What is it about?
We present patient with Partial Anomalous Pulmonary Venous Connection (PAPVC) in form of Scimitar syndrome. PAPVC is a rare congenital heart defect where some of the pulmonary veins drain into the right atrium either directly or indirectly through its venous tributaries.
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Why is it important?
The scimitar vein can often be visible on a plain chest X‑ray at the right side of the heart silhouette, as a structure resembling in shape a Turkish sword used in the times of the Ottoman Empire – a scimitar. Management depends in great deal on the magnitude and the effects of the left‑to‑right shunt. Patients with a significant shunt resulting with the right ventricular volume overload and the right sided heart dilation are candidates for surgical repair.
Perspectives
There are no widely accepted indications for surgical repair. Current European Society of Cardiology Guidelines for the management of grown‑up congenital heart disease do not specifically address PAPVC.
Leszek Drabik
Uniwersytet Jagiellonski w Krakowie
Read the Original
This page is a summary of: Partial anomalous pulmonary venous connection (RCD code: II-3C.0), Journal of Rare Cardiovascular Diseases, January 2013, SoftQ Sp. z.o.o.,
DOI: 10.20418/jrcd.vol1no2.81.
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