330.1 🎓 醫孞生版

330.1.0.1 📌 䞀頁重點

330.1.0.1.1 Indications
330.1.0.1.1.1 Most Common
  • IPF (idiopathic pulmonary fibrosis) — top indication
  • COPD (severe) — 2nd most common
  • CF (cystic fibrosis) — esp before modulator era
  • PAH (pulmonary arterial hypertension) — Group 1
  • α1-antitrypsin deficiency
  • Bronchiectasis (non-CF)
  • CTD-ILD (scleroderma, RA)
  • Sarcoidosis (stage IV)
  • HP (fibrotic)
  • Eisenmenger syndrome (heart-lung transplant)
  • Re-transplant (CLAD or graft dysfunction)
330.1.0.1.1.2 Less Common
  • LAM (after sirolimus failure)
  • PLCH
  • PAP refractory
  • Pediatric: CF, PAH, congenital heart defects
330.1.0.1.2 Patient Selection

330.1.1 Listing Criteria

COPD: - BODE index ≥ 7 - FEV1 < 20% predicted + age < 65 - Hypoxia + hypercapnia - PH

IPF: - FVC < 50% or DLCO < 35% - Significant desaturation on exercise - Hospitalization for exacerbation

CF: - FEV1 < 30% predicted - ↑ Frequency of hospitalizations - Hypoxia + hypercapnia - PH

PAH: - NYHA III-IV despite optimal PAH-specific therapy - Cardiac index < 2 L/min/m² - mRAP > 15 - 6MWD < 350 m

330.1.1.0.0.1 Contraindications

Absolute: - Active or recent malignancy (within 5 years, except non-melanoma skin) - Active uncontrolled infection (HIV with detectable viral load typically) - Active substance abuse (within 6 months for smoking, alcohol, drugs) - Severe extrathoracic comorbidities - Severe psychosocial issues (non-adherence, no support) - BMI > 30-35 (varies by center) - Severe peripheral or coronary artery disease

Relative: - Age > 70 (with good function) - BMI < 17 (cachectic) - Severe osteoporosis - Diabetes with end-organ damage - Mild renal dysfunction

330.1.1.0.1 Lung Allocation Score (LAS)
330.1.1.0.1.1 Concept
  • Replaced wait-time-based system in 2005
  • Based on:
    • Urgency (estimated wait list mortality)
    • Transplant benefit (estimated post-transplant survival vs without)
  • Higher LAS = priority allocation
330.1.1.0.1.2 Score Range
  • 0-100
  • LAS ≥ 35 considered for listing
  • Higher = more urgent
330.1.1.0.1.3 Disease-Specific LAS
  • IPF: typically higher LAS
  • COPD: typically lower LAS
  • PAH: variable
  • CF: variable
330.1.1.0.2 Surgery

330.1.2 Procedure Types

Bilateral Lung Transplant (BLT): - Preferred for most - Sequential lungs replaced - Cardiopulmonary bypass not always needed - Better long-term outcomes

Single Lung Transplant (SLT): - For COPD without significant infection - Shorter wait - Older patients - Less morbidity

Heart-Lung Transplant: - For Eisenmenger - Severe PAH with biventricular failure - Failed Fontan - Rare; donor scarcity

Living Donor (Lobar): - 2 donors → 2 lower lobes recipient - Pediatric mainly - Rare in US/Europe - More common in Japan

330.1.3 Approach

  • Sternotomy or thoracotomy
  • Sequential implantation
  • Cardiopulmonary bypass selective
  • Endobronchial intubation
  • Cold ischemia time < 6-8 hours preferred

330.1.4 Donor Selection

Standard Donors: - Age < 55 - Smoking < 20 pack-years - Adequate PaO2/FiO2 - Clear CXR - No infection - No malignancy

Extended Criteria Donors: - Older, smoker, marginal function - Used with caution - Increased donor pool

DCD (Donation After Circulatory Death): - After cardiac death (vs brain-dead) - Increasing use - Comparable outcomes

Ex-Vivo Lung Perfusion (EVLP): - Hub for donor optimization - Reassess marginal lungs - Extended preservation time - Therapeutic interventions possible (lavage, etc.)

330.1.4.0.1 Post-Transplant Care

330.1.5 Immunosuppression

Induction: - IL-2 receptor antagonist (basiliximab) OR - Anti-thymocyte globulin (ATG) - ± High-dose steroids

Maintenance Triple Therapy: - Calcineurin Inhibitor (CNI): - Tacrolimus (preferred — trough 10-15 ng/mL early, 5-10 chronic) - Cyclosporine - Antiproliferative: - Mycophenolate mofetil (MMF) (preferred) - Azathioprine - Corticosteroids: prednisone, tapered over months

Steroid-Sparing: - After chronic rejection or side effects - mTOR inhibitor (sirolimus, everolimus) — limited use

330.1.6 Prophylaxis

Bacterial: - TMP-SMX (PCP, Toxoplasma, Listeria) - Anti-MRSA / Pseudomonas peri-op

Viral: - Valganciclovir (CMV) for high-risk - Acyclovir (HSV, VZV)

Fungal: - Voriconazole or itraconazole for at-risk - Risk assessment based on donor + recipient

330.1.6.0.1 Complications

330.1.7 Primary Graft Dysfunction (PGD)

  • Within 72 hours
  • Severity: 0-3
  • Bilateral infiltrates + hypoxemia
  • Ventilator support, ECMO if severe
  • Risk for chronic dysfunction

330.1.8 Acute Rejection

Acute Cellular Rejection (ACR): - T-cell mediated - Common in first year (40-50%) - Grade A0-A4 by histology - Treatment: pulse high-dose corticosteroids; rATG for refractory

Antibody-Mediated Rejection (AMR): - Donor-specific antibodies (DSAs) - Less common, more refractory - Treatment: plasmapheresis + IVIG + rituximab + bortezomib

330.1.9 Chronic Lung Allograft Dysfunction (CLAD)

Spectrum: - BOS (bronchiolitis obliterans syndrome): small airway obliteration; obstructive pattern; small airway scarring - RAS (restrictive allograft syndrome): restrictive pattern; pleural + parenchymal scarring; worse prognosis

Risk Factors: - Acute rejection episodes - CMV infection - Pseudomonas / Aspergillus - GERD - Bronchiolitis obliterans

Treatment: - Azithromycin (Wijdicks for chronic) - Optimize immunosuppression - Treat triggers (GERD, infection) - Re-transplantation for end-stage

330.1.10 Infections

Bacterial: - Pseudomonas (especially CF) - MRSA - Other GNR

Viral: - CMV (most common viral) - Community respiratory viruses (RSV, influenza, COVID) - BK virus - EBV

Fungal: - Aspergillus (most common) - Candida - Mucormycosis

330.1.11 Malignancy

PTLD (Post-Transplant Lymphoproliferative Disorder): - EBV-driven mostly - Lymphoma spectrum - Reduce IS + rituximab + chemo

Skin Cancer: - BCC, SCC, melanoma - UV protection + surveillance

Solid Organ Cancers: - Lung, GI, oral - Surveillance

330.1.12 Renal Dysfunction

  • CNI nephrotoxicity
  • HTN-related
  • 25-30% develop ESRD
  • Renal-friendly IS regimens

330.1.13 Bone Loss

  • Steroid-induced osteoporosis
  • Bisphosphonates

330.1.14 Metabolic

  • Diabetes (steroid + CNI)
  • Hyperlipidemia
  • Hypertension
330.1.14.0.1 Outcomes

330.1.15 Survival

  • 1-year: 85%
  • 5-year: 55%
  • 10-year: 30%
  • BTT for transplant: somewhat better

330.1.16 Cause of Death

  • CLAD (1-5 year)
  • Infection (variable)
  • Cancer (long-term)
  • Cardiovascular

330.1.17 Quality of Life

  • Generally improved
  • Restrictions: immunocompromise
  • Return to activities (often)
330.1.17.0.1 Special Considerations

330.1.18 Pre-Transplant Optimization

  • Pulmonary rehabilitation
  • Smoking cessation (6 months minimum)
  • Nutrition optimization
  • Vaccinations (avoid live ones after transplant)
  • Mental health support

330.1.19 Pediatric Transplant

  • CF, PAH most common
  • Smaller donor pool
  • Living donor lobar option

330.1.20 Re-Transplantation

  • For CLAD
  • Worse outcomes than primary
  • Multidisciplinary

330.1.20.1 🩺 床邊速查

  • Indications: IPF (top), COPD, CF, PAH, α1-AT, ILD, sarcoid, bronchiectasis, Eisenmenger
  • LAS: priority allocation system
  • Bilateral lung transplant preferred
  • Heart-lung transplant for Eisenmenger
  • Immunosuppression: tacrolimus + MMF + steroid
  • PGD within 72 hours
  • CLAD = BOS + RAS (chronic dysfunction, 1-5 years)
  • CMV most common viral infection
  • Aspergillus most common fungal
  • PTLD: EBV-driven, rituximab + reduce IS