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Case of the Week
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Case-of-the-Week Information
The Case-of-the-Week is a presentation of 8 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 or TTE before Operation
IntraopPostOp Intraop TEE or TTE after Operation
IntraopEnd Intraop TEE or TTE 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
The Ring
Case#: 13
AMA CME Units: 0.25 Units
Estimated Time: 15 minutes. You need more cme units to do this COTW for CME (not required). Purchase CME Credits
Presentation:
77 y.o. male witha history of chest pain, SOB and pitting edema. The cardiac cath showed a 100% RCA, 100% Cx, and a 70% LAD lesion. The patient also had  sleep apnea and pulmonary hypertension. A   systolic ejection murmur was noted over the left upper intercostal area. The patient presents to the operating room for a CABG w/ ECC. An intraoperative TEE is below.

What is the diagnosis? What is the severity of the diagnosis? Calculate the area of the lesion. What are your recommendations, if any, to the surgeon? Are there any associated conditions with the diagnosis?
 
Loops:


Case Discussion/CME Questions
 
The TEE shows severe RAE, RVH, a pulmonary artery ring, a dilated pulmonary artery, severe pulmonary stenosis at the ring, moderate-severe pulmonary regurgitation.

The RVSP is 101 mmHg (TR Vmax 5.02 m/s, PG 101) indicating severe pulmonary hypertension. However, with the stenosis located just above the pulmonary valve the pulmonary artery was severely dilated (PA diameter 4.36-5.68 cm) from post-stenotic dilation.

The pulmonary artery ring cross sectional area was measure by 2D to be 0.7 cm2.

By continutity equation using the PA diameter below the ring as 1.78 cm, and a VTI of 25.8 below the ring and a VTI of 105 above the ring, the ring cross sectional area calculated to be 0.61 cm2.

By velocity ratio using 0.874 m/s, 4.2 m/s and 1.78 cm the cross sectional area was calculated to be 0.51 cm2.

Pulmonary artery rings/stenosis are a rare congenital condition. As the pulmonary artery narrows the pressure in the RV increases resulting in RVH and, possibly, tricuspid regurgitation. Post stenotic pulmonary artery dilation can also occur. If an atrial shunt is present, the increased RV pressure can result in reversal of the shunt from left-to-right to right-to-left. Pulmonary artery stenosis is often found with other congenital conditions such as Tetrology of Fallot, Pulmonary Atresia, Truncus Arteriosus, Pulmonary Valve Stenosis, and Patent Ductus Arteriosus. Pulmonary artery stenosis can also be secondary to congenital rubella syndrome (CRS) (PDA and/or branch pulmonary artery stenosis), Williams syndrome, or iatrogenic from pulmonary artery banding. Pulmonary artery stenosis can occur at the main pulmonary artery trunk or in one of it's branches. Aneurysms of the pulmonary artery may be associated with branch stenosis. If the stenosis occurs in a branch of the pulmonary artery, a V/Q scan will be falsely positive of thromboembolic disease. Treatment for pulmonary artery stenosis includes the following options:

  • Balloon
  • Balloon + Stent
  • Surgical


Balloon with or without stenting may not adequately treat a ring. The elasticity of the pulmonary artery may prevent a good result from the balloon procedure. Stents may increase the risk of thromboembolism in a dilated pulmonary artery. Freedom from reintervention after pulmonary artery stenting was 40% after 10 years, although the patients were very young (median age 1.8 years) at the time of the original stenting. The risk of reintervention increased with a diagnosis of Tetrology of Fallot or Truncus Arteriosus. A dilated pulmonary artery can compress the left main coronary artery. A case report of a dilated pulmonary artery trunk that compressed the left main coronary artery during systole was found on a cardiac catheterization. Dilated or aneurysmal pulmonary arteries can lead to thrombosis and embolism or dissection and hemoptysis. The natural course and the treatment of pulmonary artery aneurysms has not been defined. Most case reports of pulmonary artery aneurysms indicate a treatment for symptomatic aneurysms.

The recommendation was to excise the ring and leave the pulmonary artery and valve alone. The pulmonary valve was relatively normal and once the ring was excised and replaced with a graft, mild pulmonary regurgitation was present. The pulmonary regurgitation that was present on echo was the regurgitation through the ring.
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