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

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Presentation transcript:

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

Anatomy Surg Clin North Am 29: ,1949

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

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

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

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

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 o C SNP Isofurane +/- LV vent

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

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

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

PA Resection * Coronary artery orifice Truncal valve commissure

Ascending Aorta / Arch Reconstruction

Arch Reconstruction

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

RV-PA Conduit x

Completed Repair

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