomiting, rash, headache, and diarrhea
Therapies targeting pulmonary fibrosis
Pirfenidone	Oral	Antifibrotic agent	Decreases myocardial fibrosis	Nausea, vomiting, rash, headache, diarrhea, and dizziness
Nintedanib	Oral	Triple angiokinase inhibitor	Undetermined direct effect	Nausea, vomiting, headache, diarrhea, and anorexia

None of these medications have been specifically approved for Group 3 pulmonary hypertension as these patients may have pulmonary fibrosis and may not demonstrate vasoreactivity. Idiopathic pulmonary fibrosis, IV: intravenous, SC: subcutaneous.