Table 3.

Recommendations for Timing of Transplantation Referral in Adults With CF

Metric	International Society for Heart and Lung Transplantation	Cystic Fibrosis Foundation
FEV1 % predicted	•< 30 •Rapidly declining lung function despite maximal medical therapy with special pathogens, diabetes, or both	•< 50 with rapidly declining pulmonary function, defined as relative decline of FEV1 > 20% predicted within 12 mo •< 40 with markers of shortened survival •< 30 Note: The above recommendations refer to people 18 y of age and older. For those < 18 y of age, the recommendations are as follows: •< 50 with rapidly declining pulmonary function, defined as relative decline of FEV1 > 20% predicted within 12 mo •< 50 with markers of shortened survival •< 40
6MWT distance	< 400 m	< 400 m
Pulmonary hypertension	In absence of exacerbation •PASP > 35 mm Hg by TTE •Mean PAP > 25 mm Hg by RHC	•PASP > 50 mm Hg by TTE
Special considerations	Increased number of pulmonary exacerbations with any of the following: •Acute respiratory failure requiring NIPPV •Antimicrobial resistance •Nutritional impairment despite supplementation •Pneumothorax •Life-threatening hemoptysis despite bronchial artery embolization Special pathogens: Nontuberculous mycobacteria B cepacia complex	Markers of shortened survival regardless of FEV1 •6MWT distance < 400 m •Hypoxemia •Hypercarbia (Paco2 > 50 mm Hg) •Pulmonary hypertension (PASP > 50 mm Hg by TTE) •≥ 1 exacerbation requiring NIPPV FEV1 < 40% predicted and the following •BMI < 18 kg/m2 and concurrently working to improve nutrition •> 2 exacerbations requiring IV antibiotics •Massive hemoptysis (> 240 mL) requiring ICU admission or bronchial artery embolization •Pneumothorax Additional special considerations for •Younger age •Female sex •Short stature • Special pathogens ○ Nontuberculous mycobacteria ○ B cepacia complex ○ Scedosporium

Source: Summary recommendations per consensus guidelines by the International Society for Heart and Lung Transplantation¹² and the Cystic Fibrosis Foundation.⁴⁵ 6MWT = 6-min walk test; CF = cystic fibrosis; NIPPV = noninvasive positive pressure ventilation; PAP = pulmonary artery pressure; PASP = pulmonary artery systolic pressure; RHC = right heart catheterization; TTE = transthoracic echocardiogram.