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Double-chambered right ventricle in a 16-year-old patient with Williams syndrome

Wojciech Mądry
Wojciech Mądry
, 
Maciej A. Karolczak
Maciej A. Karolczak
 e 
Ewa Zacharska-Kokot
Ewa Zacharska-Kokot
   | 29 dic 2017
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Article Category: case-report
Pubblicato online: 29 dic 2017
Pagine: 289 - 293
Ricevuto: 26 apr 2017
Accettato: 06 giu 2017
DOI: https://doi.org/10.15557/jou.2017.0042
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© 2017 Wojciech Mądry et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial 3.0 Public License.

Fig. 1

Modified parasternal cross-sectional view displaying the left ventricle at the level of the papillary muscles, the right ventricle inflow tract, and muscle structures inside the right ventricle, dividing it into a high-pressure inflow chamber (RVI) and a low-pressure outflow chamber. Three abnormal groups of interconnected muscles are displayed: A: moderator band (hypertrophic, displaced anterior and upward; B: hypertrophic muscle bundles arising from the anterior wall of the RV, connected with the base of the anterior papillary muscle; and C: an additional, thick muscle bundle extending from the tricuspid ring – from the bottom part of the anterior leaflet – to the moderator band, to which it connects above the base of the medial papillary muscle (Lancisi). The borders of abnormal muscles are highlighted with red lines. LV: Left Ventricle, IVS: Interventricular Septum, TV: Tricuspid Valve, RVI: low-pressure inlet of the Right Ventricle
Modified parasternal cross-sectional view displaying the left ventricle at the level of the papillary muscles, the right ventricle inflow tract, and muscle structures inside the right ventricle, dividing it into a high-pressure inflow chamber (RVI) and a low-pressure outflow chamber. Three abnormal groups of interconnected muscles are displayed: A: moderator band (hypertrophic, displaced anterior and upward; B: hypertrophic muscle bundles arising from the anterior wall of the RV, connected with the base of the anterior papillary muscle; and C: an additional, thick muscle bundle extending from the tricuspid ring – from the bottom part of the anterior leaflet – to the moderator band, to which it connects above the base of the medial papillary muscle (Lancisi). The borders of abnormal muscles are highlighted with red lines. LV: Left Ventricle, IVS: Interventricular Septum, TV: Tricuspid Valve, RVI: low-pressure inlet of the Right Ventricle

Fig. 2

Modified high vascular short-axis view, oriented more sagittally than usual. A fibro muscular diaphragm formed by the above described structures, dividing the right ventricle into a high-pressure inflow chamber (RVI) and a low-pressure outflow chamber (RVOT), is visible. The flow through the diaphragm dividing the RV is visualized using color Doppler. Only a 3–4 mm communication between the two chambers of the right ventricle is visible. Ao – Aortic valve, LA Left Atrium, RA Right Atrium, TV – Tricuspid Vlave: A. moderator band; B. Hypertrophied band on the anterior wall of RV
Modified high vascular short-axis view, oriented more sagittally than usual. A fibro muscular diaphragm formed by the above described structures, dividing the right ventricle into a high-pressure inflow chamber (RVI) and a low-pressure outflow chamber (RVOT), is visible. The flow through the diaphragm dividing the RV is visualized using color Doppler. Only a 3–4 mm communication between the two chambers of the right ventricle is visible. Ao – Aortic valve, LA Left Atrium, RA Right Atrium, TV – Tricuspid Vlave: A. moderator band; B. Hypertrophied band on the anterior wall of RV

Fig. 3

Visualizations with color Doppler of the flow through the diaphragm dividing the RV; An orifice of only 3–4mm diameter is visible. Designations as in Fig. 2
Visualizations with color Doppler of the flow through the diaphragm dividing the RV; An orifice of only 3–4mm diameter is visible. Designations as in Fig. 2

Fig. 4

The measurement of the flow velocity through the RV narrowing. Calculated maximum pressure gradient of 124 mmHg
The measurement of the flow velocity through the RV narrowing. Calculated maximum pressure gradient of 124 mmHg

Fig. 5

The measurement of the velocity of tricuspid regurgitation – calculated maximum gradient RV-RA of 120 mmHg
The measurement of the velocity of tricuspid regurgitation – calculated maximum gradient RV-RA of 120 mmHg

Fig. 6

The ascending aorta in long-axis view. The walls are hyperechogenic, the proximal part of the ascending aorta seems slightly narrowed
The ascending aorta in long-axis view. The walls are hyperechogenic, the proximal part of the ascending aorta seems slightly narrowed

Fig. 7

Left ventricular long axis: the main parts of the ventricular septum are displaced and connected with a thin band of tissue – a picture suggesting a spontaneous closure of the defect located in this area. IVS: the main body of ventricular septum. CS: conal septum, LVOT: left ventricular outflow tract, RVOT: right ventricular outflow tract, arrowheads point the location of potential VSD
Left ventricular long axis: the main parts of the ventricular septum are displaced and connected with a thin band of tissue – a picture suggesting a spontaneous closure of the defect located in this area. IVS: the main body of ventricular septum. CS: conal septum, LVOT: left ventricular outflow tract, RVOT: right ventricular outflow tract, arrowheads point the location of potential VSD

Fig. 8

Color Doppler of the flow through the RVOT ten months after surgery. A view analogical to the one used in Fig. 2. Only mild turbulences of the flow are visible, no residual structures obstructing RVOT are present
Color Doppler of the flow through the RVOT ten months after surgery. A view analogical to the one used in Fig. 2. Only mild turbulences of the flow are visible, no residual structures obstructing RVOT are present

Fig. 9

Velocity of the flow recorded simultaneously with spectral Doppler does not exceed 1.67 m/s – residual maximum gradient of 11 mmHg
Velocity of the flow recorded simultaneously with spectral Doppler does not exceed 1.67 m/s – residual maximum gradient of 11 mmHg
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