1. Truncus Arteriosus - Interrupted Aortic Arch Surgical Repair Ralph S. Mosca, M.D. NYU Langone Medical Center New York

2. Anatomy Surg Clin North Am 29:1245-70,1949

3. IAA Am Heart J 58:407-13, 1959 Frequency Type B- 85-90% Type A- 5-15% Type C- <3%

4. Anatomy Echocardiography – Arch anatomy – Degree of Truncal valve stenosis or insufficiency – Coronary artery anatomy – Location and number of VSD’s – Tricuspid valve functional status – Size of atrial defect

5. Pre CPB Anesthetic Care Maintain Myocardial Perfusion Avoid – Hyperoxia – Hypocarbia – Agents that Elevate heart rate Increase myocardia oxygen demand

6. Surgical Goals Separation of pulmonary / systemic circulations Closure of septal defects Establish unobstructed aortic arch continuity Provide unobstructed pulmonary blood flow

7. Conduct of CPB Median Sternotomy Assessment thymic tissue PA’s, Coronaries Encircle RPA Dual arterial cannulation Single RA venous cannulation Encircle LPA CPB gradual cooling > 20 mins 18-20 o C SNP Isofurane +/- LV vent

8. Mobilization Extensive Dissection Truncal Root Ascending Aorta Pulmonary arteries Ductal arch- Desc Ao

9. Sequence of Procedure Myocardial / Circulatory Arrest Removal of PA’s from Truncal Root Repair IAA VSD closure Distal RV-PA connection Proximal RV-PA connection

10. Separation of Truncal Root - Arch Myocardial- Circulatory Arrest DelNido solution Topical ice slush Proposed lines of resection

11. PA Resection * Coronary artery orifice Truncal valve commissure

12. Ascending Aorta / Arch Reconstruction

13. Arch Reconstruction

14. VSD Closure Re-establish CPB Replace X-Clamp * RV incisionVSD closure

15. RV-PA Conduit x

16. Completed Repair

17. Rewarming/ Weaning CPB Gradual to 34.5 o C Avoid LV distension RA monitoring –infusion lines RA pacing wire CPB Wean Inotropes – Milrinone – RA pacing – +/- Low dose epinephrine TEE – VSD – LVOT / RVOT – Needle LV / RV