CLINICAL RESEARCH: CONGENITAL HEART DISEASE
Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging
Tal Geva, MD, FACC*,*,
Bryan M. Sandweiss, MD*,
Kimberlee Gauvreau, ScD*,
James E. Lock, MD, FACC* and
Andrew J. Powell, MD, FACC*
* Department of Cardiology, Children's Hospital; and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
Manuscript received June 17, 2003;
revised manuscript received September 16, 2003,
accepted October 6, 2003.
* Reprint requests and correspondence: Dr. Tal Geva, Department of Cardiology, Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115, USA. tal.geva{at}cardio.chboston.org
OBJECTIVES: The purpose of this study was to identify independent factors associated with impaired clinical status in late survivors of tetralogy of Fallot (TOF) repair.
BACKGROUND: Repair of TOF often results in chronic pulmonary regurgitation (PR) and right ventricular (RV) dilation, which have been linked to late morbidity and mortality. However, determinants of clinical status late after TOF repair have not been fully characterized.
METHODS: The clinical and laboratory data of 100 consecutive patients with repaired TOF (median 21 years after repair) who completed a cardiac magnetic resonance imaging protocol were analyzed. Impaired clinical status was defined as New York Heart Association (NYHA) functional class III.
RESULTS: Of the patients, 88 were in NYHA functional class I or II and 12 were in NYHA functional class III. The degree of PR and indexed RV end-diastolic volume were not associated with impaired clinical status. By multivariate analysis, a lower left ventricular (LV) ejection fraction (EF) (odds ratio [OR] = 3.88 for 10% decrease, p = 0.002) and older age at TOF repair (OR = 1.70 for 5-year increase, p = 0.013) were the strongest independent factors associated with impaired clinical status. Among RV variables, a lower RV EF was the strongest independent factors associated with poor clinical status (OR = 2.41 for 10% decrease, p = 0.01). The LV EF correlated with RV EF (r = 0.58, p < 0.001).
CONCLUSIONS: Moderate or severe LV or RV systolic dysfunction, but not PR fraction or RV diastolic dimensions, is independently associated with impaired clinical status in long-term survivors of TOF repair. The close relationship between LV EF and RV EF suggests unfavorable ventricular-ventricular interaction.
|
Abbreviations and Acronyms
| | ECG | = electrocardiogram | | EF | = ejection fraction | | LV | = left ventricle/ventricular | | MRI | = magnetic resonance imaging | | NYHA | = New York Heart Association | | PA | = pulmonary artery | | PR | = pulmonary regurgitation | | ROC | = receiver-operator characteristic | | RV | = right ventricle/ventricular | | TOF | = tetralogy of Fallot |
|
This article has been cited by other articles:

|
 |

|
 |
 
H B Grotenhuis, J Ottenkamp, L de Bruijn, J J M Westenberg, H W Vliegen, L J M Kroft, and A de Roos
Aortic elasticity and size are associated with aortic regurgitation and left ventricular dysfunction in tetralogy of Fallot after pulmonary valve replacement
Heart,
December 1, 2009;
95(23):
1931 - 1936.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. A. Warnes
Adult congenital heart disease importance of the right ventricle.
J. Am. Coll. Cardiol.,
November 17, 2009;
54(21):
1903 - 1910.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
E. Bedard, K. P. McCarthy, K. Dimopoulos, G. Giannakoulas, M. A. Gatzoulis, and S. Y. Ho
Structural abnormalities of the pulmonary trunk in tetralogy of fallot and potential clinical implications: a morphological study.
J. Am. Coll. Cardiol.,
November 10, 2009;
54(20):
1883 - 1890.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
E. W.K. Peng, S. Lilley, B. Knight, J. Sinclair, F. Lyall, K. MacArthur, J. C.S. Pollock, and M. H.D. Danton
Synergistic interaction between right ventricular mechanical dyssynchrony and pulmonary regurgitation determines early outcome following tetralogy of Fallot repair
Eur. J. Cardiothorac. Surg.,
October 1, 2009;
36(4):
694 - 702.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
K. Zervan, C. Male, T. Benesch, and U. Salzer-Muhar
Ventricular interaction in children after repair of tetralogy of Fallot: a longitudinal echocardiographic study
Eur J Echocardiogr,
July 1, 2009;
10(5):
641 - 646.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. S. Flett, M. A. Westwood, L. C. Davies, A. Mathur, and J. C. Moon
The Prognostic Implications of Cardiovascular Magnetic Resonance
Circ Cardiovasc Imaging,
May 1, 2009;
2(3):
243 - 250.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. M. Wald, I. Haber, R. Wald, A. M. Valente, A. J. Powell, and T. Geva
Effects of Regional Dysfunction and Late Gadolinium Enhancement on Global Right Ventricular Function and Exercise Capacity in Patients With Repaired Tetralogy of Fallot
Circulation,
March 17, 2009;
119(10):
1370 - 1377.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. M. Wald, A. N. Redington, A. Pereira, Y. L. Provost, N. S. Paul, E. N. Oechslin, and C. K. Silversides
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.,
February 1, 2009;
30(3):
356 - 361.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. J. Silka and Y. Bar-Cohen
Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD?: Patients With Congenital Heart Disease and a Systemic Ventricular Ejection Fraction Less Than 30% Should Undergo Prophylactic Implantation of an Implantable Cardioverter Defibrillator
Circ Arrhythm Electrophysiol,
October 1, 2008;
1(4):
298 - 306.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
N. K. Bodhey, P. Beerbaum, S. Sarikouch, S. Kropf, P. Lange, F. Berger, R. H. Anderson, and T. Kuehne
Functional Analysis of the Components of the Right Ventricle in the Setting of Tetralogy of Fallot
Circ Cardiovasc Imaging,
September 1, 2008;
1(2):
141 - 147.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. C. Fiore, M. Rodefeld, M. Turrentine, P. Vijay, T. Reynolds, J. Standeven, K. Hill, J. Bost, D. Carpenter, C. Tobin, et al.
Pulmonary Valve Replacement: A Comparison of Three Biological Valves
Ann. Thorac. Surg.,
May 1, 2008;
85(5):
1712 - 1718.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. J. Sommer, Z. M. Hijazi, and J. F. Rhodes
Pathophysiology of Congenital Heart Disease in the Adult: Part III: Complex Congenital Heart Disease
Circulation,
March 11, 2008;
117(10):
1340 - 1350.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A L Knauth, K Gauvreau, A J Powell, M J Landzberg, E P Walsh, J E Lock, P J d. Nido, and T Geva
Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair
Heart,
February 1, 2008;
94(2):
211 - 216.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
H Senzaki, Y Iwamoto, H Ishido, T Matsunaga, M Taketazu, T Kobayashi, H Asano, T Katogi, and S Kyo
Arterial haemodynamics in patients after repair of tetralogy of Fallot: influence on left ventricular after load and aortic dilatation
Heart,
January 1, 2008;
94(1):
70 - 74.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
L. Mertens
Deciphering the mystery of the leaky pulmonary valve in a new era of interventional cardiology
Eur. Heart J.,
August 1, 2007;
28(15):
1793 - 1794.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
T. Oosterhof, A. van Straten, H. W. Vliegen, F. J. Meijboom, A. P.J. van Dijk, A. M. Spijkerboer, B. J. Bouma, A. H. Zwinderman, M. G. Hazekamp, A. de Roos, et al.
Preoperative Thresholds for Pulmonary Valve Replacement in Patients With Corrected Tetralogy of Fallot Using Cardiovascular Magnetic Resonance
Circulation,
July 31, 2007;
116(5):
545 - 551.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. R Chaturvedi and A. N Redington
Pulmonary regurgitation in congenital heart disease
Heart,
July 1, 2007;
93(7):
880 - 889.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
O. Ghez, V. T. Tsang, A. Frigiola, L. Coats, A. Taylor, C. Van Doorn, P. Bonhoeffer, and M. De Leval
Right ventricular outflow tract reconstruction for pulmonary regurgitation after repair of tetralogy of Fallot.: Preliminary results
Eur. J. Cardiothorac. Surg.,
April 1, 2007;
31(4):
654 - 658.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
I. R. Henkens, A. van Straten, M. J. Schalij, M. G. Hazekamp, A. de Roos, E. E. van der Wall, and H. W. Vliegen
Predicting Outcome of Pulmonary Valve Replacement in Adult Tetralogy of Fallot Patients
Ann. Thorac. Surg.,
March 1, 2007;
83(3):
907 - 911.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. van den Berg, W. C. Hop, J. L.M. Strengers, J. C. de Jongste, L. van Osch-Gevers, F. J. Meijboom, P. M.T. Pattynama, A. J.J.C. Bogers, and W. A. Helbing
Clinical condition at mid-to-late follow-up after transatrial-transpulmonary repair of tetralogy of Fallot
J. Thorac. Cardiovasc. Surg.,
February 1, 2007;
133(2):
470 - 477.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. Ooi, N. Moorjani, G. Baliulis, B. R. Keeton, A. P. Salmon, J. L. Monro, and M. P. Haw
Medium term outcome for infant repair in tetralogy of Fallot: indicators for timing of surgery
Eur. J. Cardiothorac. Surg.,
December 1, 2006;
30(6):
917 - 922.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. Giardini, S. Specchia, G. Coutsoumbas, A. Donti, R. Formigari, R. Fattori, G. Oppido, G. Gargiulo, and F. M. Picchio
Impact of pulmonary regurgitation and right ventricular dysfunction on oxygen uptake recovery kinetics in repaired tetralogy of Fallot
Eur J Heart Fail,
November 1, 2006;
8(7):
736 - 743.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
U. K. Chowdhury, S. Sathia, R. Ray, R. Singh, K. K. Pradeep, and P. Venugopal
Histopathology of the right ventricular outflow tract and its relationship to clinical outcomes and arrhythmias in patients with tetralogy of Fallot.
J. Thorac. Cardiovasc. Surg.,
August 1, 2006;
132(2):
270 - 277.e4.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
U. K. Chowdhury, K. K. Pradeep, C. D. Patel, R. Singh, A. S. Kumar, B. Airan, G. S. Gulati, S. S. Kothari, A. Saxena, M. Kalaivani, et al.
Noninvasive Assessment of Repaired Tetralogy of Fallot by Magnetic Resonance Imaging and Dynamic Radionuclide Studies
Ann. Thorac. Surg.,
April 1, 2006;
81(4):
1436 - 1442.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. G. Williams, G. D. Pearson, R. J. Barst, J. S. Child, P. del Nido, W. M. Gersony, K. S. Kuehl, M. J. Landzberg, M. Myerson, S. R. Neish, et al.
Report of the National Heart, Lung, and Blood Institute Working Group on Research in Adult Congenital Heart Disease
J. Am. Coll. Cardiol.,
February 21, 2006;
47(4):
701 - 707.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. V. Babu-Narayan, P. J. Kilner, W. Li, J. C. Moon, O. Goktekin, P. A. Davlouros, M. Khan, S. Y. Ho, D. J. Pennell, and M. A. Gatzoulis
Ventricular Fibrosis Suggested by Cardiovascular Magnetic Resonance in Adults With Repaired Tetralogy of Fallot and Its Relationship to Adverse Markers of Clinical Outcome
Circulation,
January 24, 2006;
113(3):
405 - 413.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
K. I. Norton, C. Tong, R. B. J. Glass, and J. C. Nielsen
Cardiac MR Imaging Assessment Following Tetralogy of Fallot Repair
RadioGraphics,
January 1, 2006;
26(1):
197 - 211.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
T. Oosterhof, B. J. M. Mulder, H. W. Vliegen, and A. de Roos
Corrected Tetralogy of Fallot: Delayed Enhancement in Right Ventricular Outflow Tract
Radiology,
December 1, 2005;
237(3):
868 - 871.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. A. Warnes
The Adult With Congenital Heart Disease: Born To Be Bad?
J. Am. Coll. Cardiol.,
July 5, 2005;
46(1):
1 - 8.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
B. Bouzas, P. J. Kilner, and M. A. Gatzoulis
Pulmonary regurgitation: not a benign lesion
Eur. Heart J.,
March 1, 2005;
26(5):
433 - 439.
[Abstract]
[Full Text]
[PDF]
|
 |
|
|