1. Cardiac Case 9/15/07

4. 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%)

6. 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

8. 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 of the aortacarotid arteryductus arteriosus Arch 5 –disappears Arch 6 –right arch disappears –left arch gives off the pulmonary arteries and forms the ductus arteriosuspulmonary arteriesductus arteriosus

9. Indications for treatment Decrease in lumen diameter by > 50% at the site of coarctation and/or Pressure gradient > 20 mm Hg at rest

10. Treatment Options Surgery –End to End (extended) Anastamosis –Bypass graft –Left subclavian flap aortoplasty –Prosthetic patch aortoplasty Angioplasty Angioplasty with Stent

11. 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%)

12. 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)

13. Other Late Complications Vascular remodeling resulting in systemic hypertension –Impaired vasoreactivity –Increased intima-media thickness –Possibly associated with arch type

16. Recommendations < 6 months –Surgery 6 mo – 5 yrs (<25kg) –Surgery or Angioplasty –?angioplasty for recurrent coarctation > 5yrs (>25kg) –Stent

17. 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.