A Potpourri of cases

Surprise
Issakwisa Habakkuk Mwakyula from Tanzania sent us a case of a 26-year-old woman, a mother of four children, who presented with signs and symptoms of congestive cardiac failure and bluish discoloration of the lips, five weeks after spontaneous vaginal delivery (she delivered twins). The referral note from a tertiary hospital suggested postpartum cardiomyopathy. Is this diagnosis correct? Here is the echo:
 

Clearly, the right heart is enlarged and there is a large pericardial effusion. Is that all? Can you see that the leaflets of the tricuspid valve are in abnormal position? If you use color you will see that the origin of the TR jet is far within the apex.

 
This means that the TV is displaced toward the apex. This is a typical case of Ebstein's anomaly, a congenital abnormality in which the tricuspid valve is “dysplastic” and is located too far in the apical aspect. A rather large part of the right ventricle is “atrialized”. By the way, Issakwisa is an "old friend" of 123sonography. If you want to read more about him and the medical problems he is confronted with, check out our entry entitled: the African beauty and its beast. So a big surprise: the symptoms of the patient were not due to “peripartal cardiomyopathy”, but right heart failure and tricuspid regurgitation following Ebstein´s anomaly. Pregnancy and the large pericardial effusion certainly contributed to the problem. The only thing we had to do now was rule out an atrial septal defect, which is frequently associated with Ebstein's anomaly.
 
Clot in a sack
Aris Androulakis from Athens presented a patient with a unique thrombus. The patient, a 77-year-old woman, came to their echo lab 5 months after she had experienced an anterior myocardial infarction.
 

 
A very impressive apical thrombus, I would say. It has a jelly-like appearance. One can see the thrombus wiggle around like a pudding. The liquefied thrombus appears to be contained in a thin “sack” that holds the clot together. The follow-up study after anticoagulation therapy confirmed that it was a thrombus. It is now “almost” gone.

 
What is really inside?
Muhammad Asim Rana from the King Saud Medical City 
in Riyadh presented the stunning echo of a 56-year-old diabetic woman with fever, hypotension, tachycardia and shortness of breath. Her echo revealed a large pericardial effusion with tamponade.
 

 
On the following clip you also see the inversion of the free right atrial wall caused by the rise in intrapericardial pressure.
 

 
The RV also seems a little compressed. The most remarkable aspect is that, even though the effusion suggests a serous fluid (no fibrin), pericardiocentesis revealed pure pus.
 

Pretty impressive, 17 syringes filled with pure puss.

 
Massive mass
Pavel Kozlov from St. Petersburg in Russia presented a case of 50-year-old woman with a huge mass in the right heart that was first diagnosed 14 years ago. Interestingly, she had been asymptomatic all these years. Now, however, she developed syncope.
 

 
The mass was attached to the tricuspid valve and caused not only inflow, but also outflow tract obstruction. This can be seen on the following clips. 

 
Here is the CW-Doppler spectrum across the right ventricular outflow tract showing elevated flow velocity and, consequently, RVOT obstruction. No wonder she had an episode of syncope.

Elevated flow velocity (3.5m/s) suggesting right ventricular
flow obstruction.

 
What type of tumor is it? My notion is: if a tumor in the heart has been present for more than 14 years and the patient is still doing fine, it must be a slow-growing tumor. Thus, it is most likely not a malignancy. Pavel provided us with the answer: histology performed after surgical removal proved that the mass was indeed benign – it was a simple (but rather large) myxoma.
 

 
Resected (huge) surgical myxoma.

 
My thoughts
It always fascinates me how diverse echo is and how much there still is to learn. Were you aware of the effects of pregnancy on patients with Ebstein's anomaly - that a thrombus may look like a pudding in a sack, a pericardial effusion might look “clear” on echo but still be pure pus? Or that a mass appearing malignant at first sight turns out to be a myxoma that obstructs the right ventricular inflow as well as outflow tract? I am very grateful to all of our contestant winners for providing these new insights to me and the rest of our community. Give them a hand and post your comments below!

Best,
Thomas
PS: If you want to read more from the winners of our 123sonography case contest, check out the cases by Marei Tarek and Jose Carlos Moreno Samos