Ventricular Myocardial Deformation Imaging of Patients with Repaired Tetralogy of Fallot.

In patients with repaired tetralogy of Fallot (TOF), dysfunction of the right and left ventricles remains an important issue. Adverse right ventricular (RV) remodeling has been related to RV dilation secondary to pulmonary regurgitation, electromechanical dyssynchrony, and myocardial fibrosis. Left ventricular (LV) dysfunction is attributed among other factors to altered ventricular-ventricular interaction. Advancements in echocardiography and cardiac magnetic resonance imaging have enabled direct interrogation of myocardial deformation of both ventricles in terms of myocardial strain and strain rate. Emerging evidence suggests that myocardial deformation imaging may provide incremental information for clinical use. In children and adults with repaired TOF, there is a growing body of literature on the use of myocardial deformation imaging in the assessment of ventricular mechanics and its clinical and prognostic values. The present review aims to provide an overview of impairment in RV and LV mechanics, associations between RV and LV deformation, changes in ventricular deformation after pulmonary valve replacement, and associations between measures of RV and LV deformation and outcomes and to highlight the clinical translational potential of myocardial deformation imaging in patients with repaired TOF.

Transcatheter occlusion of complex pulmonary arteriovenous malformations in a cyanotic child.

This case report presents a 16-month-old boy with isolated mild cyanosis diagnosed to have complex pulmonary arteriovenous malformations. Three-dimensional computed tomography with volume rendering has provided the diagnosis, enabled detailed imaging, and facilitated transcatheter device occlusion of the complex arteriovenous malformations by vascular plugs and coils. Magnetic resonance of the brain revealed associated dural arteriovenous malformation. Genetic testing showed a missense disease-causing variant in the ENG gene that encodes endoglin, and the diagnosis of hereditary hemorrhagic telangiectasia was made. .

Left Ventricular Stiffness in Adolescents and Young Adults with Repaired Tetralogy of Fallot.

Left ventricular (LV) remodeling after tetralogy of Fallot (TOF) repair may influence LV stiffness. We hypothesized that LV stiffness is altered after TOF repair and related to myocardial calibrated integrated backscatter (cIB) and LV diastolic myocardial deformation. Seventy-seven TOF patients and 80 controls were studied. LV stiffness was assessed by diastolic wall strain (DWS) as defined by (LVPWsystole-LVPWdiastole)/LVPWsystole, where LVPW is LV posterior wall thickness, and stiffness index as defined by (E/e/LV end-diastolic dimension), where E and e are respectively early diastolic transmitral inflow and mitral annular velocities. Septal and LVPW cIB and LV diastolic strain rates were determined. Patients had significantly lower DWS (p < 0.001), higher stiffness index (p < 0.001), and greater cIB (p < 0.001). LV DWS correlated negatively with LV stiffness index (r = -0.31, p < 0.001), septal cIB (r = -0.21, p = 0.01), E/e ratio (r = -0.30, p < 0.001) and RV end-diastolic area (r = -0.31, p < 0.001), and positively with LV early (r = 0.33, p < 0.001) and late (r = 0.20, p = 0.01) diastolic strain rates and RV fractional area change (FAC) (r = 0.24, p = 0.003). Multivariate analysis revealed E/e (ß = -0.26, p = 0.008), RV end-diastolic area (ß = -0.20, p = 0.02), and RV FAC (ß = 0.18, p = 0.01) as significant correlates of DWS. Left ventricular stiffening occurs after TOF repair and is related to impaired LV diastolic myocardial deformation, myocardial cIB, and RV volume overload.