Advances in the management of pulmonary arterial hypertension

Abstract

The management of pulmonary arterial hypertension (PAH) has significantly evolved over the last decades in the wake of more sensitive diagnostics and specialized clinical programs that can provide focused medical care. In the current era of PAH care, 1-year survival rates have increased to 86%-90% from 65% in the 1980s, and average long-term survival has increased to 6 years from 2.8 years. The heterogeneity in the etiology and disease course has opened doors to focusing research in phenotyping the disease and understanding the pathophysiology at a cellular and genetic level. This may eventually lead to precision medicine and the development of medications that may prevent or reverse pulmonary vascular remodeling. With more insight, clinical trial designs and primary end-points may change to identify the true survival benefit of pharmacotherapy. Identifying responders from non-responders to therapy may help provide individualized patient-centered care rather than an algorithm-based approach. The purpose of this review is to highlight the latest advances in screening, diagnosis, and management of PAH.

Keywords: cardiology; heart failure; hypertension; lung transplantation; pulmonary; respiration disorders.

Figure 1

REVEAL 2.0 Risk score calculator for pulmonary arterial hypertension. BNP, brain natriuretic peptide; CTD, connective tissue disease; eGFR, estimated glomerular filtration rate; HR, heart rate; mRAP, mean right atrial pressure; NT-proBNP, N-terminal proBNP; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; SBP, systolic blood pressure.

Figure 2

Pathogenic pathways and drug targets in pulmonary arterial hypertension. BMPR-2, bone morphogenetic protein receptor 2; cAMP, cyclic AMP; GMP, guanosine monophosphate; cGMP, cyclic guanosine monophosphate; GTP, guanosine triphosphate; mPAP, mean pulmonary artery pressure; PA, pulmonary artery; PDE-5, phosphodiesterase-5; PGI2, prostaglandin I2; PVR, pulmonary vascular resistance; RV, right ventricle; sGC, soluble guanylate cyclase.