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Aortopulmonary Window

Aortopulmonary Window. Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery. Aortopulmonary Window. Definition A rare congenital heart defect with round, oval, or sometimes spiral opening between ascending aorta

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Aortopulmonary Window

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  1. Aortopulmonary Window Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

  2. Aortopulmonary Window • Definition • A rare congenital heart defect with round, oval, or • sometimes spiral opening between ascending aorta • and pulmonary artery resulting from abnormal • septation of the truncus arteriosus into the aorta • and pulmonary artery • * Aortopulmonary fistula • * Aortic septal defect • * Aorticopulmonary septal defect • * Aortopulmonary fenestration

  3. Aortopulmonary Window • 1. History • . Elliotson : 1st report in 1830 • . Dodds and Hoyle : 1st clinical Dx. in 1949 • . Gross : Successful ligation in 1952 • 2. Classification • . Type I : Between posteromedial wall of ascending • aorta and lateral wall of MPA • . Type II : Between posterior wall of ascending aorta • and origin of RPA • . Type III : Anomalous origin of RPA from postero- • lateral wall of ascending aorta

  4. Pathophysiology of AP Window • A large, nonrestrictive communication exists between the proximal ascending aorta and the main pulmonary artery, resulting in a high-flow arterial level left-to-right shunt • This leads to pulmonary overcirculation, and rapidly progressive congestive heart failure, pulmonary hypertension.

  5. Types of AP Window

  6. Aortopulmonary Window

  7. Aortopulmonary Window

  8. Pathology of AP Window • 1. Embryology and anatomy • . Failure of fusion or malalignment of conotruncal ridges • . Single large defect, rarely multiple • . Aortic origin of Rt. pulmonary artery • . Anomalous origin of Rt. & rarely Lt. coronary artery from PA • 2. Associated anomalies • . IAA, VSD, TOF, PDA, TGA, SubAS • 3. Pathophysiology • . Large left to right shunt • . CHF, pulmonary hypertension

  9. Clinical Features & Diagnostic Criteria • 1. Clinical features • . Incidence is 0.15% of CHD • . Defect is small in 10% • . No tendency to close spontaneously • . Symptoms and signs of CHD in early life • . Systolic or continuous murmur • . Early pulmonary vascular disease • 2. Diagnosis • . Two dimensional echocardiography • . Cardiac catheterization & aortography • 3. Differential diagnosis • . Large PDA, Truncus arteriosus, VSD+AR • Ruptured aneurysm of the sinus Valsalva

  10. Operative Indications & Techniques • 1. Indications • . Elective repair is advised before 3 months of age. • . Symptomatic infants may be operated on promptly. • 2. Techniques • . Division by direct suture • . Patch closure

  11. Operative Results of AP Window • 1. Mortality : low • 2. Time-related survivorship : good • The probability of surgical cure will be dependent • on the age at operation and level of pulmonary • vascular resistance. • 3. Late result • * Supravalvular pulmonary stenosis • * Subvalvar PS or supravalvar AS rarely

  12. Anomalous Origin of Right Pulmonary Artery from Ascending Aorta • Rare congenital lesion with a high mortality and morbidity • The natural history of this lesion is that of progressive heart failure with a 70% mortality rate at 1 year compared with an 84% survival rate at 1 year with surgical repair • Early surgical repair is now the standard of treatment to prevent the deleterious effects of pulmonary overcirculation and irreversible changes.

  13. Anomalous Origin of RPA from AA (A, B) Anomalous right pulmonary artery (RPA) is detached from aorta, and a double trapdoor is created on main pulmonary artery. (C, D) Anastomosis of RPA is performed. (E) Completed repair.

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