Pulmonary Hypertension in the Modern Era: Science and Clinical Practice

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Background

Pulmonary hypertension (PH) is a disease associated with abnormally elevated pulmonary pressures and right heart failure that, if untreated, leads to premature death. PH is classified into five groups based on etiology, pathological features and hemodynamic findings. Among these, pulmonary arterial hypertension (PAH) has received the greatest attention given its poor short-term prognosis and devastating impact on quality of life.

In 2018, the hemodynamic definition of PH was revised in an effort to help identify patients at earlier disease stages. Despite the availability of 14 approved drugs, PH remains a challenging disease with a high index of morbidity and mortality. At the center of this dilemma is the progressive nature of the disease which ultimately overcomes medical therapy and leaves the patient with few options for life-saving interventions.

Multiple original articles and several reviews have been published that provide an exciting new perspective on this fast-evolving field of medicine. Advances in genetics and molecular biology have revolutionized our understanding of the pathophysiological mechanisms that drive the development of vascular lesions in PAH and the genetic modifiers that increase the risk of disease development on susceptible individuals. Clinical research has also exploded in the last 5 years to encompass the study of diagnostic markers and novel therapeutics capable of improving clinical outcomes in selected patients with PH.

However, despite these exciting advances, there are still challenges that investigators and clinical practitioners currently face when dealing with PH. For instance, basic and physician scientists are limited by the imperfect animal models available to study PH as well as the difficulty of translating benchwork discoveries into clinically meaningful interventions. For clinicians, the challenge remains to identify patients at high risk of progression and death independent of hemodynamics and available therapies. Thus, the time is right for the scientific and medical communities to come together and strategize on how to address these challenges and improve our odds of finding a cure for this devastating condition.

This Research Topic represents a concerted effort to update the community on the state-of-the-art knowledge in PH and to discuss provocative questions that remain of great interest to investigators in the field. We hope to provide readers with a unique and unbiased resource that will be useful in the clinical practice and in the development of new research directions.

Keywords: pulmonary hypertension, vascular biology, hemodynamics, clinical trials, therapeutics

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