Hot topics in tetralogy of fallot

Juan Villafañe, Jeffrey A. Feinstein, Kathy J. Jenkins, Robert N. Vincent, Edward P. Walsh, Anne M. Dubin, Tal Geva, Jeffrey A. Towbin, Meryl S. Cohen, Charles Fraser, Joseph Dearani, David Rosenthal, Beth Kaufman, Thomas P. Graham

Research output: Contribution to journalReview article

85 Citations (Scopus)

Abstract

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "hot topics" to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.

Original languageEnglish (US)
Pages (from-to)2155-2166
Number of pages12
JournalJournal of the American College of Cardiology
Volume62
Issue number23
DOIs
StatePublished - Dec 10 2013

Fingerprint

Tetralogy of Fallot
Pulmonary Valve Insufficiency
Right Ventricular Function
Cardiac Arrhythmias
Magnetic Resonance Spectroscopy
Right Ventricular Dysfunction
Pulmonary Valve
Cardiac Resynchronization Therapy
Defibrillators
Congenital Heart Defects
Sudden Cardiac Death
Tachycardia
Pulmonary Artery
Aorta
Dilatation
Recurrence
Drug Therapy
Incidence

Keywords

  • arrhythmias
  • imaging
  • pulmonary regurgitation
  • pulmonary valve replacement
  • sudden cardiac death
  • tetralogy of Fallot
  • ventricular function and heart failure

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Villafañe, J., Feinstein, J. A., Jenkins, K. J., Vincent, R. N., Walsh, E. P., Dubin, A. M., ... Graham, T. P. (2013). Hot topics in tetralogy of fallot. Journal of the American College of Cardiology, 62(23), 2155-2166. https://doi.org/10.1016/j.jacc.2013.07.100

Hot topics in tetralogy of fallot. / Villafañe, Juan; Feinstein, Jeffrey A.; Jenkins, Kathy J.; Vincent, Robert N.; Walsh, Edward P.; Dubin, Anne M.; Geva, Tal; Towbin, Jeffrey A.; Cohen, Meryl S.; Fraser, Charles; Dearani, Joseph; Rosenthal, David; Kaufman, Beth; Graham, Thomas P.

In: Journal of the American College of Cardiology, Vol. 62, No. 23, 10.12.2013, p. 2155-2166.

Research output: Contribution to journalReview article

Villafañe, J, Feinstein, JA, Jenkins, KJ, Vincent, RN, Walsh, EP, Dubin, AM, Geva, T, Towbin, JA, Cohen, MS, Fraser, C, Dearani, J, Rosenthal, D, Kaufman, B & Graham, TP 2013, 'Hot topics in tetralogy of fallot', Journal of the American College of Cardiology, vol. 62, no. 23, pp. 2155-2166. https://doi.org/10.1016/j.jacc.2013.07.100
Villafañe J, Feinstein JA, Jenkins KJ, Vincent RN, Walsh EP, Dubin AM et al. Hot topics in tetralogy of fallot. Journal of the American College of Cardiology. 2013 Dec 10;62(23):2155-2166. https://doi.org/10.1016/j.jacc.2013.07.100
Villafañe, Juan ; Feinstein, Jeffrey A. ; Jenkins, Kathy J. ; Vincent, Robert N. ; Walsh, Edward P. ; Dubin, Anne M. ; Geva, Tal ; Towbin, Jeffrey A. ; Cohen, Meryl S. ; Fraser, Charles ; Dearani, Joseph ; Rosenthal, David ; Kaufman, Beth ; Graham, Thomas P. / Hot topics in tetralogy of fallot. In: Journal of the American College of Cardiology. 2013 ; Vol. 62, No. 23. pp. 2155-2166.
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AU - Dubin, Anne M.

AU - Geva, Tal

AU - Towbin, Jeffrey A.

AU - Cohen, Meryl S.

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N2 - Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "hot topics" to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.

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