1. Erle H. Austin, MD Kosair Children’s Hospital Louisville, Kentucky
A Few Examples
Erle H. Austin, MD
Kosair Children’s Hospital
Louisville, Kentucky
No Disclosures

2. Patient # 1 Newborn female 37 wks; 2.8 kg; Apgars 7/8
Heart murmur; pink with “blue spells”
Echo: PA, VSD, right aortic arch, atypical PDA
Cardiac cath at 4 days of age
Discharged age 13 days

3. 4 Days Old - Cath

4. 4 Days Old - Cath

5. 7 Months Old - Cath

6. 7 Months Old - Cath

7. 10 Months Old - Surgery Median sternotomy
Unifocalization of central PAs and MAPCAs
Small native LPA ( & tiny native RPA)
Dissected off CPB
L and R inferior MAPCAs; R superior MAPCA
Dissected on CPB
small fragile vessels; all probed at completion
VSD closure
12mm RV-PA conduit (pulmonary homograft)
Atrial septal fenestration

8. 10 Months Old - Surgery cont.
Separated from CPB with good sats/BP
TEE good function, no residual VSD
RV pressure !!! % LV pressure !!!
VSD patch fenestration
Discharged POD#11

9. 2 Years Old
Qp/Qs = 0.52

10. 7 Years Old

11. Patient # 2 Newborn male Full term; 4.4 kg; Apgars 9/9
Heart murmur; 93% sats with desat episodes
Echo: PA, VSD, right aortic arch, probable MAPCAs
Cardiac cath at 4 days of age

12. 4 Days Old - Cath

13. 5 Days Old - Surgery Median sternotomy 4mm central shunt
Ascending aorta to native central PA
Brizard et al. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 12:

14. Still on the ventilator !!!
13 Days Old
Still on the ventilator !!!

15. 14 Days Old - Cath
Qp/Qs = 8/1

16. 14 Days Old - Surgery Left Thoracotomy
Banding of large branch right MAPCA
Banding of large branch left MAPCA

17. Patient #2
Extubated 2 days later
Discharged at 2 months of age

18. 10 Months Old - Cath
Qp/Qs = 1.3/1

19. 12 Months Old - Surgery Redo left thoracotomy Redo median sternotomy
Identify left-sided MAPCAs
Ligation origin MAPCA from left subclavian
Redo median sternotomy
Unifocalization of left-sided MAPCAs
Patch angioplasty of native right PA
Placement of 8mm RV-PA Gore-Tex conduit
Discharged POD#8

20. 21 Months Old - Cath

21. Patient # 3 Newborn male 37 wk; 3.6 kg; Apgars 8/8 Fetal dx: TOF/PA
II/VI systolic murmur; Sats low 90s
Echo: TOF/PA, typical PDA, confluent 4.2mm PAs; ?MAPCAs?
PGE1
Cardiac cath at 2 days of age

22. 2 Days Old - Cath

23. 3 Days Old - Surgery Median sternotomy
Right modified BT shunt (3.5 mm)
?Ductus? Not ligated
22q11 deletion
Discharged POD#23

24. 5 Months Old - Cath