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Unnatural history of the right ventricle in patients with congenitally malformed hearts

Published online by Cambridge University Press:  01 December 2010

Leo Lopez ,
Meryl S. Cohen ,
Robert H. Anderson ,
Andrew N. Redington ,
David G. Nykanen ,
Daniel J. Penny ,
John E. Deanfield  and
Benjamin W. Eidem
Leo Lopez*
Affiliation:
Division of Pediatric Cardiology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York, United States of America
Meryl S. Cohen
Affiliation:
Division of Cardiology, Children’s Hospital of Philadelphia, The Cardiac Center, University of Philadelphia School of Medicine, Philadelphia, Pennsylvania, United States of America
Robert H. Anderson
Affiliation:
Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America
Andrew N. Redington
Affiliation:
Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Canada
David G. Nykanen
Affiliation:
Division of Pediatric Cardiology, Arnold Palmer Children’s Hospital, The Congenital Heart Institute, Orlando, Florida, United States of America
Daniel J. Penny
Affiliation:
Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia
John E. Deanfield
Affiliation:
Cardiothoracic Unit, Great Ormond Street Hospital for Sick Children, Institute of Child Health, London, United Kingdom
Benjamin W. Eidem
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, United States of America
*
Correspondence to: L. Lopez, Division of Pediatric Cardiology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York, United States of America. Tel: 1-718-741-2184; Fax: 1-718-741-2570; E-mail: llmd@llmd.net
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Abstract

The long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein’s malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.

Keywords

Tetralogy of FallotFontan circulationEbstein’s malformationcorrected transposition
Type
Original Article
Information
Cardiology in the Young , Volume 20 , Issue S3: Rare and Challenging Congenital Cardiac Lesions: an Interdisciplinary Approach , December 2010 , pp. 107 - 112
Copyright
Copyright © Cambridge University Press 2010

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References

1.Torrent-Guasp, F, Kocica, MJ, Corno, AF, et al. Towards new understanding of the heart structure and function. Eur J Cardiothorac Surg 2005; 27: 191–201.Google Scholar
2.Anderson, RH, Ho, SY, Redmann, K, Sanchez-Quintana, D, Lunkenheimer, PP. The anatomical arrangement of the myocardial cells making up the ventricular mass. Eur J Cardiothorac Surg 2005; 28: 517–525.Google Scholar
3.Wald, RM, Redington, AN, Pereira, A, et al. 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: 356–361.CrossRefGoogle ScholarPubMed
4.Chaturvedi, RR, Kilner, PJ, White, PA, Bishop, A, Szwarc, R, Redington, AN. Increased airway pressure and simulated branch pulmonary artery stenosis increase pulmonary regurgitation after repair of tetralogy of Fallot. Real-time analysis with a conductance catheter technique. Circulation 1997; 95: 643–649.CrossRefGoogle ScholarPubMed
5.Redington, AN, Penny, D, Rigby, ML, Hayes, A. Antegrade diastolic pulmonary arterial flow as a marker of right ventricular restriction after complete repair of pulmonary atresia with intact septum and critical valvar pulmonary stenosis. Cardiol Young 1992; 2: 382–386.CrossRefGoogle Scholar
6.Gatzoulis, MA, Clark, AL, Cullen, S, Newman, CG, Redington, AN. Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Restrictive physiology predicts superior exercise performance. Circulation 1995; 91: 1775–1781.Google Scholar
7.Gatzoulis, MA, Till, JA, Somerville, J, Redington, AN. Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation 1995; 92: 231–237.CrossRefGoogle ScholarPubMed
8.Gatzoulis, MA, Till, JA, Redington, AN. Depolarization-repolarization inhomogeneity after repair of tetralogy of Fallot. The substrate for malignant ventricular tachycardia? Circulation 1997; 95: 401–404.Google Scholar
9.Vogel, M, Sponring, J, Cullen, S, Deanfield, JE, Redington, AN. Regional wall motion and abnormalities of electrical depolarization and repolarization in patients after surgical repair of tetralogy of Fallot. Circulation 2001; 103: 1669–1673.Google Scholar
10.Gatzoulis, MA, Balaji, S, Webber, SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000; 356: 975–981.CrossRefGoogle ScholarPubMed
11.Davlouros, PA, Kilner, PJ, Hornung, TS, et al. 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: 2044–2052.Google Scholar
12.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: 1675–1680.CrossRefGoogle ScholarPubMed
13.D’Andrea, A, Caso, P, Sarubbi, B, et al. Right ventricular myocardial activation delay in adult patients with right bundle branch block late after repair of tetralogy of Fallot. Eur J Echocardiogr 2004; 5: 123–131.CrossRefGoogle ScholarPubMed
14.Kirsh, JA, Stephenson, EA, Redington, AN. Images in cardiovascular medicine: recovery of left ventricular systolic function after biventricular resynchronization pacing in a child with repaired tetralogy of Fallot and severe biventricular dysfunction. Circulation 2006; 113: e691–e692.CrossRefGoogle Scholar
15.Warnes, CA, Williams, RG, Bashore, TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation 2008; 118: 2395–2451.Google Scholar
16.Eyskens, B, Reybrouck, T, Bogaert, J, et al. Homograft insertion for pulmonary regurgitation after repair of tetralogy of Fallot improves cardiorespiratory exercise performance. Am J Cardiol 2000; 85: 221–225.Google Scholar
17.Therrien, J, Siu, SC, Harris, L, et al. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation 2001; 103: 2489–2494.CrossRefGoogle ScholarPubMed
18.Therrien, J, Provost, Y, Merchant, N, Williams, W, Colman, J, Webb, G. Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair. Am J Cardiol 2005; 95: 779–782.CrossRefGoogle ScholarPubMed
19.Oosterhof, T, van Straten, A, Vliegen, HW, et al. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation 2007; 116: 545–551.Google Scholar
20.Buechel, ER, Dave, HH, Kellenberger, CJ, et al. 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 2005; 26: 2721–2727.Google Scholar
21.Szabo, G, Bahrle, S. Contractility-afterload mismatch after the Fontan operation. Cardiol Young 2005; 15 (Suppl 3): 35–38.CrossRefGoogle ScholarPubMed
22.Nogaki, M, Senzaki, H, Masutani, S, et al. Ventricular energetics in Fontan circulation: evaluation with a theoretical model. Pediatr Int 2000; 42: 651–657.Google Scholar
23.Szabo, G, Buhmann, V, Graf, A, et al. Ventricular energetics after the Fontan operation: contractility-afterload mismatch. J Thorac Cardiovasc Surg 2003; 125: 1061–1069.CrossRefGoogle ScholarPubMed
24.Tanoue, Y, Sese, A, Ueno, Y, Joh, K, Hijii, T. Bidirectional Glenn procedure improves the mechanical efficiency of a total cavopulmonary connection in high-risk Fontan candidates. Circulation 2001; 103: 2176–2180.CrossRefGoogle ScholarPubMed
25.Sano, T, Ogawa, M, Taniguchi, K, et al. Assessment of ventricular contractile state and function in patients with univentricular heart. Circulation 1989; 79: 1247–1256.Google Scholar
26.Matsuda, H, Kawashima, Y, Kishimoto, H, et al. Problems in the modified Fontan operation for univentricular heart of the right ventricular type. Circulation 1987; 76: III45–III52.Google Scholar
27.Tweddell, JS, Nersesian, M, Mussatto, KA, et al. Fontan palliation in the modern era: factors impacting mortality and morbidity. Ann Thorac Surg 2009; 88: 1291–1299.CrossRefGoogle ScholarPubMed
28.Anderson, PA, Sleeper, LA, Mahony, L, et al. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol 2008; 52: 85–98.Google Scholar
29.Lubiszewska, B, Gosiewska, E, Hoffman, P, et al. Myocardial perfusion and function of the systemic right ventricle in patients after atrial switch procedure for complete transposition: long-term follow-up. J Am Coll Cardiol 2000; 36: 1365–1370.Google Scholar
30.Hauser, M, Bengel, FM, Hager, A, et al. Impaired myocardial blood flow and coronary flow reserve of the anatomical right systemic ventricle in patients with congenitally corrected transposition of the great arteries. Heart 2003; 89: 1231–1235.CrossRefGoogle ScholarPubMed
31.Babu-Narayan, SV, Goktekin, O, Moon, JC, et al. Late gadolinium enhancement cardiovascular magnetic resonance of the systemic right ventricle in adults with previous atrial redirection surgery for transposition of the great arteries. Circulation 2005; 111: 2091–2098.CrossRefGoogle ScholarPubMed
32.Winter, MM, van der Plas, MN, Bouma, BJ, Groenink, M, Bresser, P, Mulder, BJ. Mechanisms for cardiac output augmentation in patients with a systemic right ventricle. Int J Cardiol 2010; 143: 141–146.Google Scholar
33.Moons, P, Gewillig, M, Sluysmans, T, et al. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart 2004; 90: 307–313.CrossRefGoogle ScholarPubMed
34.Dos, L, Teruel, L, Ferreira, IJ, et al. Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries. Heart 2005; 91: 652–656.Google Scholar
35.Reich, O, Voriskova, M, Ruth, C, et al. Long-term ventricular performance after intra-atrial correction of transposition: left ventricular filling is the major limitation. Heart 1997; 78: 376–381.Google Scholar
36.Roos-Hesselink, JW, Meijboom, FJ, Spitaels, SE, et al. Decline in ventricular function and clinical condition after Mustard repair for transposition of the great arteries (a prospective study of 22–29 years). Eur Heart J 2004; 25: 1264–1270.CrossRefGoogle ScholarPubMed
37.Warnes, CA. Transposition of the great arteries. Circulation 2006; 114: 2699–2709.Google Scholar
38.Graham, TP Jr, Bernard, YD, Mellen, BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol 2000; 36: 255–261.CrossRefGoogle ScholarPubMed
39.Prieto, LR, Hordof, AJ, Secic, M, Rosenbaum, MS, Gersony, WM. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation 1998; 98: 997–1005.CrossRefGoogle ScholarPubMed
40.Seward, J. Ebstein’s anomaly: ultrasound imaging and haemodynamic evaluation. Echocardiography 1993; 10: 641–664.CrossRefGoogle Scholar
41.Yetman, AT, Freedom, RM, McCrindle, BW. Outcome in cyanotic neonates with Ebstein’s anomaly. Am J Cardiol 1998; 81: 749–754.Google Scholar
42.Quinonez, LG, Dearani, JA, Puga, FJ, et al. Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg 2007; 133: 1303–1310.Google Scholar
43.Brown, ML, Dearani, JA, Danielson, GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 2008; 135: 1120–1136, 1136 e1–e7.Google Scholar