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ABSTRACT
Background: Pulmonary arterial hypertension (PAH) is a serious disease characterised by progressive precapillary pulmonary hypertension. PAH affects mainly younger women and unless treated causes death within 2–3 years of diagnosis.
Scope: This review article provides an overview of the challenges in diagnosis and treatment of PAH. Literature retrieval was accessed through MEDLINE using the terms pulmonary hypertension, pulmonary arterial hypertension, diagnosis, treatment. Reference citations from publications identified were reviewed.
Diagnosis: The diagnostic process requires a series of investigations that are intended to confirm the diagnosis, to establish the category and cause of pulmonary hypertension, and to determine the severity. Diagnostic procedures include electrocardiography, chest radiography, echocardiography, lung scan, high-resolution and contrast computerised tomography, lung function and cardiac catheterisation with vasoreactivity testing. Doppler echocardiography is one of the most common diagnostic tools in patients with PAH, but right-heart catheterisation is required to establish a definitive diagnosis.
Treatment: The aims of PAH treatment should be directed not only towards symptomatic benefits, but also to improvements in survival and quality of life for the patients. Over the past few years great advances have been made in the management of PAH. Many novel drugs (endothelin receptor antagonists, prostanoids, phosphodiesterase‐5 inhibitors) have been tested in randomised trials. Before embarking on long-term therapy for PAH, reversibility testing should be performed in order to identify the small group of responders who might benefit from calcium channel blockers. Specific PAH therapy is based on functional status and should be instituted at the time of diagnosis. In NYHA class III (and class II), oral drugs are preferred. Bosentan, a dual endothelin‐1 receptor antagonist, is the first-line therapy. In NYHA class IV, intravenous prostanoids, lung transplantation and atrial septostomy remain the therapies of choice. Oral anticoagulant therapy is widely recommended for patients with PAH, although its clinical efficacy as a therapy is difficult to prove. The treatment of PAH requires a highly individualised approach to the patient; therefore patients should be referred to PAH management centres that can provide both comprehensive diagnosis and therapy.
Conclusions: Despite major improvements in PAH therapy, it is still an incurable disease. Many patients experience initial clinical benefit with specific therapy, but they remain limited and symptomatic and, according to prognostic data, continue to be at risk. Therefore combination therapy has been proposed as the concept for the treatment of PAH in the future.