Gerbode-Type Defects in Adults After Repair of Tetralogy of Fallot: A Case Series

Figures

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  Figure 1. Comparison of normal heart to hearts with variable Gerbode defects. (A) Normal heart. (B) Supravalvular (direct) defect involving membranous portion of septal wall, superior to the septal leaflet of the TV. (C) Infravalvular (indirect) defect involving membranous portion of septal wall, below the septal leaflet. (D) Both supravalvular and infravalvular defect with septal leaflet of TV. Illustration by Jessica Holland 2016, provided under CC-BY-NC-ND 4, and published by Saker et al.4

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  Figure 2. Patient 8. Two-dimensional TTE, apical 4-chamber systolic view (RV focused) without (A) and with (B) color-flow Doppler, demonstrates a mildly dilated right heart, mild to moderate TR (white arrow), and a GtD (yellow arrow). Continuous-wave Doppler spectral display demonstrates a transtricuspid gradient of 41 mm Hg (C) and a maximal gradient between the LV and the RA of 82 mm Hg (D). LA, Left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

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  Figure 3. Patient 6. Two-dimensional TTE, apical 4-chamber view (RV focused) with color-flow Doppler in early (A) and mid-late (B) systole, demonstrates moderate right ventricular dilation, mild TR (white arrow), and a GtD (yellow arrow). Continuous-wave Doppler spectral display demonstrates a transtricuspid gradient of 39 mm Hg (C) and a maximal gradient between the LV and the RA of 84 mm Hg (D). LA, Left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

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  Figure 4. Patient 9. Two-dimensional TTE, apical 4-chamber systolic view (RV focused) without (A) and with (B) color-flow Doppler, demonstrates mild right ventricular dilation and trace TR; parasternal short-axis mid-systolic view (C) demonstrates a GtD (yellow arrow) with continuous-wave Doppler spectral display confirming a gradient between the LVOT and RA up to 100 mm Hg (D). AoR, Aortic root; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

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  Figure 5. Patient 4. Two-dimensional TTE, apical 4-chamber early systolic view without (A) and with (B) color-flow Doppler, demonstrates moderate right ventricular dilation and 2 eccentric jets originating from the LV; one jet is directed toward the right ventricular apex consistent with a residual VSD (green arrow); it is unclear whether the second eccentric jet at the level of the TV (asterisk) represents a residual VSD or a GtD (yellow arrow). Two-dimensional TTE, subcostal, modified short-axis view without (D) and with (E) color-flow Doppler, more clearly demonstrates a GtD with direct connection from the LVOT to the RA (supravalvular type). Continuous-wave Doppler spectral display demonstrates a transtricuspid gradient of 32 mm Hg (C) and a maximal gradient between the LVOT and RA of 117 mm Hg (F). AoR, Aortic root; LA, left atrium; LVOT, left ventricular outflow tract; LV, left ventricle; RA, right atrium; RV, right ventricle.

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  Figure 6. Patient 4. CMR, steady-state free precession cine image, oblique sagittal RVOT view, demonstrates a small GtD with left-to-right flow (red arrow). RA, Right atrium; RV, right ventricle; VSD Patch, ventricular septal defect patch.

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  Figure 7. Graphical abstract documenting late complications after repair of TOF and pitfalls to diagnose a GtD. (A) Illustration of TOF repair with transannular patch from the RVOT across the PV annulus to the main PA. (B) Supravalvular (direct) GtD with direct communication from the LV to the RA (curved arrow), above the septal leaflet of the TV. (C) Two-dimensional TTE, apical 4-chamber systolic view with color-flow Doppler, demonstrates TR (white arrow) and a GtD (yellow arrow). LA, Left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle; RVSP, right ventricular systolic pressure; VSD, ventricular septal defect. ∗VSD patch; Illustration B by Jessica Holland 2016, provided under CC-BY-NC-ND 4.0 and published by Saker et al.4 Illustration A taken from http://www.chd-diagrams.com.