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.
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.


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/m(2) vs. 118 ± 30 mL/m(2)), 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).


CONCLUSIONS
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.
Department of Radiology, Children's Mercy Hospital and Clinics, 2401 Gillham Road, Kansas City, MO, 64108, USA, sschan@cmh.edu.
Sherwin S
SS
Chan
Kevin K
KK
Whitehead
Timothy S
TS
Kim
Gregory L
GL
Fu
Marc S
MS
Keller
Mark A
MA
Fogel
Matthew A
MA
Harris
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006352:Heart Ventricles / Q000473:pathology*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D012587:Scimitar Syndrome / Q000150:complications* / Q000473:pathology; D012720:Severity of Illness Index; D013771:Tetralogy of Fallot / Q000150:complications* / Q000601:surgery*; D018497:Ventricular Dysfunction, Right / Q000150:complications* / Q000473:pathology*; D055815:Young Adult