Introduction on A Practical Clinical Approach to the Diagnosis and Treatment of Patients with Pulmonary Hypertension
Pulmonary hypertension (PH) is said to occur when the mean pulmonary arterial pressure (mPAP) exceeds 25 mmHg at rest or 30 mmHg during exercise. There are many diverse causes of this condition but the term pulmonary arterial hypertension (PAH) is used to describe a rare group of diseases that share histopathological similarities in the small muscularised pulmonary arterioles leading to vascular remodelling known as plexogenic pulmonary arteriopathy (PPA). As a consequence, there is a progressive elevation in the pulmonary vascular resistance (PVR) that, if untreated, leads to death as a consequence of progressive right heart failure. Currently, there is no effective cure for PAH and the majority of treatments available either stabilise the condition or slow the rate of progression.1,2
Unfortunately, PH is often not considered or misdiagnosed and a median time of 14 months from symptom onset to diagnosis has been reported.3
It is essential that there is an improvement in the awareness of PH among health care professionals and that clinicians managing these complex patients know how to correctly interpret the investigations necessary to reach the diagnosis. Furthermore once the diagnosis has been made, patients should be referred to a specialist PH centre to enable appropriate therapeutic intervention to be made as soon as is possible.4