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Pediatric Lung Transplantation
Assoc. Prof. Figen Gülen MDEge University Medical Faculty Division of Pediatric Allergy and Pulmonologyfigen.gulen@ege.edu.tr
Transplantation
Radical treatment option for end-stage organ failure
Lung Transplantation
In spite of medical treatments; * End-stage lung disease * Life-threatening pulmonary vascular disease
1963 ; first lung transplantation
History
1950 Heart-Lung1963 Adult lung1980 Canada (J.Cooper), England,
USA (Heart-Lung) 1986 Pediatric Lung1990 Only lung (Pasque et al.)
Single lung, single lobe 1998 Turkey Ö. Oto (Heart-Lung)
2005 USA27.527 organ transplantation
1405 Lung32 Heart-Lung
The US Organ Procurement and Transplant.
Network (OPTN/SRTR 2005)
2000-2002 Pediatric Transplantation
Mallory G.B.Eur.Res.J. 2004; 24:839-45
Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.
Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.
Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.
ISHLT (International Society for Heart and Lung Transplantation)
Chronic obstructive lung diseaseIdiopathic Pulm.FibrosisCystic FibrosisPrimary Pulm. HypertensionAlpha-1 antityripsin deficiencyBronchiectasisEisenmenger Synd.Re-TransplantationOthers
ISHLT. J.Hearth. Lung. Trans. 2006; 25: 745,755
Adult indications
Contraindications
AbsoluteRelative
Absolute Contraindications
Severe scoliosis, Thorax deformitySevere tracheomegaly and malasia Liver, kidney, left vent. insufficiencyActive malignancy (last 2 years)Irreversible neuromuscular diseaseActive systemic vascular disease
Absolute Contraindications Burcholderia cepacia genomovar-3 Lower respiratory infectionHepatitis B-C, HIV (unresponsive to treatment)Active viral inf.Active TuberculosisBacteriemia or septicemia
Am.J.of Transplasnt 2007;7:285-92J.Hearth Lung Transplant 2006;25:745-55
Eur .Res.J. 2004;24:839-45
Absolute Contraindications
Incompatible with medical monitoring and treatment ,Without social security ,Patients with psychiatric disorders, or non-cooperative .
Am.J.of Transplasnt 2007;7:285-92j.Hearth Lung Transplant 2006;25:745-55
Eur .Res.J. 2004;24:839-45
Relative Contraindications
Symptomatic osteopenia, osteoporosisPneumonectomyResistant and high-virulence organisms in airway (colonization) Lower respiratory infection with B.Cepacia genomovar (except genomovar 3)Severe malnutritionPleurodesis25/30 kg/m2 < BMI18/20 kg/m2 > BMIMechanical ventilation
Am.J.of Transplasnt 2007;7:285-92J.Hearth Lung Transplant 2006;25:745-55Eur .Res.J. 2004;24:839-45
Criteria for entry into the listExpected survival 6 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
Other Criterias
Patients on Technological supportNitric-oxide inhalationExtracorporeal membran oxygenation
Reviews neededBlood examination
Hemogram, HemostasisBlood groupUrea, Ions, creatininBlood glucoseLiver functionsLipidsThyroid functionsHLA typing
Radiological examinations
Chest x-rayThorax, abdominal tomographyAbdominal ultrasoundVentilation/Perfusion scintigraphySinusal tomography (for CF)
Functional ExaminationsLung
Pulmonary function tests, diffusion capacityBlood gases6 sec. walking test
HeartEKG, ECHO, bone-mineral density
Searching for InfectionsSputum, culture-antibiogramDetection of mycobacteriaPPDUrine analysisCulture for MRSAHIV, Hepatitis B and C serologyEBVChlamydia pneumoniaeVaricella zoster serology
Evaluation of malignancy
Sputum cytologyPapanicolau smearProstate-specific antigenMammographyBlood in stool
Assessment of autoimmunity
ANA, RFDNA antibodyANCACreatinin kinaseImmunoglobulins
ConsultationsDentalGastroDermatologyPsychiatryNutritionEar-Nose-Throat (ENT)Physiotherapy
Patient Selection Criterias
Cystic Fibrosis
Basic illness requirements for lung transplantation in children, adolescent and young adults
CF and BronchiectasisFEV1 < %30 and progressive declinePCO2 > 55 mmHgPO2 < 50 mmHg
increased egzazerbation despite treatment with antibiotics Refractory and / or recurrent pneumothoraxUncontrolled Hemoptysis
Am. J.of Transpl. 2007;7:285-92
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
Pulmonary Vascular Disease
Uncontrolled progressive pulmonary HTDeterioration of quality of lifeSevere hypoxemia
Donor Selection
ABO compatibilitySizeChest radiographyArterial blood gasesBronchoscopy
ABO compatibilitySizeChest radiographyArterial blood gasesBronchoscopy
Applied Techniques
Single lung or lobeDouble lungHeart-lung transplantation
Donors
Cadaveric (Heart-Lung, double-lung)Live (one lung, lobe)
For Children, a living donor is preferred
Ischemic time for organs to be transplanted to a maximum of 4-5 hours.
Pediatric Single Lung TranplantationIdiopatic Pulmonary Fibrosis
(isolated lung disease)Pulmonary Hypertension (isolated)
Pediatric Heart-LungSevere heart failure including Left ventriclepulmonary hypertension + structural heart defects untreatable with surgery
Bilateral Lung Transplantation
Pulmonary FibrosisPrimary pulmonary hypertensionpulmonary hypertension + heart defects untreatable with surgeryInterstitiel lung diseaseCystic FibrosisSurfactant protein B deficiencyAlveolar proteinosisBronchiolitis obliterans
TCH LUNG TRANSPLANT TEAMPhysiciansGeorge Mallory, M.D. DirectorE. Dean McKenzie, M.D. Surgical DirectorOkan Elidemir, M.D. Pediatric Transplant PulmonologistJeff Heinle, M.D. Pediatric Cardiothoracic SurgeonDavid Morales, M.D. Pediatric Cardiothoracic SurgeonDean Andropolous, M.D. Pediatric CT Anesthesiologist
Additional Clinical PersonnelPegg Dobmeier, RN, BSN Transplant CoordinatorTonya Jack, RN, BSN Transplant CoordinatorBrady Moffet, Ph.D. Transplant Pharmacist Michelle Lawson, LMSW Social Work Katherine Rushing, CCLS Child Life SpecialistMichele Burns, RD NutritionistPatricia Harris, RRT Pulmonary Diagnostic LabDanita Czyzewski, Ph.D. PsychologyKatie Wilkinson, RPT Physical TherapySteve Habetz, RPT Physical Therapy
TCH LUNG TRANSPLANT TEAMTCH LUNG TRANSPLANT TEAM
Pre-surgery induction (triple drug) PO cyclosporine 300-400 ng/ml orIV-PO tacrolimus 10-15 ng/mlIV azathioprine 2-3 mg/dIV-PO mycophenolate mofenil 15mg/kg BIDIV 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
AntimicrobialsExcept CF
1. jenerasyon sefalosporinCF
IV 2 antipsödomonalAccording to culture
Aspergillus Fumigatus :I.V. Low-dose amphotericine-BIf CMV (+) IV gancyclovirPneumocystis CariniiTrimethoprim-sulfametaxole
Complications
SurgeryAnastomotic strictures Gastrointestinal dysmotility 50% ArrhythmiaInfectious1-6 months viral, fungal infection (24-46%)1-12 months CMV, EBV, P.cariniiLate stage bacterial infection
Post-dilatationBronchial AnastomoticStricture
Immunologic ComplicationsAcute rejection: 1 week-1 monthAsymptomaticFever, dyspnea, hypoxiaX-ray, bilateral infiltrasyonDecline in FEV1 and FVC
Diagnosis: Bronchoscopy, BAL,TB. Biopsy (A0-A4 grade)
Chronic rejection: Several months later
Am.J.of Transplant 2007;285-92
Acute rejectionOften seen after transplantation Reported over 50%Symptoms of fever and malaise However, many patients with minimal symptomsTransbronchial biopsy:
sensitivity specifity
Acute rejection %88 %91Chronic rejection %60 %100
Bronchiolitis ObliteransThe most feared complication of long-term50% in the first five yearsPost-transplant deaths: 40% in the first yearDevelopment is not clear Lower risk of developing in live-donortransplant Diagnosis: biopsy, HRCT, Vent. / Perf. Scintg.Treatment: immunosuppressive
Eur.resp. J. 2004;24:839-45Am.J.of Transplant 2007;7:285-89
Followed by transbronchial biopsy
Malignancy
6.5% in the first 1 yearIn five years, 8.5%LymphomaEBV infection is a riskTreatment: reduction of immunosuppressionCD20 monoclonal antibody
Survival
mean /yearPediatric all ages 3.5Adolescent 5.4Adult 3.8Infant 7.1
J.of Hearth Lung transplant 2002;21:827-40
Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.
Early death:% 56 Acute rejection% 8 Infection
Late death:% 62 Bronchiolitis Obliterans% 22 Infection% 14 Malignancy
Ann Surgery 2002;236:270-76
Paul Aurora, Leah B. Edwards et al. J Heart Lung Transplant 2009; 28: 1023-30.
8 April 2009
M.P.Birth date: 14.06.1994 Admission date: 07.04.2008Complaints: CoughDifficulty breathingWheezing
Diagnosis: Bronchiolitis obliterans+ Bronchiectasis+ Pulmonary Hypertansion
Pulmonary Function Tests
Before Bronchodilat. After Bronchodilat.
FVC 28 % 30 %FEV 1 23 % 24 %
MEF 25 - 75 12 % 13 %
Blood gases: pH: 7.44 pO2: 64 pCO2:48 HCO3: 29 Sat: % 93
DOUBLE - LUNG TRANSPLANTATION
08.04.2009
Postop 15th minute
Treatment
Prednisolon
Mycophenolate Mofetil
Cyclosporine
Basiliximab
Postop 24th hours
Postop 48th hours
Postop 19th day
Post op 6th week
Postop 6th week
Postop 12th month
Postop 12th month
Problems in one year
CMV infection (two times)
Diarrhea (parasytic inf)
Psychiatric problems
Lung Transplantation,is a promising treatment modality despite
it’s all difficulties.Perhaps the most dramatic response;
March 2009 March 2010
Ege Heart-Lung Transplantation Team Adult; Heart and Vascular Surgery
CardiologyPulmonologyThoracic Surgery
Pediatrics; Pediatric SurgeryPulmonologyCardiology
Others: Infectious DiseasesPathologyPhysiotherapyPsychology
Ege Heart-Lung Transplantation Team Adult; Heart and Vascular Surgery
CardiologyPulmonologyThoracic Surgery
Pediatrics; Pediatric SurgeryPulmonologyCardiology
Others: Infectious DiseasesPathologyPhysiotherapyPsychology
Thank you Thank you
for your attentionfor your attention
Assoc. Prof. Figen GAssoc. Prof. Figen Güülen MDlen MD
figen.gulen@ege.edu.trfigen.gulen@ege.edu.tr
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