Pulmonary Arterial Hypertension Risk Assessment

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Questions and Answers

What is the focus of the gestalt risk assessment in PAH patients?

Determining the effectiveness of treatment options

Evaluating the patient's age and medical history

Calculating the risk of pulmonary complications

Assessing the potential for clinical worsening or death (correct)

Which of the following variables is NOT used in the calculated risk assessment for 1-year mortality?

Blood Pressure Level (correct)

Cardiac Index (CI)

6-Minute Walk Distance (6MWD)

WHO Functional Class (WHO FC)

How many variables are used to calculate the risk of 1-year mortality in PAH patients?

6 (correct)

Which of the following is included in the calculated risk assessment for PAH?

N-terminal pro b-type natriuretic peptide (BNP) Signup and view all the answers

What does RAP stand for in the context of risk assessment for PAH?

Right Atrial Pressure Signup and view all the answers

What is the BUN level reported in the basic metabolic panel?

16 mg/dL Signup and view all the answers

Which test result indicates a normal bicarbonate level?

27 mEq/L Signup and view all the answers

What is the significance of a negative pulmonary vasoreactivity test?

It indicates no improvement with vasodilators. Signup and view all the answers

What CI value is reported in the results?

2.8 L/min/m2 Signup and view all the answers

Which tool should be used to assess risk at diagnosis for pulmonary arterial hypertension?

REVEAL 2.0 Signup and view all the answers

What is the reported PAWP (pulmonary arterial wedge pressure)?

12 mm Hg Signup and view all the answers

Which electrolyte level is reported as 4.5 mmol/L?

Potassium Signup and view all the answers

What is the mean PAP (pulmonary artery pressure) reported?

40 mm Hg Signup and view all the answers

What is the classification of Max's score in the 3-Strata Risk Model?

Intermediate Signup and view all the answers

What is the primary purpose of the COMPERA 3-Strata Risk Model?

To assess risk in patients with PAH Signup and view all the answers

Which year was the Comprehensive PAH Risk Assessment: Three-Strata Model published?

2022 Signup and view all the answers

Who are the authors of the Comprehensive PAH Risk Assessment: Three-Strata Model?

Humbert M, et al. Signup and view all the answers

Which score indicates a low-risk classification in the 3-Strata Risk Model?

2 points Signup and view all the answers

What type of document is the 2022 ESC/ERS PH Guidelines considered?

Clinical guideline Signup and view all the answers

What is the significant restriction mentioned regarding the provided materials?

They are strictly for personal use. Signup and view all the answers

Which future publication is referenced in relation to treatment algorithms for PAH?

Eur Respir J. 2024 Signup and view all the answers

What is the frequency of the medication Furosemide?

Daily Signup and view all the answers

Which medication was started most recently?

Tadalafil Signup and view all the answers

How many medications were started 4 months ago?

Two Signup and view all the answers

What is the dosage of Macitentan prescribed?

10 mg Signup and view all the answers

Which of the following medications is taken daily?

Furosemide Signup and view all the answers

What medication was started alongside Macitentan?

Tadalafil Signup and view all the answers

What is the prescribed frequency for Losartan?

Daily Signup and view all the answers

How long has Tadalafil been prescribed?

3 months Signup and view all the answers

What is the primary indication for Sotatercept SC Injection?

Treatment of adults with PAH Signup and view all the answers

When was Sotatercept SC Injection FDA approved?

March 2024 Signup and view all the answers

Which of the following outcomes does Sotatercept aim to improve in PAH patients?

Increase exercise capacity Signup and view all the answers

Which combination therapy is mentioned for the treatment of PAH?

Macitentan/Tadalafil Signup and view all the answers

What is the primary purpose of the materials mentioned in the content?

For educational resource only Signup and view all the answers

Study Notes

### Patient History

Patient presented 3 months ago for a PH center test.
Patient's lab results revealed normal renal function, normal electrolytes, a normal blood glucose level.
The right heart catheterization (RHC) results indicate mild pulmonary hypertension (PH) with a mean pulmonary artery pressure (PAP) of 40 mm Hg.
The pulmonary vascular resistance (PVR) is elevated at 5.5 Wood units (WU).
The pulmonary vasoreactivity test was negative, indicating the patient has PH that is not reversible with vasodilators.

### Assessment of Risk for PAH Progression

There are various tools used to assess risk of disease progression in PAH.
Gestalt risk is a qualitative assessment of risk based on a clinician's overall impression of the patient.
Calculated risk uses specific lab and clinical variables to assess risk and is more objective.
The COMPERA 3-Strata Risk Model stratifies risk into three levels (low, intermediate, and high) by using specific scores.
ESC/ERS 4-Strata model is widely used to define risk in PH, however, it has been recommended to use the newer 3-Strata model.
For comprehensive risk assessment, the 2022 ESC/ERS PH Guidelines recommend a Three-Strata Model which considers multiple factors such as clinical presentation, functional class, hemodynamics, and biomarker levels.

Treatment and Medications

The patient is currently on a combination of medications for PAH, including:
- Furosemide (diuretic)
- Losartan (angiotensin II receptor blocker)
- Macitentan (endothelin receptor antagonist)
- Tadalafil (phosphodiesterase type 5 inhibitor)

### Tests

There are several clinical tests used for the diagnosis, evaluation, and treatment of PAH.
The most common tests include right heart catheterization (RHC), 6-minute walk test (6MWT), echocardiogram, and brain natriuretic peptide (BNP) levels.

### New Treatments for PAH

Sotatercept is a novel treatment for PAH approved by the FDA in March 2024.
Sotatercept is classified as a soluble activin receptor IIA ligand trap.
It has been proven to improve exercise capacity, improve WHO functional class (WHO FC), and reduce the risk of clinical worsening events in patients with PAH.
Macitentan/Tadalafil Single-Tablet Combination Therapy is also available for the treatment of PAH. This combination therapy was studied in the DUE trial and may provide further beneficial effects for patients with PAH.

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Description

This quiz focuses on the assessment of risk for pulmonary arterial hypertension (PAH) progression using different models and tools. It covers patient history, lab results, and the significance of gestalts vs. calculated risks. Test your knowledge on evaluating and stratifying the risk for PAH.