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      Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support

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      Journal of Cardiovascular Magnetic Resonance

      BioMed Central

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          Abstract

          Surgical management of tetralogy of Fallot (TOF) results in anatomic and functional abnormalities in the majority of patients. Although right ventricular volume load due to severe pulmonary regurgitation can be tolerated for many years, there is now evidence that the compensatory mechanisms of the right ventricular myocardium ultimately fail and that if the volume load is not eliminated or reduced by pulmonary valve replacement the dysfunction might be irreversible. Cardiovascular magnetic resonance (CMR) has evolved during the last 2 decades as the reference standard imaging modality to assess the anatomic and functional sequelae in patients with repaired TOF. This article reviews the pathophysiology of chronic right ventricular volume load after TOF repair and the risks and benefits of pulmonary valve replacement. The CMR techniques used to comprehensively evaluate the patient with repaired TOF are reviewed and the role of CMR in supporting clinical decisions regarding pulmonary valve replacement is discussed.

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          Most cited references 137

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          Normal human left and right ventricular dimensions for MRI as assessed by turbo gradient echo and steady-state free precession imaging sequences.

          To establish normal ranges of left ventricular (LV) and right ventricular (RV) dimensions as determined by the current pulse sequences in cardiac magnetic resonance imaging (MRI). Sixty normal subjects (30 male and 30 female; age range, 20-65) were examined; both turbo gradient echo (TGE) and steady-state free precession (SSFP) pulse sequences were used to obtain contiguous short-axis cine data sets from the ventricular apex to the base of the heart. The LV and RV volumes and LV mass were calculated by modified Simpson's rule. Normal ranges were established and indexed to both body surface area (BSA) and height. There were statistically significant differences in the measurements between the genders and between TGE and SSFP pulse sequences. For TGE the LV end-diastolic volume (EDV)/BSA (mL/m(2)) in males was 74.4 +/- 14.6 and in females was 70.9 +/- 11.7, while in SSFP in males it was 82.3 +/- 14.7 and in females it was 77.7 +/- 10.8. For the TGE the LV mass/BSA (g/m(2)) in males was 77.8 +/- 9.1 and in females it was 61.5 +/- 7.5, while in SSFP in males it was 64.7 +/- 9.3 and in females it was 52.0 +/- 7.4. For TGE the RV EDV/BSA (mL/m(2)) in males was 78.4 +/- 14.0 and in females it was 67.5 +/- 12.7, while in SSFP in males it was 86.2 +/- 14.1 and in females it was 75.2 +/- 13.8. We have provided normal ranges that are gender specific as well as data that can be used for age-specific normal ranges for both SSFP and TGE pulse sequences. Copyright 2003 Wiley-Liss, Inc.
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            Normal human left and right ventricular and left atrial dimensions using steady state free precession magnetic resonance imaging.

            The aim of this project was to establish a database of left and right ventricular and left atrial dimensions in healthy volunteers using steady-state free precession cardiac magnetic resonance imaging, the clinical technique of choice, across a wide age range. 108 healthy volunteers (63 male, 45 female) underwent cardiac magnetic resonance imaging using steady-state free precession sequences. Manual analysis was performed by 2 experienced observers. Left and right ventricular volumes and left ventricular mass were larger in males than females: LV end-diastolic volume 160 +/- 29 mL vs. 135 +/- 26 mL, LV end-systolic volume 50 +/- 16 mL vs. 42 +/- 12 mL; RV end-diastolic volume 190 +/- 33 mL vs. 148 +/- 35 mL, RV end-systolic volume 78 +/- 20 mL vs. 56 +/- 18 mL (p < .05 for all). Normalization of values to body surface area removed the statistical differences for LV volumes, but not for LV mass or RV volumes. With increased age, males showed a significant decrease in volume and mass indices for both ventricles, while female values remained unchanged. Compared to females, males had significantly larger maximal left atrial volumes (103 +/- 30 mL vs. 89 +/- 21 mL, p = .01) and left atrial stroke volumes (58 +/- 23 mL vs. 48 +/- 15 mL, p = .01). There was no difference in left atrial ejection fraction between the sexes. We have produced a large database of age-related normal ranges for left and right ventricular function and left atrial function in males and females. This will allow accurate interpretation of clinical and research datasets.
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              Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot.

              Although corrective surgery for tetralogy of Fallot has been available for more than 30 years, the occurrence of late sudden death in patients in whom surgery was apparently successful remains worrisome. We studied long-term survival among 163 patients who survived 30 days after complete repair of tetralogy of Fallot, examining follow-up hospital records and death certificates when relevant. The overall 32-year actuarial survival rate among all patients who survived surgery was 86 percent, as compared with an expected rate of 96 percent in a control population matched for age and sex (P < 0.01). Thirty-year actuarial survival rates were calculated for the patient subgroups. The survival rates among patients less than 5 years old, 5 to 7 years old, and 8 to 11 years old were 90, 93, and 91 percent, respectively--slightly less than the expected rates (P < 0.001, P = 0.06, and P = 0.02). Among patients 12 years old or older at the time of surgery, the survival rate was 76 percent, as compared with an expected rate of 93 percent (P < 0.001). The performance of a palliative Blalock-Taussig shunt procedure before repair, unlike the performance of a Waterston or Potts shunt procedure, was not associated with reduced long-term survival, nor was the need for a trans-annular patch at the time of surgery. Independent predictors of long-term survival were older age at operation (P = 0.02) and a higher ratio of right ventricular to left ventricular systolic pressure after surgery (P = 0.008). Late sudden death from cardiac causes occurred in 10 patients during the 32-year period. Among patients with surgically repaired tetralogy of Fallot, the rate of long-term survival after the postoperative period is excellent but remains lower than that in the general population. The risk of late sudden death is small.
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                Author and article information

                Journal
                J Cardiovasc Magn Reson
                Journal of Cardiovascular Magnetic Resonance
                BioMed Central
                1097-6647
                1532-429X
                2011
                20 January 2011
                : 13
                : 1
                : 9
                Affiliations
                [1 ]Department of Cardiology, Children's Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, MA, USA
                Article
                1532-429X-13-9
                10.1186/1532-429X-13-9
                3036629
                21251297
                Copyright ©2011 Geva; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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                Review

                Cardiovascular Medicine

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