Lung transplantation is a therapeutic consideration for many patients with nonmalignant end-stage lung disease, and it prolongs survival and improves quality of life in appropriately selected recipients. Since 1985 more than 25,000 procedures have been recorded worldwide, and ~2200 transplants have been reported annually in recent years.
The indications span the gamut of lung diseases. The most common indications in the last few years have been chronic obstructive pulmonary disease (COPD), ~30%; idiopathic pulmonary fibrosis (IPF), ~30%; cystic fibrosis (CF), ~15%; α1-antitrypsin deficiency emphysema, ~3%; and idiopathic pulmonary arterial hypertension (IPAH), ~2%. Other diseases have accounted for the balance of primary indications, and retransplantation has accounted for ~4% of procedures.
Transplantation should be considered when other therapeutic options have been exhausted and when the patient's prognosis is expected to improve as a result of the procedure. Survival rates after transplantation can be compared with predictive indices for the patient's disease, but each patient's clinical course must be integrated into the assessment, too. Moreover, quality of life is a primary motive for transplantation for many patients, and the prospect of improved quality-adjusted survival is often attractive even if the survival advantage itself may be marginal.
Disease-specific consensus guidelines for referring patients for evaluation and for proceeding with transplantation are summarized in Table 266-1 and are linked to clinical, physiologic, radiographic, and pathologic features that influence the prognosis of the respective diseases. Candidates for lung transplantation are also thoroughly screened for comorbidities that might affect the outcome adversely. Conditions such as systemic hypertension, diabetes mellitus, gastroesophageal reflux, and osteoporosis are not unusual, but if uncomplicated and adequately managed, they do not disqualify patients from transplantation. The upper age limit is ~65–70 years at most centers.
Table 266-1 Disease-Specific Guidelines for Referral and Transplantation |Favorite Table|Download (.pdf)
Table 266-1 Disease-Specific Guidelines for Referral and Transplantation
|Chronic Obstructive Pulmonary Disease|
BODE index >5
BODE index 7–10
any of the following criteria:
Hospitalization for exacerbation, with PaCO2 >50 mmHg
Pulmonary hypertension or cor pulmonale despite oxygen therapy
FEV1<20% with either DLCO <20% or diffuse emphysema
FEV1<30% or rapidly declining FEV1
Hospitalization in ICU for exacerbation
Increasing frequency of exacerbations
Refractory or recurrent pneumothorax
Recurrent hemoptysis not controlled by bronchial artery embolization
Oxygen-dependent respiratory failure
|Idiopathic Pulmonary Fibrosis|
Pathologic or radiographic evidence of UIP regardless of vital capacity
Pathologic or radiographic evidence of UIP
any of the following criteria
Decrement in FVC ≥10% during 6 months of follow-up
Decrease in SpO2 below 88% during a 6-min walk test
Honeycombing on HRCT (fibrosis score >2)
|Idiopathic Pulmonary Arterial Hypertension|
NYHA functional class III or IV regardless of therapy
Rapidly progressive disease
Failing therapy with intravenous epoprostenol (or equivalent drug)
Persistent NYHA ...