One-Stage Repair of Aortic Coarctation & Intracardiac Defects SNU Children’s Hospital One-Stage Repair of Aortic Coarctation & Intracardiac Defects Yong Jin Kim, M.D. Department of Thoracic & Cardiovascular Surgery Seoul National University Hospital Seoul, Korea CoA

Morphology of Coarctation SNU Children’s Hospital Morphology of Coarctation CoA

Repair of CoA with Intracardiac Defects SNU Children’s Hospital Repair of CoA with Intracardiac Defects Controversies still exist about optimal surgical treatment Methods of repair Coarctation repair alone Coarctation repair with PA banding One-stage repair of associated defects CoA

Advantages of One-stage Repair SNU Children’s Hospital Advantages of One-stage Repair Avoid complications of longstanding disease Benefits in the perioperative period Ease of repair in arch hypoplasia Lower recurrence rate Benefits for complete anatomic repair Overall wellbeing in the future development CoA

One-Stage Repair of CoA with Associated Defects SNU Children’s Hospital One-Stage Repair of CoA with Associated Defects The time of CPB, TCA, ACC Relief of LVOT obstruction Residual diseases Residual coarctation Residual subaortic stenosis Residual intracardiac defects CoA

Subaortic Stenosis in Coarctation SNU Children’s Hospital Subaortic Stenosis in Coarctation Reasons of underestimation Presence of nonrestrictive VSD Aortic arch obstruction Hemodynamic status Criteria by anatomic measurement Diastolic ratio of descending aorta to LVOT below 1.0 is indicative , severe below 0.6 LVOT value less than 4-5 mm in neonate CoA

Surgical Technique of Aortic Arch Reconstruction SNU Children’s Hospital Surgical Technique of Aortic Arch Reconstruction Wide mobilization of aorta & arch vessels Careful trimming of all the ductal tissue Elimination of anastomosis to the isthmus beyond the left subclavian artery Extended end-to-end or side anastomosis proximal to arch hypoplasia CoA

SNU Children’s Hospital Operative Procedure Extended end-to-end anastomosis CoA

SNU Children’s Hospital Operative Procedure Extended end-to-side anastomosis CoA

Experience of One-stage Repair SNU Children’s Hospital Experience of One-stage Repair Seoul National University Children’s Hospital CoA

SNU Children’s Hospital Purpose To evaluate the effectiveness of surgical treatment mortality, morbidity and outcome 66 patients who underwent one-stage transsternal repair of coarctation and associated defects. CoA

SNU Children’s Hospital Patient Profiles Duration : Sept. 1989 - Dec. 1999 Number : 66 patients Sex : 40 male, 26 female Age : 67 ± 82 d ( 5 d - 530 d ) Bwt (kg) : 4.1 ± 0.2 Kg (1.8 - 9.8 Kg) CoA

SNU Children’s Hospital Distribution Type of lesion No. of No. of patient tubular hypoplasia Group 1 CoA, minor defects 8 ( 12.1%) 1 (12 %) Group 2 CoA, VSD* 46 ( 69.7%) 33 (72 %) Group 3 CoA, complicated defects 12 ( 18.2%) 6 (50 %) Total 66 (100 %) 40 (61 %) CoA

SNU Children’s Hospital Associated Anomalies in CoA with minor defects (n=8) ASD + PDA 5 Anomalous origin of RPA + PDA 2 ASD + AS (bicuspid AV) 1 CoA

SNU Children’s Hospital Associated Anomalies in CoA with VSD (n=46) PDA 42 ASD 18 Aortic stenosis 2 Coronary artery anomaly 1 Tricuspid valve straddling 1 Congenital tracheal stenosis 1 CoA

SNU Children’s Hospital Types of Isolated VSD n=46 Type of VSD No. of patients Perimembranous 28 with extension 14 with posterior malalignment 14 (6)* Subarterial 17 with subaortic stenosis 3 (3)* Multiple 1 * Enlargement of VSD, resection of conal septum was done CoA

Associated Anomalies in CoA with complicated defects (n=12) SNU Children’s Hospital Associated Anomalies in CoA with complicated defects (n=12) TOF 2 Shone’s syndrome 2 Parachute MV + SAS + supravalvular AS 1 MSR + AS(bicuspid) 1 TGA with VSD 2 DORV with subaortic VSD 1 Single atrium, VSD, systemic venous anomaly 1 Lt SVC with unroofed CS, AS, VSD 2 HLHS 2 CoA

SNU Children’s Hospital Surgical Methods (1) Operative technique : simultaneous repair of CoA & associated defects through the transsternal approach Conduction of CPB Intermittent cold crystalloid or blood cardioplegia Deep hypothermic circulatory arrest CPB time (min) : 131 ± 38 (86 - 335) ACC time (min) : 60 ± 16 (21 - 117) TCA time (min) : 37 ± 14 (20 - 72) CoA

SNU Children’s Hospital Surgical Methods (2) Type of operation No. of patient Patch angioplasty 5 ( 7.6%) R & A* 12 (18.2%) ERAA** 49 (74.2%) Total 66 * R & A = resection & anastomosis ** ERAA = extended end-to-end anastomosis CoA

SNU Children’s Hospital Mortality Group early death late death Gr 1 (n= 8) 0 1 Gr 2 (n=46) 5 ( 10.8 %) 1 Gr 3 (n=12) 2 ( 16.7 %) 2 Total (n=66) 7 ( 10.6 %) 4 CoA

SNU Children’s Hospital Causes of Early Death Pneumonia, sepsis, multiorgan failure (POD #20) Remaining AS & AR, LCO (POD # 8) Residual SAS, myocardial failure (POD # 1) Myocardial failure, Pulm. HT (POD # 1) Myocardial failure, residual SAS (POD # 1) Afterload mismatch, LV failure, Pulm. HT (POD # 0) Mediastinitis, sepsis (POD #11) CoA

Actuarial Survival Rate SNU Children’s Hospital Actuarial Survival Rate 96.6% 94.7% 92.9% CoA

SNU Children’s Hospital Complications n= 66 Complication No.of patient Diaphragmatic palsy 4 Hypoxic encephalopathy 3 Pneumonia 3 Transient seizure 2 Arrhythmia 3 Mediastinitis 2 Chylothorax 2 Pericardial effusion 2 CoA

Risk Factors for Hospital Mortality SNU Children’s Hospital Risk Factors for Hospital Mortality Variables Group Mean or Mortality p-Value Age at Op. survivor 76 ± 88d± 88d 0.055 mortality 28 ± 19d ACC survivor 59 ± 17min 0.390 mortality 67 ± 22min SAS (+) 2/ 8 25.0% 0.877 (-) 9/58 15.5% Complicated defects (+) 4/14 28.6% 0.552 (-) 7/52 13.5% Arch hypoplasia (+) 7/40 17.5% 0.496 (-) 4/26 15.4%

SNU Children’s Hospital Follow-up Results (1) Follow-up Total 59 patients Duration (mo) : 30.4 ± 33.5 ( 8 - 127 ) Late death (4 / 59 survivors, 6.8%) Asphyxia during seizure, respiratory failure CHF, febrile seizure, respiratory failure Intestinal strangulation (malrotation) Pneumonia CoA

SNU Children’s Hospital Follow-up Results (2) Residual coarctation (2/55, 3%) Color Doppler (> v = 2.25m/s), Pr gradient (>20mmHg) Two, borderline degree (interval 12, 32mo) No additional procedure Reoperation (2/55, 3%) Konno operation due to recurrent subaortic stenosis (interval 44mo) Permanent pacemaker insertion due to heart block (interval 7 years) CoA

SNU Children’s Hospital Conclusions One-stage transsternal repair of aortic coarctation & cardiac defects is a good surgical option in selected cases. This approach may be applicable to following conditions ; Patients with little benefits from relief of CoA alone. Size & type of VSD, unlikely to close spontaneously. CoA with minor, major associated defects repaired. CoA with severe hypoplasia of aortic arch. CoA