Functional Health Status in Adult Survivors of Operative Repair of Tetralogy of Fallot

Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada.
The American journal of cardiology (Impact Factor: 3.43). 03/2012; 109(6):873-80. DOI: 10.1016/j.amjcard.2011.10.051
Source: PubMed

ABSTRACT We aimed to determine late functional health status of the growing adult population with repaired tetralogy of Fallot (TOF). We studied all 840 patients with TOF born from 1927 through 1984 who survived to adulthood (> 18 years of age). Clinical follow-up was by chart review, telephone interview (n = 706), and echocardiographic reports (n = 339). Functional health status was assessed using Short Form-36 (SF-36) surveys (n = 396) indexed to normative data. Risk of reoperation was low (≈ 1%/year) but increased beyond age 40 years. At latest follow-up moderate or severe pulmonary regurgitation was common (54%) and right ventricular outflow tract stenosis presented in 1/3. Consequently, evidence of right ventricular dilatation and dysfunction and tricuspid regurgitation was typical. Left-sided abnormalities were also common: hypertrophy (p < 0.0001) and outflow tract dilation (p < 0.0001) with at least mild aortic regurgitation in > 50%. Cardiorespiratory symptoms were reported in 45% (palpitations 27%, dyspnea 21%, chest pain 17%). SF-36 scores were significantly below normal for 4 physical domains (p < 0.001). Decrements in physical functioning were associated particularly with older age at follow-up (p < 0.0001), associated syndromes/lesions, reoperations, ventricular dysfunction, tricuspid regurgitation, residual septal defects, and cardiorespiratory symptomatology. Echocardiographic abnormalities were more common in older patients (p < 0.0001). All 3 SF-36 domains specific to psychosocial well-being were normal. In conclusion, despite excellent survival prospects, physical compromise is common in adults with repaired TOF. Greater decrements in older patients may reflect late deterioration with advancing age or cohort effects related to historical management. Efforts to limit ventricular and outflow tract dysfunction may translate into improved late functional status.

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    ABSTRACT: After repair of tetralogy of Fallot, right ventricular (RV) mass and mass:volume ratio may reflect RV remodeling and adverse outcomes. This study aimed to evaluate the relation of RV mass to functional health status and subsequent adverse RV remodeling and to determine whether RV mass measurement in systole could improve reproducibility. In 53 patients with tetralogy of Fallot (median 29 years old) who previously underwent cardio-vascular magnetic resonance and completed the Short Form 36, version 2 (Optum, Eden Prairie, MN), short-axis images were analyzed for RV end-diastolic volume and diastolic and systolic mass, indexed to body surface area. The most recent subsequent cardiovascular magnetic resonance study (before pulmonary valve or conduit replacement) was evaluated for change in RV end-diastolic volume and ejection fraction. Diastolic indexed mass 37.3 g/m 2 (odds ratio 7.6, p [ 0.02) predicted decreased general health scores. In patients with normal RV ejection fraction, indexed mass correlated with Physical Component Summary and general health scores. RV diastolic mass:volume ratio >0.2 had a strong association with subsequent increase in RV end-diastolic volume (odds ratio 26.1, p [ 0.002). Systolic RV mass measurement had excellent correlation with diastolic measure-ment (r [ 0.97, p <0.0001), but did not improve intraobserver or interobserver variability. In conclusion, RV mass relates to functional health status and adverse RV remodeling and can be measured with good reproducibility. RV mass should be routinely evaluated in this population and is best measured in diastole; further study is necessary to evaluate longitudinal changes in functional health status and RV parameters. Ó 2014 Elsevier Inc. All rights reserved. (Am J Cardiol 2014;114:1896e1901) After repair of tetralogy of Fallot (TOF), patients often have free pulmonary insufficiency and a varying degree of right ventricular (RV) outflow tract obstruction, but the optimal timing of pulmonary valve replacement is unclear. Much attention has focused on ventricular size and function, 1e3 but recent data suggest that RV mass, specif-ically mass:volume ratio, may be an important marker of ventricular remodeling, as it predicts adverse outcomes in patients with repaired TOF. 4 The potential relation between RV mass and functional health status has not been demon-strated. In addition, although RV mass measurement by cardiovascular magnetic resonance (CMR) has been shown to accurately reflect actual RV weight on autopsy, 5 several studies have shown lesser reproducibility of RV mass measurement than other parameters, 6e11 which may be related to the thin wall of the RV in diastole. It is unclear whether this measurement would be more reliable in sys-tole, 12 when the RV thickens, and borders may be more clearly defined. This study aimed to determine whether RV mass in patients with repaired TOF is related to functional health status and RV remodeling in this population, and whether measurement variability would improve with measurement in systole rather than diastole.
    The American Journal of Cardiology 09/2014; 114(12):1896. DOI:10.1016/j.amjcard.2014.09.027 · 3.43 Impact Factor
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    ABSTRACT: This year marks the 60(th) year since the first successful intracardiac repair of tetralogy of Fallot (TOF).(1) The repair, largely unchanged over decades, consists of VSD closure and the relief of variable forms of right ventricular (RV) obstruction, usually with a trans-annular patch (TAP), and usually resulting in free pulmonary insufficiency (PI). Early in the experience with TOF repair, attention was on quantity of life - lifting the early dip in the survival curve. Palliative shunts were widely used, to permit repair at a safer, older age. The dividends from a full relief of obstruction included excellent function for decades for patients formerly suffering morbid or lethal disease. In this issue of Circulation, Cuypers and colleagues report findings of the third decennial follow up of a cohort of early TOF repairs, the longest prospective study of this population to date.(2) As such longitudinal evidence accrues, we are continually called upon to reexamine both ends of the treatment timeline, though it is a wobbly yardstick that attempts to inform today's best practices by measuring the sequelae of yesterday's.
    Circulation 10/2014; DOI:10.1161/CIRCULATIONAHA.114.013270 · 14.95 Impact Factor
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    ABSTRACT: -Prospective data on long-term survival and clinical outcome beyond 30 years after surgical correction of Tetralogy of Fallot (ToF) is non-existing.
    Circulation 10/2014; DOI:10.1161/CIRCULATIONAHA.114.009454 · 14.95 Impact Factor