Early diagnosis and management of complications associated with Fontan physiology hold the most promise for improving longevity. Imaging after stage 3 repair can assess anatomy, ventricular and valve function, blood flow, and myocardial fibrosis. Anatomic considerations include the presence of thrombus in the circuit, the patency of the extracardiac conduit, stenoses within the caval veins and pulmonary arteries, and the size and location of aortopulmonary collaterals and venovenous collaterals. Considerations for assessment of flow include quantification of the degree of aortopulmonary and venovenous collateralization, the relative flow within the 2 branch pulmonary arteries, and valvular regurgitation. Considerations for assessment of ventricular function and morphology include screening for systolic and diastolic dysfunction, ventricular type and shape, and myocardial characterization. Arteriography Coronary with Ventriculography Clinical indications for this examination include investigating symptoms associated with decreased ventricular function such as unexplained volume retention, fatigue, exercise limitation, and cyanosis. Cardiac catheterization with coronary angiography can evaluate coronary artery anatomy, and ventriculography can be used to estimate the function of the single ventricle and valvular flow. Cardiac catheterization is also helpful to assess the presence and severity of aortopulmonary, systemic-pulmonary venous, and systemic venovenous collaterals and also provides access to perform embolization of clinically significant collaterals. Cardiac catheterization is also suggested in the Congenital or Acquired Heart Disease assessment of patients with failing Fontan physiology and features of Fontan-associated liver disease. Imaging of a patent fenestration is seen well with direct angiography on cardiac catheterization [52].
Congenital or Acquired Heart Disease. Early diagnosis and management of complications associated with Fontan physiology hold the most promise for improving longevity. Imaging after stage 3 repair can assess anatomy, ventricular and valve function, blood flow, and myocardial fibrosis. Anatomic considerations include the presence of thrombus in the circuit, the patency of the extracardiac conduit, stenoses within the caval veins and pulmonary arteries, and the size and location of aortopulmonary collaterals and venovenous collaterals. Considerations for assessment of flow include quantification of the degree of aortopulmonary and venovenous collateralization, the relative flow within the 2 branch pulmonary arteries, and valvular regurgitation. Considerations for assessment of ventricular function and morphology include screening for systolic and diastolic dysfunction, ventricular type and shape, and myocardial characterization. Arteriography Coronary with Ventriculography Clinical indications for this examination include investigating symptoms associated with decreased ventricular function such as unexplained volume retention, fatigue, exercise limitation, and cyanosis. Cardiac catheterization with coronary angiography can evaluate coronary artery anatomy, and ventriculography can be used to estimate the function of the single ventricle and valvular flow. Cardiac catheterization is also helpful to assess the presence and severity of aortopulmonary, systemic-pulmonary venous, and systemic venovenous collaterals and also provides access to perform embolization of clinically significant collaterals. Cardiac catheterization is also suggested in the Congenital or Acquired Heart Disease assessment of patients with failing Fontan physiology and features of Fontan-associated liver disease. Imaging of a patent fenestration is seen well with direct angiography on cardiac catheterization [52].