Abstract
Background
There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure.
Materials and methods
We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences.
Results
There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m2 vs. 118 ± 30 mL/m2), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16).
Conclusion
Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation.
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Supplementary Video S1
A 4-month-old boy with repaired tetralogy of Fallot and partial anomalous pulmonary venous connection. Four-chamber balanced steady-state free procession cine MRI shows paradoxical motion of the intraventricular septum and compression of the left ventricle by the right ventricle. The right ventricle is also much larger than the left ventricle (RVEDVi [indexed right ventricle end diastolic volume] =135 mL/m2). (MP4 12 kb)
Supplementary Video S2
A 6-month-old boy (same patient as in Supplementary Video S1) with repaired tetralogy of Fallot and repaired partial anomalous pulmonary venous connection. Four-chamber balanced steady-state free procession cine MRI after repair of the anomalous pulmonary venous connection shows that the paradoxical motion of the intraventricular septum has resolved and that the left ventricle and right venticle are now equal in size (RVEDVi [indexed right ventricle end diastolic volume] = 94 mL/m2). The right ventricle contraction has markedly improved. (MP4 10 kb)
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Chan, S.S., Whitehead, K.K., Kim, T.S. et al. Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation. Pediatr Radiol 45, 1465–1471 (2015). https://doi.org/10.1007/s00247-015-3358-0
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DOI: https://doi.org/10.1007/s00247-015-3358-0