Towards early detection of pulmonary hypertension: a call to arms

Abstract

The more powerful the obstacle, the more glory we have in overcoming it – Mascarille, Moliere, L’Etourdi ou Les Contretemps , Act V, Scene XI Emerging evidence demonstrates that early detection alters outcomes in systemic sclerosis-related pulmonary arterial hypertension (PAH) [1]. The endeavours of the pulmonary hypertension community over the last 20 years have improved survival with modern treatment strategies but prognosis remains poor [2]. On average, even in the current era, patients have been symptomatic for >2 years by the time the diagnosis of pulmonary hypertension is made [3, 4]. If our understanding of the pathobiology of PAH is correct, then by the time of diagnosis, there will have been extensive remodelling and/or obliteration of a very significant proportion of the small muscular pulmonary arteries in our patients. The imperative over the coming decade for those of us treating pulmonary hypertension is to improve our ability to diagnose it early, when permanent damage to the pulmonary circulation is limited, leading to improved outcomes and, potentially, a normal quality of life with modern therapy. In response to these facts, patient-led support groups in Europe, North America and Australia have instigated campaigns not only to raise awareness of the existence of PAH but also to encourage individuals with unexplained breathlessness or fatigue to pursue a diagnosis with their doctors. This will inevitably lead to increasing referrals to pulmonary vascular clinics of patients with unexplained dyspnoea, syncope or fatigue. In some of these individuals, investigation will confirm a diagnosis of established PAH and, in others, alternative causes of their symptoms will be diagnosed. However, clinicians will be faced with symptomatic patients who have a moderate-to-high pre-test probability of pulmonary vascular disease based on clinical history or examination in whom echocardiography and other assessments at rest …