Introduction
Patients and Methods
Patients
Laboratory Assessment
Liver Ultrasound and Transient Elastography
Hemodynamic Assessment
Statistical Analysis
Results
Patient Characteristics
Demographic assessment | No. (%)/median [IQR] |
---|---|
Patient age (years) | 16.0 (14.2) |
Age at Fontan operation (years) | 3.5 (3.3) |
Duration of Fontan circulation (years) | 10.3 (14.7) |
Gender | |
Male | 71 (49.0) |
Underlying anatomy | |
Tricuspid atresia | 45 (31.0) |
Double inlet left ventricle | 21 (14.5) |
Hypoplastic left heart syndrome | 22 (15.2) |
Complex transposition of great arteries | 11 (7.6) |
Unbalanced AVSD | 16 (11.0) |
RV hypoplasia with PA/IVS | 11 (7.6) |
Other | 19 (13.1) |
Predominant ventricular morphology | |
Left | 95 (66.5) |
Right | 50 (34.5) |
Fontan type | |
Intracardiac lateral tunnel | 22 (15.2) |
Extracardiac conduit | 109 (75.2) |
Other | 14 (9.7) |
Fenestration | |
Primary | 61 (14.5) |
Secondary | 6 (4.1) |
PLE | 12 (8.3) |
Laboratory Assessment
Hepatic assessment | Median (IQR) | No. (%) abnormal |
---|---|---|
Laboratory analysis | ||
AST (U/l) | 36.6 (13.5) | 27/133 (20.3) |
ALT (U/l) | 34.7 (16.5) | 31/133 (23.3) |
γGT (U/l) | 71.1 (43.0) | 115/133 (86.5) |
Bilirubin (mg/dl) | 1.0 (0.6) | 32/130 (24.6) |
Thrombocytes (K/µl) | 207.2 (87.0) | 37/133 (27.8) |
Fibrotest® | ||
F0 | 6/101 (5.9) | |
F1 | 8/101 (7.9) | |
F2 | 49/101 (48.5) | |
F3 | 24/101 (23.8) | |
F4 | 14/101 (13.9) | |
Liver ultrasound findings | ||
Hepatomegaly | 17/117 (14.5) | |
Splenomegaly | 38/117 (32.5) | |
Heterogeneous parenchymal echotexture | 83/117 (70.9) | |
Lobar atrophy/hypertrophy | 27/117 (23.1) | |
Liver vein dilatation | 69/117 (59.0) | |
Abnormal liver vein architecture | 32/117 (27.4) | |
Hyperechogenic lesions | 10/117 (8.5) | |
Surface nodularity | 18/117 (15.4) | |
Ascites | 13/117 (11.1) | |
Transient elastography | 27.7 (14.5) | |
Hemodynamic assessment | ||
Impairment of ventricular function | ||
None/mild | 122/145 (84.1) | |
Moderate | 20/145 (13.8) | |
Severe | 3/145 (2.1) | |
AV valve insufficiency | ||
None/mild | 122/145 (84.1) | |
Moderate | 21/145 (14.5) | |
Severe | 2/145 (1.4) | |
PAP (mmHg) | 12.0 (4.0) | |
EDP (mmHg) | 8.0 (3.8) | |
TPG (mmHg) | 3.5 (3.0) | |
LVP (mmHg) | 12.0 (5.0) | |
LVWP (mmHg) | 13.0 (4.0) |
Liver Ultrasound and Transient Elastography
Hemodynamic Assessment
The Impact of Fontan Duration on Hemodynamics and FALD
Risk Factor Analysis for the Development of FALD
Variable | Univariable analysis | Multivariable analysis | ||||
---|---|---|---|---|---|---|
OR | 95% CI | P value | OR | 95% CI | P value | |
Systemic ventricle | 0.83 | 0.35–2.0 | 0.681 | |||
Fontan type | 0.36 | 0.11–1.14 | 0.082 | 0.3 | 0.05–0.12 | 0.443 |
Sinus rhythm | 0.24 | 0.06–0.41 | 0.008 | 0.21 | 0.08–0.26 | 0.298 |
Fontan duration | 0.78 | 0.72–0.89 | < 0.001 | 0.77 | 0.68–0.87 | < 0.001 |