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

Abstract

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.

Myocardial strain-curve deformation patterns after Fontan operation

“…In general, strain derived parameters performed better than EF and ventricular size indices in predicting clinical events individually in both SLV and SRV patients. Our findings align with previous results in morphological normal hearts 6 and single ventricle studies 2 , 13 demonstrating strain as a more sensitive marker of subclinical systolic dysfunction.…”

“…Consequently, adaptation to adverse loading conditions is different as well 16 . In patients with SRV morphology, the myocardial strain patterns are dynamically changing during 17 and also after single ventricle palliation 13 . In morphologically normal right ventricle, GLS is the predominant mode of global strain, even in patients with pulmonary hypertension with a GCS/GLS ratio < 1 18 , 19 .…”

“…In morphologically normal right ventricle, GLS is the predominant mode of global strain, even in patients with pulmonary hypertension with a GCS/GLS ratio < 1 18 , 19 . In patients with hypoplastic left heart syndrome and its pathologic variants, the GCS/GLS ratio can approach 1.0 and can fully reverse in some patients late after Fontan operation 1 , 2 , 13 . In our study, we showed that d EF/ d GCS slope is steeper than d EF/ d GLS slope in SRV patients, suggesting that circumferential strain is a larger contributor to overall pump function.…”

Cardiovascular magnetic resonance reference values of right ventricular volumetric variables in patients with hypoplastic left heart syndrome

“…A current position statement for patients with a Fontan circulation recommends cardiovascular magnetic resonance (CMR) imaging to monitor serial changes in ventricular function [4] . This is supported by previous CMR studies from our group that showed a significant increase in RV volumes as well as a decrease in RV global longitudinal strain in HLHS patients after TCPC completion during serial follow-up and confirmed that CMR is well suited for serial RV follow-up [5] , [6] .…”

Narrative review of single ventricle: where are we after 40 years?

“…The introduction of technological innovations such as three-dimensional echocardiography ( 48 , 49 ) and global longitudinal strain analysis of single ventricle physiology ( 50 ) using low radiation-dose computed tomography ( 51 - 53 ) can provide accurate anatomic delineation of congenital heart defects. Functional assessment of myocardial function, in addition to the echocardiographic investigations, with magnetic resonance imaging ( 54 , 55 ) and the introduction of techniques merging different diagnostic imaging modalities ( 56 ) facilitate operative decision-making, together with 3-D modeling and printing of the heart structures ( 57 , 58 ), as well as computational design of the planned surgical procedure with evaluation of the obtainable sizes of ventricular inflows/outflows and ventricular volumes ( 59 ).…”