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AMA Category 1 Credit for Ohysicians Only.
ASRT CME Not Available
Case-of-the-Week Information
The Case-of-the-Month is a presentation of one or more video loops to present an important topic in echocardiography. Please reveiw the image or video loops and then answer the questions below. After you have answered the questions you can view the explanation and obtain CME credit (if available).

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Loop Text Key  
Preop Preop TEE or TTE
Intraop An Intraop TEE
IntraopPreOp Intraop TEE before Operation
IntraopPostOp Intraop TEE after Operation
IntraopEnd Intraop TEE at End of Anesthesia
Post Op Postop TEE or TTE
Case of the Month Information Table
IntraopEnd is usually a TEE after the Operation and after some event occured to show a change in the TEE
Crazy Conduits and More
Case#: 21
AMA CME Units: 0.25 Units
Estimated Time: 15 minutes
CME Requirements: Active CME Session and must be a paid, active subscriber to iTEE or advanced TEE AND have enough CME credits in your bank. AMA CME ONLY.
Presentation: 28 yo female with history of Noonan's Syndrome, severe tricuspid regurgitation and an atrial septal defect (left to right shunt ratio 1.6). She presents for tricuspid valve repair/replacement and closure of the atrial septal defect. She has been having increasing shortness of breath.

What is your assessment of the echocardiographic study?
Please review the cause of her tricuspid regurgitation.
Please review the cause of her left to right shunt.
Case Discussion/CME Questions
The echocardiographic study shows Ebstein's anomaly, perisistent left superior vena cava, and parital anomalous pulmonary venous return via the left superior vena cava.

The tricuspid valve insertion point is more than 15 mm from the base of the heart where the mitral valve annlulus is located. In fact, the insertion point is about 3 cm from the mitral valve insertion point. The septal leaftlet of the tricupid vale inserts in the septal wall of the heart. On the views of the left ventricle the coronary sinus is enlarged and a persistent left superior vena cava is present on the upper esophageal views as well as some of the views of the tricuspid valve. The coronary sinus is markedly enlarged. A bubble test shows the right atrium filling from the coronary sinus (when injected into a left arm IV) and no bubbles made it to the left atrium.  A left to right shunt was not present on the bubble study as evidenced by the absence of a negative contrast jet in the right atrium. Therefore, an anomalous pulmonary venous return must be present as a rule out diagnosis. The tricuspid regurgitation was severe. The pulmonary and aortic regurgitation was mild.

Ebstein's anomaly is a rare heart defect where tricuspid valve is located in the right ventricle. A significant portion of the right ventricle base become 'atrialized'. The leaflets are often larger and may obstruct flow into the RVOT and pulmonary artery leading to right sided congestive heart failure signs and symptoms. In most cases an atrial septal defect is present leading to a right-to-left shunt because of the higher right sided pressures. Ebstein's anomaly has been associated with multiple other cardiac congenital lesions such as atrial septal defect, persistent left superior vena cava, anomalous pulmonary venous return, bicuspid pulmonary valve, bicuspid aortic valve, patent foramen ovale, pulmonary stenosis, pulmonary atresia, ventricular septal defect and more.

Repair of the tricuspid valve is difficult because the whole valve will need to be excised and reimplanted. Nagdyman used the two largest leaflets and made a bicuspid tricuspid valve and had about a 10% mortality rate. All of the patients who died were > 50 y.o. and HYHA Class III or IV. Improvement in the NYHA class occurred in most of the patients.

van Noord used 3D echocardiography to visualize the septal leaflet and the RVOT and to find a bicuspid pulmonary valve. Using 3D he was able to locate and visualize the areas where the leaflets exhibited malcoaptation. Hharucha used 3D echocardiography to differential between Ebstein's anomaly and tricuspid dysplasia.

Sometimes the repair may involve vertical plication and the coronary blood flow to the right ventricle may be affected. The triangle of Koch containing the AV node may be affected during the repair or replacement of the tricuspid valve.

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