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Making a diagnosis in PAH

CORRESPONDENCE: J. S. R. Gibbs, National Pulmonary Hypertension Service, Dept of Cardiology, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK. Fax: 44 2083832331. E-mail: s.gibbs{at}imperial.ac.uk

Poor survival among patients with untreated pulmonary arterial hypertension (PAH) means that timely and accurate diagnosis is of paramount importance. However, the nonspecific nature of PAH symptoms, which include breathlessness and fatigue, make PAH a considerable diagnostic challenge. As a result, many patients are passed among different physicians and are only correctly diagnosed at relatively advanced stages of disease, when they are already significantly compromised and are finding even the basic activities of daily living difficult. Over recent years, the advent of targeted therapies has changed PAH from a rapidly fatal disease to a serious but treatable condition. This makes the need for obtaining a rapid and accurate diagnosis all the more urgent.

The diagnostic process itself requires a reason for clinical suspicion and a series of investigations that are intended to confirm the diagnosis, to establish the cause of pulmonary hypertension and to determine the severity. The present paper reviews the routine investigations available to the physician to diagnose PAH, including electrocardiography, chest radiography, transthoracic Doppler echocardiography and right heart catheterisation, and highlights some of the diagnostic pitfalls encountered en route to an accurate diagnosis of PAH.

KEYWORDS: Diagnosis, pulmonary arterial hypertension