- 期刊
Right Ventricular Cardiomyopathy in Repaired Tetralogy of Fallot: A Clinical Mimic of Arrhythmogenic Right Ventricular Cardiomyopathy
摘要
We aimed to study the clinical and imaging features in patients with tetralogy of Fallot (TF) after transannular patch repair, and determine the parameters of right ventricular dysfunction necessitating pulmonary valve replacement (PVR). Ten out of 149 patients, seven male and three female, were enrolled in this retrospective study. They were 17.0 ± 4.4 years old, repaired by transannular patch at 36.0 ± 26.9 months old, and divided into two groups, including two patients with sustained ventricular tachycardia (VT) in Group A and eight without sustained VT in Group B. Chest radiograms, electrocardiograms, echocardiograms, angiography, and cardiac magnetic resonance imaging (MRI) were reviewed to analyze structural dysfunctions and abnormalities of the right ventricle (RV). All ten patients presented with structural dysfunctions/abnormalities of the RV, repolarization/depolarization/conduction abnormalities, and arrhythmias. Two patients survived PVR, in whom RV outflow tract aneurysm, RV end-diastolic volume index, and RV end-systolic volume index were significantly larger than those without PVR (54.9 ± 2.9 mm vs. 39.9 ± 5.1 mm; 210.6 ± 54.3 mL/m^2 vs. 103.6 ± 20.0 mL/m^2; 146.5 ± 10.6 mL/m^2 vs. 73.6 ± 21.2 mL/m^2; respectively) (p value = 0.044). Mean corrected QT interval was longer in patients necessitating PVR than those waiving PVR (515.5 ± 9.2 ms vs. 485.3 ± 15.6 ms; p value = 0.044). A phenotype of RV cardiomyopathy could be seen in repaired TF patients. Electrocardiograms, echocardiograms, angiography, and cardiac MRI could be informative of its presence, and appropriate management could be applied to prevent further RV failure, VT, and sudden cardiac death.
延伸閱讀
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