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Coarctation Of Aorta

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  • 1. Coarctation of Aorta Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery
  • 2. Coarctation of Aorta
    • 1. Definition
    • A congenital narrowing of upper descending thoracic aorta
    • adjacent to the site of attachment of ductus arteriosus
    • 2. History
    • Morgagni : 1st description in 1760
    • Bonnett : postductal & preductal type in 1903
    • Crafoord : 1st coarctation repair in 1944
    • Vorsschulte : prosthetic onlay graft or vertical incision
    • and transverse closure in 1957
    • Waldhausen : subclavian patch aortoplasty in 1966
  • 3. Coarctation of Aorta
    • Developmental factor
    • 1. Underdevelopment or hypoplasia of aortic
    • arch or isthmus
    • Definition of hypoplasia
    • * Proximal arch : 60% of ascending aorta
    • * Distal arch : 50% of ascending aorta
    • * Isthmus : 40% of ascending aorta
    • 2. Presence of ectopic ductal tissue in the aorta
  • 4. Aortic Arch Hypoplasia
    • Definition
    • Hypoplastic arch has higher ratio of elastin lamellae to vessel diameter & increase in collagen and decrease in alpha-actin-positive cell that may hinder the ability of arch to distend .
    • 1. 50% reduction of terminal end of ascending aorta, sometimes,
    • because of small ascending aorta in coarctation, descending
    • thoracic aorta is compared.
    • 2. Transverse arch diameter less than body weight in Kg plus 1
    • 3. Z-value less than –2 or more
  • 5. Coarctation of Aorta
    • Morphology
    • 1. Localized stenosis
    • * More than 50% reduction in cross sectional area
    • * Shelf, projection, infolding of aortic media into the
    • lumen opposite the ductus arteriosus
    • * Usually intimal hypertrophy ( intimal veil ) extends
    • the shelf circumferentially and further narrows the
    • lumen (Rodbard)
    • 2. Tubular hypoplasia
    • * Severe with lesser narrowing
    • * Proximal aortic & arterial wall
    • * Distal aortic arch narrowing
    • * Fetal flow pattern (Rudolph)
  • 6. Coarctation of Aorta
    • Evolution
  • 7. Coarctation of Aorta
    • Pathophysiology
    • Narrowed aorta produces increased left ventricular afterload and wall stress, left ventricular hypertrophy, and congestive heart failure.
    • Systemic perfusion is dependent on the ductal flow and collateralization in severe coarctation
  • 8. Coarctation of Aorta
    • Associated pathology
    • 1. Collateral circulation
    • * Inflow : primary from branches of both subclavian arteries
    • . internal mammary artery . vertebral artery
    • . costocervical trunk . thyrocervical trunk
    • * Outflow : into descending aorta, two pairs of intercostal arteries
    • 2. Aneurysm formation of intercostal arteries
    • * 3rd, & 4th rib notching * rare before 10 years of age
    • 3. Coronary artery dilatation and tortuosity
    • * due to LVH
    • 4. Aortic valve
    • * bicuspid (27-45%) * stenosis ( 6 - 7%)
    • 5. Intracranial aneurysm
    • * berry type intracranial aneurysm in some patients
    • 6. Associated cardiac anomaly
    • * 85% of neonates presenting COA
  • 9. CoA Localized
  • 10. CoA Tubular Hypoplasia PDA Distal arch
  • 11. Coarctation of Aorta
    • Natural history
    • 1. Incidence
    • * 5-8% of CHD (5 per 10000 live births)
    • * Isolated CoA (82% of total CoA) ; male:female = 2:1
    • CoA + VSD 11%, COA + other cardiac anomalies 7%
    • * Complex CoA ; no sex difference
    • 2. Survival of pure CoA
    • * 15% : CHF in neonate or infancy
    • * 85% : survive late childhood without operation
    • * 65% : survive 3rd decade of life (2% at 60 years)
    • 3. Bacterial endocarditis : common in 1 st 5 decades
    • 4. Aortic rupture : 2~3rd decade
    • 5. Intracranial lesion : subarachnoid hemorrhage(cong. Berry
    • aneurysm)
  • 12. Collaterals in CoA
  • 13. Coarctation of Aorta
    • Clinical features & diagnosis
    • 1. Infancy
    • 1) Closure of ductus (7-10 days) produces severe obstruction
    • 2) Ductus arteriosus remains patent - differential cyanosis
    • 3) Associated intracardiac defect - more severe, early onset
    • 4) Degree of collateral circulation
    • 2. Childhood
    • * Asymptomatic without significant associated lesion
    • * Hypertension (90%) * Cardiomegaly (33%)
    • * Rib notching (15%)
    • 3. Adolescence and adult
    • * Hypertension ; very common * Valvar heart disease
    • * Heart failure at 30 years of age
    • 4. Associated syndrome
    • * Turner syndrome (XO) : 2% * Von Recklinghausen’s D
    • * Noonan’s syndrome or congenital rubella
  • 14. Coarctation of Aorta
    • Indications for operation
    • 1. Reduction of luminal diameter greater
    • than 50% at any age
    • 2. Upper body hypertension over 150mmHg
    • in young infant ( not in heart failure )
    • 3. CoA with congestive heart failure
    • at any age
  • 15. Coarctation of Aorta
    • Techniques of operation
    • 1. Subclavian flap aortoplasty
    • Neonate, infant and child up to 10 years
    • 2. End-to-end anastomosis
    • Preferred in any age group
    • * Extended end-to-end anastomosis
    • * Radically extended end-to-end anastomosis
    • 3. Patch angioplasty or graft replacement
  • 16. Prevention of Recoarctation
    • Ideal operative procedure
    • Successfully address transverse arch hypoplasia (if present),
    • Resection of all ductal tissue, and
    • Prevention of residual circumferential scarring at the aortic anastomotic sit.
    • Factors
    • Younger age at operation
    • Presence of aortic arch hypoplasia remain risk factors for recoarctation
  • 17. Regional Cerebral Perfusion
    • Technique
    • We begin full-flow CPB at a calculated baseline of 150 mL · kg–1 · min–1 and, after snare placement on the proximal brachiocephalic vessels, initiate RLFP by reducing pump flow to 50% of baseline.
    • We make further adjustments such that baseline cerebral blood flow velocity as measured by transcranial Doppler and cerebral oximetrics as measured by NIRS are optimally maintained.
    • RLFP provides consistent cerebral circulatory support and that this support is bilateral, despite being applied to the inominate artery.
  • 18. Pediatric Cardiac Surgery
    • Neurologic complications
    • Incidence of 2.3% for overt clinical presentation & up to 60% when sensitive magnetic resonance imaging is applied in heart surgery of infants & children.
    • In control of the arch proximal to the left carotid artery, during COA surgery, this assumes that collateral blood flow and completeness of the circle of Willis allows for a favorable and even distribution of cerebral blood flow.
    • But patients undergoing coarctation repair, proximal occlusion of the aortic arch results in transient but significant impairment in contralateral cerebral oxygen balance
  • 19. Blood Supply to Spinal Cord The most important blood supply to spinal cord comes from spinal artery, a minor supply is from Adamkiewicz artery
  • 20. CoA Exposure
  • 21. CoA LSCA flap
  • 22. CoA Patch Augmentation
  • 23. CoA Subclavian Artery Flap
  • 24. CoA End-to-End Anastomosis
  • 25. CoA Extended end-to-end Anastomosis
  • 26. Coactation of Aorta Resection and Anastomosis
  • 27. Coactation of Aorta Resection & Extended end-to-end Anastomosis
  • 28. Coarctation of Aorta End-to-Side Anastomosis
  • 29. Coarctation of Aorta Enlargement of VSD, Resection of Conal Septum
  • 30. CoA + VSD, One-stage Repair
  • 31. CoA + VSD, One-stage Repair
  • 32. Coarctation of Aorta End-to-Side Anastomosis Opening of Resected Segment
  • 33. Coactation of Aorta
    • Operative results
    • Hospital mortality
    • Causes of early death are
    • acute and chronic cardiac failure or severe
    • pulmonary insufficiency
    • Incremental risk factor for death
    • 1) Older age
    • 2) Hypoplastic left heart class
    • 3) Techniques of operation
  • 34. Coactation of Aorta
    • Operative results
    • Mobidity
    • 1) Paraplegia (0.2 ~ 1.5%)
    • 2) Hypertension and abdominal pain
    • 3) Persistent or recurrent coarctation
    • - more than 20mmHg
    • - high incidence in young
    • 4) Upper body hypertension without resting gradient
    • - increased vascular activity in the forearm
    • - age at operation is risk factor
    • 5) Late aneurysm formation
    • - higher in onlay patch technique
    • 6) Valvular disease
    • 7) Congestive heart failure with hypertension
    • 8) Bacterial endocarditis
  • 35. Coactation of Aorta
    • Special features of postoperative care
    • 1. Systemic arterial hypertension
    • Usually, but infant or young child doesn’t
    • need to be treated.
    • 2. Abdominal pain
    • Usually mild abdominal discomfort for a few days,
    • and prominent in 5 - 10%.
    • Control hypertension, nasogastric decompression,
    • IV maintain
    • 3. Chylothorax
    • 5%
  • 36. Coactation of Aorta Repair
    • Postoperative hypertension
    • Sealy
    • Altered baroreceptor response with increased excretion of epinephrine or norepinephrine
    • Rocchin
    • Sympathetic nervous system in early phase, and renin-angiotensin system in late phase
  • 37. Coactation of Aorta Repair
    • Paraplegia
    • 1. Duration of spinal cord ischemia
    • 2. Duration of intercostal artery ischemia
    • 3. Intraoperative proximal hypotension
    • 4. Postoperative hypotension
    • 5. Hyperthermia during operation
    • 6. Anastomosis with tension
    • 7. Acidosis in the perioperative periods
  • 38. Coactation of Aorta
    • Special situation & controversies
    • 1. CoA proximal to left subclavian artery
    • * 1% of all COA
    • * reverse subclavian flap
    • * abdominal CoA : 0.5 ~ 2%
    • 2. Mild or moderate coarctation
    • * degenerative change prone to occur
    • 3. Prevention of paraplegia
    • * Collateral circulation, hypothermia(< 45min at 33 deg C)
    • * Descending aortic pressure under 50mmHg after clamp
    • 4. Recurrent coarctation
    • Increased mortality and morbidity
    • 5. CoA with VSD or other anomalies
    • Increased mortality and morbidity
  • 39. Coactation of Aorta
    • Balloon dilatation
    • The role of balloon dilatation is controversial because of early restenosis, the need for multiple interventions, potential limb ischemia, and the increased risk of aneurysm formation
    • The mechanism for early restenosis in neonates may be related to multiple factors including ductal tissue constriction or recoil, isthmus hypoplasia, intimal hyperplasia as a result of smooth muscle cell proliferation, and matrix protein production with arterial remodeling are involved in restenosis

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