The Emerging Role of Stem/Progenitor Cells in Pulmonary Vascular Disease

Saima Rajabali,Duncan J Stewart

doi:10.4172/2155-9880.s8-004

Abstract

Pulmonary hypertension is a severe disease characterized by small pulmonary artery obstruction from vascular proliferation and remodeling leading to elevated mean pulmonary arterial pressure, increased pulmonary vascular resistance, right ventricular failure and death. Current treatments include prostacyclin analogs, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors, which largely address mechanisms of endothelial dysfunction that were identified over 2 decades ago. Despite advances in understanding the disease mechanisms and the development of new pharmacological therapies, the prognosis of pulmonary hypertension remains poor. Recent advances in stem cell biology have unraveled the potential of stem/progenitor cells to repair damaged organs and offer the possibility for cell-based treatment for intractable diseases. This review summarizes the emerging role of stem/progenitor cells in the pathophysiology and the treatment of pulmonary hypertension.

Highlights

Pulmonary hypertension [1] is defined by an increase in pulmonary arterial pressure exceeding 25 mmHg at rest [2]
Evidence suggests that CFU-Hill and circulating angiogenic cells” (CACs) are not true endothelial progenitor cells (EPCs), but modified myeloid lineage cells that participate in neoangiogenesis
Since we have developed a greater understanding of the pathophysiology, diagnosis and treatment of pulmonary vascular disease

Summary

Introduction

Pulmonary hypertension [1] is defined by an increase in pulmonary arterial pressure exceeding 25 mmHg at rest [2]. Pulmonary arterial hypertension (PAH) represents a subset of PH and is characterized by pulmonary arterial obstruction, increased pulmonary vascular resistance, leading to right ventricular failure and death. Despite advances in therapeutic interventions targeting the vascular endothelium including prostacyclins, endothelin receptor blockers and phosphodiesterase type 5 inhibitors; the mortality and morbidity of PAH remain high. Increasingly human studies, have advanced our understanding about the pathogenesis of PAH and enabled the development of novel pharmacological therapies. While at present there is no perfect preclinical model that completely recapitulates human PAH [4], all models have provided invaluable insight into the pathophysiology of PH, including the emerging role of stem/progenitor cells

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