Validation of Cardiac Magnetic Resonance Sequences in Patients With Single Ventricles

Single ventricle defects make up the severe end of the congenital heart disease spectrum. The Fontan operation leads to a complete redirection of systemic venous blood outside of the heart and directly into the lungs. Patients with single ventricles suffer from multiple complications. Their survival has improved over the past decades, but is still severely compromised compared to the general population. Their evaluation includes echocardiography and functional status by history and/or exercise testing. In longer intervals or if echocardiography does not allow visualization of all cardiovascular structures, cardiac magnetic resonance (CMR) is employed. Many patients also undergo more invasive cardiac catheterization. In single ventricle patients, cardiac imaging has to address the questions of the patency of the Fontan pathways, i.e. all systemic veins, the Fontan conduit, and the pulmonary arteries, and of the function of the single ventricle (including myocardial function and valve function). By using conventional imaging methods in Fontan patients, Ghelani et al. identified a CMR-based ventricular end-diastolic volume of \> 125 ml/m2 and an echocardiographic global circumferential strain (GCS) value of higher than -17% to be strong predictors for a combined adverse outcome of death or heart transplantation. While interobserver reproducibility of single ventricle ejection fraction is similarly high by echocardiography, CMR is better in reliably measuring ventricular mass and diastolic volume and can provide additional information by MR feature tracking (strain), T1 mapping, and 4D flow measurements. Several substances that can be measured in the peripheral blood are being increasingly investigated as biomarkers of heart failure. In conclusion, several advanced CMR sequences and new biomarkers have a potential role in the assessment and risk stratification of single ventricle patients. Every single published study has elucidated a particular use and aspect of th