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01.09.2015 | Original Article

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

verfasst von: Sherwin S. Chan, Kevin K. Whitehead, Timothy S. Kim, Gregory L. Fu, Marc S. Keller, Mark A. Fogel, Matthew A. Harris

Erschienen in: Pediatric Radiology | Ausgabe 10/2015

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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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Vohra HA, Adamson L, Haw MP (2008) Is early primary repair for correction of tetralogy of Fallot comparable to surgery after 6 months of age? Interact Cardiovasc Thorac Surg 7:698–701CrossRefPubMed

Kirklin JW, Wallace RB, McGoon DC et al (1965) Early and late results after intracardiac repair of Tetralogy of Fallot. 5-Year review of 337 patients. Ann Surg 162:578–589PubMedCentralCrossRefPubMed

Bacha EA, Scheule AM, Zurakowski D et al (2001) Long-term results after early primary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 122:154–161CrossRefPubMed

Parry AJ, McElhinney DB, Kung GC et al (2000) Elective primary repair of acyanotic tetralogy of Fallot in early infancy: overall outcome and impact on the pulmonary valve. J Am Coll Cardiol 36:2279–2283CrossRefPubMed

Pigula FA, Khalil PN, Mayer JE et al (1999) Repair of tetralogy of Fallot in neonates and young infants. Circulation 100:II157–II161CrossRefPubMed

Nollert G, Fischlein T, Bouterwek S et al (1997) Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol 30:1374–1383CrossRefPubMed

Bajolle F, Zaffran S, Losay J et al (2009) Conotruncal defects associated with anomalous pulmonary venous connections. Arch Cardiovasc Dis 102:105–110CrossRefPubMed

Redington AN, Raine J, Shinebourne EA et al (1990) Tetralogy of Fallot with anomalous pulmonary venous connections: a rare but clinically important association. Br Heart J 64:325–328PubMedCentralCrossRefPubMed

Helbing WA, Niezen RA, Le Cessie S et al (1996) Right ventricular diastolic function in children with pulmonary regurgitation after repair of tetralogy of Fallot: volumetric evaluation by magnetic resonance velocity mapping. J Am Coll Cardiol 28:1827–1835CrossRefPubMed

10.

Niezen RA, Helbing WA, van der Wall EE et al (1996) Biventricular systolic function and mass studied with MR imaging in children with pulmonary regurgitation after repair for tetralogy of Fallot. Radiology 201:135–140CrossRefPubMed

11.

Murphy JG, Gersh BJ, Mair DD et al (1993) Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 329:593–599CrossRefPubMed

12.

Geva T, Sandweiss BM, Gauvreau K et al (2004) Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging. J Am Coll Cardiol 43:1068–1074CrossRefPubMed

13.

Geva T (2011) Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson 13:9PubMedCentralCrossRefPubMed

14.

Maskatia SA, Spinner JA, Morris SA et al (2013) Effect of branch pulmonary artery stenosis on right ventricular volume overload in patients with tetralogy of fallot after initial surgical repair. Am J Cardiol 111:1355–1360CrossRefPubMed

15.

Maskatia SA, Spinner JA, Nutting AC et al (2013) Impact of obesity on ventricular size and function in children, adolescents and adults with Tetralogy of Fallot after initial repair. Am J Cardiol 112:594–598CrossRefPubMed

16.

Buechel EV, Kaiser T, Jackson C et al (2009) Normal right- and left ventricular volumes and myocardial mass in children measured by steady state free precession cardiovascular magnetic resonance. J Cardiovasc Magn Reson 11:19PubMedCentralCrossRefPubMed

17.

Buechel ER, Dave HH, Kellenberger CJ et al (2005) Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance. Eur Heart J 26:2721–2727CrossRefPubMed

18.

Sarikouch S, Boethig D, Peters B et al (2013) Poorer right ventricular systolic function and exercise capacity in women after repair of tetralogy of fallot: a sex comparison of standard deviation scores based on sex-specific reference values in healthy control subjects. Circ Cardiovasc Imaging 6:924–933CrossRefPubMed

19.

Piazza L, Chessa M, Giamberti A et al (2012) Timing of pulmonary valve replacement after tetralogy of Fallot repair. Expert Rev Cardiovasc Ther 10:917–923CrossRefPubMed

20.

Rominger MB, Bachmann GF, Pabst W et al (1999) Right ventricular volumes and ejection fraction with fast cine MR imaging in breath-hold technique: applicability, normal values from 52 volunteers, and evaluation of 325 adult cardiac patients. J Magn Reson Imaging 10:908–918CrossRefPubMed

21.

Ucar T, Fitoz S, Tutar E et al (2008) Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections. Int J Cardiovasc Imaging 24:229–235CrossRefPubMed

Titel: 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
verfasst von: Sherwin S. Chan
Kevin K. Whitehead
Timothy S. Kim
Gregory L. Fu
Marc S. Keller
Mark A. Fogel
Matthew A. Harris
Publikationsdatum: 01.09.2015
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Radiology / Ausgabe 10/2015
Print ISSN: 0301-0449
Elektronische ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-015-3358-0

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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