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243: LUNG TRANSPLANT: PEDIATRIC

Kay Tasso, PhD, PT, PCS

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

  • 6C: Impaired Ventilation, Respiration/Gas Exchange, and Aerobic Capacity/Endurance Associated With Airway Clearance Dysfunction

PATIENT PRESENTATION

A 16-year-old female is referred to a local outpatient physical therapy clinic 4 months after receiving a double-lung transplant due to cystic fibrosis (CF). She was receiving physical therapy three times a week at the hospital where she received the transplant. Her goal is to progress to swimming as her primary aerobic workout but has been working to progress her exercise tolerance and endurance using a treadmill and stationary bike. She also wants to increase her overall strength.

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 are congenital heart disease (CHD), peripheral vascular disease (PVD), and CF

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

General Considerations

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

  • Long-term outcomes are limited

  • Contraindications to lung transplant

    • Malignancy

    • Sepsis

    • Tuberculosis

    • Hepatitis

    • Neuromuscular disease

Demographics2

  • Forty-four lung transplants in 2011

  • Males more common than females

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

  • Double-lung transplants 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

FIGURE 243-1

Number of patients awaiting lung transplants and number of lung transplants performed each year. Data derived from Scientific Registry of Transplant Recipients, 2002 (http://www.ustransplant.org).

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FIGURE 243-2

Different types of tissue transplants (grafts). (From Chandrasoma P, Taylor CR. Concise Pathology 3rd ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

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Possible Contributing Causes

  • Pediatric3

    • CHD

    • Surfactant dysfunction

    • Idiopathic pulmonary fibrosis (IPF)

    • Bronchiolitis obliterans (BO)

    • Pulmonary vascular disorder

    • Chronic lung disease of infancy

    • Pulmonary fibrosis/Idiopathic pulmonary fibrosis (IPF)

    • Pulmonary vein anomalies

    • CF

  • Adult

    • Pulmonary hypertension

    • Emphysema

    • CF

    • Alpha-1 antitrypsin ...

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