Aortic root dilatation in adults with surgically repaired tetralogy of Fallot: A multicenter cross-sectional study

François Pierre Mongeon, Michelle Z. Gurvitz, Craig Broberg, Jamil Aboulhosn, Alexander R. Opotowsky, Joseph D. Kay, Anne Marie Valente, Michael G. Earing, George K. Lui, Susan M. Fernandes, Deborah R. Gersony, Stephen C. Cook, Jennifer Grando Ting, Michelle J. Nickolaus, Michael J. Landzberg, Paul Khairy

    Research output: Contribution to journalArticle

    49 Citations (Scopus)

    Abstract

    BACKGROUND - : Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS - : A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%). CONCLUSIONS - : Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

    Original languageEnglish (US)
    Pages (from-to)172-179
    Number of pages8
    JournalCirculation
    Volume127
    Issue number2
    DOIs
    StatePublished - Jan 15 2013

    Fingerprint

    Tetralogy of Fallot
    Dilatation
    Cross-Sectional Studies
    Aortic Valve Insufficiency
    Confidence Intervals
    Pulmonary Atresia
    Sinus of Valsalva
    Diastole
    Sex Ratio
    Thoracic Aorta
    Multivariate Analysis
    Odds Ratio
    Pathology

    Keywords

    • aorta
    • aortic regurgitation
    • congenital
    • heart defects
    • tetralogy of Fallot

    ASJC Scopus subject areas

    • Physiology (medical)
    • Cardiology and Cardiovascular Medicine

    Cite this

    Aortic root dilatation in adults with surgically repaired tetralogy of Fallot : A multicenter cross-sectional study. / Mongeon, François Pierre; Gurvitz, Michelle Z.; Broberg, Craig; Aboulhosn, Jamil; Opotowsky, Alexander R.; Kay, Joseph D.; Valente, Anne Marie; Earing, Michael G.; Lui, George K.; Fernandes, Susan M.; Gersony, Deborah R.; Cook, Stephen C.; Ting, Jennifer Grando; Nickolaus, Michelle J.; Landzberg, Michael J.; Khairy, Paul.

    In: Circulation, Vol. 127, No. 2, 15.01.2013, p. 172-179.

    Research output: Contribution to journalArticle

    Mongeon, FP, Gurvitz, MZ, Broberg, C, Aboulhosn, J, Opotowsky, AR, Kay, JD, Valente, AM, Earing, MG, Lui, GK, Fernandes, SM, Gersony, DR, Cook, SC, Ting, JG, Nickolaus, MJ, Landzberg, MJ & Khairy, P 2013, 'Aortic root dilatation in adults with surgically repaired tetralogy of Fallot: A multicenter cross-sectional study', Circulation, vol. 127, no. 2, pp. 172-179. https://doi.org/10.1161/CIRCULATIONAHA.112.129585
    Mongeon, François Pierre ; Gurvitz, Michelle Z. ; Broberg, Craig ; Aboulhosn, Jamil ; Opotowsky, Alexander R. ; Kay, Joseph D. ; Valente, Anne Marie ; Earing, Michael G. ; Lui, George K. ; Fernandes, Susan M. ; Gersony, Deborah R. ; Cook, Stephen C. ; Ting, Jennifer Grando ; Nickolaus, Michelle J. ; Landzberg, Michael J. ; Khairy, Paul. / Aortic root dilatation in adults with surgically repaired tetralogy of Fallot : A multicenter cross-sectional study. In: Circulation. 2013 ; Vol. 127, No. 2. pp. 172-179.
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    abstract = "BACKGROUND - : Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS - : A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9{\%} (95{\%} confidence interval, 26.9{\%}-30.9{\%}). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95{\%} confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6{\%} (95{\%} confidence interval, 5.3{\%}-7.9{\%}). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5{\%} (95{\%} confidence interval, 2.7{\%}-4.2{\%}). CONCLUSIONS - : Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.",
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    T1 - Aortic root dilatation in adults with surgically repaired tetralogy of Fallot

    T2 - A multicenter cross-sectional study

    AU - Mongeon, François Pierre

    AU - Gurvitz, Michelle Z.

    AU - Broberg, Craig

    AU - Aboulhosn, Jamil

    AU - Opotowsky, Alexander R.

    AU - Kay, Joseph D.

    AU - Valente, Anne Marie

    AU - Earing, Michael G.

    AU - Lui, George K.

    AU - Fernandes, Susan M.

    AU - Gersony, Deborah R.

    AU - Cook, Stephen C.

    AU - Ting, Jennifer Grando

    AU - Nickolaus, Michelle J.

    AU - Landzberg, Michael J.

    AU - Khairy, Paul

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    N2 - BACKGROUND - : Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS - : A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%). CONCLUSIONS - : Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

    AB - BACKGROUND - : Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS - : A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%). CONCLUSIONS - : Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

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    KW - aortic regurgitation

    KW - congenital

    KW - heart defects

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