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Right ventricular function in patients with pulmonary regurgitation with versus without tetralogy of Fallot.
Am Heart J. 2019 07; 213:8-17.AH

Abstract

BACKGROUND

Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes.

METHODS

Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups.

RESULTS

The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 ± 6.4% vs. 48.5 ± 7.2%, P = .81) and exercise capacity (% predicted peak VO2:82.5 ± 17.7% vs. 75.6 ± 20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:r = -0.39, P = .04, rToF:r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:r = -0.77, P < .001, rToF:r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 ± 3.9 vs.-25.7 ± 4.4, P < .001).

CONCLUSIONS

Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.

Authors+Show Affiliations

Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Division of Pediatrics, P. Universidad Catolica de Chile, Santiago, Chile.Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Division of Pediatric Cardiology, Perth Children´s Hospital, Perth, Australia.Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada.Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada.Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada.Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada. Electronic address: mark.friedberg@sickkids.ca.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

31071505

Citation

Larios, Guillermo, et al. "Right Ventricular Function in Patients With Pulmonary Regurgitation With Versus Without Tetralogy of Fallot." American Heart Journal, vol. 213, 2019, pp. 8-17.
Larios G, Yim D, Dragulescu A, et al. Right ventricular function in patients with pulmonary regurgitation with versus without tetralogy of Fallot. Am Heart J. 2019;213:8-17.
Larios, G., Yim, D., Dragulescu, A., Mertens, L., Grosse-Wortmann, L., & Friedberg, M. K. (2019). Right ventricular function in patients with pulmonary regurgitation with versus without tetralogy of Fallot. American Heart Journal, 213, 8-17. https://doi.org/10.1016/j.ahj.2019.03.012
Larios G, et al. Right Ventricular Function in Patients With Pulmonary Regurgitation With Versus Without Tetralogy of Fallot. Am Heart J. 2019;213:8-17. PubMed PMID: 31071505.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Right ventricular function in patients with pulmonary regurgitation with versus without tetralogy of Fallot. AU - Larios,Guillermo, AU - Yim,Deane, AU - Dragulescu,Andreea, AU - Mertens,Luc, AU - Grosse-Wortmann,Lars, AU - Friedberg,Mark K, Y1 - 2019/04/08/ PY - 2018/12/03/received PY - 2019/03/27/accepted PY - 2019/5/10/pubmed PY - 2020/3/5/medline PY - 2019/5/10/entrez SP - 8 EP - 17 JF - American heart journal JO - Am Heart J VL - 213 N2 - BACKGROUND: Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. METHODS: Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. RESULTS: The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 ± 6.4% vs. 48.5 ± 7.2%, P = .81) and exercise capacity (% predicted peak VO2:82.5 ± 17.7% vs. 75.6 ± 20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:r = -0.39, P = .04, rToF:r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:r = -0.77, P < .001, rToF:r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 ± 3.9 vs.-25.7 ± 4.4, P < .001). CONCLUSIONS: Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration. SN - 1097-6744 UR - https://www.unboundmedicine.com/medline/citation/31071505/Right_ventricular_function_in_patients_with_pulmonary_regurgitation_with_versus_without_tetralogy_of_Fallot_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0002-8703(19)30078-X DB - PRIME DP - Unbound Medicine ER -