1. Pulmonary atresia with VSD Presenter: 吳承諭 Supervisor: 王玠能醫師
INTERN SEMINAR
Pulmonary atresia with VSD
Presenter: 吳承諭
Supervisor: 王玠能醫師

2. OUTLINE
Case presentation
Discussion
Pulmonary atresia
Prognosis

3. BASIC INFORMATION Name:陳O豪 Gender: male Patient ID:143xxxxxxx
Date of Birth:2011/10/31
Age: 5 year old
Height: 111.8cm
Weight: 18.2kg

4. HOSPITAL COURSE Before birth
GA 22wks, level II sonar  suspected tetralogy of Fallot
No gestational diabetes mellitus.
No preeclampsia or gestational hypertension

5. HOSPITAL COURSE Before birth Birth
GA 39+3 week, NSD, Apgar score: 8->10,BBW: 3695 gm
PE:
SaO2: 85% under room air
Lip: mild cyanosis
Nail back: mild cyanosis
Heart: RHB, continuous murmur grade 2/6 over LSB, S2 single

6. INITIAL CXR (2011/10/31)

7. INITIAL ECHO (2011/10/31) RV
Aorta LV LA
Parastenal long axis view
Modified Parastenal short axis view 沒有看到PA 隱約看到LPA和RPA absent of RVOT
Suprastenal arch view: No PDA, 疑似MACAP
Imp:
Pulmonary atresia with Ventricular Septal Defect RV

8. INITIAL CTA (2011/11/01) PAVSD. One MAPCA from left CCA to MPA.
Short segmental confluence of RPA and LPA.
Native RPA: 2.6mm near hilum.
Native proxiaml LPA, markedly focal stenosis
One short MAPCA (The upper one from descending thoarcic aorta)
One MAPCA (The lower one from descending thoarcic aorta)

9. HOSPITAL COURSE Before birth Birth 2 month old
acute cyanosis and dyspnea when feeding
SaO2 down to 70% under O2
Arranged Cath
Three MAPCAs

10. HOSPITAL COURSE Before birth Birth 2 month old 3 month old
Modified Right Blalock–Taussig shunt , 5mm in this 5.4 kg baby
After surgery: SaO2:79-80%
less cyanosis was noted, cyanosis happened only during exertion.

11. HOSPITAL COURSE Before birth Birth 2 month old 3 month old
Right side unifocalization

12. HOSPITAL COURSE
1 Y 7 M old
Left side unifocalization

13. HOSPITAL COURSE 1 Y 7 M old 2 Y 8M old
Due to follow up CTA showed LPA is small
 Modified B-T shunt, left side

14. HOSPITAL COURSE 1 Y 7 M old 2 Y 8M old 3 Y 7 M old
CTA showed bil. B-T shunt stenosis
 balloon dilatation

15. HOSPITAL COURSE 1 Y 7 M old 2 Y 8M old 3 Y 7 M old 4 Y 8M old
BW: 16.8kg
Hybrid: Rastelli operation+stent
Bilateral BT shunt transection,
VSD repair,
RV-PA continuity reconstruction
with hand-made valved conduit
PA angioplasty and stent implantation
After surgery, SaO2 95 %
Now adequate activity in his daily life !!!

16. DISSUSSION: Pulmonary atresia with VSD

17. EMBRYOLOGY OF HEART
Pulmonary artery  originate from the truncus arteriosus and the sixth pharyngeal arch.
Aortopulmonary collaterals originate from aorta dorsalis

18. ETIOLOGY and EPIDEMIOLOGY
% of all congenital cardiac malformations
Slighly more prevalent in males than in females
Genetic factors: siblings and in offsprings of adults with tetralogy of Fallot
DiGeorge syndrome (Del22q11)
Trisomy 21

19. CLASSIFICATION
Type A: Native PAs present, pulmonary vascular supply through PDA and no APCs. 
Type B: Native PAs and APCs present 
Type C: No native PAs, pulmonary blood supply through APCs only
Aortopulmonary collaterals

20. CLINICAL PRESENTATION
Central cyanosis
S 1 is normal; S 2 is always single.
A grade 3/6 systolic murmur usually is audible along the lower left sternal border
If systemic-to-pulmonary collateral arteries are present, continuous murmurs may be diffusely audible over the entire chest and back.
systolic murmur: TR

21. MANAGMENT
Stanford 2009

22. DIAGNOSIS Echocardiography: characterize the intracardiac anatomy
Cardiac catheterization:
detailed roadmap of all sources of pulmonary blood flow
identify the origins and contributions of all aortopulmonary (AP) collaterals

23. GOALS OF SURGICAL MANAGEMENT
Complete Unifocalization: 3-6 months
Intracardiac Repair: close VSD
Mean pulmonary artery pressure < 25 mmhg
RV/LV pressure ratio less than 0.5

24. PROGNOSIS PA/VSD TOF 10-year 60% 97% 20-year 50% 94% 30-year 53% 89%
Overall survival rate
PA/VSD
TOF
10-year
60%
97%
20-year
50%
94%
30-year
53%
89%
Long-term outcome after treatment of pulmonary atresia with ventricular septal defect: nationwide study of 109 patients born in 1970–2007, Anu K. Kaskinen et al
European Journal of Cardio-Thoracic Surgery 49 (2016)
Long-Term Survival in patients with repair of Tetralogy of Fallot: 36-Year follow-up of 490 survivors of the first year after surgical repair, Georg Nollert et al
Journal of the American college of cardiology 1997

25. What Factors Effect Prognosis??

27. Higher McGoon index and more lung segments supplied by true pulmonary arteries increased:
the probability of repair
the survival of PA + VSD.
Overall survival was no difference: PA/VSD v.s PA/VSD/MAPCAs
PA/VSD/MAPCAs  higher risk of remaining palliated
Of the palliated patient, with MAPCAs had better survival rate than without MAPCAs

28. J Thorac cardiovasc Surg. 2010 Nov

29. weighing more than 5 kg, regardless of their age
Neonatal age and low body weight (3 kg) at unifocalization were significantly associated with mortality
weighing more than 5 kg, regardless of their age

30. The annals of thoracic surgery, 2012 Nov

31. With genetic syndrome had lower 20-year survival than without syndrome(P<0.01)
Del22q11 had higher 10-year survial rate than other genetic syndrome(P<0.06)

32. BACK TO OUR CASE  Prognosis not the best, but great
This case is a boy
Type B PA/VSD/MACPAs
Received the first surgery at 5 kg
No genetic syndrome
 Prognosis not the best, but great

33. TAKE HOME MASSEGE Embryology of PA and MACAPS
(truncus arteriosus + 6th pharyngeal arch vs aorta dorsalis )
PA with VSD and MACAPs clinical presentation
Single S2(absent RVOT), systolic murmur(TR), continuous murmurs (MACAPs)
Central cyanosis (right to left shunt)
Prognosis:
Native PA
Unifocalization time
Genetic syndrome

34. THANKS FOR YOUR ATTENTION