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Interrupted Aortic Arch

Interrupted Aortic Arch. Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery. Interrupted Aortic Arch. 1. Definition A complete luminal & anatomic discontinuity between the two segments of the aortic arch 2. History

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Interrupted Aortic Arch

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  1. Interrupted Aortic Arch Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

  2. Interrupted Aortic Arch • 1. Definition • A complete luminal & anatomic discontinuity between • the two segments of the aortic arch • 2. History • Steidely : 1st description in 1778 • Celoria and Patton : A, B, C type in 1959 • Samson : 1st successful operation • in 1955 • 3. Classification • Type A (40%), Type B (55%), Type C ( 5%)

  3. Pathophysiology of IAA • Luminal interruption between the ascending and descending aorta is found, and distal blood flow is dependent on a patent ductus arteriosus. • Spontaneous ductal closure results in systemic hypoperfusion, metabolic acidosis , and end-organ failure • Associated cardiac anomalies are common, particularly a nonrestrictive VSD, and IAA comprises less than 1% of all CHD

  4. Embryonic Origin of Aortic Arch • 1. Ascending aorta : Embryonic conotruncus • 2. Proximal arch : Embryonic aortic sac • 3. Distal arch : 4th embryonic aortic arch • 4. Isthmus : Junction of 6th embryonic • arch with left dorsal aorta & • 4th embryonic aortic arch

  5. 3 Components of Aortic Arch

  6. Morphology of IAA • 1. Aortic arch • * Anomalies of brachiocephalic vessels are frequent. • * Rare interruption in right aortic arch • 2. Left ventricular outflow anomalies • * Bicuspid in 30-50% • * Aortic and subaortic stenosis • * Aortic ring is frequently small • 3. Coexisting cardiac anomalies • * PDA is almost always • * Large VSD is nearly always • * Truncus arteriosus, aortopulmonary window • 4. Associated syndrome • * DiGeorge’s syndrome (absence of thymic tissue) • - hypocalcemia & immunologic problem (Type B)

  7. Types of IAA

  8. IAA

  9. Clinical Features in IAA • 1. Clinical features • * Critically ill neonate in severe CHF • * Cardiac murmurs are not specific. • * Closing PDA ; anuria & metabolic acidosis • 2. Natural History • * 1~4% of CHD in autopsy ( 1.3% of CHD in infancy ) • * Median age of death : 4~10 days • * 90% death in the 1st. year even though PDA • * Pulmonary vascular disease after one year

  10. DiGeorge’s Syndrome • 1. Complete type • * dysmorphic facial feature • : hypertelorism, carp-shaped mouth, • micrognathia, notched ear pinnae • * hypocalcemia • * Low T lymphocyte CD4 • * IAA • 2. Partial type • * Facial feature with hypocalcemia and IAA in • the absence of any deficit of CD4 T lymphocyte

  11. Berry Syndrome • Interrupted aortic arch • Distal aortopulmonary septal defect • RPA from ascending aorta • Intact ventricular septum

  12. Operation of IAA • 1. Indication • * Urgent operation is advisable, preferably • by a one-stage. • 2. Technique • * Direct anastomosis (open or non-open technique) • * Interposition of graft • * One stage repair • Median sternotomy and circulatory arrest, • IAA and associated anomaly repair

  13. IAA Type B Operative Procedure

  14. Early Operative Results of IAA Mortality & morbidity 1) Early death Acute or subacute heart failure without or with multisystem failure. One stage repair : under 10% IAA repair & second repair : 12-47% mortality • 2) Late • Survival : good • Aortic anastomotic narrowing • Development of subaortic stenosis (44-85%)

  15. Operative Results of IAA • 1. Survival • Early death • Time-related survival • 2. Modes of death • 3. Incremental risk factors for premature death • 1) Coexisting cardiac anomalies • 2) Location of interruption • 3) Preoperative condition • 4) Earlier date of operation • 4. Left ventricular outflow obstruction • 5. Persistent or redeveloped aortic arch obstruction

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