• Jana Schlangen, Colin Petko, Jan H Hansen, Miriam Michel, Christopher Hart, Anselm Uebing, Gunther Fischer, Kolja Becker, and Hans-Heiner Kramer.
• From the Department for Congenital Heart Disease and Paediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany (J.S., C.P., J.H.H., M.M., C.H., G.F., K.B., H.-H.K.); Department of Pediatric, Fetal and Congenital Cardiology, Hawaii Permanente Medical Group, Kaiser Permanente Moanalua Medical Center, Honolulu, HI (C.P.); and Adult Congenital Heart Disease Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom (A.U.). jana.schlangen@pedcard.uni-kiel.de.
• Circ Cardiovasc Imaging. 2014 Nov 1; 7 (6): 880-6.

BackgroundAssessment of systemic right ventricular (RV) function in patients with hypoplastic left heart syndrome is important during long-term follow-up after Fontan repair. Traditional echocardiographic parameters to evaluate systolic ventricular function are affected by loading conditions. The only generally accepted load-independent parameter of systolic function, end systolic elastance (Ees), requires invasive catheterization. Therefore, we sought to determine if parameters obtained by 2-dimensional speckle tracking (2DST) were affected by acute changes in preload and correlated with catheterization-derived indices of RV contractility in hypoplastic left heart syndrome patients after Fontan palliation.Methods And ResultsFifty-two patients with hypoplastic left heart syndrome (median age, 6.6; range 2.9-22.2 years) were prospectively enrolled to have echocardiography and conductance catheter studies performed simultaneously. We compared traditional echo, 2-dimensional speckle tracking and catheterization-derived parameters during different states of preload at baseline and during dobutamine infusion. Global longitudinal strain (S) showed a tendency to decrease with preload reduction, whereas global longitudinal strain rate (SR) did not change (S: -17.7 ± 3.4% versus -16.9 ± 3.8%, P=0.08; SR: -1.30 ± 0.29 versus -1.34 ± 0.34 s(-1), P=0.3). S did not change with dobutamine infusion (-17.7 ± 3.4% versus -18.4 ± 3.9%, P=0.24), whereas SR increased significantly (-1.30 ± 0.29 versus -2.26 ± 0.49 s(-1), P<0.001). RV Ees correlated with SR (rs= -0.47, P<0.001), but not with S (rs=0.07, P=0.5) or other echocardiographic parameters.ConclusionsIn contrast to S, SR was not affected by preload and correlated with Ees of the systemic RV. SR may be a useful noninvasive surrogate of RV contractility and suitable for follow-up of patients with hypoplastic left heart syndrome after Fontan palliation.© 2014 American Heart Association, Inc.

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