1. Xinwei Du M.D. Shanghai Children’s Medical Center
Single Ventricle Physiology with Pulmonary Hypertension: Decisions and Challenges
Xinwei Du M.D.
Shanghai Children’s Medical Center

2. Single Ventricle Physiology
With restrictive pulmonary blood flow
With neither restrictive pulmonary nor systemic blood flow
W/O restrictive pulmonary blood flow but with restrictive systemic blood flow

3. Single Ventricle Physiology
Normal
Heart
Fontan

4. PVR Success of Fontan operation depends on lower PVR
Even slight increases in PVR sig. reduce the transpulmonary blood flow → failing Fontan
Pulmonary arteries
Pulmonary vascular bed
Pulmonary venous return
Atrial septum

5. Surgical Techniques in Stage I Palliation
PA Banding
DKS or Norwood
Palliative Switch

6. PA Banding
Must have an obstruction-free SVOT anatomy
R. A. Jonas

7. Why do DKS？ <2cm2/m2 Increasing restriction at BVF
Obstruction to systemic outlet
Decreased ventricular compliance
R. A. Jonas
<2cm2/m2

8. DKS
DHCA or DHLF
Source of pulmonary flow can be a B-T or Sano shunt

9. Lesions with Obstruction at BVF
DILV with discordant ventriculoarterial connection
TA with discordant ventriculoarterial connection
DORV with MA

10. PAB+APW
The distance between the pulmonary valve to the bifurcation should be long enough for AP window creation

11. Palliative Switch Coronary pattern should be Yacoub A
Aortic-pulmonary position should be anterior-posterior
Aortic root should not be too small

12. SCMC Data –
72 patients

13. Stage I Palliation
Hospital Motality = 2.8% (2/72)

15. Re-operation before Stage II Glenn
Rebanding 5
8.4% (5/59 )
Shunt Revision 1
7.6 (1/13)
Other (Bleeding, Chylothorax) 2

16. 9 Patients Failed at stage I Palliation
2 hospital motality
7 fail to pursue Glenn
Present late (after 6m)
Post-op. AV valve regurgitation 4
Heterotaxy
mPAP > 25mmHg

17. Pulmonary Vasodilator after Fontan
PVRI > 3.0 Wood units. m2
mPAP > 15 mmHg
CVP > 20mmHg
TPG > 10 mmHg
Desaturation
Persistent PE

18. Iloprost therapy after Fontan Effect iloprost on TPG (mmHg) (*P=0.000)
TPG1: Before iloprost (14.5 ± 2.7)
TPG2: Before extubation (9.5± 2.3)
TPG3: After extubation 30min later (8.1± 1.9)
SCMC data

19. Iloprost therapy after Fontan Effect iloprost on CVP (mmHg) (*P=0.000)
CVP1: Before iloprost (19.8 ± 5.2)
CVP2: Before extubation (12.4 ± 2.6)
CVP3: After extubation 30min later (11.4 ± 2.4)
SCMC data

20. Oral Bosentan after Fontan
40 patients
6 months follow-up

21. Conclusion
Stage I palliation is the key point to treat single ventricle physiology with unrestricted pulmonary blood flow
Early diagnosis ensure a good long term outcome
Pulmonary vasodilators help to improve cardiac performance in patients with high PVR postoperatively

22. Thank you !