La necesidad de la detección temprana de la hipertensión arterial pulmonar

Abstract

Pulmonary arterial hypertension (PAH), Group 1 of the pulmonary hypertension classification, is a rare, highly complex and progressive disorder that ultimately leads to premature death. PAH causes significant physical, social, occupational, and emotional inconveniences among affected patients and their caregivers. Early diagnosis and initiation of the most optimal treatment possible are required in order to achieve the best results, trying to stop vascular remodeling and right heart failure; however, the clinical presentation of PAH is nonspecific and is often associated with other comorbidities, leading to delayed diagnosis or misdiagnosis. In recent decades, a greater understanding of the pathophysiology of PAH has led to changes in its definition, diagnosis, and treatment. In addition, contemporary PAH registries have shown higher survival rates among PAH patients and have allowed the development of risk calculation tools that are now used to drive therapeutic targets. To date, multiple PAH-specific treatments have been developed that target all 3 pathways that contribute to the pathogenesis of PAH endothelial dysfunction (prostacyclin, endothelin, and nitric oxide pathways). Because PAH is subdivided into 7 subgroups, it is essential that individuals be properly grouped to optimize treatment efficacy and complication prevention and improve outcome. Since the impairment of health-related quality of life in PAH is multifactorial, it is important for patients to be educated in the pathology, participate in the clinical decision-making process, and have access to multidisciplinary care, which will improve compliance with medical indications. There is convincing evidence that screening for PAH in high-risk populations will enable earlier diagnosis and intervention, offering a promising opportunity to improve patient outcomes. However, detection methods commonly used in clinical practice have limitations and a combination of tools or parameters may be required to improve the sensitivity and selectivity of current programs. The creation of detection algorithms for patients at risk of PAH with systemic sclerosis has increased the speed and specificity of diagnosis, potentially improving survival, and although the costs of detection remain significant, they are irrelevant given the high cost of treatment of this disease in advanced stages. The development and validation of detection algorithms for PAH of other aetiologies is required. Screening for PAH in asymptomatic patients at risk and developing screening-based approaches in symptomatic patients, where diagnosis is rarely considered, are needed to improve detection rates and reduce time to diagnosis. There is a clear and unmet need for improvements in the diagnosis, characterization and management of patients with PAH. PAH is a rapidly progressing disease, even in patients with mild symptoms, and timely therapeutic intervention is essential to influence the long-term prognosis of patients with PAH.