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Single Ventricle Physiology with Pulmonary Hypertension: Decisions and Challenges

Single Ventricle Physiology with Pulmonary Hypertension: Decisions and Challenges. Xinwei Du M.D. Shanghai Children ’ s Medical Center. Single Ventricle Physiology. With restrictive pulmonary blood flow With neither restrictive pulmonary nor systemic blood flow

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Single Ventricle Physiology with Pulmonary Hypertension: Decisions and Challenges

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  1. Single Ventricle Physiology with Pulmonary Hypertension: Decisions and Challenges Xinwei DuM.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 Fontan Normal Heart

  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 Techniquesin 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？ 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 withObstruction 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 • 2002.1 – 2014.12 • 72 patients

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

  14. Re-operation before Stage II Glenn

  15. 9 Patients Failed at stage I Palliation • 2 hospital motality • 7 fail to pursue Glenn • Present late (after 6m) 5 • Post-op. AV valve regurgitation 4 • Heterotaxy 5 • mPAP > 25mmHg 6

  16. Pulmonary Vasodilatorafter Fontan • PVRI > 3.0 Wood units. m2 • mPAP > 15 mmHg • CVP > 20mmHg • TPG > 10 mmHg • Desaturation • Persistent PE

  17. 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

  18. 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

  19. Oral Bosentanafter Fontan • 40 patients • 6 months follow-up

  20. 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

  21. Thank you !

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