Partial anomalous pulmonary venous connection (RCD code: II-3C.0)

Leszek Wrotniak, Grzegorz Kopeć, Lidia Tomkiewicz‑Pająk, Leszek Drabik, Anna Kabłak‑Ziembicka, Tadeusz Przewłocki, Piotr Podolec

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A 48-year-old woman was admitted to our institution with a year’s history of uncharacteristic chest pain episodes and decrease in exercise tolerance. A CT lung scan performed nine months earlier revealed an anomalous vein arising from the right lower pulmonary lobe and draining into the inferior vena cava. A chest radiogram performed 2 months prior to the admission showed a curved shape adjacent to the right heart border. Right heart catheterization revealed: normal systolic, diastolic and mean pulmonary artery pressures, normal pulmonary resistance and insignificant shunt volume. Pulmonary artery angiography demonstrated an anastomotic vessel between the aberrant pulmonary vein and the right upper pulmonary vein. The patient was diagnosed with Partial Anomalous Pulmonary Venous Connection in form of Scimitar syndrome. Given a mild character of symptoms, no signs of the right-sided heart volume overload, insignificant shunt fraction and normal pulmonary circulation parameters, the patient was qualified to a conservative treatment with a close medical observation. JRCD2013; 1 (2): 39–41

Key words: partial anomalous pulmonary venous connection, scimitar syndrome, left-to-right shunt, pulmonary hypertension


partial anomalous pulmonary venous connection; scimitar syndrome; left-to-right shunt; pulmonary hypertension


Healey JE Jr. An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Surg 1952; 23: 433.

Haramati LB, Moche IE, Rivera VT, et al. Computed tomography of partial anomalous pulmonary venous connection in adults. J Comput Assist Tomogr 20 03; 27: 743–749.

Ho ML, Bhalla S, Bierhals A, et al. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging 2009; 24: 89–95.

Alsoufi B, Cai S, Van Arsdell GS, et al. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg. 2007; 84: 2020–2026.

Vida VL, Padalino MA, Boccuzzo G, et al. Scimitar Syndrome: A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study. Circulation. 2010; 122: 1159–1166.

Michael A. Gatzoulis, Gary D. Webb, Piers E. F. Daubeney,. Diagnosis and Management of Adult Congenital Heart Disease. Churchill Livingstone; 2 edition.



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