title: Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: The delay study.
abstract: Survival rates for patients with idiopathic pulmonary arterial hypertension (IPAH) have improved with the introduction of PAH-specific therapies. However, the time between patient-reported onset of symptoms and a definitive diagnosis of IPAH is consistently delayed. We conducted a retrospective, multi-center, descriptive investigation in order to (a) understand what factors contribute to persistent diagnostic delays, and (b) examine the time from initial symptom onset to a definitive diagnosis of IPAH. Between January 2007 and December 2008, we enrolled consecutively diagnosed adults with IPAH from four tertiary referral centers in Australia. Screening of patient records and "one-on-one" interviews were used to determine the time from patient-described initial symptoms to a diagnosis of IPAH, confirmed by right heart catheterization (RHC). Thirty-two participants (69% female) were studied. Mean age at symptom onset was 56 ± 16.4 years and 96% reported exertional dyspnea. Mean time from symptom onset to diagnosis was 47 ± 34 months with patients subsequently aged 60 ± 17.3 years. Patients reported 5.3 ± 3.8 GP visits and 3.0 ± 2.1 specialist reviews before being seen at a pulmonary hypertension (PH) center. Advanced age, number of general practitioner (GP) visits, heart rate, and systolic blood pressure at the time of diagnosis were significantly associated with the observed delay. We found a significant delay of 3.9 years from symptom onset to a diagnosis of IPAH in Australia. Exertional dyspnea is the most common presenting symptom. Current practice within Australia does not appear to have the specific capacity for timely, multi-factorial evaluation of breathlessness and potential IPAH.
authors: Monash University, Epidemiology and Preventive Medicine, The Alfred Hospital, Melbourne, Australia.
authors: Geoff
authors: G
authors: Strange
authors: Eli
authors: E
authors: Gabbay
authors: Fiona
authors: F
authors: Kermeen
authors: Trevor
authors: T
authors: Williams
authors: Melinda
authors: M
authors: Carrington
authors: Simon
authors: S
authors: Stewart
authors: Anne
authors: A
authors: Keogh
keywords: diagnosis; epidemiology; pulmonary arterial hypertension; pulmonary hypertension
references: Respir Med. 2010 Jun;104(6):902-10
references: 20149617
references: Am J Respir Crit Care Med. 2002 Mar 15;165(6):800-4
references: 11897647
references: Curr Opin Cardiol. 2004 Nov;19(6):575-81
references: 15502501
references: Arch Intern Med. 2008 May 12;168(9):959-68
references: 18474760
references: Intern Med J. 2011 Mar;41(3):227-34
references: 20002851
references: N Engl J Med. 1996 Feb 1;334(5):296-301
references: 8532025
references: Am J Respir Crit Care Med. 2006 May 1;173(9):1023-30
references: 16456139
references: Am J Respir Crit Care Med. 2003 Feb 15;167(4):580-6
references: 12446266
references: N Engl J Med. 2002 Mar 21;346(12):896-903
references: 11907289
references: Aust Fam Physician. 2005 Jul;34(7):520-1
references: 15999160
references: Ann Intern Med. 1987 Aug;107(2):216-23
references: 3605900
references: J Am Coll Cardiol. 2004 Apr 7;43(7):1149-53
references: 15063421
references: J Am Coll Cardiol. 2003 Jun 18;41(12):2119-25
references: 12821234
references: N Engl J Med. 2002 Aug 1;347(5):322-9
references: 12151469
references: Am Heart J. 2006 Apr;151(4):851.e1-5
references: 16569546
references: Eur Respir J. 2007 Jul;30(1):104-9
references: 17360728
references: Chest. 2004 Jul;126(1 Suppl):14S-34S
references: 15249493
references: Circulation. 2002 Sep 17;106(12):1477-82
references: 12234951
references: Lancet. 2008 Jun 21;371(9630):2093-100
references: 18572079
references: Heart. 1998 Aug;80(2):151-5
references: 9813561
references: Swiss Med Wkly. 2008 Jun 28;138(25-26):379-84
references: 18587690
references: Circulation. 2006 Jul 4;114(1):48-54
references: 16801459
references: Eur Heart J. 2009 Oct;30(20):2493-537
references: 19713419
references: Eur Respir J. 2005 Feb;25(2):244-9
references: 15684287
references: J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):40S-47S
references: 15194177
references: Lancet. 2001 Oct 6;358(9288):1119-23
references: 11597664
references: Chest. 2011 Jul;140(1):19-26
references: 21393391
references: Chest. 2004 Jul;126(1 Suppl):4S-6S
references: 15249490
references: Circulation. 2008 Jun 10;117(23):3010-9
references: 18506008
references: Am J Respir Crit Care Med. 2005 Jun 1;171(11):1292-7
references: 15750042
references: Respir Med. 2009 Aug;103(8):1136-42
references: 19403296
references: Ann Intern Med. 1990 Apr 1;112(7):485-91
references: 2107780
references: J Cardiovasc Nurs. 2010 Mar-Apr;25(2):106-14
references: 20142752
references: Chest. 1987 May;91(5):675-81
references: 3105966
references: BMC Public Health. 2011 Jan 12;11:33
references: 21226957
references: Circulation. 2010 Jul 13;122(2):156-63
references: 20585011
references: N Engl J Med. 2005 Nov 17;353(20):2148-57
references: 16291984
references: J Am Coll Cardiol. 2005 Aug 2;46(3):529-35
references: 16053970
references: Am J Respir Crit Care Med. 2004 Feb 15;169(4):441-7
references: 14630619
references: Am Fam Physician. 2001 May 1;63(9):1789-98
references: 11352291
references: J Am Coll Cardiol. 2002 Aug 21;40(4):780-8
references: 12204511
references: Ann Intern Med. 2000 Mar 21;132(6):425-34
references: 10733441