Ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia.

Ectopia cordis, defined as partial or complete displacement of the heart outside of the thoracic cavity, is a rare congenital malformation. If not surgically corrected during the early years of life, ectopia cordis can prove to be a fatal abnormality. However, due to the presence of multiple intracardiac and extracardiac malformations, a corrective surgery might not always be successful. The pathology of ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia is discussed, highlighting the complexities involved in such a rare disorder.

Hypertrophic cardiomyopathy with right ventricular outflow tract and left ventricular intracavitary obstruction.

Obstruction of the right ventricular outflow tract (RVOT) is a rare finding in hypertrophic cardiomyopathy (HCM) patients unlike left ventricular outflow tract (LVOT) obstruction. Although there are guidelines that aid in clinical decision making in patients with LVOT obstruction, there are none addressing RVOT obstruction. As RVOT obstruction may pose serious clinical implications similar to LVOT obstruction, appropriate medical and surgical management is very important. A unique phenotype of HCM with RVOT obstruction in conjunction with left ventricle (LV) intracavitary obstruction is discussed.

Evaluation of myocardial mechanics with three-dimensional speckle tracking echocardiography in heart transplant recipients: comparison with two-dimensional speckle tracking and relationship with clinical variables.

AIMS: Two-dimensional speckle-tracking echocardiography (2D-STE) is limited by its inability to track tissue motion in three dimensions. This is particularly relevant in heart transplant recipients, in whom marked translational motion of the transplanted heart is present. We aimed to compare 3-dimensional (3D)- and 2D-STE-derived strain parameters, and to identify clinical features associated with myocardial mechanics in transplant recipients. METHODS AND RESULTS: In 36 heart transplant recipients, global and regional left-ventricular (LV) longitudinal and circumferential strain (LSt and CSt), and radial displacement (RDisp) were obtained by 3D- and 2D-STE, and their results were compared. 3D-STE deformation from a subset of transplant recipients with preserved ejection fraction was compared with a control group of 25 subjects matched by gender, age, history of hypertension, and ejection fraction. Associations between global LSt and CSt and clinical, echocardiographic, and haemodynamic parameters in transplant recipients were investigated. 3D-STE yielded lower magnitude of global LSt compared with 2D-STE (-13 ± 3 vs. -16 ± 3%, P < 0.001). The inferolateral wall was a source of variation between 3D- and 2D-STE both for LSt and CSt. Inferolateral wall 3D-STE-derived RDisp was greater than that observed in control subjects (7.4 ± 1.2 vs. 6.5 ± 1.7 mm, P = 0.03), while anteroseptal RDisp was lower than controls (4.2 ± 1.0 vs. 7.3 ± 1.6 mm, P < 0.001). Multiple regression analysis demonstrated that 3D-STE-derived LSt was independently associated with NYHA class (P < 0.001), while 2D-STE-derived LSt was not. CONCLUSION: Examination of LV mechanics by 3D- and 2D-STE deformation parameters in heart transplant recipients yields significantly discordant results. 3D-STE-derived LSt is independently associated with NYHA class, suggesting a clinically important relationship between functional status and myocardial mechanics.

Left ventricular twist mechanics in hypertrophic cardiomyopathy assessed by three-dimensional speckle tracking echocardiography.

Left ventricular (LV) twist represents a phenomenon that links systolic contraction with diastolic relaxation and plays a major role in cardiac physiology; thus, the study of twist mechanics is of particular interest in hypertrophic cardiomyopathy (HC). Three-dimensional speckle tracking echocardiography (3D-STE) has the potential to overcome the limitations of 2-dimensional imaging and provide a greater understanding of LV twist in HC. We aimed to examine LV twist mechanics in HC using 3D-STE. Echocardiograms from subjects with a diagnosis of HC were examined for 3D-STE analysis. Age- and gender-matched healthy subjects were tested as a control group. Forty patients with HC (age 37 ± 16 years; 42.5% women) and 40 control subjects (age 35 ± 10 years; 42.5% women) were examined. Compared with the controls, the patients with HC showed increased peak LV twist (16.5 ± 4.7° vs 12.0 ± 3.9°, p <0.001) mainly because of increased apical rotation of those with LV outflow tract obstruction (obstruction, 12.7 ± 4.4° vs nonobstruction, 9.7 ± 2.8°, p = 0.02). In addition, the patients with HC displayed onset of torsion recoil occurring closer to the aortic valve closure (94 ± 6% vs 85 ± 6%, p <0.001; time normalized by the length of systole), limited completion of untwist during early diastole (31 ± 12% vs 62 ± 15%, p <0.001), and delayed peak untwist velocity (22 ± 7% vs 13 ± 9%, p <0.001; time normalized by the length of diastole). In conclusion, the evaluation of twist mechanics using 3D-STE provides novel insight regarding alterations in LV mechanics in patients with HC. Elucidating the characteristics of the wringing motion of the heart might help to broaden the understanding of the hyperdynamic contraction and impaired relaxation observed in these patients.