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2015-07-07-22 Answer to the case of the week #399 © Kamel www.TheFetus.net 


Answer to the case of the week #399

July 16, 2015 - July 30, 2015 

Rasha Kamel, MD1; Islam Badr, M.Sc.2; Sameh Abdel Latif Abdel Salam, M.Sc.3

1 Professor of obstetrics & Gynecology, Cairo University, Egypt;
2 Fetal medicine unit, Cairo university, Egypt;
3 Radiodiagnosis department, Kasr Alainy teaching hospitals, Cairo university, Egypt.

 

Case report


22 years old woman G2, P1 was referred to us at the 37th week of gestation due to abnormal four chamber view of the heart. No history of medical diseases or familial conditions.

Ultrasound examination revealed the following findings:

Images 1, 2, 3, and video 1: Diastolic and systolic frames show cardiomegaly, aortic overriding and hypertrophy of myocardium. Video 1: shows cardiomegaly with hypertrophy of the myocardium, aortic overriding with huge aneurismal dilatation of the main pulmonary trunk, the pulmonary valve annulus is hypoplastic with rudimentary valve. The great vessels are normally related with anterior displacement of the aorta.

 

 

Images 4, 5: the images shows pulmonary artery (Pulm) arising from the right ventricle (RV); stenotic pulmonary valve anulus (PV) with huge aneurismal dilatation of the pulmonary artery (Pulm).

 

Videos 2, 3: shows aortic overriding with normal relation and crossing of the great vessels with anterior displacement of the aorta.

 


Image 6, 7, 8, 9, 10, 11: the images show huge dilatation of the main pulmonary trunk and the pulmonary branches

 

 

 

Videos 4, 5: the video 4 shows normally related great vessels with dilatation of the main pulmonary trunk and the pulmonary branches; the video 5 shows pulmonary artery arising from the right ventricle - hypoplastic pulmonary valve annulus and the aneurismal dilatation is evident.

 


Images 12, 13, and video 6: right parasagittal section showing relation between the aorta and the hugely dilated right pulmonary artery. The anterior displacement of the aorta is evident too.

 



Image 14: Color Doppler showing overriding of the aorta.



Image 15: Pulsed wave Doppler showing both stenosis and regurgitation across the pulmonary valve (Yin Yang flow).



Discussion

The images and videos above revealed:

- huge aneurismal dilatation of the main pulmonary trunk and its major branches with consequent severe compression on fetal lungs causing marked pulmonary hypoplasia;

- arterial duct could not be traced due to its probable agenesis;

- pulmonary valve was seen dysplastic with rudimentary leaflets and small pulmonary annulus showing severe stenosis and regurgitation on Doppler study (Yin Yang flow);

- ascending aorta was seen overriding a large outlet subaortic ventricular septal defect arising from both ventricles through the defect;

- normal arrangement of the great vessels in mediastinum with slight anterior displacement of the ascending aorta in relation to the aneurysmatic dilatation of the main pulmonary trunk;

- evidence of marked right ventricular myocardial hypertrophy with multiple deep myocardial crypts.

Our final diagnosis was Tetralogy of Fallot with absent pulmonary valve syndrome.

The baby was delivered at term but died after one hour despite of intensive care probably due to bronchomalacia.

 

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