scholarly journals Myocardial Perfusion in Hypoplastic Left Heart Syndrome: Risk Factors for the Right Ventricular Microcirculation

2021 ◽  
Author(s):  
Carsten Rickers ◽  
Philip Wegner ◽  
Michael Silberbach ◽  
Erin Madriago ◽  
Dominik Daniel Gabbert ◽  
...  
Keyword(s):  
Risk Factors ◽  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Coronary Microcirculation ◽  
Total Cavopulmonary Connection ◽  
Gadolinium Enhancement ◽  
Coronary Conductance ◽  
Systemic Ventricle ◽  
Left Heart Syndrome ◽  
Cavopulmonary Connection

Background: The status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle’s coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS. Methods: One hundred nineteen HLHS patients (median age, 4.80 years) and 34 healthy volunteers (median age, 5.50 years) underwent follow-up cardiac magnetic resonance imaging ≈1.8 years after total cavopulmonary connection. Right ventricle volumes and function, myocardial perfusion, diffuse fibrosis, and late gadolinium enhancement were assessed in 4 anatomic HLHS subtypes. Myocardial blood flow (MBF) was quantified at rest and during adenosine-induced hyperemia. Coronary conductance was estimated from MBF at rest and catheter-based measurements of mean aortic pressure (n=99). Results: Hyperemic MBF in the systemic ventricle was lower in HLHS compared with controls (1.89±0.57 versus 2.70±0.84 mL/g per min; P <0.001), while MBF at rest normalized by the rate-pressure product, was similar (1.25±0.36 versus 1.19±0.33; P =0.446). Independent risk factors for a reduced hyperemic MBF were an HLHS subtype with mitral stenosis and aortic atresia ( P =0.017), late gadolinium enhancement ( P =0.042), right ventricular diastolic dysfunction ( P =0.005), and increasing age at total cavopulmonary connection ( P =0.022). The coronary conductance correlated negatively with systemic blood oxygen saturation (r, −0.29; P =0.02). The frequency of late gadolinium enhancement increased with age at total cavopulmonary connection ( P =0.014). Conclusions: The coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. These hypothesis-generating findings on HLHS-specific risk factors for microvascular dysfunction suggest a potential benefit from early relief of frank cyanosis by total cavopulmonary connection.

scholarly journals Characteristics of Patients With Arrhythmogenic Left Ventricular Cardiomyopathy

2020 ◽  
Vol 13 (12) ◽  
Author(s):  
Michela Casella ◽  
Alessio Gasperetti ◽  
Rita Sicuso ◽  
Edoardo Conte ◽  
Valentina Catto ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Genetic Variants ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
Voltage Mapping ◽  
Fatty Replacement

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce. Methods: Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV. Results: Twenty-five patients ALVC (53 [48–59] years, 60% male) were enrolled. T wave inversion in infero-lateral and left precordial leads were the most common ECG abnormalities. Overall arrhythmic burden at study inclusion was 56%. Cardiac magnetic resonance showed LV late gadolinium enhancement in the LV lateral and posterior basal segments in all patients. In 72% of the patients an invasive evaluation was performed, in which electroanatomical voltage mapping and electroanatomical voltage mapping-guided endomyocardial biopsy showed low endocardial voltages and fibro-fatty replacement in areas of late gadolinium enhancement presence. Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were identified in 12/25 of the cohort presenting pathogenic/likely pathogenic variants. A definite/borderline 2010 International Task Force Criteria arrhythmogenic right ventricular cardiomyopathy diagnosis was reached only in 11/25 patients. Conclusions: ALVC presents with a preferential involvement of the lateral and postero-lateral basal LV and is associated mostly with variants in desmoplakin and desmoglein-2 genes. An amendment to the current International Task Force Criteria is reasonable to better diagnose patients with ALVC.

Novel plasma biomarkers in arrhythmogenic cardiomyopathy: the role of ST2 and GDF-15 in predicting biventricular involvement

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
D Akdis ◽  
L Chen ◽  
A.M Saguner ◽  
N Zhang ◽  
J Gawinecka ◽  
...  
Keyword(s):  
Heart Failure ◽  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Validation Cohort ◽  
Muscle Disease ◽  
Funding Source ◽  
Gadolinium Enhancement ◽  
Plasma Biomarkers ◽  
Combined Use

Abstract Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease characterized by fibrofatty replacement of the myocardium and ventricular arrhythmias. Biventricular (BiV) involvement in ARVC may lead to heart failure. Purpose This study aimed to investigate the role of novel plasma biomarkers soluble (s)ST2, Galectin-3 (Gal-3) and GDF-15 in predicting BiV involvement and adverse outcomes in ARVC patients. Methods ARVC patients from two independent cohorts were studied. 108 patients were included from the discovery cohort and 47 patients were included from a second validation cohort. All patients had a definite ARVC diagnosis at time of blood withdrawal. sST2, Gal-3 and GDF-15 were independently correlated with NT-proBNP, left ventricular (LV) ejection fraction, late gadolinium enhancement by cardiac magnetic resonance (CMR) imaging and clinical outcome. Results ARVC patients with LV involvement had higher levels of sST2 and GDF-15 as compared to controls and patients with isolated right ventricular involvement. sST2 and GDF-15 significantly correlated to late gadolinium enhancement on CMR and also correlated to adverse heart failure outcomes. Gal-3 was elevated in ARVC patients with and without LV involvement as compared to controls. The combined use of the three biomarkers (NT-proBNP, sST2 and GDF-15) showed the best performance in predicting LV involvement in both the discovery and the validation cohort. Plasma drawn from coronary arteries and coronary sinus showed a transmyocardial elevation of sST2. Conclusion Our study shows that sST2 and GDF-15 may predict BiV involvement and the combined use of NT-proBNP, sST2 and GDF-15 shows the best prediction of LV involvement in ARVC. Transmyocardial elevation of sST2 suggests that this biomarker is produced by myocardial tissue in ARVC. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Fuwai ARVC Program was supported by CAMS Innovation Fund for Medical Sciences and the National Natural Science Foundation of China, Zurich ARVC Program was supported by grants from the Schwyzer Foundation and Baugarten Foundation

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