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: 3.28).
03/2012; 109(6):873-80. DOI: 10.1016/j.amjcard.2011.10.051
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.
Available from: Karen P McCarthy
- "The challenge is to obtain a spatially pleasing reconstruction in three dimensions. The patient' s prognosis clearly depends on the results  , which must be carefully assessed by, for example, direct pressure measurement and/or epicardial echocardiography . The spatial orientation of the pulmonary arteries can vary greatly depending on the underlying diagnosis, including aligned, V-shaped and discontinuous. "
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ABSTRACT: Surgical repair of pulmonary artery (PA) branches encompasses many different clinical scenarios and technical challenges. The most common, such as bifurcation and central PA reconstruction, are described, as well as the challenges of complex and peripheral reconstruction.
Multimedia Manual of Cardiothoracic Surgery 01/2013; 2013:mmt014. DOI:10.1093/mmcts/mmt014
Available from: PubMed Central
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ABSTRACT: Tetralogy of Fallot (TOF) is an important lesion for all pediatric and congenital heart surgeons. In designing the most appropriate operation for children with TOF, the postoperative physiology should be taken into account, both in the short and long term. The balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) may be critical for preservation of ventricular function. A unified repair strategy that limits both residual PS and PI is presented, along with supporting experimental evidence, a strategy for dealing with coronary anomalies, and comments regarding best timing of operation.
Annals of Pediatric Cardiology 07/2008; 1(2):93-100. DOI:10.4103/0974-2069.43873
Available from: Jimmy C Lu
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ABSTRACT: After repair of tetralogy of Fallot, the left ventricular ejection fraction and the right ventricular ejection fraction are associated with clinical status and outcomes, but the relation of strain, a potentially earlier marker of dysfunction, to quality of life has not been evaluated. In 58 patients with tetralogy of Fallot (median age 29 years, interquartile range 20 to 41) who underwent cardiovascular magnetic resonance imaging and completed the Short Form 36, Version 2 (a validated quality-of-life assessment), left ventricular global circumferential strain, left ventricular global longitudinal strain, and right ventricular free wall longitudinal strain (RVLSFW) were measured from cine images using feature-tracking software. Age-adjusted z score ≤-1 for the physical component summary or subscales of physical functioning, role-physical, and general health was considered a clinically significant decrease in quality of life. Patients with RVLSFW less than the median had increased odds of decreased physical functioning (odds ratio [OR] 5.4, p = 0.01) and general health (OR 3.5, p = 0.04) subscale scores, which remained significant in patients with right ventricular ejection fractions ≥45% (physical functioning: OR 9.5, p = 0.03; general health: OR 5.9, p = 0.04). Left ventricular global circumferential strain and left ventricular global longitudinal strain did not predict decreased quality of life in this population. Intraobserver and interobserver variability was acceptable for left ventricular global circumferential strain (coefficients of variation 9.5% and 10.0%, respectively) but lower for left ventricular global longitudinal strain (coefficients of variation 17.2% and 16.8%, respectively) and poor for RVLSFW (coefficients of variation 19.9% and 28.8%, respectively). In conclusion, RVLSFW appears to have discriminative ability in this population for decreased quality of life and may yield incremental prognostic value beyond global right ventricular ejection fraction assessment, but further study is needed to evaluate methods to limit variability.
The American journal of cardiology 03/2013; 111(11). DOI:10.1016/j.amjcard.2013.01.336 · 3.28 Impact Factor
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