Abstract
Ventricular dysfunction is a well-known complication in single ventricle patients in Fontan circulation. As studies exclusively examining patients with a single left ventricle (SLV) are sparse, we assessed left ventricular (LV) function in SLV patients by using 2D-cardiovascular magnetic resonance (CMR) feature tracking (2D-CMR-FT) and 2D-speckle tracking echocardiography (2D-STE). 54 SLV patients (11.4, 3.1–38.1 years) and 35 age-matched controls (12.3, 6.3–25.8 years) were included. LV global longitudinal, circumferential and radial strain (GLS, GCS, GRS) and strain rate (GLSR, GCSR, GRSR) were measured using 2D-CMR-FT. LV volumes, ejection fraction (LVEF) and mass were determined from short axis images. 2D-STE was applied in patients to measure peak systolic GLS and GLSR. In a subgroup analysis, we compared double inlet left ventricle (DILV) with tricuspid atresia (TA) patients. The population consisted of 19 DILV patients, 24 TA patients and 11 patients with diverse diagnoses. 52 patients were in NYHA class I and 2 patients were in class II. Most SLV patients had a normal systolic function but median LVEF in patients was lower compared to controls (55.6% vs. 61.2%, p = 0.0001). 2D-CMR-FT demonstrated reduced GLS, GCS and GCSR values in patients compared to controls. LVEF correlated with GS values in patients (p < 0.05). There was no significant difference between GLS values from 2D-CMR-FT and 2D-STE in the patient group. LVEF, LV volumes, GS and GSR (from 2D-CMR-FT) were not significantly different between DILV and TA patients. Although most SLV patients had a preserved EF derived by CMR, our results suggest that, LV deformation and function may behave differently in SLV patients compared to healthy subjects.
Highlights
Since the introduction of the Fontan operation, life expectancy of single ventricle (SV) patients steadily improved, and an increasing number of SV patients is reaching adolescence and adulthood [1,2,3]
Registry data indicate that patients with a single left ventricle (SLV) seem to have a better freedom from late Fontan failure compared to patients with a single right ventricle [3]
This retrospective study was approved by the ethics committee of the medical faculty of the Christian-Albrechts University Kiel (No D555/19) and included all 54 SLV patients after Fontan completion, who received a cardiovascular magnetic resonance (CMR) examination as part of a routine clinical follow up during 2010–2019. 35 age-matched healthy controls were included for comparison
Summary
Since the introduction of the Fontan operation, life expectancy of single ventricle (SV) patients steadily improved, and an increasing number of SV patients is reaching adolescence and adulthood [1,2,3]. Despite this success, we must remind ourselves that the Fontan procedure is a palliative approach that can be regarded as a bridge to cardiac transplantation with limited exceptions. Abnormal hemodynamics due to unfavorable volume and/ or pressure load of the SV, as well as the stepwise surgical procedures, might be a cause for SV dysfunction and heart failure [8]. More recently associations between myocardial fibrosis and adverse SV function have been demonstrated [9, 10]
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