An Unusual Equatorial Constriction of the Ventricular Cone

Abstract

A 25-year-old woman with a recent diagnosis of congenital heart disease and probable endocarditis was referred to our institution. During our evaluation we observed an unusual deformation of both ventricles. We discuss its possible origin as revealed by printing of a three-dimensional model. (Level of Difficulty: Advanced.).

Keywords: CMR, cardiac magnetic resonance; CT, computed tomography; computed tomography; congenital heart defect; papillary muscles; three-dimensional printing.

Graphical abstract

Figure 1

Electrocardiographic-Gated Multiplanar Computed Tomography Angiography (Retrospective Protocol) Computed tomography showing a remarkable deformation in the walls of the ventricles (curved white arrows), abnormal grouping of papillary muscles (black arrows), and excessive trabeculation (red asterisk). (A) 4-chamber view. (B) 3-chamber view. (C) Right heart chambers and outflow tract, subvalvar pulmonary stenosis is also observed (D) 2-chamber view. (E) Level of the deformation in the short axis, confirming the abnormal arrangement of the papillary muscles. (F) Volumetric reconstruction. Ao = aorta; LA = left atrium; LV = left ventricle; PA = pulmonary artery; PT = pulmonary trunk; RA = right atrium; RV = right ventricle.

Figure 2

3-Dimensional Printing Model (A) Frontal view, in the chest X-ray, the notable deformation cannot be observed, however, when superimposing the 3D model, the deformation is shown on the lower wall of the right ventricle (RV) (curved arrow). (B) View of the base of the heart showing that the constriction (curved arrow) is marked in both ventricles, giving the appearance of 4 ventricles. (C) View from above showing deformation and stenosis at the infundibular level (asterisk). (D) View of the lateral wall of the left ventricle (LV) showing the oblique path of the deformation. ICV = inferior caval vein; LAA = left atrial appendage; RAA = right atrial appendage; RPA = right branch of pulmonary trunk; RCA = dominant right coronary artery; SCV = superior caval vein; other abbreviations as in Figure 1.

Figure 3

Cardiac Magnetic Resonance and Computed Tomography (A) True fast imaging with steady-state free precession sequence showing accumulation of fatty tissue (asterisk) outside the ventricular walls, at the level of the deformation, more evident in the left ventricle (LV). (B) Steady-state free precession sequence of 2 chambers showing the deformation of the wall around the LV and the accumulation of fatty tissue (asterisk) at this level. (C) Computed tomography view modified for the right ventricle (RV), showing the constriction of the walls of both ventricles (arrows). Abbreviations as in Figure 1.