Skip to main content
Erschienen in: Pediatric Cardiology 4/2019

06.02.2019 | Original Article

Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein’s Anomaly

verfasst von: Christian Meierhofer, Andreas Kühn, Jan Müller, Nerejda Shehu, Alfred Hager, Stefan Martinoff, Heiko Stern, Peter Ewert, Manfred Vogt

Erschienen in: Pediatric Cardiology | Ausgabe 4/2019

Einloggen, um Zugang zu erhalten

Abstract

Background

Exercise capacity is a well-defined marker of outcome in congenital heart disease. We analyzed seventeen cardiovascular magnetic resonance (CMR) derived parameters and their correlation to exercise capacity in patients with Ebstein’s anomaly (EA).

Methods

Fifty-four surgery free patients, age 5 to 69 years (median 30 years) prospectively underwent CMR examination and cardiopulmonary exercise testing (CPET). The following volume/flow parameters were compared with peak oxygen uptake as the percentage of normal (peakVO2%) using univariate and multivariate analysis: right and left ventricular ejection fraction (RVEF and LVEF), the indexed end-diastolic and end-systolic volumes (RVEDVi, RVESVi, LVEDVi, and LVESVi), the indexed stroke volumes (RVSVi and LVSVi), the total normalized right and left heart volumes; the total right to left heart volume ratio (R/L-ratio). The indexed antegrade flow (ante), indexed net flow (net) as well as cardiac index (CI) in the aorta (Ao) and pulmonary artery (PA) were used.

Results

RVEF (R2 0.2788), indexed flow PA net (R2 0.2330), and PA ante (R2 0.1912) showed the best correlation with peakVO2% (all p < 0.001) in the univariate model. Further significant correlation could also be demonstrated with CI-PA, LVEF, LVSVi, Aorta net, RVESVi, and Aorta ante. Multivariate analysis for RVEF and indexed net flow PA revealed a R2 of 0.4350.

Conclusion

Functional CMR parameters as RVEF and LVEF and flow data of cardiac forward flow correlate to peakVO2%. Evaluation of the indexed net flow in the pulmonary artery and the overall function of the right ventricle best predicts the maximal exercise capacity in patients with EA.
Literatur
1.
Zurück zum Zitat Fratz S, Janello C, Muller D et al (2013) The functional right ventricle and tricuspid regurgitation in Ebstein’s anomaly. Int J Cardiol 167:258–261CrossRefPubMed Fratz S, Janello C, Muller D et al (2013) The functional right ventricle and tricuspid regurgitation in Ebstein’s anomaly. Int J Cardiol 167:258–261CrossRefPubMed
2.
Zurück zum Zitat Attenhofer Jost CH, Connolly HM, Dearani JA et al (2007) Ebstein’s anomaly. Circulation 115:277–285CrossRefPubMed Attenhofer Jost CH, Connolly HM, Dearani JA et al (2007) Ebstein’s anomaly. Circulation 115:277–285CrossRefPubMed
3.
Zurück zum Zitat Kilner PJ, Geva T, Kaemmerer H et al (2010) Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology. Eur Heart J 31:794–805CrossRefPubMedPubMedCentral Kilner PJ, Geva T, Kaemmerer H et al (2010) Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology. Eur Heart J 31:794–805CrossRefPubMedPubMedCentral
4.
Zurück zum Zitat Tobler D, Yalonetsky S, Crean AM et al (2013) Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol 165:146–150CrossRefPubMed Tobler D, Yalonetsky S, Crean AM et al (2013) Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol 165:146–150CrossRefPubMed
5.
Zurück zum Zitat Lee CM, Sheehan FH, Bouzas B et al (2013) The shape and function of the right ventricle in Ebstein’s anomaly. Int J Cardiol 167:704–710CrossRefPubMed Lee CM, Sheehan FH, Bouzas B et al (2013) The shape and function of the right ventricle in Ebstein’s anomaly. Int J Cardiol 167:704–710CrossRefPubMed
6.
Zurück zum Zitat Gutberlet M, Oellinger H, Ewert P et al (2000) Pre- and postoperative evaluation of ventricular function, muscle mass and valve morphology by magnetic resonance tomography in Ebstein’s anomaly. Rofo 172:436–442CrossRefPubMed Gutberlet M, Oellinger H, Ewert P et al (2000) Pre- and postoperative evaluation of ventricular function, muscle mass and valve morphology by magnetic resonance tomography in Ebstein’s anomaly. Rofo 172:436–442CrossRefPubMed
7.
Zurück zum Zitat Kempny A, Dimopoulos K, Uebing A et al (2012) Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life—single centre experience and review of published data. Eur Heart J 33:1386–1396CrossRefPubMed Kempny A, Dimopoulos K, Uebing A et al (2012) Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life—single centre experience and review of published data. Eur Heart J 33:1386–1396CrossRefPubMed
8.
Zurück zum Zitat Mueller GC, Sarikouch S, Beerbaum P et al (2013) Health-related quality of life compared with cardiopulmonary exercise testing at the midterm follow-up visit after tetralogy of Fallot repair: a study of the German competence network for congenital heart defects. Pediatr Cardiol 34:1081–1087CrossRefPubMed Mueller GC, Sarikouch S, Beerbaum P et al (2013) Health-related quality of life compared with cardiopulmonary exercise testing at the midterm follow-up visit after tetralogy of Fallot repair: a study of the German competence network for congenital heart defects. Pediatr Cardiol 34:1081–1087CrossRefPubMed
9.
Zurück zum Zitat Muller J, Hager A, Diller GP et al (2015) Peak oxygen uptake, ventilatory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol 196:158–164CrossRefPubMed Muller J, Hager A, Diller GP et al (2015) Peak oxygen uptake, ventilatory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol 196:158–164CrossRefPubMed
10.
Zurück zum Zitat Radojevic J, Inuzuka R, Alonso-Gonzalez R et al (2013) Peak oxygen uptake correlates with disease severity and predicts outcome in adult patients with Ebstein’s anomaly of the tricuspid valve. Int J Cardiol 163:305–308CrossRefPubMed Radojevic J, Inuzuka R, Alonso-Gonzalez R et al (2013) Peak oxygen uptake correlates with disease severity and predicts outcome in adult patients with Ebstein’s anomaly of the tricuspid valve. Int J Cardiol 163:305–308CrossRefPubMed
11.
Zurück zum Zitat Kuhn A, Meierhofer C, Rutz T et al (2015) Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein’s anomaly: comparison with CMR-derived ejection fraction in 49 patients. Eur Heart J Cardiovasc Imaging 17:930–935 Kuhn A, Meierhofer C, Rutz T et al (2015) Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein’s anomaly: comparison with CMR-derived ejection fraction in 49 patients. Eur Heart J Cardiovasc Imaging 17:930–935
12.
Zurück zum Zitat Goleski PJ, Sheehan FH, Chen SS et al (2014) The shape and function of the left ventricle in Ebstein’s anomaly. Int J Cardiol 171:404–412CrossRefPubMed Goleski PJ, Sheehan FH, Chen SS et al (2014) The shape and function of the left ventricle in Ebstein’s anomaly. Int J Cardiol 171:404–412CrossRefPubMed
13.
Zurück zum Zitat Muller J, Heck PB, Ewert P et al (2016) Noninvasive screening for pulmonary hypertension by exercise testing in congenital heart disease. Ann Thorac Surg 103:1544–1549 Muller J, Heck PB, Ewert P et al (2016) Noninvasive screening for pulmonary hypertension by exercise testing in congenital heart disease. Ann Thorac Surg 103:1544–1549
14.
Zurück zum Zitat Muller J, Ewert P, Hager A (2015) Only slow decline in exercise capacity in the natural history of patients with congenital heart disease: a longitudinal study in 522 patients. Eur J Prev Cardiol 22:113–118CrossRefPubMed Muller J, Ewert P, Hager A (2015) Only slow decline in exercise capacity in the natural history of patients with congenital heart disease: a longitudinal study in 522 patients. Eur J Prev Cardiol 22:113–118CrossRefPubMed
15.
Zurück zum Zitat Muller J, Hess J, Hager A (2014) Sense of coherence, rather than exercise capacity, is the stronger predictor to obtain health-related quality of life in adults with congenital heart disease. Eur J Prev Cardiol 21:949–955CrossRefPubMed Muller J, Hess J, Hager A (2014) Sense of coherence, rather than exercise capacity, is the stronger predictor to obtain health-related quality of life in adults with congenital heart disease. Eur J Prev Cardiol 21:949–955CrossRefPubMed
16.
Zurück zum Zitat Fratz S, Schuhbaeck A, Buchner C et al (2009) Comparison of accuracy of axial slices versus short-axis slices for measuring ventricular volumes by cardiac magnetic resonance in patients with corrected tetralogy of fallot. Am J Cardiol 103:1764–1769CrossRefPubMed Fratz S, Schuhbaeck A, Buchner C et al (2009) Comparison of accuracy of axial slices versus short-axis slices for measuring ventricular volumes by cardiac magnetic resonance in patients with corrected tetralogy of fallot. Am J Cardiol 103:1764–1769CrossRefPubMed
17.
Zurück zum Zitat Fratz S, Hess J, Schuhbaeck A et al (2008) Routine clinical cardiovascular magnetic resonance in paediatric and adult congenital heart disease: patients, protocols, questions asked and contributions made. J Cardiovasc Magn Reson 10:46CrossRefPubMedPubMedCentral Fratz S, Hess J, Schuhbaeck A et al (2008) Routine clinical cardiovascular magnetic resonance in paediatric and adult congenital heart disease: patients, protocols, questions asked and contributions made. J Cardiovasc Magn Reson 10:46CrossRefPubMedPubMedCentral
18.
Zurück zum Zitat Meierhofer C, Lyko C, Schneider EP et al (2015) Baseline correction does not improve flow quantification in phase-contrast velocity measurement for routine clinical practice. Clin Imaging 39:427–431CrossRefPubMed Meierhofer C, Lyko C, Schneider EP et al (2015) Baseline correction does not improve flow quantification in phase-contrast velocity measurement for routine clinical practice. Clin Imaging 39:427–431CrossRefPubMed
19.
Zurück zum Zitat Muller J, Kuhn A, Tropschuh A et al (2016) Exercise performance in Ebstein’s anomaly in the course of time: deterioration in native patients and preserved function after tricuspid valve surgery. Int J Cardiol 218:79–82CrossRefPubMed Muller J, Kuhn A, Tropschuh A et al (2016) Exercise performance in Ebstein’s anomaly in the course of time: deterioration in native patients and preserved function after tricuspid valve surgery. Int J Cardiol 218:79–82CrossRefPubMed
20.
Zurück zum Zitat Kuhn A, De Pasquale Meyer G, Muller J et al (2013) Tricuspid valve surgery improves cardiac output and exercise performance in patients with Ebstein’s anomaly. Int J Cardiol 166:494–498CrossRefPubMed Kuhn A, De Pasquale Meyer G, Muller J et al (2013) Tricuspid valve surgery improves cardiac output and exercise performance in patients with Ebstein’s anomaly. Int J Cardiol 166:494–498CrossRefPubMed
21.
Zurück zum Zitat Hosch O, Ngyuen TT, Lauerer P et al (2015) BNP and haematological parameters are markers of severity of Ebstein’s anomaly: correlation with CMR and cardiopulmonary exercise testing. Eur Heart J Cardiovasc Imaging 16:670–675PubMed Hosch O, Ngyuen TT, Lauerer P et al (2015) BNP and haematological parameters are markers of severity of Ebstein’s anomaly: correlation with CMR and cardiopulmonary exercise testing. Eur Heart J Cardiovasc Imaging 16:670–675PubMed
22.
Zurück zum Zitat Lange R, Burri M, Eschenbach LK et al (2015) Da Silva’s cone repair for Ebstein’s anomaly: effect on right ventricular size and function. Eur J Cardiothorac Surg 48:316–320 (Discussion 20–1)CrossRefPubMed Lange R, Burri M, Eschenbach LK et al (2015) Da Silva’s cone repair for Ebstein’s anomaly: effect on right ventricular size and function. Eur J Cardiothorac Surg 48:316–320 (Discussion 20–1)CrossRefPubMed
23.
Zurück zum Zitat Ibrahim M, Tsang VT, Caruana M et al (2015) Cone reconstruction for Ebstein’s anomaly: patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg 149:1144–1150CrossRefPubMed Ibrahim M, Tsang VT, Caruana M et al (2015) Cone reconstruction for Ebstein’s anomaly: patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg 149:1144–1150CrossRefPubMed
24.
Zurück zum Zitat Chen SS, Dimopoulos K, Sheehan FH et al (2016) Physiologic determinants of exercise capacity in patients with different types of right-sided regurgitant lesions: Ebstein’s malformation with tricuspid regurgitation and repaired tetralogy of Fallot with pulmonary regurgitation. Int J Cardiol 205:1–5CrossRefPubMed Chen SS, Dimopoulos K, Sheehan FH et al (2016) Physiologic determinants of exercise capacity in patients with different types of right-sided regurgitant lesions: Ebstein’s malformation with tricuspid regurgitation and repaired tetralogy of Fallot with pulmonary regurgitation. Int J Cardiol 205:1–5CrossRefPubMed
Metadaten
Titel
Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein’s Anomaly
verfasst von
Christian Meierhofer
Andreas Kühn
Jan Müller
Nerejda Shehu
Alfred Hager
Stefan Martinoff
Heiko Stern
Peter Ewert
Manfred Vogt
Publikationsdatum
06.02.2019
Verlag
Springer US
Erschienen in
Pediatric Cardiology / Ausgabe 4/2019
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-019-02066-7

Weitere Artikel der Ausgabe 4/2019

Pediatric Cardiology 4/2019 Zur Ausgabe

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.