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Restrictive physiology in repaired Tetralogy of Fallot: Prevalence, significance and pathophysiology
Journal of Cardiovascular Magnetic Resonance volume 12, Article number: P15 (2010)
Introduction
Restrictive physiology of the right ventricle(RV) has been associated with a better long-term prognosis in patients afterTetralogy of Fallot(TOF) repair presumably because the decreased compliance prevents dilation of RV. However, there is another report that restrictive physiology is related to poor exercise tolerance. The presence of end diastolic forward flow(EDFF) in the pulmonary artery in patients with pulmonary regurgitation is commonly used to diagnose "restriction" of the RV.
Purpose
To investigate the cause, prevalence and clinical significance of EDFF to prognosis of the in patients with repaired TOF.
Methods
From Aug 2007 to Jan 2009, a total 93 magnetic resonance imaging (MRI) examinations were performed in patients with repaired TOF. Out of them 43 examinations were excluded for analysis in this retrospective study because of significant RV outflow obstruction or other causes. A total 50 patients (mean age = 13.0, M:F = 26:24) are divided into two group; Group 1 with EDFF and Group 2 without EDFF. We also analyzed the data from 12 normal subjects as controls. We evaluated the timing of flow patterns of the ascending aorta and pulmonary artery, mitral and tricuspid inflow, obtained by phase contrast MRI. We also collected the results of the volumetric assessment within the same MRI, the most recent exercise test, electrocardiogram. The date and type of repair was recorded.
Results
EDFF was present in 33 patients (group 1) out of 50 and not observed in the control groups. Group 1 reached 69% of the predicted maximum oxygen consumption while group 2 achieved 58% (p = 0.01). There was no significant difference in ejection fraction (49% vs. 46%, respectively, p = 0.33) and right ventricular end-diastolic volume (168 ml/m2 vs.166 ml/m2, respectively, p = 0.92) between groups 1 and 2. Descriptors of the aortic, pulmonic, mitral and tricuspid inflow patterns were similar between the two groups. Group 1 showed a significant delay of onset of the tricuspid inflow and lower tricuspid inflow e/a ratio of 1.25 than 2.58, when compared to normal subjects (p = < 0.001) Figure 1.
Typical flow pattern of aorta (red), pulmonary artery (blue), mitral (brown) and tricuspid valves (green) in repaired TOF. Note the pulmonary regurgitation (arrowhead) and end-diastolic forward flow (arrow) which is related with a wave of tricuspid inflow (A).
Conclusion
The presence of EDFF is associated with with better exercise tolerance. Patients with EDFF express diastolic dysfunction, as evidenced by a delayed onset of tricuspid valve inflow and a lower the e/a ratio.
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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Whal, L., Roche, S.L., Yoo, S. et al. Restrictive physiology in repaired Tetralogy of Fallot: Prevalence, significance and pathophysiology. J Cardiovasc Magn Reson 12, P15 (2010). https://doi.org/10.1186/1532-429X-12-S1-P15
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Keywords
- Right Ventricle
- Exercise Tolerance
- Contrast Magnetic Resonance Imaging
- Maximum Oxygen Consumption
- Pulmonary Regurgitation
