Abstract

Pulmonary arterial hypertension (PAH) is a progressive and fatal lung disease of multifactorial etiology. Most of the available drugs and FDA-approved therapies for treating pulmonary hypertension attempt to overcome the imbalance between vasoactive and vasodilator mediators, and restore the endothelial cell function. Traditional medications for treating PAH include the prostacyclin analogs and receptor agonists, phosphodiesterase 5 inhibitors, endothelin-receptor antagonists, and cGMP activators. While the current FDA-approved drugs showed improvements in quality of life and hemodynamic parameters, they have shown only very limited beneficial effects on survival and disease progression. None of them offers a cure against PAH, and the median survival rate remains less than three years from diagnosis. Extensive research efforts have led to the emergence of innovative therapeutic approaches in the area of PAH. In this review, we provide an overview of the current FDA-approved therapies in PAH and discuss the associated clinical trials and reported-side effects. As recent studies have led to the emergence of innovative therapeutic approaches in the area of PAH, we also focus on the latest promising therapies in preclinical studies such as stem cell-based therapies, gene transfer, and epigenetic therapies.

Highlights

  • Pulmonary arterial hypertension (PAH) is a progressive and fatal lung disease of multifactorial etiology (Montani et al, 2013) (Fig. 1)

  • Group IV refers to chronic thromboembolic pulmonary hypertension (CTEPH), which may occur when blood clots affect the flow of blood in the pulmonary arteries and other chronic pulmonary artery obstructions (Simonneau et al, 2013)

  • As intensive research efforts have led to the emergence of innovative therapeutic approaches in the area of PH, we focus on the latest emerging and promising therapies in preclinical studies such as stem cell-based therapies, gene therapies, and epigenetic therapies

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Summary

Introduction

Pulmonary arterial hypertension (PAH) is a progressive and fatal lung disease of multifactorial etiology (Montani et al, 2013) (Fig. 1). In PAH, increased pressure in the vessels is associated with pulmonary artery vascular remodeling, obstruction of small arteries (Lai et al, 2014), and an increase in the vascular resistance to blood flow through the lungs. Abnormal endothelial cell proliferation, along with exuberant neoangiogenesis, leads to the formation of glomeruloid structures known as plexiform lesions. They are described as common pathological features of the pulmonary vessels of patients with PAH. The proliferation and migration of the pulmonary artery smooth muscle cells (PASMCs) contribute to the muscularization of the small pulmonary arteries and, to vascular obstruction, which progressively increases vascular resistance in PH (Shimoda and Laurie, 2013). Despite the available treatments and ongoing research efforts, there is currently no curative treatment curative treatment against PAH

Diagnosis of pH
Classification of PH
Prostacyclin analogs and receptor agonists
Phosphodiesterase inhibitors
Endothelin-receptor antagonists
Calcium sensitizing agent
Combination therapy
Emerging therapies
Stem Cell Therapy
Gene therapy
Epigenetic medicines
Findings
Conclusion
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