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Condition/Disorder Synonyms

  • Single-lung transplant

  • Double-lung transplant

  • Living donor lobar transplant

ICD-9-CM Code

  • V42.6 Lung replaced by transplant

ICD-10-CM Code

  • Z94.2 Lung transplant status

Preferred Practice Pattern

Key Features

Description

  • Diseased or defective lung (single-lung transplant) or lungs (double-lung transplant), or a portion thereof is removed

  • Healthy partial lung from a living donor or complete donor lung(s) from a deceased person is implanted

Essentials of Diagnosis

  • Most common causes in children under 1 year of age is congenital heart disease (CHD), peripheral vascular disease (PVD), and cystic fibrosis (CF)

  • Lung transplants considered when patient death is imminent within 1 to 2 years and all other medical options have been exhausted and are ineffective1

General Considerations

  • Timing of transplant for over 12 years of age depends on lung allocation score (LAS)

  • Long term outcomes are limited

  • Contraindications to lung transplant

    • Malignancy

    • Sepsis

    • Tuberculosis

    • Hepatitis

    • Neuromuscular disease

Demographics2

  • 44 lung transplants in 2011 at the Mayo clinic in Florida

  • Males more common than females

  • 0.02% pediatric lung transplants (<18 years old)

  • Double lung transplants are 5 to 6 times more common than single lung transplants

Clinical Findings

Signs and Symptoms

  • Respiratory failure

  • Unable to tolerate exercise

  • Hypoxemia

  • Carbon dioxide retention

  • Failure to thrive

  • Abnormal pulmonary function tests (PFTs)

  • Syncope

  • Cyanosis

Functional Implications

  • Developmental delays

  • Dependent for ADLs

  • Immunosuppression

Possible Contributing Causes

  • Pediatric4

    • Congenital heart disease (CHD)

    • Surfactant dysfunction

    • Idiopathic pulmonary disorders (IPH)

    • Bronchiolitis obliterans (BO)

    • Pulmonary vascular disorder

    • Chronic lung disease of infancy

    • Pulmonary fibrosis

    • Pulmonary vein anomalies

    • Cystic fibrosis

  • Adult

    • Pulmonary hypertension

    • Emphysema

    • Cystic fibrosis

    • Alpha-1 antitrypsin deficiency1

    • Idiopathic pulmonary fibrosis3

    • Interstitial lung disease3

    • Chronic obstructive pulmonary disease3

Differential Diagnosis

  • Post surgical issues

    • Rejection

    • Infection

    • Bronchiolitis obliterans

Means of Confirmation or Diagnosis

Laboratory Tests

  • Complete blood count (CBC)

  • Arterial blood gases

  • Prothrombin time (PT)

  • Liver function test

  • Partial thromboplastin time (PTT)

  • PFTs

    • Forced expiratory volume in one second (FEV1)

    • Arterial oxygen tension (PaO2)

    • Arterial carbon dioxide tension (PaCO2)

  • HIV

  • Immunoglobulin G levels (IgG)

Imaging

  • X-ray

  • Chest CT scan

  • Echocardiography

Diagnostic Procedures

  • Electrocardiogram (EKG)

  • Lung biopsy

  • Bronchoscopy

  • Transbronchial biopsy to assess for rejection

  • Thoracosternotomy3

  • Cardiac catheterization

  • Thoracotomy

Treatment

Medication

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