Michal Schäfer1, Kathryn K Collins2, Lorna P Browne3, D Dunbar Ivy2, Steven Abman4, Richard Friesen2, Benjamin Frank2, Brian Fonseca2, Michael DiMaria2, Kendall S Hunter2, Uyen Truong2, Johannes C von Alvensleben2. 1. Division of Cardiology, Heart Institute, Children's Hospital Colorado, Anschutz Medical Campus, University of Colorado Denver, Aurora, Colorado. Electronic address: michal.schafer@ucdenver.edu. 2. Division of Cardiology, Heart Institute, Children's Hospital Colorado, Anschutz Medical Campus, University of Colorado Denver, Aurora, Colorado. 3. Department of Radiology, Breathing Institute, Children's Hospital Colorado, Anschutz Medical Campus, University of Colorado Denver, Aurora, Colorado. 4. Division of Pulmonology, Breathing Institute, Children's Hospital Colorado, Anschutz Medical Campus, University of Colorado Denver, Aurora, Colorado.
Abstract
BACKGROUND: Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony. METHODS: Fifty-six children with PAH and comprehensive CMR evaluation were stratified based on QRS duration z-score, with electrical dyssynchrony defined as z-score ≥2. Comprehensive biventricular volumetric, dyssynchrony, and strain analysis was performed. RESULTS: Nineteen PAH patients had or developed electrical dyssynchrony. Patients with electrical dyssynchrony had significantly reduced RV ejection fraction (35% vs 50%, p = 0.003) and greater end-diastolic (168 vs 112 ml/m2, p = 0.041) and end-systolic (119 vs 57, ml/m2, p = 0.026) volumes. Patients with electrical dyssynchrony had reduced RV longitudinal strain (-14% vs -19%, p = 0.007), LV circumferential strain measured at the free wall (-19% vs -22%, p = 0.047), and the LV longitudinal strain in the septal region (-10% vs -15%, p = 0.0268). LV mechanical intraventricular dyssynchrony was reduced in patients with electrical dyssynchrony at the LV free wall (43 vs 19 ms, p = 0.019). CONCLUSIONS: The electrical dyssynchrony is associated with the reduced LV strain, enlarged RV volumes, and reduced biventricular function in children with PAH. CMR assessment of biventricular mechanical function with respect to QRS duration may help to detect pathophysiologic processes associated with progressed PAH.
BACKGROUND: Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony. METHODS: Fifty-six children with PAH and comprehensive CMR evaluation were stratified based on QRS duration z-score, with electrical dyssynchrony defined as z-score ≥2. Comprehensive biventricular volumetric, dyssynchrony, and strain analysis was performed. RESULTS: Nineteen PAH patients had or developed electrical dyssynchrony. Patients with electrical dyssynchrony had significantly reduced RV ejection fraction (35% vs 50%, p = 0.003) and greater end-diastolic (168 vs 112 ml/m2, p = 0.041) and end-systolic (119 vs 57, ml/m2, p = 0.026) volumes. Patients with electrical dyssynchrony had reduced RV longitudinal strain (-14% vs -19%, p = 0.007), LV circumferential strain measured at the free wall (-19% vs -22%, p = 0.047), and the LV longitudinal strain in the septal region (-10% vs -15%, p = 0.0268). LV mechanical intraventricular dyssynchrony was reduced in patients with electrical dyssynchrony at the LV free wall (43 vs 19 ms, p = 0.019). CONCLUSIONS: The electrical dyssynchrony is associated with the reduced LV strain, enlarged RV volumes, and reduced biventricular function in children with PAH. CMR assessment of biventricular mechanical function with respect to QRS duration may help to detect pathophysiologic processes associated with progressed PAH.
Authors: Benjamin S Frank; Michal Schäfer; Johannes M Douwes; D Dunbar Ivy; Steven H Abman; Jesse A Davidson; Sandra Burzlaff; Max B Mitchell; Gareth J Morgan; Lorna P Browne; Alex J Barker; Uyen Truong; Johannes C von Alvensleben Journal: Am J Physiol Heart Circ Physiol Date: 2019-12-20 Impact factor: 4.733
Authors: Michal Schäfer; Petter Bjornstad; Benjamin S Frank; Amy Baumgartner; Uyen Truong; Daniel Enge; Johannes C von Alvensleben; Max B Mitchell; D Dunbar Ivy; Alex J Barker; Jane E B Reusch; Kristen J Nadeau Journal: Am J Cardiol Date: 2020-05-13 Impact factor: 2.778
Authors: Michal Schäfer; Alex J Barker; Gareth J Morgan; James Jaggers; Matthew L Stone; Lorna P Browne; D Dunbar Ivy; Max B Mitchell Journal: Int J Cardiovasc Imaging Date: 2020-01-06 Impact factor: 2.357
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
Authors: Michal Schäfer; Benjamin S Frank; D Dunbar Ivy; Max B Mitchell; Kathryn K Collins; Pei-Ni Jone; Johannes C von Alvensleben Journal: J Am Heart Assoc Date: 2022-03-01 Impact factor: 6.106
Authors: Sabrina Schweintzger; Stefan Kurath-Koller; Ante Burmas; Gernot Grangl; Andrea Fandl; Nathalie Noessler; Alexander Avian; Andreas Gamillscheg; Philippe Chouvarine; Georg Hansmann; Martin Koestenberger Journal: Front Cardiovasc Med Date: 2022-07-14