Cardiac Case 9/15/07. Coarctation of the Aorta Congenital narrowing of the thoracic aorta; typically distal to the left subclavian artery. M:F – 2:1.

Cardiac Case

9/15/07

Coarctation of the Aorta

• Congenital narrowing of the thoracic aorta; typically distal to the left subclavian artery.

• M:F – 2:1

• 6-8% in pts with congenital heart disease

• Associated anomalies– Bicuspid aortic valve (50-60%); VSD (25%);

PDA; TGA

• Increased incidence in Turner’s (10-15%)

Pathophysiology

• 1. Hemodynamic Theory– Lesions which decrease blood flow through left

ventricular outflow in the fetus causes a decrease flow across the aortic isthmus

– e.g. assoc anomalies – VSD, biscuspid aortic valve, LV outflow obstruction

• 2. Ductal Sling Theory– Secondary to migration of ductus smooth

muscle cells into the periductal aorta• Ductal smooth muscle cells seen on histo

• Arches 1 and 2– Stapedial Artery

• Arch 3– Internal Carotid Arteries

•Arch 4– right arch forms the right subclavian

artery – left arch constitutes the arch of the aorta

between the origin of the left carotid artery and the termination of the ductus arteriosus.

• Arch 5– disappears

•Arch 6– right arch disappears

– left arch gives off the pulmonary arteries and forms the ductus arteriosus

Indications for treatment

• Decrease in lumen diameter by > 50% at the site of coarctation

and/or

• Pressure gradient > 20 mm Hg at rest

Treatment Options

• Surgery– End to End (extended) Anastamosis– Bypass graft – Left subclavian flap aortoplasty– Prosthetic patch aortoplasty

• Angioplasty

• Angioplasty with Stent

Complications of Surgical Repair

• Post-op paradoxical hypertension

• Recurrent laryngeal nerve or phrenic nerve inury

• Steal phenomenon (w/ subclavian flap)

• Aneurysm (w/ patch aortoplasty)

• Recoarctation (5-10%)

Complications of Stents• Technical

– Stent migration or fracture; baloon rupture, overlap of brachiocephalic vessels

• Aortic– Intimal tears, dissection, rupture

• Peripheral– CVA, peripheral emboli, access vessel injuries

• Recoarctation (less than angioplasty alone)

Other Late Complications

• Vascular remodeling resulting in systemic hypertension– Impaired vasoreactivity– Increased intima-media thickness– Possibly associated with arch type

Recommendations

• < 6 months– Surgery

• 6 mo – 5 yrs (<25kg)– Surgery or Angioplasty– ?angioplasty for recurrent coarctation

• > 5yrs (>25kg)– Stent

References

• Ou, P., Celermajer, D., et al. Vascular Remodeling After Successful Repair of Coarctation. Journal of the American College of Cardiology. Vol 49, No. 8, 2007.

• Agarwala, B., Bacha, E., et al. Management of Coarctation of the Aorta. UpToDate 2007.

• Shih, M., Tholpady, A., et al. Surgical and Endovascular Repair of Aortic Coarctation: Normal Findings and Appearance of Complications on CT Angiography and MR angiography. American Journal of Radiology, Vol 187, 2006.

• Abbruzzese, P., Aidala, E. Aortic Coarctation: An overview. Journal of Cardiovascular Medicine 2007, 8:123-128.

• Golden, A., Hellenbrand, W., Coarctation of the Aorta: Stenting in Children and Adults. Catheterization and Cardiovascular Interventions. 69: 289-299, 2007.