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Pediatric Lung Transplantation

Pediatric Lung Transplantation. Assoc. Prof. Figen Gülen MD Ege University Medical Faculty Division of Pediatric Allergy and Pulmonology figen.gulen@ege.edu.tr. Transplantation. Radical treatment option for end-stage organ failure. Lung Transplantation. In spite of medical treatments;

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Pediatric Lung Transplantation

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  1. Pediatric Lung Transplantation Assoc. Prof.Figen GülenMD Ege University Medical Faculty Division of Pediatric Allergy and Pulmonology figen.gulen@ege.edu.tr

  2. Transplantation Radical treatment option for end-stage organ failure

  3. Lung Transplantation In spite of medical treatments; * End-stage lung disease * Life-threatening pulmonary vascular disease

  4. 1963 ; first lung transplantation

  5. History 1950 Heart-Lung 1963 Adult lung 1980 Canada (J.Cooper), England, USA (Heart-Lung) 1986 Pediatric Lung 1990 Only lung (Pasque et al.) Single lung, single lobe 1998 Turkey Ö. Oto (Heart-Lung)

  6. 2005 USA 27.527 organ transplantation 1405 Lung 32 Heart-Lung The US Organ Procurement and Transplant. Network (OPTN/SRTR 2005)

  7. 2000-2002 Pediatric Transplantation Mallory G.B.Eur.Res.J. 2004; 24:839-45

  8. Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.

  9. Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.

  10. Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.

  11. ISHLT (International Society for Heart and Lung Transplantation)

  12. Adult indications • Chronic obstructive lung disease • Idiopathic Pulm.Fibrosis • Cystic Fibrosis • Primary Pulm. Hypertension • Alpha-1 antityripsin deficiency • Bronchiectasis • Eisenmenger Synd. • Re-Transplantation • Others ISHLT. J.Hearth. Lung. Trans. 2006; 25: 745,755

  13. Contraindications • Absolute • Relative

  14. Absolute Contraindications • Severe scoliosis, Thorax deformity • Severe tracheomegaly and malasia • Liver, kidney, left vent. insufficiency • Active malignancy (last 2 years) • Irreversible neuromuscular disease • Active systemic vascular disease

  15. Absolute Contraindications • Burcholderia cepacia genomovar-3 Lower respiratory infection • Hepatitis B-C, HIV (unresponsive to treatment) • Active viral inf. • Active Tuberculosis • Bacteriemia or septicemia Am.J.of Transplasnt 2007;7:285-92 J.Hearth Lung Transplant 2006;25:745-55 Eur .Res.J. 2004;24:839-45

  16. Absolute Contraindications • Incompatible with medical monitoring and treatment, • Without social security, • Patients with psychiatric disorders, or non-cooperative. Am.J.of Transplasnt 2007;7:285-92 j.Hearth Lung Transplant 2006;25:745-55 Eur .Res.J. 2004;24:839-45

  17. Relative Contraindications • Symptomatic osteopenia, osteoporosis • Pneumonectomy • Resistant and high-virulence organisms in airway (colonization) • Lower respiratory infection with B.Cepacia genomovar (except genomovar 3) • Severe malnutrition • Pleurodesis • 25/30 kg/m2 < BMI • 18/20 kg/m2 > BMI • Mechanical ventilation Am.J.of Transplasnt 2007;7:285-92 J.Hearth Lung Transplant 2006;25:745-55 Eur .Res.J. 2004;24:839-45

  18. Criteria for entry into the list Expected survival6 months-2 years, New-York Hearth association (NYHA) class III and IV levels function NYHA Functional Classification • NYHA Class III: moderate, significant limitation in physical activity, but the rest is easy, less activity than usual causes symptoms• NYHA Class IV: severe, can not do any physical activity without any problems,orthopnea or paroxysmal Symptoms such as nocturnal dyspnea can be found at rest

  19. Other Criterias Patients on Technological support Nitric-oxide inhalation Extracorporeal membran oxygenation

  20. Reviews needed Blood examination • Hemogram, Hemostasis • Blood group • Urea, Ions, creatinin • Blood glucose • Liver functions • Lipids • Thyroid functions • HLA typing

  21. Radiological examinations • Chest x-ray • Thorax, abdominal tomography • Abdominal ultrasound • Ventilation/Perfusion scintigraphy • Sinusal tomography (for CF)

  22. Functional Examinations Lung Pulmonary function tests, diffusion capacity Blood gases 6 sec. walking test Heart EKG, ECHO, bone-mineral density

  23. Searching for Infections • Sputum, culture-antibiogram • Detection of mycobacteria • PPD • Urine analysis • Culture for MRSA • HIV, Hepatitis B and C serology • EBV • Chlamydia pneumoniae • Varicella zoster serology

  24. Evaluation of malignancy • Sputum cytology • Papanicolau smear • Prostate-specific antigen • Mammography • Blood in stool

  25. Assessment of autoimmunity • ANA, RF • DNA antibody • ANCA • Creatinin kinase • Immunoglobulins

  26. Consultations • Dental •  Gastro • Dermatology • Psychiatry • Nutrition • Ear-Nose-Throat (ENT) • Physiotherapy

  27. Patient Selection Criterias

  28. Cystic Fibrosis • Basic illness requirements for lung transplantation in children, adolescent and young adults

  29. CF and Bronchiectasis • FEV1 < %30 and progressive decline • PCO2 > 55 mmHg • PO2 < 50 mmHg • increased egzazerbation despite treatment with antibiotics • Refractory and / or recurrent pneumothorax • Uncontrolled Hemoptysis Am. J.of Transpl. 2007;7:285-92

  30. Idiopathic Pulmonary Hypertension • NYHA or WHO class III-IV findings • Low response to 6 sec. walking test • Uncontrolled syncope • Hemoptysis • Right heart failure Hearth, Lung Transplant 2006;25:745-55

  31. Pulmonary Vascular Disease • Uncontrolled progressive pulmonary HT • Deterioration of quality of life • Severe hypoxemia

  32. Donor Selection • ABO compatibility • Size • Chest radiography • Arterial blood gases • Bronchoscopy

  33. Applied Techniques • Single lung or lobe • Double lung • Heart-lung transplantation

  34. Donors • Cadaveric (Heart-Lung, double-lung) • Live (one lung, lobe) For Children, a living donor is preferred

  35. Ischemic time for organs to be transplanted to a maximum of 4-5 hours.

  36. Pediatric Single Lung Tranplantation Idiopatic Pulmonary Fibrosis (isolated lung disease) Pulmonary Hypertension (isolated) • Pediatric Heart-Lung Severe heart failure including Left ventricle pulmonary hypertension + structural heart defects untreatable with surgery

  37. Bilateral Lung Transplantation • Pulmonary Fibrosis • Primary pulmonary hypertension • pulmonary hypertension + heart defects untreatable with surgery • Interstitiel lung disease • Cystic Fibrosis • Surfactant protein B deficiency • Alveolar proteinosis • Bronchiolitis obliterans

  38. TCH LUNG TRANSPLANT TEAM Physicians George Mallory, M.D. Director E. Dean McKenzie, M.D.Surgical Director Okan Elidemir, M.D. Pediatric Transplant Pulmonologist Jeff Heinle, M.D. Pediatric Cardiothoracic Surgeon David Morales, M.D. Pediatric Cardiothoracic Surgeon Dean Andropolous, M.D. Pediatric CT Anesthesiologist Additional Clinical Personnel Pegg Dobmeier, RN, BSN Transplant Coordinator Tonya Jack, RN, BSN Transplant Coordinator Brady Moffet, Ph.D. Transplant Pharmacist Michelle Lawson, LMSW Social Work Katherine Rushing, CCLS Child Life Specialist Michele Burns, RD Nutritionist Patricia Harris, RRT Pulmonary Diagnostic Lab Danita Czyzewski, Ph.D. Psychology Katie Wilkinson, RPT Physical Therapy Steve Habetz, RPT Physical Therapy

  39. TCH LUNG TRANSPLANT TEAM

  40. Pre-surgery induction (triple drug) PO cyclosporine 300-400 ng/ml or IV-PO tacrolimus 10-15 ng/ml IV azathioprine 2-3 mg/d IV-PO mycophenolate mofenil 15mg/kg BID IV prednisolon 0.5-1 mg/kg (Cyclosporine + Azothioprine + Steroid) for 3 months Continuation with Tacrolimus + MMF + Prednisolon Am.J.of transplant 2007;7:285-92

  41. Antimicrobials Except CF 1. jenerasyon sefalosporin CF IV 2 antipsödomonal According to culture Aspergillus Fumigatus : I.V. Low-dose amphotericine-B If CMV (+) IV gancyclovir Pneumocystis Carinii Trimethoprim-sulfametaxole

  42. Bronchial Anastomotic Stricture Post-dilatation Complications Surgery Anastomotic strictures Gastrointestinal dysmotility 50% ArrhythmiaInfectious1-6 months viral, fungal infection (24-46%)1-12 months CMV, EBV, P.cariniiLate stage bacterial infection

  43. Immunologic Complications Acute rejection: 1 week-1 month Asymptomatic Fever, dyspnea, hypoxia X-ray, bilateral infiltrasyon Decline in FEV1 and FVC Diagnosis: Bronchoscopy, BAL, TB. Biopsy (A0-A4 grade) Chronic rejection: Several months later Am.J.of Transplant 2007;285-92

  44. Acute rejection • Often seen after transplantation • Reported over 50% • Symptoms of fever and malaise • However, many patients with minimal symptoms • Transbronchial biopsy:

  45. Bronchiolitis Obliterans The most feared complication of long-term 50% in the first five years Post-transplant deaths: 40% in the first year Development is not clear Lower risk of developing in live-donor transplant Diagnosis: biopsy, HRCT, Vent. / Perf. Scintg. Treatment: immunosuppressiveEur.resp. J. 2004;24:839-45 Am.J.of Transplant 2007;7:285-89

  46. Followed by transbronchial biopsy

  47. Malignancy 6.5% in the first 1 year In five years, 8.5% Lymphoma EBV infection is a risk Treatment: reduction of immunosuppressionCD20 monoclonal antibody

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