Double Aortic Arch with TOF

Double Aortic Arch with TOF and Anorectal

MalformationDepartment of CTVS

P.G.I.M.E.R. & Dr. RML HospitalNew Delhi

Patient2 ½ yr. / Female

Underwent colostomy for ARM in neonatal period.

Referred to RMLH for intracardiac repair prior to colostomy closure.

LT. Kidney congenitally absent. (KFT normal)

Echo & Cath StudyTOF.Adequate sized PAs.Val.+Inf. PS (PG-65).Double Aortic Arch.

CT Cardiac AngioDouble Aortic Arch (Right dominant) with

mild tracheal compression RCCA and RSCA from rt. archLSCA and LCCA from lt. arch.Right sided DTAB/L SVC with no bridging vein

Diagnosis & TreatmentTOF/Inf. PS/MPA stenosis/Confluent and

adequate branch PAs/ No MAPCA or CoA or PDA/ normal coronaries/ Double Aortic Arch (Rt. Arch Dominant)/ B/L SVC.

FS-11, HS-7.5

Sx : Total Correction + Lt. arch division.

Aproach Double Aortic Arch

Isolated Associated with cardiac (Thoracotomy) Anomalies

Backer CL, 1989 (Median Sternotomy) 45 years analysis Zhou Dan etal – 15

cases

Single Stage with 2 incisions- (AIIMS)

S. Talwar, Anubhav Gupta etal – 2 cases (Division of Arch by posterolateral

thoracotomy and Intracardiac repair via sternotomy)

Two stage

B Pankaj et al - I/C repair f/b –Arch division on POD7

(Intracardiac repair via median sternotomy, could not divide Arch, - Divided Arch via thoracotomy later)

Our PlanSingle stage procedure via median

sternotomy

If not able to divide Arch, then Intracardiac Repair first , followed by Division of Arch via thoracotomy in second stage.

Per operative findingsProximal Arch dissected.

CPB to facilitate dissection. RCCA, LCCA, LSCA & Left arch identified.

PDA identified, ligated and divided. 

Intra cardiac Repair

Post Op recovery uneventful

Discharged on POD 4

Follow up – Doing well

Double Aortic Arch with TOF is rare anomaly and based on review of literature and our experience Single stage procedure is Advocated

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