CTICasesAlveolar Capillary Dysplasia with Unroofed Coronary Sinus (Right-to-Left Shunt)
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Imaging findings
Significant right heart enlargement and hypertrophy. Associated lung collapse. An unroofed coronary sinus is present, establishing continuity with the left atrium.
Key takeaways
Alveolar capillary dysplasia is a fatal disease of gas exchange unit maldevelopment. An unroofed coronary sinus can act as an atrial septal defect. In this patient, due to severe pulmonary hypertension and high right heart pressures, the shunt across the unroofed coronary sinus was reversed, functioning as a right-to-left shunt.
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