Atrial Appendage Anastomosis in Hypoplastic Left Heart Syndrome With Restrictive Atrial Septum

Figures

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  Figure 1. Fetal Echocardiogram at 20 Weeks, 3 Days (A) A 4-chamber view demonstrating the atrial septum bowing from left to right and a mildly hypoplastic mitral valve (4 mm; Z = −2). The left ventricle (LV) was dilated, with severely diminished function. The aortic valve measured normally (3 mm; Z = −1.7), but was thickened, dysplastic, and stenotic (Video 1). (B) A 3-vessel view demonstrating retrograde flow in the mildly hypoplastic transverse arch (Video 2). (C) and (D) Color and pulse-wave Doppler imaging in the right pulmonary vein (RPV) showed an antegrade-to-reverse velocity time integral ratio (A:R VTI) of 3.1:1 (Video 3). Ao = aorta; DA = descending aorta; LA = left atrium.

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  Figure 2. Fetal Echocardiogram at 34 Weeks, 2 Days (A) A 4-chamber view demonstrating a non–apex-forming dilated, hypertrophied left ventricle with significant endocardial fibroelastosis. The mitral valve measured 9.8 mm (Z = −0.6). The aortic valve measured 3.1 mm (Z = −4.8), peak gradient 90 mm Hg (Video 4). (B) A 3-vessel view demonstrating a hypoplastic aortic arch with retrograde flow in the transverse arch (Video 5). (C) Color Doppler imaging of the mitral valve demonstrating severe mitral regurgitation (peak gradient 67 mm Hg) (Video 6) and (D) pulse-wave Doppler imaging of the left lower pulmonary vein demonstrating and an antegrade-to-reverse velocity time integral ratio (A:R VTI) of 1.5.

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  Figure 3. Intraoperative Atrial Appendage Anastomosis This is also shown in Video 7. RAA = right atrial appendage; LAA = left atrial appendage.

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  Figure 4. Intraoperative Transesophageal Images During Superior Cavopulmonary Surgery The mitral regurgitation, which had been severe in utero and was not addressed at the time of the Norwood operation, was now trivial (arrow). LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.