FIGURE LEGEND
Figure 1 . Panel A:
12-leads ECG showing sinus rhythm (52 bpm), left and right ventricular
hypertrophy and repolarization abnormalities. Panel B:
Posterior-anterior Chest X-ray documenting situs solitus and levocardia
with significant cardiac enlargement and abnormal cardiothoracic ratio
(greater than 50%).Figure 2 . Echocardiogram.
Panel A: diastolic frame of parasternal short axis view at the level of
the papillary muscles. Panel B: diastolic frame of apical 4 chamber
view. Severe RV hypertrophy and small LV cavity size, due to the
ventricular interdependence and RV overload, are evident. Panel C:
parasternal short axis at the level of great vessels with color-Doppler
twin view and measurement of the severely narrowed right ventricular
outflow-tract (RVOT) diameter at infundibular level. Panel D: CW Doppler
interrogation of the obstructed RVOT with maximum gradient of 185
mmHg.Figure 3 . Coronary-CT
Angiography (CCTA). Panel A: Coronal oblique multi-planar reconstruction
(MPR) well shows the right partial anomalous pulmonary venous return
(PAPVR) with the right superior pulmonary vein draining into the
superior Vena Cava (SVC) (green arrow). Panel B-C: MPR (B) and 3D (C)
volume rendering (VR) of the RVOT well depict the significant
infundibular stenosis (yellow arrows). Panel D: 3D VR-slab along the
vertical long axis (VLA) of the RV well shows the significant
hypertrophy of the RV walls (>13mm), papillary muscles, and
the severe obstruction of the RVOT (infundibular stenosis).
Supplementary Material: The following supplementary material is
available online - Movie Clips AVI:
Video 1. Transthoracic echocardiogram video clips. Panel A:
parasternal long axis view. Panel B: short axis view at the level of the
papillary muscles. Both clips show the severe RV hypertrophy with
anomalous muscle bundles and ventricular septum flattening, causing
small LV cavity size due to ventricular interdependence. Panel C:
parasternal short axis at the level of great vessels and
color-Doppler twin views focused on RVOT with Color flow Doppler mosaic
pattern consistent with severe RVOT obstruction. Panel D: parasternal
short axis at the level of great vessels and color-Doppler twin views
focused on VSD documenting the right-to-left shunt.
Video 2 . 3D VR video along the VLA of the RV shows the right
PAPVR with the right superior pulmonary vein draining into the SVC, the
right atriomegaly, hypertrophy of RV walls (>13mm),
papillary muscles, and the severe narrowing of RVOT (infundibular
stenosis).
Video 3 . MPR video of the RVOT well shows the significant
infundibular stenosis.