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Contemporary PAH registries vary in their study populations, study design, and cohorts. Significant progress in the field of PAH pathophysiology and treatment have occurred in the 2 decades since the NIH registry. PAH-specific therapies were not available at this time, and registry participants had a median survival of 2.8 years (1 year, 68% 3 year, 48% and 5 year, 34%). The registry largely consisted of females, found a mean age of PAH presentation of 36 years, and was primarily Caucasian. 5,18 This registry included 187 individuals having PAH of various etiologies. 7 The National Institutes of Health (NIH) was a landmark registry that collected PAH data between 19. However, it can occur in males and is often associated with worse clinical outcomes. Generally, PAH affects women aged between 30 and 60 years. 1 Idiopathic, heritable, and anorexigen-induced PAH make up 52.6% of all PAH cases. PAH is a rare disorder found in 15 to 50 persons per million within the United States and Europe. 16 A separate group of individuals with long-term response to calcium channel blockers was included in the updated clinical classification based on accumulating improved survival data in this small subset of patients. 1,2 Typically, individuals with PVOD/PCH have similar clinical presentations and hemodynamic profiles as those with PAH however, they have poorer prognosis, limited response to PAH treatment, and are at high risk for developing pulmonary edema from PAH therapeutics. Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH) were moved to a subset within group 1 as opposed to the prior iteration of the WSPH, where they were designated as group 1. As a result, the management of PAH is rapidly evolving.Ī small subset of patients with PAH presenting with overt features of venous/capillary involvement was also recognized as a distinct category. The past 2 decades have been marked by significant advancements leading to novel therapeutics and improved understanding of the pathogenesis of PAH. Patients with PAH also must undergo a thorough evaluation to ascertain the severity of disease and future risk, and ideally have access to treatment at specialized care centers. Due to the progressive nature of PAH, it is crucial that the disease is diagnosed early with an accurate classification. 3 Currently, there is no cure, and the majority of patients with PAH go on to develop right heart dysfunction leading to death. 2 Pulmonary arterial hypertension (PAH), which corresponds to group 1 PH, and a focus of this article, is a complex and devastating disease that causes progressive vasoconstriction and vascular remodeling of the distal pulmonary arteries.

1 The World Symposium on Pulmonary Hypertension (WSPH) categorizes pulmonary hypertension into 5 groups (Table 1). Pulmonary hypertension (PH) describes a group of severe pulmonary vascular disorders characterized by elevated mean pulmonary arterial pressure (mPAP) at rest.

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The purpose of this review is to update healthcare professionals on the management of PAH with the most current information on epidemiology, pathophysiology, clinical presentation, and diagnostic considerations. As health-related quality of life for PAH is multifactorial, it is important that patients are involved in the clinical decision-making process and have access to multidisciplinary care. Because PAH is classified into 7 subgroups, it is essential that individuals are grouped appropriately for the efficacy of treatment and avoidance of harm. To date, multiple PAH-specific therapies have been developed, and all currently target one of 3 pathways that contribute to the endothelial dysfunction pathogenesis of PAH (prostacyclin, endothelin, and nitric oxide pathways). Additionally, contemporary PAH registries have shown greater survival rates among patients with PAH and have allowed for the development of risk calculator tools that are now used to drive therapeutic goals. In the past decades, increased understanding of the pathobiology of PAH has led to changes in its definition. Early diagnosis and initiation of treatment is required for best outcomes however, the clinical presentation of PAH is nonspecific and frequently overlaps with several other conditions, often leading to a delay in diagnosis or misdiagnosis.

PAH causes significant physical, social, work, and emotional burdens among affected patients and their caregivers. Group 1 pulmonary hypertension (or pulmonary arterial hypertension) is a rare, highly complex, and progressive disorder that is incurable and ultimately can lead to premature death.

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