Abstract

Pulmonary hypertension (PH) is a rare disease characterized by considerable morbidity and mortality. Significant progress has been recently achieved in enhancing the identification, diagnosis, and treatment of the disease, as evidenced by the latest guideline. Several additional medical conditions can complicate the overall understanding of the patient's condition, making the diagnosis even more difficult. Genetic and molecular factors, certain toxic drugs (such as methamphetamines, desatinib, or anorexigens), systemic disorders, or other predisposing conditions lead to the remodeling of distal pulmonary arterioles, resulting in pulmonary hypertension. Non-invasive investigations are initially undertaken in suspected cases based on cardiac biomarkers, lung function, and echocardiograms. Nowadays, the definition of pulmonary hypertension (PH) has recently changed, now including patients with mean pulmonary artery pressure >20 mmHg, and hemodynamic evaluation with right heart catheterization remains the diagnostic gold standard. Beyond new medically targeted therapies, there is a greater appreciation for the importance of supervised training in stable PH and the possible role of interventional therapies in select cases. The landscape of PH is in constant change, characterized by progress, innovation, and new medical opportunities.

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