Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland.
BACKGROUND
Incident pulmonary arterial hypertension was underrepresented in most pulmonary hypertension registries and may have a different disease profile to prevalent disease.


OBJECTIVE
To determine the characteristics and outcome of a purely incident, treatment-naive cohort of idiopathic, heritable, and anorexigen-associated pulmonary arterial hypertension and to determine the changes in presentations and survival over the past decade in the United Kingdom and Ireland.


METHODS
All consecutive newly diagnosed patients from 2001 to 2009 were identified prospectively.


RESULTS
A total of 482 patients (93% idiopathic, 5% heritable, and 2% anorexigen-associated pulmonary arterial hypertension) were diagnosed, giving rise to an estimated incidence of 1.1 cases per million per year and prevalence of 6.6 cases per million in 2009. Younger patients (age ≤ 50 yrs) had shorter duration of symptoms, fewer comorbidities, better functional and exercise capacity, higher percent diffusing capacity of carbon monoxide, more severe hemodynamic impairment, but better survival compared with older patients. In comparison with the earlier cohorts, patients diagnosed in 2007-2009 were older, more obese, had lower percent diffusing capacity of carbon monoxide,(,) and more comorbidities, but better survival. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) equation, REVEAL risk score, and Pulmonary Hypertension Connection Registry survival equation accurately predicted survival of our incident cohort at 1 year.


CONCLUSIONS
This study highlights the influence of age on phenotypes of incident pulmonary arterial hypertension and has shown the changes in demographics and epidemiology over the past decade in a national setting. The results suggest that there may be two subtypes of patients: the younger subtype with more severe hemodynamic impairment but better survival, compared with the older subtype who has more comorbidities.
Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Agamemnon Street, Glasgow, UK.
Yi
Y
Ling
Martin K
MK
Johnson
David G
DG
Kiely
Robin
R
Condliffe
Charlie A
CA
Elliot
J Simon R
JS
Gibbs
Luke S
LS
Howard
Joanna
J
Pepke-Zaba
Karen K K
KK
Sheares
Paul A
PA
Corris
Andrew J
AJ
Fisher
James L
JL
Lordan
Sean
S
Gaine
J Gerry
JG
Coghlan
S John
SJ
Wort
Michael A
MA
Gatzoulis
Andrew J
AJ
Peacock
D000328:Adult; D017677:Age Distribution; D000368:Aged; D015897:Comorbidity; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000401:mortality / Q000503:physiopathology; D015994:Incidence; D007494:Ireland / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012042:Registries / Q000706:statistics & numerical data; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D006113:United Kingdom / Q000453:epidemiology