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2013-10-12-09 Case of the week #356 © Krasnov 


Answer to the case of the week #356

October 24, 2013 - November 07, 2013


Alexander Krasnov, MD*; Andrew Averyanov, MD*; Irina Glazkova, MD*; Andrew Kurkevich, MD**; Larisa Golovakha, MD*; Anatoliy Malov, MD*** .

*    Donetsk`s Regional Specialized Center of Medical Genetics and Prenatal Diagnostics, Ukraine;
**  Scientific and Practical Medical Center of Pediatric Cardiology and Cardiac Surgery, Ukraine;
*** Donetsk National Medical University, Ukraine.

Case report

A 31-year old woman (G4P1) presented to our center at 12 weeks of pregnancy. Her family history was unremarkable and biochemical screening in the first trimester was normal (PAPP-A -0,93 MoM, Beta-hCG-0,56 MoM).

Our initial ultrasonographic examination raised suspicion of a congenital heart defect. Anatomical changes of the ventricles and major vessels were similar to those seen in tetralogy of Fallot.

Second ultrasonographic examination was carried out at 21 weeks and revealed aorto-left-ventricular tunnel (see schematic drawings 1, 2) with dysplastic monocuspid aortic valve.

The parents opted for the termination of the pregnancy. Following 
histopathological examination confirmed our diagnosis.

Drawings 1, 2: The drawings show principle of the aorto-ventricular tunnel - the tunnel represents a paravalvar communication between the aorta and the left ventricle (LV) and connects the ascending aorta above the sinotubular junction to the left (most of the cases) or right ventricle.

RA - right atrium; RV - right ventricle; LA - left atrium; LV - left ventricle; Ao - aorta; PA - pulmonary artery; green arrow passes via the aorto-ventricular tunnel connecting the aorta and left ventricle.


Images 1-4:12 weeks of pregnancy; the images show normal nuchal translucency (NT) - image 1; enlargement of the left ventricle of the heart - image 2; small pulmonary artery and large aorta - image 3, 4.



Videos 1, 2: 12 weeks of gestation; video 1 shows enlargement of the left ventricle of the heart; video 2 shows turbulent flow within the aorta.


Images 5-6: 21 weeks of pregnancy; the images show four-chamber view of the heart with cardiomegaly.


Images 7, 8: 21 weeks of pregnancy; the images show pulmonary artery (PA) and pulsed Doppler flow within the PA.


Images 9, 10, 11: 21 weeks of pregnancy; the images show left outflow tract of the heart - stenotic part of the aorta at the level of dysplastic semilunar valves and enlargement of the ascending aorta can be seen. The image 11 shows pulse Doppler flow within the aorta.


Image 12: 21 weeks of pregnancy; the image shows 3-vessel view of the heart with dilated aorta.

Images 13-17: 21 weeks of pregnancy; the image 13 shows ascending aorta with parallel aorto-ventricular tunnel (calipers are placed within the tunnel). Images 14-17 show color Doppler and pulse Doppler turbulent flow within the aorta and aorto-ventricular tunnel.



Videos 3, 4, and 5: The video 3 shows dilated left ventricle at the level of the four-chamber view of the heart; the video 4 shows ascending aorta with narrow part at the level of the dysplastic semilunar valve, bypassed by the aorto-ventricular tunnel, and dilated ascending part behind the valve. The video 5 represents a sweep encompassing four-chamber view, five-chamber view and three-vessel view of the heart. Initial part of the 
aorta is narrowed at the level of the dysplastic semilunar valve bypassing by the aorto-ventricular tunnel and the ascending part distal to semilunar valve is enlarged.


Videos 6, 7, and 8: the videos show color and pulsed Doppler flows at the level of the aorto-left ventricular tunnel. To and fro movement of the blood via the tunnel is well depicted by color Doppler in the videos 7 and 8.


Images 18-21: The images show fetal heart during histopathological examination. A probe is placed within the aorto-left-ventricular tunnel on the image 20. The image 21 shows the probe placed in the aortic root under monocuspid valve.



Aorto-left-ventricular (or aorto-right-ventricular) tunnel is an extremely rare heart defect in which there is a direct connection between the left (more often) or right ventricle and ascending aorta, bypassing the aortic valve. The incidence of aorto-left ventricular tunnel has been variably estimated to be around 0.1% of congenital heart defects. Associated anomalies, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases.

The diagnosis is usually suspected after 18 weeks of gestation, but certain suspicion of the aorto-ventricular tunnel may arise even during the first trimester screening as it was in our case.

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