1. Right Ventricular Failure After Tetralogy of Fallot Repair
Robert Jeenchen Chen, MD, MPH
Cardiovascular Surgery
National Taiwan Univ Hosp, Taipei

2. Case Presentation 1y2m/o female baby (DOB:06/23/2000)
Chief Complaint: Acrocyanosis, poor oral intake, hypoactivity for days
Past History:
Down syndrome, growth<3rd %
TOF s/p modified B-T shunt at 2 m/o

3. Past History--Echo Before B-T Shunt
Severe RVOT narrowing, scant antegrade flow, PG=78
Hypoplastic MPA (annulus=2 mm)
PDA 2.9 mm
McGoon index=1.37
VSD, 6.2 mm, perimembranous
ASD, 2.9 mm at fossa ovalis

4. Past History- Cardiac Cath Before B-T Shunt
IVC: ///(43%)
RA: //0.5(40.3%)
SVC: ///(62.5%)
RV: 60/5/(60.6%)
Ao: 90/40/65(71.9%)
Extreme TOF
Hypoplastic PA
McGoon index =( )/6.96 =1.3
Large PDA from LSCA
Right aortic arch

5. Op (2 m/o): Palliative Aorto-Pulmonary Shunting
Right modified Blalock-Taussig shunt
PA-SCA
3.5 mm GoreTex graft

6. Past History--Cardiac Echo One Month After B-T Shunt
RVOT narrowing, scant antegrade. PG=55
Patent B-T shunt
McGoon=1.6
PDA closed
ASD, slit
VSD, 7.8 mm
TR, PG=39
RPA juxta-shunt 2.6/6.1 mm
LPA 3 mm, juxta-ductal stenosis

7. Past History--Cardiac Echo Four Months After B-T Shunt
RVOTO
Patent B-T shunt
McGoon=2.0
ASD, slit
VSD, 7 mm
TR, PG=40
RPA 5.9 mm, mild juxta-shunt stenosis
LPA 6.9 mm

8. Present Illness (1 Y/O) Followed up at OPD
Acrocyanosis, poor oral intake, hypo-activity for days
Generalized cyanosis (SpO2=45%) at NTUH
Echo: B-T shunt occlusion!
Emergent op!

9. Op (1 Y/O): Total Correction
Infundibulectomy
ASD fenestration
VSD patch repair
Trans-annular patch (TAP) augmentation of MPA till LPA orifice
Occluded B-T shunt

10. Course and Treatment Postop low CO High inotrope support Anuria
Hepatomegaly
Inhaled NO improved condition

11. Course and Treatment
Echo (POD-1): dilated RV, good LV contractility, TR with PG=23.8 mmHg, CVP= 17 mm Hg, no AR
Impression: RV failure
Op (POD-2): ASD fenestration and VSD fenestration

12. Course and Treatment– Echo on POD-23
RA, RV chamber enlargement
ASD s/p repair and fenestration, 5 mm
VSD s/p repair and fenestration, PG=21 mmHg
s/p infundubulectomy, TAP of MPA
No PG over RVOT or PV
Severe PR, PG=5 mmHg
Severe TR, PG=40 mmHg

13. Course and Treatment Lung edema, on diuretics
Pleural effusion, frequently tapped
Hepatomegaly (2-4 fb below RCM)
Respiratory distress with skin mottling
MRSE sepsis in 7/2001!
Cath (7/6): RVOTO, large Qp/Qs
CPR on 7/7/2001

14. Course and Treatment—Cath One Month After TC
IVC 54.7% (55.1%)
RA //19 (68.6%)
LA //19 (87.5%)
LV 90/19 (88.8%)
SVC (55.4%)
RPA 75/13/40 (79.1%)
MPA 75/15/40 (80.5%)
RV 75/16 (72.7%)
LPA 31/16/24 (81.8%)
MPA 75/16/37, distal RPA 40/15/26
Qp/Qs=3.8
VSD
Juxta-ductal LPA stenosis (PG=44)
Shunt-related RPA stenosis (PG=35)
Severe PR
Pulmonary HTN
RVSP/LVSP=0.83

15. Re-Op-- 33 Days After TC VSD fenestration (post. inf) repair
1. LPA stenosis, 3 mm
2. MPA s/p TAP augmentation
3. RPA juxta-shunt stenosis, 3.5 mm
VSD fenestration (post. inf) repair
ASD fenestration downsize from 8 to 5 mm
MPA to LPA patch augmentation
RPA patch augmentation

16. Course and Treatment—Echo Two Month After TC
Mild RA, RV chamber enlargement
VSD, PG=31 mmHg
Mild PS, PG=10 mmHg
Moderate PR, PG=4 mmHg
TR, PG=33 mmHg
ASD, 1 mm
Pleural and pericardial effusion

17. Course and Treatment Febrile pleural effusion Elevated CRP=7.83
Uncontrolled by specific antibiotics
CT (8/2): loculated pleural effusion with atelectasis (L't 50 cc, R't 20 cc)
Op (8/6): lung empyema debridement and adhesiolysis

18. Course and Treatment—Echo Nine Weeks After TC
Enlarged LA, RA, and RV chambers
PFO 2.7 mm
VSD, repaired, PG=27
Moderate TR, PG=53 mmHg
RVOT without significant stenosis
Moderate PR, PG=9 mmHg
Mild LPA stenosis, PG=12 mmHg
Right pleural effusion, massive ascites

19. WBC

20. Worsening LVEF

21. McGoon Index

22. Case Summary: TOF Birth: cyanosis 1.5 m/o: Mod B-T shunt
3 m/o: PDA occluded
5.5 m/o: Shunt-dependent pulmonary flow
11.5 m/o: Emergent total correction
11.5 m/o: ASD and VSD fenestration for postop RV failure
1 y/o: VSD Qp/Qs=3.8, pulmonary HTN
1 y/o: Downsizing of ASD and VSD fenestration, re-augmentation of PA
1.2 y/o: RV failure (pleural effusion, ascites)

23. RV Failure After TOF Repair
Discussion
RV Failure After TOF Repair

24. Controversies Palliation or primary total correction?
PFO and VSD sizes?
A 2nd palliation rather than TC?
Myocardium injury during resuscitation?
Trans-annular patch and RV dysfunction?

25. Early TC Advantages Reduce hypoxia changes
VSD closure: reduce late Cx (CHF, cerebral emboli/abscess, IE)
Decrease RVP: reduce RVH
Adequate PA flow: enhance growth
Abolish progressive RVOT stenosis
Separate MAPCA: reduce risk of PVRD
Rudolph AM: Congenital Diseases of the Heart 2001: 544-5

26. Early TC Advantages: Avoid Complications of Palliation
Reduce risk for high PVR due to shunt
PA stenosis/distortion not problem
Prevent shunt-related LV failure
Rudolph AM: Congenital Diseases of the Heart 2001: 544-5

27. Early TC Disadvantages
RVOT stenosis relief: inadequate PA size, large VSD L-R shunt
Coronary artery crossing RVOT
Trans-annular patch causes severe PR
Palliation allows growth of pulmonary artery and annulus
Rudolph AM: Congenital Diseases of the Heart 2001: 545-6

28. ASD and VSD Fenestration in TOF/PA
Open VSD: markedly increases pulmonary blood flow -> sudden CHF and lung edema.
PFO (ASD) in TOF: allows mild right-to-left shunt-> maintains systemic C.O. and not lead to RV failure.
Mavroudis & Backer. Pediatric Cardiac Surgery, 2nd ed.
Ch.21:

29. VSD Fenestration in TOF/PA
RV Failure (RVP>LVP): VSD has to be fenestrated; closed later when PA growth suffices
Residual VSD: poorly tolerated; must be closed if Qp/Qs>1.5.
Mavroudis & Backer. Pediatric Cardiac Surgery, 2nd ed.
Ch.21: , 287-9

30. RV Failure Cause: Trans-annular Patch (TAP)?
Direct closure: less late adverse events, less PR, smaller RVEDD/LVEDD
TAP & RV patch: long-term PR & RV dilatation
Circulation.  102 (19 Suppl 3):III116-22, 2000

31. RV Failure Cause: Myocardial Injury
Acute RV restriction after TOF repair: low CO, long ICU stay
N=11, serial prospective, doppler echo
Cardiac troponin T, NO-x, iron metabolism and antioxidants
Restrictive (N=4) vs non-restrictive: longer ICU stay, higher trop-T, higher NO-x, higher iron loading, and less iron-binding antioxidant activity
Circulation.  100(14):1540-7, 1999

32. What to Change Next Time?
TOF/PA, McGoon index=1.3
Earlier total correction before B-T shunt occlusion
ASD and VSD fenestrations at the total correction
VSD closed later when PA larger or Qp/Qs>1.5

33. What Next for This Case? Trisomy 21 Clear consciousness
Recurrent Infections
Refractory RV failure
Prolonged intubation
Inotrope dependency
Infection eradication
Nutrition support
Diuretics
Ventilator weaning
Re-operation

34. Thank You!