Delays in Diagnosis

PAH Is a Silently Progressive Disease That Can Lead to Right Heart Failure1-3

2.8 YEARS

The average delay between symptom onset and diagnosis4*

Hourglass
Hourglass

The consequences of late diagnosis and treatment can be severe5-7

Increased risk of:

  • Right heart failure
  • Hospitalizations
  • Death
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Decrease in:

  • Physical ability
  • Quality of life

The race to manage PAH progression involves multiple pathways1,7:

  • Nitric oxide pathway
  • Endothelin pathway
  • Prostacyclin pathway
  • Activin signaling pathway

Once PAH is diagnosed, there is no time to lose—

PROGRESSION HAS ALREADY BEGUN8

An RHC is required to confirm diagnosis of PAH by assessing key hemodynamic characteristics including mPAP, PAWP, and PVR.1

Hemodynamic definitions of PH and PAH1

The definitions are based on hemodynamic assessment by an RHC

  • PH mPAP >20 mmHg
  • PAH mPAP >20 mmHg PAWP ≤15 mmHg PVR >2 WU

Common symptoms of PAH include dyspnea on exertion, fatigue, chest pain, edema, presyncope/syncope, and dizziness. Since PAH symptoms may present like other lung conditions, an RHC can help distinguish it from other cardiac or pulmonary etiologies.9,10

Learn about the pathways

involved in the treatment of PAH

Explore treatment guidelines

based on clinical evidence and expert consensus

*

REVEAL was a US-based, observational registry involving 55 academic- and community-based treatment centers. 3515 patients enrolled between March 2006 and December 2009. REVEAL was funded and sponsored by Actelion Pharmaceuticals US, Inc.1,6

mPAP=mean pulmonary arterial pressure; PAH=pulmonary arterial hypertension; PAWP=pulmonary arterial wedge pressure; PH=pulmonary hypertension; PVR=pulmonary vascular resistance; REVEAL=Registry to Evaluate Early and Long-term PAH Disease Management; RHC=right heart catheterization; WU=Wood units.

References: 1. Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2022;43(38):3618-3731. 2. Lai Y-C, Potoka KC, Champion HC, et al. Pulmonary arterial hypertension: the clinical syndrome. Circ Res. 2014;115(1):115-130. 3. Lau EMT, Humbert M, Celermajer DS. Early detection of pulmonary arterial hypertension. Nat Rev Cardiol. 2015;12(3):143-155. 4. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376-387. 5. Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest. 2011;140(1):19-26. 6. Didden E-M, Lee E, Wyckmans J, et al. Time to diagnosis of pulmonary hypertension and diagnostic burden: a retrospective analysis of nationwide US healthcare data. Pulm Circ. 2023;13(1):e12188. 7. Chin KM, Gaine SP, Gerges C, et al. Treatment algorithm for pulmonary arterial hypertension. Eur Respir J. 2024;64(4):2401325. 8. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2):1700740. doi:10.1183/13993003.00740-2017 9. Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest. 2011;140(1):19-26. doi:10.1378/chest.10-1166. 10. Bostwick D, Hatton ND, Mayeux JD, et al. The approach to the patients with chronic dyspnea of unclear etiology. Adv Pulm Hypertens. 2018;16(3):103-111.