Isolated hypoplasia of the right ventricle is a rare congenital anomaly. The complex defect comprises also a communication between the atria in the form of an oval window or secondary atrial septal defect. The possibility of a communication between the atria via the coronary sinus having in such cases an additional orifice in the left atrium cannot be excluded. In cases of isolated right ventricular hypoplasia the right-to-left shunting goes via the interatrial communication. To eliminate the arterial hypoxemia the interatrial communication has to be closed. When an isolated hypoplasia of the right ventricle is combined with a communication of the coronary sinus with the left atrium, the coronary sinus orifice in the right atrium may be closed by suturing.
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