Skip to main content
Top
Published in: Journal of Cardiovascular Magnetic Resonance 1/2017

Open Access 01-12-2016 | Research

Vicious circle between progressive right ventricular dilatation and pulmonary regurgitation in patients after tetralogy of Fallot repair? Right heart enlargement promotes flow reversal in the left pulmonary artery

Authors: Atsuko Kato, Christian Drolet, Shi-Joon Yoo, Andrew N. Redington, Lars Grosse-Wortmann

Published in: Journal of Cardiovascular Magnetic Resonance | Issue 1/2017

Login to get access

Abstract

Background

The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well understood. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR.

Methods

Forty-eight Cardiovascular Magnetic Resonance (CMR) studies in patients after TOF repair were analyzed. In addition to the routine blood flow and ventricular volume quantification cardiac angle between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured on an axial image. Statistical analysis was performed to compare flow parameters between RPA and LPA and to assess correlation among right ventricular volume, pulmonary blood flow parameters and lung area.

Results

There was no difference between LPA and RPA diameters. The LPA showed significantly less total forward flow (2.49 ± 0.87 L/min/m2 vs 2.86 ± 0.89 L/min/m2; p = 0.02), smaller net forward flow (1.40 ± 0.51 vs 1.89 ± 0.60 mL/min/m2; p = <0.001), and greater regurgitant fraction (RF) (34 ± 10 % vs 43 ± 12 %; p = 0.001) than the RPA. There was no difference in regurgitant flow volume between RPA and LPA (p = 0.29). Indexed right ventricular end-diastolic volume (RVEDVi) correlated with LPA RF (R = 0.48, p < 0.001), but not with RPA RF (p = 0.09). Larger RVEDVi correlated with a more leftward cardiac axis (R = 0.46, p < 0.001) and with smaller left lung area (R = −0.58, p < 0.001). LPA RF, but not RPA RF, correlated inversely with left lung area (R = −0.34, p = 0.02). The follow-up CMRs in 20 patients showed a correlation of the rate of RV enlargement with the rates of LPA RF worsening (R = 0.50, p = 0.03), and of increasing left lung compression (R = −0.55, p = 0.012).

Conclusion

An enlarged and levorotated heart is associated with left lung compression and impaired flow into the left lung.
Literature
1.
go back to reference Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, McGoon DC, Kirklin JW, Danielson GK. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329(9):593–9.CrossRefPubMed Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, McGoon DC, Kirklin JW, Danielson GK. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329(9):593–9.CrossRefPubMed
2.
go back to reference Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC and others. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356(9234):975–81. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC and others. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356(9234):975–81.
3.
go back to reference Rowe SA, Zahka KG, Manolio TA, Horneffer PJ, Kidd L. Lung function and pulmonary regurgitation limit exercise capacity in postoperative tetralogy of Fallot. J Am Coll Cardiol. 1991;17(2):461–6.CrossRefPubMed Rowe SA, Zahka KG, Manolio TA, Horneffer PJ, Kidd L. Lung function and pulmonary regurgitation limit exercise capacity in postoperative tetralogy of Fallot. J Am Coll Cardiol. 1991;17(2):461–6.CrossRefPubMed
4.
go back to reference Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol. 2002;40(9):1675–80.CrossRefPubMed Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol. 2002;40(9):1675–80.CrossRefPubMed
5.
go back to reference Redington AN. Determinants and assessment of pulmonary regurgitation in tetralogy of Fallot: practice and pitfalls. Cardiol Clin. 2006;24(4):631–9. vii.CrossRefPubMed Redington AN. Determinants and assessment of pulmonary regurgitation in tetralogy of Fallot: practice and pitfalls. Cardiol Clin. 2006;24(4):631–9. vii.CrossRefPubMed
6.
go back to reference Wald RM, Redington AN, Pereira A, Provost YL, Paul NS, Oechslin EN, Silversides CK. Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot repair: should we be measuring regurgitant fraction or regurgitant volume? Eur Heart J. 2009;30(3):356–61.CrossRefPubMed Wald RM, Redington AN, Pereira A, Provost YL, Paul NS, Oechslin EN, Silversides CK. Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot repair: should we be measuring regurgitant fraction or regurgitant volume? Eur Heart J. 2009;30(3):356–61.CrossRefPubMed
7.
go back to reference d’Udekem Y, Ovaert C, Grandjean F, Gerin V, Cailteux M, Shango-Lody P, Vliers A, Sluysmans T, Robert A, Rubay J. Tetralogy of Fallot: transannular and right ventricular patching equally affect late functional status. Circulation. 2000;102(19 Suppl 3):III116–22.PubMed d’Udekem Y, Ovaert C, Grandjean F, Gerin V, Cailteux M, Shango-Lody P, Vliers A, Sluysmans T, Robert A, Rubay J. Tetralogy of Fallot: transannular and right ventricular patching equally affect late functional status. Circulation. 2000;102(19 Suppl 3):III116–22.PubMed
8.
go back to reference Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JC, Smith GC, Tat T, Pennell DJ, Gatzoulis MA. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction. J Am Coll Cardiol. 2002;40(11):2044–52.CrossRefPubMed Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JC, Smith GC, Tat T, Pennell DJ, Gatzoulis MA. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction. J Am Coll Cardiol. 2002;40(11):2044–52.CrossRefPubMed
9.
go back to reference Niezen RA, Helbing WA, van der Wall EE, van der Geest RJ, Rebergen SA, de Roos A. Biventricular systolic function and mass studied with MR imaging in children with pulmonary regurgitation after repair for tetralogy of Fallot. Radiology. 1996;201(1):135–40.CrossRefPubMed Niezen RA, Helbing WA, van der Wall EE, van der Geest RJ, Rebergen SA, de Roos A. Biventricular systolic function and mass studied with MR imaging in children with pulmonary regurgitation after repair for tetralogy of Fallot. Radiology. 1996;201(1):135–40.CrossRefPubMed
10.
go back to reference Boechat MI, Ratib O, Williams PL, Gomes AS, Child JS, Allada V. Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 2005;25(6):1535–46.CrossRefPubMed Boechat MI, Ratib O, Williams PL, Gomes AS, Child JS, Allada V. Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 2005;25(6):1535–46.CrossRefPubMed
11.
go back to reference Lewis MJ, O’Connor DS, Rozenshtien A, Ye S, Einstein AJ, Ginns JM, Rosenbaum MS. Usefulness of magnetic resonance imaging to guide referral for pulmonary valve replacement in repaired tetralogy of Fallot. Am J Cardiol. 2014;114(9):1406–11.CrossRefPubMed Lewis MJ, O’Connor DS, Rozenshtien A, Ye S, Einstein AJ, Ginns JM, Rosenbaum MS. Usefulness of magnetic resonance imaging to guide referral for pulmonary valve replacement in repaired tetralogy of Fallot. Am J Cardiol. 2014;114(9):1406–11.CrossRefPubMed
12.
go back to reference Geva T. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson. 2011;13:9.CrossRefPubMedPubMedCentral Geva T. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson. 2011;13:9.CrossRefPubMedPubMedCentral
13.
go back to reference Kang IS, Redington AN, Benson LN, Macgowan C, Valsangiacomo ER, Roman K, Kellenberger CJ, Yoo SJ. Differential regurgitation in branch pulmonary arteries after repair of tetralogy of Fallot: a phase-contrast cine magnetic resonance study. Circulation. 2003;107(23):2938–43.CrossRefPubMed Kang IS, Redington AN, Benson LN, Macgowan C, Valsangiacomo ER, Roman K, Kellenberger CJ, Yoo SJ. Differential regurgitation in branch pulmonary arteries after repair of tetralogy of Fallot: a phase-contrast cine magnetic resonance study. Circulation. 2003;107(23):2938–43.CrossRefPubMed
14.
go back to reference Harris MA, Weinberg PM, Whitehead KK, Fogel MA. Usefulness of branch pulmonary artery regurgitant fraction to estimate the relative right and left pulmonary vascular resistances in congenital heart disease. Am J Cardiol. 2005;95(12):1514–7.CrossRefPubMed Harris MA, Weinberg PM, Whitehead KK, Fogel MA. Usefulness of branch pulmonary artery regurgitant fraction to estimate the relative right and left pulmonary vascular resistances in congenital heart disease. Am J Cardiol. 2005;95(12):1514–7.CrossRefPubMed
15.
go back to reference Bland JM, Altman DG. Statistical methods for assessing agreement between two methods of clinical measurement. Lancet. 1986;1(8476):307–10.CrossRefPubMed Bland JM, Altman DG. Statistical methods for assessing agreement between two methods of clinical measurement. Lancet. 1986;1(8476):307–10.CrossRefPubMed
16.
go back to reference Powell AJ, Maier SE, Chung T, Geva T. Phase-velocity cine magnetic resonance imaging measurement of pulsatile blood flow in children and young adults: in vitro and in vivo validation. Pediatr Cardiol. 2000;21(2):104–10.CrossRefPubMed Powell AJ, Maier SE, Chung T, Geva T. Phase-velocity cine magnetic resonance imaging measurement of pulsatile blood flow in children and young adults: in vitro and in vivo validation. Pediatr Cardiol. 2000;21(2):104–10.CrossRefPubMed
17.
go back to reference Harris MA, Whitehead KK, Gillespie MJ, Liu TY, Cosulich MT, Shin DC, Goldmuntz E, Weinberg PM, Fogel MA. Differential branch pulmonary artery regurgitant fraction is a function of differential pulmonary arterial anatomy and pulmonary vascular resistance. JACC Cardiovasc Imaging. 2011;4(5):506–13.CrossRefPubMed Harris MA, Whitehead KK, Gillespie MJ, Liu TY, Cosulich MT, Shin DC, Goldmuntz E, Weinberg PM, Fogel MA. Differential branch pulmonary artery regurgitant fraction is a function of differential pulmonary arterial anatomy and pulmonary vascular resistance. JACC Cardiovasc Imaging. 2011;4(5):506–13.CrossRefPubMed
18.
go back to reference Wu MT, Huang YL, Hsieh KS, Huang JT, Peng NJ, Pan JY, Huang JS, Yang TL. Influence of pulmonary regurgitation inequality on differential perfusion of the lungs in tetralogy of Fallot after repair: a phase-contrast magnetic resonance imaging and perfusion scintigraphy study. J Am Coll Cardiol. 2007;49(18):1880–6.CrossRefPubMed Wu MT, Huang YL, Hsieh KS, Huang JT, Peng NJ, Pan JY, Huang JS, Yang TL. Influence of pulmonary regurgitation inequality on differential perfusion of the lungs in tetralogy of Fallot after repair: a phase-contrast magnetic resonance imaging and perfusion scintigraphy study. J Am Coll Cardiol. 2007;49(18):1880–6.CrossRefPubMed
19.
go back to reference Voser EM, Kellenberger CJ, Buechel ER. Effects of pulmonary regurgitation on distensibility and flow of the branch pulmonary arteries in tetralogy of Fallot. Pediatr Cardiol. 2013;34(5):1118–24.CrossRefPubMed Voser EM, Kellenberger CJ, Buechel ER. Effects of pulmonary regurgitation on distensibility and flow of the branch pulmonary arteries in tetralogy of Fallot. Pediatr Cardiol. 2013;34(5):1118–24.CrossRefPubMed
20.
go back to reference Ristow B, Ahmed S, Wang L, Liu H, Angeja BG, Whooley MA, Schiller NB. Pulmonary regurgitation End-diastolic gradient is a Doppler marker of cardiac status: data from the heart and soul study. J Am Soc Echocardiogr. 2005;18(9):885–91.CrossRefPubMedPubMedCentral Ristow B, Ahmed S, Wang L, Liu H, Angeja BG, Whooley MA, Schiller NB. Pulmonary regurgitation End-diastolic gradient is a Doppler marker of cardiac status: data from the heart and soul study. J Am Soc Echocardiogr. 2005;18(9):885–91.CrossRefPubMedPubMedCentral
21.
go back to reference Hart S, Devendra G, Kim Y, Flamm S, Kalahasti V, Arruda J, Walker E, Boonyasirinant T, Bolen M, Setser R and others. PINOT NOIR: Pulmonic INsufficiency imprOvemenT with Nitric Oxide Inhalational Response. J Cardiovasc Magn Reson. 2013;15(1):75. Hart S, Devendra G, Kim Y, Flamm S, Kalahasti V, Arruda J, Walker E, Boonyasirinant T, Bolen M, Setser R and others. PINOT NOIR: Pulmonic INsufficiency imprOvemenT with Nitric Oxide Inhalational Response. J Cardiovasc Magn Reson. 2013;15(1):75.
22.
go back to reference Bogren HG, Klipstein RH, Mohiaddin RH, Firmin DN, Underwood SR, Rees RS, Longmore DB. Pulmonary artery distensibility and blood flow patterns: a magnetic resonance study of normal subjects and of patients with pulmonary arterial hypertension. Am Heart J. 1989;118(5 Pt 1):990–9.CrossRefPubMed Bogren HG, Klipstein RH, Mohiaddin RH, Firmin DN, Underwood SR, Rees RS, Longmore DB. Pulmonary artery distensibility and blood flow patterns: a magnetic resonance study of normal subjects and of patients with pulmonary arterial hypertension. Am Heart J. 1989;118(5 Pt 1):990–9.CrossRefPubMed
23.
go back to reference Johnson RJ, Haworth SG. Pulmonary vascular and alveolar development in tetralogy of Fallot: a recommendation for early correction. Thorax. 1982;37(12):893–901.CrossRefPubMedPubMedCentral Johnson RJ, Haworth SG. Pulmonary vascular and alveolar development in tetralogy of Fallot: a recommendation for early correction. Thorax. 1982;37(12):893–901.CrossRefPubMedPubMedCentral
24.
go back to reference Bokma JP, Winter MM, Oosterhof T, Vliegen HW, van Dijk AP, Hazekamp MG, Koolbergen DR, Groenink M, Mulder BJ, Bouma BJ. Severe tricuspid regurgitation is predictive for adverse events in tetralogy of Fallot. Heart. 2015;101(10):794–9.CrossRefPubMed Bokma JP, Winter MM, Oosterhof T, Vliegen HW, van Dijk AP, Hazekamp MG, Koolbergen DR, Groenink M, Mulder BJ, Bouma BJ. Severe tricuspid regurgitation is predictive for adverse events in tetralogy of Fallot. Heart. 2015;101(10):794–9.CrossRefPubMed
25.
26.
go back to reference Agostoni P, Cattadori G, Guazzi M, Palermo P, Bussotti M, Marenzi G. Cardiomegaly as a possible cause of lung dysfunction in patients with heart failure. Am Heart J. 2000;140(5):e24.CrossRefPubMed Agostoni P, Cattadori G, Guazzi M, Palermo P, Bussotti M, Marenzi G. Cardiomegaly as a possible cause of lung dysfunction in patients with heart failure. Am Heart J. 2000;140(5):e24.CrossRefPubMed
Metadata
Title
Vicious circle between progressive right ventricular dilatation and pulmonary regurgitation in patients after tetralogy of Fallot repair? Right heart enlargement promotes flow reversal in the left pulmonary artery
Authors
Atsuko Kato
Christian Drolet
Shi-Joon Yoo
Andrew N. Redington
Lars Grosse-Wortmann
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Journal of Cardiovascular Magnetic Resonance / Issue 1/2017
Electronic ISSN: 1532-429X
DOI
https://doi.org/10.1186/s12968-016-0254-1

Other articles of this Issue 1/2017

Journal of Cardiovascular Magnetic Resonance 1/2017 Go to the issue