Systemic Venous Anomalies in Patients with Pulmonary Atresia: A CT angiographic Study


Sofia Sabouri 1 , Mohammad Ali Karimi 1 , * , Hadi Mahdavirad 1

1 Department of Radiology, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, IR Iran

How to Cite: Sabouri S, Karimi M A, Mahdavirad H. Systemic Venous Anomalies in Patients with Pulmonary Atresia: A CT angiographic Study, Iran J Radiol. Online ahead of Print ; 11(30th Iranian Congress of Radiology):e21433. doi: 10.5812/iranjradiol.21433.


Iranian Journal of Radiology: 11 (30th Iranian Congress of Radiology); e21433
Published Online: February 28, 2014
Article Type: Research Article


Background: Associated anomalies in pulmonary atresia (PA) result in poor prognosis and more complicated surgeries.

Objectives: There is no study about the prevalence of systemic venous anomalies (SVAs) in patients with PA. The aim of this study was to determine the types and prevalence of systemic venous anomalies in patients with PA by CT angiography.

Patients and Methods: CT angiography images of 90 patients with PA (mean age: 6.6 years; 60% male) were reviewed by a cardiovascular radiologist and types and frequencies of SVAs were determined.

Results: PA was isolated in (2.2%), and associated with VSD in (42.2%), large VSD or single ventricle in (12.2%), or complex cardiac anomalies in (43.3%) of cases. The frequencies of SVC, IVC, innominate vein, Azygos and hepatic veins anomalies were 73.3%, 47.8%, 40%, 17.8%, and 16.7%, respectively. The most common type (34.4%) of SVC anomalies was bilateral SVC with drainage of RSVC to right-sided atrium and LSVC to coronary sinus to right-sided atrium. The most common type (16.7%) of IVC anomalies was left IVC (LIVC) to left atrium, followed by Interrupted RIVC with Azygos continuation to RSVC (9%). Innominate vein was absent in 39% of cases and retroaortic innominate vein was seen in one patient. IVC continuation with Azygos and left Azygos to LSVC were seen in 16.7% and 15.6% of cases, respectively. All 15 cases of anomalous hepatic veins, including separate entry to right (n=11) or left (n=4) -sided atrium, were in association with IVC anomalies. In patients with PA and complex cardiac anomalies, IVC anomalies were significantly higher than other types of PA (P = 0.001).

Conclusions: SVAs are common in pulmonary atresia; SVC anomalies are seen in more than two-third and IVC anomalies are seen in about half of these patients. Familiarity with SVAs associated with PA is important for detailed interpretation of CT angiographies of these patients and surgery planning.

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