Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study

BACKGROUND: As right ventricular dysfunction is a major cause of adverse outcome in patients with hypoplastic left heart syndrome, the aim was to assess right ventricular function and deformation after Fontan completion by performing 2‐dimensional cardiovascular magnetic resonance feature tracking in serial cardiovascular magnetic resonance studies. METHODS AND RESULTS: Cardiovascular magnetic resonance examinations of 108 patients with hypoplastic left heart syndrome (female: 31) were analyzed. Short‐axis cine images were used for right ventricular volumetry. Two‐dimensional cardiovascular magnetic resonance feature tracking was performed using long‐axis and short‐axis cine images to measure myocardial global longitudinal, circumferential, and radial strain. All patients had at least 2 cardiovascular magnetic resonance examinations after Fontan completion and 41 patients had 3 examinations. Global strain values and right ventricular ejection fraction decreased from the first to the third examination with a significant decline in global longitudinal strain from the first examination to the second examination (median, first, and third quartile: −18.8%, [−20.5;−16.5] versus −16.9%, [−19.3;−14.7]) and from the first to the third examination in 41 patients (−18.6%, [−20.9;−15.7] versus −15.8%, [−18.7;−12.6]; P‐values <0.004). Right ventricular ejection fraction decreased significantly from the first to the third examination (55.4%, [49.8;59.3] versus 50.2%, [45.0;55.9]; P<0.002) and from the second to the third examination (53.8%, [47.2;58.7] versus 50.2%, [45.0;55.9]; P<0.0002). CONCLUSIONS: Serial assessment of cardiovascular magnetic resonance studies in patients with hypoplastic left heart syndrome after Fontan completion demonstrates a significant reduction in global strain values and right ventricular ejection fraction at follow‐up. The significant reduction in global longitudinal strain between the first 2 examinations with non‐significant changes in right ventricular ejection fraction suggest that global longitudinal strain measured by 2‐dimensional cardiovascular magnetic resonance feature tracking might be a superior technique for the detection of changes in myocardial function.