TABLE 1.
Studies using liver biopsy to diagnose the FALD
| References | Study | N (n) | Population | Age at Ex (y)a | Duration after Fontan op (y)a | Reason for liver bx | Scoring system | Anatomy | Systemic ventricle | Fontan type | Imaging modality | Correlation with histopathology |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Kiesewetter et al11 | Retro. | 11 | Both | 24.6 ± 8 | 14.1 ± 5.0 | Clinical evaluation | METAVIR Fibrosis Score | TA DILV |
No mention | AP LT TCPC |
CT | Fibrosis score ≤2 vs. >3 –zonal enchancement (n) 3/4 vs. 1/7, (p = 0.033) –reticular enhancement (n) 2/4 vs. 6/7 (p = 0.183) |
| Kutty et al12 | Pros. | 41 | Both | 13.8 ± 6.3 | 11 ± 6 | To confirm the image | No mention | No mention | RV 39% LV 61% |
LT 54% EC 46% |
SWE | Fibrosis score <2 vs. ≥2 13.4 ± 1.3 vs. 19.8 ± 2.6 kPa, (p = 0.002) |
| Wu et al13 | Pros. | 50 (10) | Both | 13.1 (2.4–57.7) | 9.9 (0.1–32.5) | To confirm the image | METAVIR fibrosis staging | TA 18% DILV 16% HLHS 32% Heterotaxy 6% |
RV 54% LV 46% |
AP 8% LT 82% EC 8% |
TE | 1. Portal fibrosis score ≤2 vs. >3 34.8 (14.3–66.5) vs. 21.1 (16.8–22.6) kPa, (p = 0.14) 2. Central fibrosis score ≤2 vs. >3 14.3, 16.8 vs. 26.0 (21.1–66.5) kPa (p = 0.05) |
| Poterucha et al14 | Retro. | 50 | Adults | 25 (21–33) | 22 (16–26) | Clinical evaluation | Ishak Scheuer Schwartz |
TA 24% PA with IVS 2% DILV 32% DORV 16% DIRV 2% AVSD 12% Heterotaxy 12% |
RV 40% LV 60% |
AP 40% LT 34% EC 26% |
MRE | Correlation coefficientb
R = 0.74 (p =0.02) |
| Wu et al15 | Retro. | 68 | Both | 23.2 (5.0–52.7) | 18.1 (1.2–32.7) | Clinical evaluation | METAVIR fibrosis staging | TA 21% DILV 23% HLHS 10% Heterotaxy 19% others 26% |
RV 37% LV 56% mix 7% |
AP 38% LT 50% EC 6% |
||
| Evans et al16 | Retro. | 30 | Both | 17 (6–45) | 15 (1–29) | Clinical evaluation | Modified Scheuer staging and sinusoidal fibrosis staging | No mention | No mention | No mention | SWE | Correlation coefficientb
R = 0.6 (p = 0.002) |
| Goldberg et al3 | Retro. | 67 | Both | 17.3 ± 4.5 | 14.9 ± 4.5 | Routine screening | Calculate quantitative collagen deposition by Leica Biosystems | No mention | RV 55% LV or mixed 44% |
LT 60% EC 36% |
||
| Munsterman et al17 | Pros. | 38 | Adults | 27 ± 6.6 | 21.4 ± 5.5 | Routine screening | Fontan-specific fibrosis scores and collagen proportion area | TA and PA 47% DILV 29% DORV 5% HLHS 10% DORV 5% Heterotaxy 3% |
No mention | AP 37% LT 18% EC 40% |
TE | Fibrosis score ≤2 vs. >3 21.3 (14.3–29.1) vs. 26.0 (15.1–28.9) kPa (p = 0.511) |
| Silva-Sepulveda et al18 | Retro | 49 | Both | 17.8 (5–39) | 15.2 (2–33) | Routine screening | Ishak fibrosis stage congestive hepatic fibrosis score and Modified Ishak congestive hepatic fibrosis | TA 18% HLHS 14% PA w IVS 12% DILV 12% DORV 10% AVSD 10% Heterotaxy 16% Others 6% |
RV 39% LV 61% |
AP 4% LT 59% EC 37% |
MRE | Correlation coefficientb
R = 0.53 (p =0.004) |
| Patel et al19 | Retro | 57 | Ped | 14.6 ± 2.8 | 11.4 ± 2.9 | Routine screening | Congestive Hepatic Fibrosis Score | No mention | RV 56% LV or Mixed 44% |
EC 93% | ||
| Emamaullee et al20 | Retro | 106 | Ped | 14.4 ± 3.5 | 10.8 ± 3.6 | Routine screening | Congestive Hepatic Fibrosis Score | No mention | RV 52% LV 44.% |
LT 6% EC 94% |
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| Borquez et al21 | Retro | 125 | Both | 15 (2–50.5) | 12.7 (1−31) | Routine screening | Ishak fibrosis stage and Congestive hepatic fibrosis score and Modified Ishak congestive hepatic fibrosis | TA 32% Heterotaxy 14% HLHS 27% PA wIVS 14% DILV 17% DORV 8% AVSD 8% Others 6% |
RV 51% LV 46% |
No mention | ||
| Shin et al22 | Retro | 45 | Adults | 25.9 ± 6.5 | 20.8 ± 4.8 | Routine screening | Batts and Ludwig scoring system | No mention | RV 42.2% | AP 27% LT 49% EC 24% |
TE | Fibrosis score ≤2 vs. >3 24.8 ± 20.6 vs. 23.3 ± 8.2 kPa (p = 0.85) |
| Bütikofer et al23 | Pros. | 50 | Adults | 25.9 (19.5–34.0) | 21.8 (16.7–27.8) | Clinical evaluation | Congestive Hepatic Fibrosis Score | No mention | RV 20% LV 80% |
AP 38% TCPC 62% |
TE | Fibrosis score ≤2 vs. >3 23.4 (13.0–33.63) vs. 21.3 (17.3–26) kPa (p = 0.911) |
| Martin de Miguel et al24 | Retro | 159 (31) | Adults | 31.5 ± 9.3 | Not assessed | Clinical evaluation | Congestive Hepatic Fibrosis Score | TA 33% DILV 30% DORV 12% PA w IVS 11% HLHS 4% Heterotaxy 10% others 11% |
RV 22% | AP 58% LT 20% EC 9% |
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| Jarasvaraparn et al25 | Retro | 66 | Both | 24.3 ± 9.3 | 20.3 ± 7.1 | Routine screening and clinical evaluation | Congestive Hepatic Fibrosis Score | No mention | No mention | No mention | TE | Fibrosis score ≤2 vs. >3 25.66 ± 15.88 vs. 28.35 ± 15.84 kPa (p = 0.67) |
a
Mean ± SD or median (range or IQR).
b
refer to the Pearson correlation coefficient
Abbreviations: AP, atriopulmonary; AVSD, atrioventricular septal defect; Bx, biopsy; DILV, double inlet left ventricle; DIRV, double inlet right ventricle; DORV, double outlet right ventricle; EC, extracardiac conduit; Ex, examination; IVS, intact ventricular septum; LT, lateral tunnel; LV, left ventricle; MRE, magnetic resonance elastography; N, total number of patients; n, number of cases that received a liver biopsy if not all patients underwent the testing; PA, pulmonary atresia; Ped, pediatrics; Prosp, prospective; Retro, retrospective; RV, right ventricle; SWE, shear wave elastography; TA, tricuspid atresia; TE, transient elastography.