Table 7. ISHLT indications for lung transplant referral and listing in cystic fibrosis.
| Referral |
| FEV1 ≤30% |
| Patient with advanced disease and rapidly falling FEV1 despite optimal treatment, especially female patients infected with NTM or BCC |
| 6MWD <400 m |
| PH in absence of hypoxic exacerbation |
| Increased frequency of exacerbations with: |
| ARF requiring NIV |
| Increased antibiotic resistance and poor clinical recovery |
| Pneumothorax |
| Worsening nutritional status |
| Life threatening hemoptysis despite bronchial embolization |
| Listing |
| Chronic respiratory failure |
| With hypoxia PaO2 <60 mmHg |
| With hypercapnia PaCO2 >50 mmHg |
| Long-term NIV therapy |
| PH |
| Frequent hospitalizations |
| Rapid lung function decline |
| WHO functional class IV |
FEV1, forced expiratory volume in 1 second; NTM, non-tuberculous mycobacteria; BCC, Burkholderia cepacia complex; 6MWD, six-minute walk distance; PH, pulmonary hypertension; ARF, acute respiratory failure; NIV, noninvasive ventilation; PaO2, partial pressure of oxygen; PaCO2, partial pressure of carbon dioxide; PH, pulmonary hypertension; WHO, World Health Organization.