Pei-Ni Jone1, Michal Schäfer2, Ling Li2, Mary Craft2, D Dunbar Ivy2, Shelby Kutty2. 1. From 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. 2. From 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.).
Abstract
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.
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.
Authors: Wei Hui; Mohamed Y Abd El Rahman; Fatima Dsebissowa; Axel Rentzsch; Matthias Gutberlet; Vladimir Alexi-Meskishvili; Roland Hetzer; Peter E Lange; Hashim Abdul-Khaliq Journal: Cardiol Young Date: 2004-10 Impact factor: 1.093
Authors: Mark-Jan Ploegstra; Marcus T R Roofthooft; Johannes M Douwes; Beatrijs Bartelds; Nynke J Elzenga; Dick van de Weerd; Hans L Hillege; Rolf M F Berger Journal: Circ Cardiovasc Imaging Date: 2014-12-31 Impact factor: 7.792
Authors: Mark-Jan Ploegstra; Willemijn M H Zijlstra; Johannes M Douwes; Hans L Hillege; Rolf M F Berger Journal: Int J Cardiol Date: 2015-01-27 Impact factor: 4.164
Authors: Ronald J Raymond; Alan L Hinderliter; Park W Willis; David Ralph; Edgar J Caldwell; William Williams; Neil A Ettinger; Nicholas S Hill; Warren R Summer; Bennett de Boisblanc; Todd Schwartz; Gary Koch; Linda M Clayton; Maria M Jöbsis; James W Crow; Walker Long Journal: J Am Coll Cardiol Date: 2002-04-03 Impact factor: 24.094
Authors: Michal Schäfer; D Dunbar Ivy; Steven H Abman; Kurt Stenmark; Lorna P Browne; Alex J Barker; Max B Mitchell; Gareth J Morgan; Neil Wilson; Anar Shah; Madhukar Kollengode; Nivedita Naresh; Brian Fonseca; Michael DiMaria; J Kern Buckner; Kendall S Hunter; Vitaly Kheyfets; Brett E Fenster; Uyen Truong Journal: Am J Physiol Heart Circ Physiol Date: 2019-03-01 Impact factor: 4.733
Authors: David G Kiely; David Levin; Paul Hassoun; David D Ivy; Pei-Ni Jone; Jumaa Bwika; Steven M Kawut; Jim Lordan; Angela Lungu; Jeremy Mazurek; Shahin Moledina; Horst Olschewski; Andrew Peacock; Goverdhan Dutt Puri; Farbod Rahaghi; Michal Schafer; Mark Schiebler; Nicholas Screaton; Merryn Tawhai; Edwin Jr Van Beek; Anton Vonk-Noordegraaf; Rebecca R Vanderpool; John Wort; Lan Zhao; Jim Wild; Jens Vogel-Claussen; Andrew J Swift Journal: Pulm Circ Date: 2019-03-18 Impact factor: 3.017