Eisenmenger syndrome usually arises when a large intra/extra-cardiac connection, such as a VSD (as in figure) is left unrepaired. Initially, the blood flows from the left to the right side of the heart. Over time, the pulmonary vascular resistance can rise to exceed the systemic vascular resistance and then the flow through the defect reverses; this results in cyanosis, erythrocytosis and a variety of consequences, as shown. | Celermajer, D. (2017). Eisenmenger syndrome: a rare malady that continues to fascinate. European Heart Journal, 38, 2068–2069.

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