From Fetal Diagnosis to Staged Percutaneous Palliation

Figures

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  Visual Summary. Pathway for Management Decisions in TVD and PVD With an Intact Interventricular Septum Patients presenting with severe tricuspid regurgitation, right heart failure, a small RV, and low anterograde pulmonary flow are directed toward functional univentricular palliation. Conversely, when the pulmonary valve area is adequate, a patent ductus arteriosus is maintained, and good RV and tricuspid valve dimensions are present, a staged strategy aiming for biventricular repair is feasible. The lower panel illustrates Doppler flow profiles after percutaneous pulmonary valvuloplasty and ductal stenting, promoting RV remodeling and adequate pulmonary circulation. DA = ductus arteriosus; HF = heart failure; IVS = interventricular septum; LA = left atrium; PVD = pulmonary valve dysplasia; RA = right atrium; RV = right ventricle; TR = tricuspid regurgitation; TV = tricuspid valve; TVD = tricuspid valve dysplasia.

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  Figure 1. Fetal Echocardiographic Images at 32 Weeks of Gestation Showing Features of Tricuspid Valve Dysplasia and Pulmonary Valve Dysplasia With Intact Interventricular Septum (Top Left) Color Doppler 4-chamber imaging reveals severe tricuspid regurgitation with retrograde flow into the right atrium. (Top Right) Three-vessels and trachea view showing reversed blood flow from the aortic arch to the pulmonary artery. (Bottom Left) Measurement of the hypoplastic bipartite right ventricle area. (Bottom Right) Measurement of the tricuspid valve annulus, with a z-score of −2.41, indicating borderline right ventricular development. AA = aortic arch; AD = descending aorta; DA = ductus arteriosus; PA = pulmonary artery; RA = right atrium; RV = right ventricle; SVC = superior vena cava; T = trachea.

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  Figure 2. Fluoroscopic Images During Staged Percutaneous Palliation (Top Panels) Angiography of the right ventricle showing critical pulmonary valve stenosis with minimal anterograde flow; guidewire crossing the pulmonary valve. (Bottom Left) Balloon pulmonary valvuloplasty using a balloon-to-annulus ratio of 1.3. (Bottom Right) Ductal stenting with a 3.0 × 18 mm stent deployed in the ductus arteriosus to maintain adequate pulmonary blood flow. DA = ductus arteriosus; PV = pulmonary valve.

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  Figure 3. Transthoracic Echocardiography at the Time of Discharge (Top Left) Subcostal 4-chamber view shows a hypoplastic bipartite right ventricle and a dilated right atrium. (Top Right) Color Doppler imaging across the pulmonary valve demonstrates severe stenosis with minimal antegrade flow. (Bottom Left) Color Doppler assessment of the tricuspid valve shows severe to moderate regurgitation. (Bottom Right) Color Doppler of the ductus arteriosus demonstrating shunting. TV = tricuspid valve; other abbreviations as in Figures 1 and 2.

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  Figure 4. Follow-Up Transthoracic Echocardiography at Age 18 Months (Top Panels) Parasternal views showing the stent of the ductus arteriosus, with sustained retrograde flow into the pulmonary arteries. (Bottom Panels) Apical 4-chamber views demonstrating right ventricular growth and reduced tricuspid valve regurgitation, supporting successful right ventricular remodeling and progressive transition toward biventricular circulation. Abbreviations as in Figure 1.