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WHAT IS PH? : EPIDEMIOLOGY

DEFINITION AND CLASSIFICATION / PATHOPHYSIOLOGY / EPIDEMIOLOGY / CLINICAL PRESENTATION / MANAGEMENT / NATURAL HISTORY


Data on the epidemiology of PAH varies between studies and depends upon the population in question and the aetiology of the condition. The UK National Audit on Pulmonary Hypertension 2012 showed the prevalence of PAH in Scotland to be 48.5 cases per million population. Throughout the UK, the audit showed the proportion of patients in different subcategories of PAH to be as below:

 

Subcategory of PAH  %
Idiopathic 34.0
Heritable 1.5
Drug induced 0.7
Connective tissue disease 28.0
Congenital heart disease 30.0
Portal hypertension 3.6
Haemolytic anaemia 1.2
HIV 0.9 
PPHN

0.1 

 

 

 

The proportion of patients with the condition affected by PAH varies widely and is summarized in the table below.

 

Condition % with PAH
Systemic sclerosis 12
Congenital heart disease 15-29
Portal hypertension 2
HIV 0.5

 

 

 

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