Echocardiographic Assessment Shows Promise as Risk Predictor in PAH

Over a 10-year period of hospitalizations at the Johns Hopkins Hospital medical intensive care unit (ICU), patients who were also enrolled in the Johns Hopkins Pulmonary Hypertension Registry because of their pulmonary arterial hypertension (PAH) exhibited a significant risk of death (26%). The 1-year mortality rate was even higher in those who were discharged alive (38.6%).

These retrospective analysis results, published in JACC Advances,1 had the investigators conclude that comprehensive echocardiographic assessment using right heart metrics can be an extremely valuable risk-prediction tool for critically ill patients living with PAH, a population considered at high risk of adverse survival outcomes. Their study years were January 2010 through December 2020.

This overall study population comprised 102 patients, and of this group, 77 had adequate echocardiographic data; the most common subtype of PAH was connective tissue disease–associated PAH (n = 45), followed by idiopathic PAH (n = 20), and other (n = 12). There were Diagnosis was confirmed using the following: mean pulmonary arterial pressure above 20 mm Hg at rest, maximum pulmonary capillary wedge pressure of 15 mm Hg, and pulmonary vascular resistance above 2 WU, all according to 2022 European Society of Cardiology/European Respiratory Society 2022 guidelines.2

Patients who died in hospital were slightly older vs those with info on 1-year mortality (58 vs 56 years), most nonsurvivors were White (67% and 75%, respectively) or female (86% and 80%). World Health Organization class III disease was the most common disease class (48% and 55% of survivors and nonsurvivors, respectively, in those with info on 1-year mortality and 51% and 55% of the in-hospital mortality cohort). At baseline, 11% to 35% had a history of invasive ventilation (P = .04), and 65% to 75% had a history of noninvasive ventilation at baseline (P = .29). Also, tricuspid regurgitation was typically moderate or severe in the 1-year mortality group (P = .047) and the in-hospital mortality group (P = .08). Median (IQR) follow-up was 365 days (328.5-365) in the 1-year mortality survivors, 30.1 days (6-91.8) in the 1-year mortality nonsurvivors, 6 days (3-8) in the in-hospital mortality survivors, and 3.5 days (2-10.5) in the in-hospital mortality nonsurvivors.

Close to 40% of patients with right heart failure and pulmonary arterial hypertension (PAH) who remain critically ill and require admittance to the intensive care unit die within 1 year of that hospitalization. | Image Credit: © Pepermpron-stock.adobe.com

Overall, nonsurvivors vs survivors with 1-year mortality exhibited the following:

• Worsened tricuspid regurgitation (P = .047
• Lower left ventricular end-diastolic volume (LVEDV): 47 vs 61 mL (P = .01)
• Significantly reduced tricuspid annular plane systolic excursion (TAPSE): 11 vs 17 mm (P < .001)
• Impaired global right ventricular (RV) free wall strain (RVFWSglobal): –14% vs –11% (P = .01)
• Reduced right atrium (RA) reservoir: 15% vs 20% (P = .045)

In-hospital nonsurvivors vs survivors exhibited the following:

• Worse RV systolic function with lower TAPSE: 11 vs 15 mm (P = .01)
• Greater RVFWSglobal: –13% vs –10% (P = .01)
• Reduced RA reservoir: 13% vs 19% (P = .01)
• Reduced RA booster strain: –11%% vs –6% (P = .01)
• Reduced RV/pulmonary artery systolic pressure (PASP): 0.12 vs 0.20 mm Hg (P = .03)

Cox regression adjusted and unadjusted also identified each 1 mm increase in TAPSE and greater LVEDV as reducing mortality risk (HR, 0.87 and 0.98, respectively), and RVFWSglobal and RA conduit strain as increasing mortality (HR, 1.17 and 1.19, respectively). Further, each 1 mm increase in TAPSE remained significantly protective (P = .003), RVFWSglobal /PASP demonstrated a robust protective association (P = .02), while RVFWSglobal had an independent association with greater 1-year mortality (P = .046).

Lastly, the study authors incorporated machine learning to identify nonlinear predictors of outcomes, and they found that RV dysfunction and uncoupling, LV diastolic dysfunction and ventricular interdependence, and abnormal myocardial mechanics were the top predictors of 1-year mortality.

“Comparative analyses between survivors and nonsurvivors at both 1-year and in-hospital time points revealed distinct echocardiographic and clinical profiles,” they explained. “In-hospital nonsurvivors demonstrated more pronounced hemodynamic instability…reinforcing the prognostic significance of both ventricular and atrial performance during acute decompensation.”

Altogether, their findings, they continued, “highlight the central role of echocardiographic markers, in prognosticating outcomes among critically ill patients with PAH.”

However, there are limitations on these results. The sample size was small, the single-center analysis lacked external validation, echocardiographic variable measures were not repeated during hospitalization, and clinical advancements during the study period may have affected observed associations.

References

1. Mombeini H, Jani VP, Malhi J, et al. Advanced echocardiographic assessment predicts 1-year mortality in critically ill patients with pulmonary arterial hypertension. JACC Adv. 2025:102080. doi:10.1016/j.jacadv.2025.102080
2. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237.