Case of the Week #603

Case of the Week #603

Halil Mesut
 on 05/15/2024
 on Wednesday, May 15th, 2024
I think the primary cause in this case is "unguarded tricupid valve". This is a variant of tricuspid valve dysplasia. There is dysplasia in the cord tendineae or papillary muscles. and the majority of them are associated with pulmonary atresia. In these cases, the right ventricle and atrium are severely dilated. There is severe regurgitation in the valve. However, in cases of pulmonary atresia without VSD, the right ventricular cavity is normal or small in size, the ventricular wall is thick and fibroelastosis is observed in the endocardium.
#1 - 
05/16/2024
Murat Cagan said I also agree with Dr Mesut. I do not think that this fetus's primary pathology is pulmonary atresia. This case is characterized by enlarged right chambers due to tricuspid dysplasia. Pulmonary artery progressed to hypoplasia/atresia due to decreased pressure and volume transmitted from the right ventricle to the pulmonary artery. One of the acceptable answers should be tricuspid dysplasia and giant/enlarged right atrium.
#2 - 
05/19/2024
Hien Nguyen Van said I also think that primary pathology is tricuspid dysplasia
#3 - 
07/10/2024
Frantisek Grochal said Hi guys,
Thank you for your contributive opinions, but it is like a riddle asking “Which came first, the chicken or the egg? “. The mother was first time seen at 37 weeks in advanced stage of the anomaly. I believe that at 20 week the findings would have been clearer and giving us more space for such speculations. The baby was born two days later and was seen by cardiologists of our National Institute for Cardiovascular Diseases. Moreover, the baby has underwent several surgical operations, and both surgeons and cardiologists have come to agreement on the diagnosis of PA-IVS.
If someone said that it was tricuspid dysplasia, or unguarded tricuspid valve, we think the answer was not complete and we didn’t give him a point. But when someone said it was tricuspid dysplasia (or severe tricuspid regurgitation) with pulmonary atresia, we considered the answer as complete and gave him the point.
It is good that cases like this rises discussions. It means that you think of other differential diagnoses, and your in-depth knowledge of the problematic is wide. We believe that people like you have their own interesting cases in their practice, and we and our solvers will be thankful if you find some time and effort to prepare them as COWs that can be posted at our website. Thank you for your input and remarks and do not hesitate to send us your own interesting cases.

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