Table 5:
Standardized exception points in the United States (Modified from [19])
| Indication | Criteria | eMELD points assigned |
|---|---|---|
| Hepatopulmonary syndrome | 1. Presence of portal hypertension (ascites, varices, splenomegaly, or thrombocytopenia) 2. Presence of pulmonary shunt 3. PaO2 <60 mmHg on room air within 30 days of initial request 4. No clinically significant underlying primary pulmonary disease |
3 points below MMaT (>18 yrs) MMAt (12–18 yrs) MPaT (<12 y) |
| Portopulmonary hypertension | 1. Initial MPAP level ≥35 mmHg and initial PVR level ≥240 dynes.s.cm−5 2. No other causes of pulmonary hypertension 3. Initial transpulmonary gradient to correct for volume overload 4. Documentation of treatment 5. Post treatment MPAP <35 mmHg and post treatment PVR <400 dynes.s.cm−5 within 30 days of exception request 6. Presence of portal hypertension |
3 points below MMaT (>18 y) MMAt (12–18 y) MPaT (<12 y) |
| Familial amyloid polyneuropathy | 1. Listed for heart transplant or echocardiogram within 30 days of initial exception showing ejection fraction <40% 2. Patient can walk without assistance 3. Confirmation of TTR gene mutation 4. Biopsy proven amyloid |
3 points below MMaT (>18 y) MMAt (12–18 y) MPaT (<12 y) |
| Cystic fibrosis | 1. Diagnosis confirmed by genetic analysis 2. FEV1 <40% predicted within 30 days of initial exception request |
3 points below MMaT (>18 y) MMAt (12–18 y) MPaT (<12 y) |
| Cholangiocarcinoma | Unresectable hilar cholangiocarcinoma without extrahepatic spread; protocol includes neoadjuvant therapy and laparoscopy One of the following: • Biopsy/cytology results showing malignancy • CA 19–9 >100 U/mL in absence of cholangitis • Aneuploidy • Hilar mass <3 cm in radial diameter |
3 points below MMaT (>18 y)
MMAt (12–18 y) MPaT (<12 y) |
| Metabolic disease | Urea cycle disorder or organic acidemia |
MMAt (12–18 y)
MPaT (<12 y) Status 1B if not transplanted in 30 days |
| Primary hyperoxaluria | 1. Listed for kidney transplant 2. AGT deficiency proven 3. eGFR <25 ml/min on two occasions at least 42 days apart |
MMaT (>18 y)
3 points above MMAt (12–18 y) 3 points above MPaT (<12 y) |
MMaT = Median Model for End-Stage Liver Disease at Transplant; PaO2 = Partial pressure of oxygen; MPAP = Mean pulmonary arterial pressure; PVR = Pulmonary vascular resistance; TTR = Transthyretin; FEV1 = Forced expiratory volume at 1 second; CA 19-9 = Carbohydrate antigen 19-9; AGT = Alanine glyoxylate aminotransferase; eGFR = estimated glomerular filtration rate