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ASRT CME is not available.
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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 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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Cleveland Clinic Pulmonary Stenosis
Surgical management of infants with isolated supravalvular pulmonary stenosis: case reports.
A case of left main systolic compression caused by a dilated pulmonary artery in a patient with congenital pulmonic stenosis.
Long-term outcomes of intraoperative pulmonary artery stent placement for congenital heart disease.
A case of isolated peripheral pulmonary artery branch stenosis associated with multiple pulmonary artery aneurysms.
Congenital rubella syndrome with autistic disorder.
Pulmonary artery stents: long-term follow-up.
Congenital pulmonary artery stenoses masquerading as chronic thromboembolic disease.
An update on cardiovascular malformations in congenital rubella syndrome.