Truncus Arteriosus - Interrupted Aortic ArchSurgical Repair

Ralph S. Mosca, M.D.NYU Langone Medical Center

New York

Anatomy

Surg Clin North Am 29:1245-70,1949

IAA

Am Heart J 58:407-13, 1959

Frequency

Type B- 85-90%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 SternotomyAssessment

thymic tissuePA’s, Coronaries

Encircle RPADual arterial cannulationSingle RA venous cannulationEncircle LPACPB

gradual cooling > 20 mins18-20oCSNPIsofurane

+/- LV vent

Mobilization

Extensive DissectionTruncal RootAscending AortaPulmonary arteriesDuctal 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 orificeTruncal valve commissure

Ascending Aorta / Arch Reconstruction

Arch Reconstruction

VSD Closure

Re-establish CPBReplace X-Clamp

*

RV incision VSD closure

RV-PA Conduit

x

Completed Repair

Rewarming/ Weaning CPB

• Gradual to 34.5oC• 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