[II-YIA-03] Evaluation of Fontan failure using the severity of FALD as secondary organ disease
Introduction:
Despite improved survival, Fontan palliated patients are prone to failure of the Fontan circulation andsecond organ dysfunction in the form of Fontan-associated liver disease (FALD). In this study weestablished a graduation of FALD based on laboratory and ultrasonographic liver assessment andanalyzed its applicability for definition of Fontan failure.
Methods:
Liver assessment was performed in 90 consecutive patients with a median age of 17.3 years [IQR14.9]. The extend of FALD was graded as mild, moderate and severe due to laboratory parameters(liver enzymes, Fibrotest®), liver ultrasound and liver stiffness measurement (Fibroscan®).Hemodynamic assessment was performed using echocardiography and cardiac catheterization.Fontan failure was defined as active protein-losing enteropathy, impaired cardiopulmonary exercisecapacity measured by spiroergometry with a VO2max below 45 % of the age adjusted standard valueor more than 2 hospitalisation periods due to cardiopulmonary decompensation within 12 months.
Results:
FALD was graded as mild, moderate and severe in 46, 34 and 10 patients (51.1 %; 37.8 %; 11.1 %).The extend of FALD significantly correlated with exercise capacity (p<0.001) and systolic ventricularfunction based on echocardiography (p=0.003). Invasive pressure measurement revealed a strongcorrelation between the extend of FALD and Fontan pressure (p<0.001), mean pulmonary arterypressure (p=0.002) and end-diastolic ventricular pressure (p=0.003). Fontan failure was detected in 17patients (18.9 %) and correlated with severity of FALD (p<0.001). Death occurred in 4 patients withfailing Fontan circulation, FALD was graded moderate in 1 and severe in 3 patients (p=0.004).
Conclusion:
Detection of FALD is mandatory for Fontan surveillance and monitoring of the failing Fontan. Severityof FALD significantly correlates with exercise capacity, ventricular function and hemodynamics ofFontan palliated patients. FALD graduation might be a useful diagnostic for definition of Fontan failureand evaluation for heart transplant.
Despite improved survival, Fontan palliated patients are prone to failure of the Fontan circulation andsecond organ dysfunction in the form of Fontan-associated liver disease (FALD). In this study weestablished a graduation of FALD based on laboratory and ultrasonographic liver assessment andanalyzed its applicability for definition of Fontan failure.
Methods:
Liver assessment was performed in 90 consecutive patients with a median age of 17.3 years [IQR14.9]. The extend of FALD was graded as mild, moderate and severe due to laboratory parameters(liver enzymes, Fibrotest®), liver ultrasound and liver stiffness measurement (Fibroscan®).Hemodynamic assessment was performed using echocardiography and cardiac catheterization.Fontan failure was defined as active protein-losing enteropathy, impaired cardiopulmonary exercisecapacity measured by spiroergometry with a VO2max below 45 % of the age adjusted standard valueor more than 2 hospitalisation periods due to cardiopulmonary decompensation within 12 months.
Results:
FALD was graded as mild, moderate and severe in 46, 34 and 10 patients (51.1 %; 37.8 %; 11.1 %).The extend of FALD significantly correlated with exercise capacity (p<0.001) and systolic ventricularfunction based on echocardiography (p=0.003). Invasive pressure measurement revealed a strongcorrelation between the extend of FALD and Fontan pressure (p<0.001), mean pulmonary arterypressure (p=0.002) and end-diastolic ventricular pressure (p=0.003). Fontan failure was detected in 17patients (18.9 %) and correlated with severity of FALD (p<0.001). Death occurred in 4 patients withfailing Fontan circulation, FALD was graded moderate in 1 and severe in 3 patients (p=0.004).
Conclusion:
Detection of FALD is mandatory for Fontan surveillance and monitoring of the failing Fontan. Severityof FALD significantly correlates with exercise capacity, ventricular function and hemodynamics ofFontan palliated patients. FALD graduation might be a useful diagnostic for definition of Fontan failureand evaluation for heart transplant.