CoA

SNU Children’s Hospital

Yong Jin Kim, M.D.

Department of Thoracic & Cardiovascular SurgerySeoul National University Hospital

Seoul, Korea

One-Stage Repair of Aortic Coarctation & Intracardiac Defects

CoA

SNU Children’s Hospital

Morphology of Coarctation

CoA

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

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

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

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

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 ProcedureExtended end-to-end anastomosis

CoA

SNU Children’s Hospital

Operative ProcedureExtended end-to-side anastomosis

CoA

SNU Children’s Hospital

Experience of One-stage Repair

Seoul National University Children’s Hospital

SNU Children’s Hospital

CoA

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

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)

Patient Profiles

SNU Children’s Hospital

CoA

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 Anomaliesin 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 Anomaliesin 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

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

n=46

SNU Children’s Hospital

CoA

Associated Anomaliesin 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)

SNU Children’s Hospital

CoA

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

Surgical Methods (2)

CoA

SNU Children’s Hospital

Mortality

Group early death late death

Gr 1 (n= 8) 0 1Gr 2 (n=46) 5 ( 10.8 %) 1Gr 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

SNU Children’s Hospital

Actuarial Survival Rate

Months

140120100806040200

Cum

ula

tive S

urv

ival

1.00

.90

.80

.70

Survival Function

Censored

96.6%94.7%

92.9%

CoA

SNU Children’s Hospital

Complications

Complication No.of patient

Diaphragmatic palsy 4Hypoxic encephalopathy 3Pneumonia 3Transient seizure 2Arrhythmia 3Mediastinitis 2Chylothorax 2Pericardial effusion 2

n= 66

Risk Factors for Hospital Mortality

Variables Group Mean or Mortality p-Value

Age at Op. survivor 76 ± 88d± 88d 0.055

mortality 28 ± 19dACC survivor 59 ± 17min

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

SNU Children’s Hospital

CoA

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 (inter

val 44mo)

– Permanent pacemaker insertion due to heart block (interval 7 years)

SNU Children’s Hospital

CoA

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.