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2017-08-26  Case of the week # 455  © Olga Ivanitskaya

Answer to the case of the week # 455
September 28, 2017 - October 12, 2017
Olga Ivanitskaya, MD, Elena Andreeva, MD.

Moscow Regional Research Institute of Obstetrics and Gynecology, Medical-Genetics Department, Moscow, Russia

Case report

A 29-year-old woman (G2P1), with non-contributive history, was referred to our department after her second trimester scan to rule out a cardiac anomaly. The patient’s first child was healthy. No anomalies were detected during the 12 weeks scan and the patients prenatal course was unremarkable.

At 24 weeks we found a VSD, dilated pulmonary artery and aorta with diameter equal to the superior vena cava.
There was a common root of these two vessels with predominant turbulent blood flow from the right ventricle and the normal aortic arch was absent. Diagnosis of truncus arteriosus with interruption of the aortic arch was determined (type A4 according to Van Praagh classification).

Parents decided to continue the pregnancy and diagnosis was confirmed after birth. At the 4th day of life the first stage of surgical correction was performed (narrowing of the right and left pulmonary arteries and placing a stent into the ductus arteriosus 
to maintain it open). Now baby is stable and waiting for the second stage of the surgical treatment.  

Images 1, 2 and 3: 2D images of 4CV and 3VTV showing a common root with dilated pulmonary artery and interrupted aortic arch distal to the left common carotid artery.

Images 4 and 5: Doppler images show turbulent flow in these two vessels.


Videos 1, 2 and 3: common trunk arising from the right ventricle and absent aortic arch.


Image 6: Van Praagh classification includes 4 types (Expert Rev Cardiovasc Ther. 2012;10(12):1497-1516): 

- A1 - there is a short MPA segment of the trunk from which both branch pulmonary arteries arise. 
- A2 - there is no MPA segment and both branch PA come off directly from the truncus. 
- A3 - either the LPA or RPA are absent and collateral vessels perfuse the respective lung. 
- A4 - the aortic arch is interrupted and a large patent ductus arteriosus provides blood flow to the descending aorta, like in our case. 

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