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Update on Pediatric Cardiac Transplantation

Update on Pediatric Cardiac Transplantation. Dr Jameel Al-ata Consultant & Assistant Professor of Pediatrics & Pediatric Cardiology Taif April 2007. Introduction.

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Update on Pediatric Cardiac Transplantation

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  1. Update on Pediatric Cardiac Transplantation Dr Jameel Al-ata Consultant & Assistant Professor of Pediatrics & Pediatric Cardiology Taif April 2007

  2. Introduction • Orthotopic pediatric heart transplantation is well established for infants & children with severe forms of CHD or cardiomyopathies. • The one month , 1 y , 5 y , & 10 y survival rate is 90% , 85% , 75% , & 65% respective

  3. Indication • Heart transplant is indicated when life expectancy is less than 1-2 y. OR unacceptable quality of sec to End-stage heart disease. • CMP , CHD with ventricular failure are primary indications. • HLHS , HIV , & hepatitis are controversial indications.

  4. DIAGNOSIS IN PEDIATRIC HEART TRANSPLANT RECIPIENTS (Age: < 1 Year) 1988-1995 1/1996-6/2005 J Heart Lung Transplant 2006;25:893-903

  5. DIAGNOSIS IN PEDIATRIC HEART TRANSPLANT RECIPIENTS (Age: 1-10 Years)

  6. DIAGNOSIS IN PEDIATRIC HEART TRANSPLANT RECIPIENTS (Age: 11-17 Years)

  7. Pre-transplant considerations

  8. Pre-transplant medical considerations • Malnutrition &growth failure are common (anorexia , vomiting , mal-absorption , & hyper-metabolic state). • Co-morbid conditions like PLE , renal & chronic liver disease may be contributing to the poor nutritional state.

  9. Immunization • Prior to transplantation Immunization records must be reviewed and vaccines given according to recommendations. • Influenza vaccination should be yearly. • Measles & varicella vaccine should be given( if not immune ) & titers checked 6-8 weeks. • Hepatitis,B vaccine should also be given. • Pneumococcal vaccine is recommended even over 2 years of age.

  10. Waiting list • Waiting time varies according to case severity , blood type , & recipient body WT. • In the U.S. organ procurement & transplantation network 2001 annual report the median time to transplantation for a 4 year old was 191 days when listed with 84 same age range. ( 190 days for less than 1 year old listed with 142 patients)

  11. Pre-transplant Surgical considerations • Nearly 50% of refered cases are Coronary Heart Disease most of which undergone multiple palliations. • In experienced centers , even those with pulmonary arteries stenosis , anomalies of system & pulmonary venous drainage & or atrial arrangement abnormalities have nearly comparable survival to cardiomyopathies.

  12. Surgical considerations: • High output failure may be sec to failure to recognize important aorto-pulmonary collateral circulation in transplanted cyanotic CHD patient. • PLE , ch liver disease & pulmonary. AVMs poses additional premorbid challenges to the failed fontan transplantation patient. • Results of transplantation for ACHD are poor ( unclear reasons ).

  13. Surgical condition • PVR less than 10 woods units is acceptable , but poses increased risk of acute RV failure ( compared to less than 6 ). • ECMO can be used to bridge infants and small children ( not more than 2 wks because of increased risk of complications ). • Ventricular assist devices can a successfull bridge for the older child.

  14. AGE DISTRIBUTION OF PEDIATRIC HEART RECIPIENTS(Transplants: January 1996 - June 2005) Number of Transplants ISHLT 2006 J Heart Lung Transplant 2006;25:893-903

  15. Survival after Pediatric Heart Transplantation • 10 y actuarial survival rate between 1982 & 2001 more than 50% ( ISHLT report ). • Infants have higher mortality in first few months , with better outcome if they survive the 1st year. • Adolescents have annual survival decrement rate of 4%

  16. PEDIATRIC HEART TRANSPLANTATIONKaplan-Meier Survival (1/1982-6/2004) J Heart Lung Transplant 2006;25:893-903

  17. PEDIATRIC HEART TRANSPLANTATION Kaplan-Meier Survival by Era (1/1982-6/2004) J Heart Lung Transplant 2006;25:893-903

  18. Risk Factors

  19. PEDIATRIC HEART TRANSPLANTS (1/1995-6/2004)Risk Factors For 1 Year Mortality J Heart Lung Transplant 2006;25:893-903

  20. PEDIATRIC HEART TRANSPLANTS (1/1995-6/2004)Borderline Significant Risk Factors For 1 Year Mortality JHeart Lung Transplant 2006;25:893-903

  21. PEDIATRIC HEART TRANSPLANTS (1/1995-6/2004) Factors Not Significant for 1 Year Mortality • Recipient Factors: • IV inotropes, sternotomy, thoracotomy, history of malignancy, height, recent infection, age, PA pressure, cardiac output, pulmonary vascular resistance.

  22. PEDIATRIC HEART TRANSPLANTS (1/1995-6/2004) Factors Not Significant for 1 Year Mortality • Donor Factors: • Gender, history of hypertension, height, clinical infection, history of diabetes • Transplant Factors: • CMV mismatch, ABO identical/compatible, ischemia time, HLA mismatch, transplant center volume

  23. PEDIATRIC HEART TRANSPLANTS (1/1995-6/2000)Risk Factors For 5 Year Mortality Conditional on 1 Year Survival

  24. PEDIATRIC HEART TRANSPLANTS (1/1995-6/2000) Factors Not Significant for Conditional 5 Year Mortality • Recipient Factors: • History of malignancy, recent infection, hospitalized at time of transplant, bilirubin, creatinine, cardiac output, pulmonary vascular resistance, PRA, sternotomy, ventilator, VAD, age, PA pressures

  25. PEDIATRIC HEART TRANSPLANTS (1/1995-6/2000) Factors Not Significant for Conditional 5 Year Mortality • Donor Factors: • Cause of death, history of hypertension, weight, height, age, gender, clinical infection at donation • Transplant Factors: • Donor/recipient weight ratio, year of transplant, CMV mismatch, transplant center volume, induction use, treated for infection prior to discharge, dialysis prior to discharge

  26. Long term management post Pediatric Heart Transplantation

  27. PEDIATRIC HEART RECIPIENTS Functional Status of Surviving Recipients (Follow-ups: April 1994 - June 2005) J Heart Lung Transplant 2006;25:893-903

  28. Early issues • Immunosuppressive therapy needed for life of the graft. • To prevent host immune response to donor antigens & minimize toxicity ( nephrotoxicity , bone marrow suppression , hyperlipidemia , diabetes …..etc ).

  29. Immunosuppressive agents • Triple protocol ( calcineurin inhibitro e.g. cyclosporine or tacrolimus plus MMF ( replacing azathiop ) and steroids ( weaned within 1st year ). • Rapamycin as rescue therapy for acute rejection.

  30. PEDIATRIC HEART RECIPIENTS Induction Immunosuppression (Transplants: January 2001 - June 2005) JHeart Lung Transplant 2006;25:893-903

  31. PEDIATRIC HEART RECIPIENTS Maintenance Immunosuppression at Time of Follow-up(Follow-ups: January 2001 - June 2005) J Heart Lung Transplant 2006;25:893-903

  32. Morbidity

  33. POST-HEART TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 1 Year Post-Transplant (Follow-ups: April 1994 - June 2005)

  34. POST-HEART TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 5 Years Post-Transplant (Follow-ups: April 1994 - June 2005)

  35. POST-HEART TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 8 Years Post-Transplant (Follow-ups: April 1994 - June 2005)

  36. FREEDOM FROM CORONARY ARTERY VASCULOPATHY For Pediatric Heart Recipients (Follow-ups: April 1994 - June 2005)

  37. Renal Dysfunction & Sys Hypertension • 73% n. renal function at 5 y • Factors for decreased renal function include; low COP, ischemia/ repefusion & calcineurin inhibitant. • 2/5 have decreased glomerular filtration at long term follow up. • Aggressive high blood pressure therapy and use of non nephrotoxic agents ( mmf ) promotes renal function preservation • A small number may need renal transplant • 60% at 5 y will need at least 1 antihypertensive

  38. FREEDOM FROM SEVERE RENAL DYSFUNCTION*For Pediatric Heart Recipients (Follow-ups: April 1994 - June 2005)

  39. Rejection • 2 /3 recipients are free at 1 m. , but < 1/3 at 1 year. • Risk factors include; older age at transplant , af-am race CMV & previous rejection. • Usually no symptoms. • Mild to moderate rejection DX. At surv. Endomyocardial biopsies.

  40. S & S of rejection • Fatigue , decreased appetite,nausea,abdominal pain, rapid including in weight., fussiness & poor feeding. • Tachycardia, irregular rhythm,fever,gallop & hepatomegally.

  41. Chronic rejection( graft vasculopathy) • Accelerated coronary vasculopathy is the leading cause of death in late survivors. • Is due to myointimal prolifration involving the entire vessel including intra myo.branch • Angiography is not sensitive for mild forms. • 75% overall prevalence by IVUS. AT 5 Y. • Ectopy, pre-syncope, syncope, interm oedema, ex intolerance & rarely chest pain are some symptoms. • Rapamycin prevents it in animals.

  42. Cause of Death • Acute allograft failure 1st 30 days • Acute cellular rejection & infections 1-5 y • Chronic rejection causing heart or pt. Loss beyond 5 y.

  43. Other issues • Growth • Osteoporosis • Exercise • Psychosocial • Noncompliance

  44. Summary • Pediatric heart transplantation is effective • Multidisciplinary approach is needed • Vasculopathy is a major obstacle • Much needed in KSA.

  45. THANK YOU

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