BACKGROUND: There are very few validated prognostic markers in pediatric pulmonary hypertension. Cardiac MRI is a useful, noninvasive method for determining prognosis in adults. The present study is the first to assess its prognostic value in children. METHODS AND RESULTS: A total of 100 children with pulmonary hypertension (median, 10.4 years; range, 0.5-17.6 years) were evaluated (idiopathic, n=60; repaired congenital heart disease, n=22; miscellaneous, n=18). In all patients, ventricular volumes and great vessel flow were measured. Volumetric data were obtained using retrospectively gated cine imaging (n=37) or real-time imaging (n=63), depending on the patient's ability to hold his or her breath. During a median follow-up of 1.9 years, 11 patients died and 3 received lung transplantation. Of the cardiac MR parameters measured, right ventricular ejection fraction and left ventricular stroke volume index were most strongly predictive of survival on univariate analysis (2.6- and 2.5-fold increase in mortality for every 1-SD decrease, respectively; P<0.05). These results were reflected in good separation of tertile-based Kaplan-Meier survival curves for these variables. CONCLUSIONS: Cardiac MR measures correlate with clinical status and prognosis in children with pulmonary hypertension. Cardiac MR is feasible and may be useful in clinical decision making in pediatric pulmonary hypertension.
BACKGROUND: There are very few validated prognostic markers in pediatric pulmonary hypertension. Cardiac MRI is a useful, noninvasive method for determining prognosis in adults. The present study is the first to assess its prognostic value in children. METHODS AND RESULTS: A total of 100 children with pulmonary hypertension (median, 10.4 years; range, 0.5-17.6 years) were evaluated (idiopathic, n=60; repaired congenital heart disease, n=22; miscellaneous, n=18). In all patients, ventricular volumes and great vessel flow were measured. Volumetric data were obtained using retrospectively gated cine imaging (n=37) or real-time imaging (n=63), depending on the patient's ability to hold his or her breath. During a median follow-up of 1.9 years, 11 patients died and 3 received lung transplantation. Of the cardiac MR parameters measured, right ventricular ejection fraction and left ventricular stroke volume index were most strongly predictive of survival on univariate analysis (2.6- and 2.5-fold increase in mortality for every 1-SD decrease, respectively; P<0.05). These results were reflected in good separation of tertile-based Kaplan-Meier survival curves for these variables. CONCLUSIONS: Cardiac MR measures correlate with clinical status and prognosis in children with pulmonary hypertension. Cardiac MR is feasible and may be useful in clinical decision making in pediatric pulmonary hypertension.
Authors: Michal Schäfer; D Dunbar Ivy; Steven H Abman; Alex J Barker; Lorna P Browne; Brian Fonseca; Vitaly Kheyfets; Kendall S Hunter; Uyen Truong Journal: Circ Cardiovasc Imaging Date: 2017-02 Impact factor: 7.792
Authors: Martin Koestenberger; Mark K Friedberg; Eirik Nestaas; Ina Michel-Behnke; Georg Hansmann Journal: Pulm Circ Date: 2016-03 Impact factor: 3.017
Authors: Richard M Friesen; Michal Schäfer; D Dunbar Ivy; Steven H Abman; Kurt Stenmark; Lorna P Browne; Alex J Barker; Kendall S Hunter; Uyen Truong Journal: Eur Heart J Cardiovasc Imaging Date: 2019-02-01 Impact factor: 6.875
Authors: K T N Breeman; M Dufva; M J Ploegstra; V Kheyfets; T P Willems; J Wigger; K S Hunter; D D Ivy; R M F Berger; U Truong Journal: Int J Cardiol Date: 2019-05-10 Impact factor: 4.164
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: Melanie J Brewis; Alessandro Bellofiore; Rebecca R Vanderpool; Naomi C Chesler; Martin K Johnson; Robert Naeije; Andrew J Peacock Journal: Int J Cardiol Date: 2016-05-13 Impact factor: 4.164