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Right Atrial Deformation in Predicting Outcomes in Pediatric Pulmonary Hypertension
Pei-Ni Jone1, Michal Schäfer2, Ling Li2
1From the Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora (P.-N.J., M.S., D.D.I.); and Pediatric Cardiology, Children's Hospital and Medical Center, University of Nebraska, Omaha (L.L., M.C., S.K.). pei-ni.jone@childrenscolorado.org.
Insights
Right atrial (RA) function is impaired in pediatric pulmonary hypertension (PH) patients, with altered deformation properties impacting outcomes. RA reservoir and pump function predict clinical outcomes in pediatric PH.
Area of Science:
- Cardiology
- Pediatric Medicine
- Medical Imaging
Background:
- Elevated right atrial (RA) pressure is a mortality risk factor in pulmonary hypertension (PH).
- RA size is prognostic of adverse outcomes in PH.
- Limited data exists on phasic RA function (reservoir, conduit, pump) in pediatric PH.
Purpose of the Study:
- Evaluate RA function in pediatric PH patients compared to controls.
- Correlate RA deformation indices with Doppler indices of diastolic dysfunction, functional capacity, biomarkers, hemodynamics, and right ventricular function.
- Assess the predictive value of RA deformation indices for clinical outcomes in pediatric PH.
Main Methods:
- Utilized 2-dimensional speckle tracking (2DCPA) to assess RA and right ventricular deformation indices in 66 PH patients and 36 controls.
- Measured RA strain, strain rates, emptying fraction, and right ventricular longitudinal strain.
- Analyzed associations between RA function, invasive hemodynamics, and clinical outcomes.
Main Results:
- RA function was significantly impaired in pediatric PH patients compared to controls (P<0.001).
- Significant associations were found between RA function and invasive hemodynamics (P<0.01).
- RA reservoir function, pump function, rate of RA filling, and minimum atrial volume predicted adverse clinical outcomes (P<0.01).
Conclusions:
- Pediatric PH patients exhibit significantly altered RA deformation properties.
- Worsening RA reservoir and conduit functions correlate with right ventricular diastolic dysfunction.
- RA reservoir function, pump function, rate of atrial filling, and minimum atrial volume are significant outcome predictors in pediatric PH.
Background:
Elevated right atrial (RA) pressure is a risk factor for mortality, and RA size is prognostic of adverse outcomes in pulmonary hypertension (PH). There is limited data on phasic RA function (reservoir, conduit, and pump) in pediatric PH. We sought to evaluate (1) the RA function in pediatric PH patients compared with controls, (2) compare the RA deformation indices with Doppler indices of diastolic dysfunction, functional capacity, biomarkers, invasive hemodynamics, and right ventricular functional indices, and (3) evaluate the potential of RA deformation indices to predict clinical outcomes.
Methods And Results:
Sixty-six PH patients (mean age 7.9±4.7 years) were compared with 36 controls (7.7±4.4 years). RA and right ventricular deformation indices were obtained using 2-dimensional speckle tracking (2DCPA; TomTec, Germany). RA strain, strain rates, emptying fraction, and right ventricular longitudinal strain were measured. RA function was impaired in PH patients versus controls (P<0.001). There were significant associations between RA function with invasive hemodynamics (P<0.01). RA reservoir, pump function, the rate of RA filling, and atrial minimum volume predicted adverse clinical outcomes (hazard ratio [HR], 0.15; confidence interval [CI], 0.03-0.73; P<0.01; HR, 0.05; CI, 0.003-0.43; P<0.004; HR, 0.04; CI, 0.006-0.56; P<0.01; and HR, 8.6; CI, 1.6-37.2; P<0.01, respectively).
Conclusions:
RA deformation properties are significantly altered in pediatric PH patients. Progressive worsening of RA reservoir and conduit functions is related to changes in right ventricular diastolic dysfunction. RA reservoir function, pump function, the rate of atrial filling, and atrial minimum volume emerged as outcome predictors in pediatric PH.

