Improved Risk Prediction Using a Refined European Guidelines Instrument in Pulmonary Arterial Hypertension Related to...

Area of Science:

• Cardiology
• Pulmonary Hypertension
• Congenital Heart Disease

Background:

• European guidelines recommend goal-oriented treatment for pulmonary arterial hypertension (PAH) using validated risk assessment instruments.
• The general PAH risk instrument's discriminatory ability in adult congenital heart disease (CHD) PAH populations requires investigation.
• Tricuspid annular plane systolic excursion (TAPSE) and revised thresholds may enhance risk prediction in PAH-CHD.

Purpose of the Study:

• To evaluate the general PAH risk instrument's discriminatory ability in adult PAH-CHD patients.
• To explore the utility of TAPSE and revised thresholds for improving risk stratification in this population.
• To propose a refined risk instrument for PAH-CHD.

Main Methods:

• A cohort of 223 adult PAH-CHD patients from 5 European centers were assessed at baseline and follow-up (4-18 months).
• Patients were initially classified using the general PAH guidelines instrument.
• Revised thresholds for N-terminal pro-brain natriuretic peptide, 6-minute walk distance, and TAPSE were applied for reclassification.

Main Results:

• The general PAH instrument showed limited discriminatory value, with no significant survival difference between risk groups.
• Revised thresholds and TAPSE significantly improved risk discrimination (ROC increase from 0.648 to 0.701, p=0.001), reclassifying 29% of patients.
• Higher proportions of low-risk variables and improvement to a low-risk profile at follow-up correlated with better survival.

Conclusions:

• The external validity of the general PAH risk instrument is limited in the PAH-CHD population.
• A refined risk instrument incorporating TAPSE and adjusted thresholds demonstrates improved discrimination for PAH-CHD.
• This refined tool can aid in more accurate risk stratification and potentially improve outcomes for PAH-CHD patients.