Published Studies in PAH

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Recent publications on best practices in PAH patient monitoring and treatment

PAH Assessment of risk: Insights from an International Survey of Clinical Practice article thumbnail

Assessment of Risk of Disease Progression in Pulmonary Arterial Hypertension: Insights From an International Survey of Clinical PracticeSimons JE, Mann EB, Pierozynski A.
Adv Ther. 2019;36(9):2351-2363.

Study Design

  • Chart review of 90 PAH-experienced cardiologists and pulmonologists who managed ≥7 patients with PAH

Key Insights

  • Clinical gestalt and formal risk calculations were compared for 365 patient charts
  • When calculated, risk status aligned with gestalt in less than half (45%) the charts evaluated
  • Of patients assessed to be low risk by gestalt, 80% were assigned to a higher risk category after formal risk assessment
Is your FC II patient low risk article thumbnail

Risk Assessment in Patients With Functional Class II Pulmonary Arterial Hypertension: Comparison of Physician Gestalt With ESC/ERS and the REVEAL 2.0 Risk ScoreSahay S, Tonelli AR, Selej M, Watson Z, Benza RL.
PLoS One. 2020;15(11):e0241504.

Study Design

  • Retrospective chart analysis of 153 FC II patients on mono or dual therapy (ERA/PDE-5i)
  • PAH-experienced physicians were surveyed for their gestalt assessment of risk, and then risk was calculated independently using COMPERA, the French Noninvasive method, and REVEAL 2.0

Key Insights

  • More than half of FC II patients were classified as intermediate- or high-risk status when risk was formally calculated
  • Factors that led to incongruent risk assessments included less frequent Echos at follow up, higher physician-reported patient activity level, and HCP-reported patient symptomatic stability
Risk Reduction and Hemodynamics with Initial Combination Therapy in Pulmonary Arterial Hypertension article thumbnail

Risk Reduction and Hemodynamics With Initial Combination Therapy in Pulmonary Arterial HypertensionBadagliacca R, D’Alto M, Ghio S, et al.
Am J Respir Crit Care Med. 2021;203(4):484-492.

Study Design

  • Retrospective analysis of 181 treatment-naive patients initiated on ERA + PDE-5i therapy who received RHC at baseline and at 6-month follow-up

Key Insights

  • Initial ERA + PDE-5i therapy decreased PVR by an average of 35%, and one-third of patients had a decrease in PVR <25%
  • Low-risk status was achieved or maintained in only 35% of patients according to REVEAL 2.0 scores
  • Patients who were able to achieve low-risk status had greater PVR reductions
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Prediction of Health Quality of Life and Hospitalization in PAH: The Pulmonary Hypertension Association Registry (PHAR) article thumbnail

Prediction of Health-Related Quality of Life and Hospitalization in Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry (PHAR)Min J, Badesch D, Chakinala M, et al.
Am J Respir Crit Care Med. 2021; 203(6)761-764.

Study Design

  • 869 patients with PAH enrolled in PHAR (2015-2019) were included in analyses to determine if higher risk status was associated with worse HRQoL or increased hospitalizations
  • Risk was calculated using COMPERA and REVEAL 2.0; HRQoL was determined using the Medical Outcome Study Short Form-12 and emPHasis-10

Key Insights

  • Higher risk status was associated with worse disease-specific HRQoL and increased hospitalizations
  • More refined risk stratification with improved risk calculation techniques could allow for targeted strategies to improve HRQoL and reduce hospitalizations
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The Growing Role of Echocardiography in Pulmonary Arterial Hypertension Risk Stratification: The Missing Piece article thumbnail

The Growing Role of Echocardiography in Pulmonary Arterial Hypertension Risk Stratification: The Missing PieceMiotti C, Papa S, Manzi G, et al.
J Clin Med. 2021; 10(4):619.

Study Design

  • Literature review on the use of Echo parameters in PAH and their current and future roles in determining patient prognosis

Key Insights

  • Echo-derived parameters show how a patient’s RV is adapting to increased afterload and can indicate disease progression before worsening symptoms or 6MWD
  • Many Echo parameters have proven to be prognostic in PAH, including right heart reverse remodeling (RHRR), a recently identified key parameter in evaluating a patient’s response to PAH therapy
  • Adding Echo parameters to current risk assessment tools could allow for improved prognostication and optimized therapeutic management
Current Clinical Utilization of Risk Assessment Tools in Pulmonary Arterial Hypertension: A Descriptive Survey article thumbnail

Current Clinical Utilization of Risk Assessment Tools in Pulmonary Arterial Hypertension: A Descriptive SurveyWilson M, Keeley J, Kingman M, Wang J, Rogers F.
Pulm Circ. 2020; 10(3):2045894020950186.

Study Design

  • In 2019, 121 treatment decision makers of the PHA providers’ network were surveyed on their use of risk assessment tools

Key Insights

  • 59% of treatment decision makers reported using formal risk assessment tools
  • Risk tools were most commonly used at the time of diagnosis (54%) and of worsening symptoms (42%)
  • Fewer than 1 in 5 used formal risk tools at all of the following: the time of diagnosis, worsening symptoms, changing medications, repeat Echo, and repeat RHC

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CO=cardiac output; Echo=echocardiogram; ERA=endothelin receptor antagonist; ESC/ERS=European Society of Cardiology/European Respiratory Society; FC=Functional Class; HRQoL=health-related quality of life; PAH=pulmonary arterial hypertension; PDE-5i=phosphodiesterase-5 inhibitor; PHA=Pulmonary Hypertension Association; PVR=pulmonary vascular resistance; RHC=right heart catheterization; RV=right ventricle.