scholarly journals Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Andrea Pisesky ◽  
Marjolein J. E. Reichert ◽  
Charlotte de Lange ◽  
Mike Seed ◽  
Shi-Joon Yoo ◽  
...  

Abstract Background The extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study. Methods This retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynamics in young Fontan patients to controls. Results Fifty-five Fontan patients and 44 healthy controls were included (median age 14 years (range 7–17 years) vs 13 years (range 4–14 years), p = 0.057). Fontan patients had a higher indexed end-diastolic ventricular volume (EDVI 129 ml/m2 vs 93 ml/m2, p < 0.001), and lower ejection fraction (EF 45% vs 58%, p < 0.001), circumferential (CS − 23.5% vs − 30.8%, p < 0.001), radial (6.4% vs 8.2%, p < 0.001), and longitudinal strain (− 13.3% vs − 24.8%, p < 0.001). Compared to healthy controls, Fontan patients had higher extracellular volume fraction (ECV) (26.3% vs 20.6%, p < 0.001) and native T1 (1041 ms vs 986 ms, p < 0.001). Patients with a dominant right ventricle demonstrated larger ventricles (EDVI 146 ml/m2 vs 120 ml/m2, p = 0.03), lower EF (41% vs 47%, p = 0.008), worse CS (− 20.1% vs − 25.6%, p = 0.003), and a trend towards higher ECV (28.3% versus 24.1%, p = 0.09). Worse EF and CS correlated with longer cumulative bypass (R = − 0.36, p = 0.003 and R = 0.46, p < 0.001), cross-clamp (R = − 0.41, p = 0.001 and R = 0.40, p = 0.003) and circulatory arrest times (R = − 0.42, p < 0.001 and R = 0.27, p = 0.03). T1 correlated with aortopulmonary collateral (APC) flow (R = 0.36, p = 0.009) which, in the linear regression model, was independent of ventricular morphology (p = 0.9) and EDVI (p = 0.2). The composite outcome (cardiac readmission, cardiac reintervention, Fontan failure or any clinically significant arrhythmia) was associated with increased native T1 (1063 ms vs 1026 ms, p = 0.029) and EDVI (146 ml/m2 vs 118 ml/m2, p = 0.013), as well as decreased EF (42% vs 46%, p = 0.045) and worse CS (− 22% vs − 25%, p = 0.029). APC flow (HR 5.5 CI 1.9–16.2, p = 0.002) was independently associated with the composite outcome, independent of ventricular morphology (HR 0.71 CI 0.30–1.69 p = 0.44) and T1 (HR1.006 CI 1.0–1.13, p = 0.07). Conclusions Pediatric Fontan patients have ventricular dysfunction, altered myocardial mechanics and increased fibrotic remodeling. Cumulative exposure to cardiopulmonary bypass and increased aortopulmonary collateral flow are associated with myocardial dysfunction and fibrosis. Cardiac dysfunction, fibrosis, and collateral flow are associated with adverse outcomes.

Cardiology ◽  
2017 ◽  
Vol 138 (4) ◽  
pp. 207-217 ◽  
Author(s):  
Sophie Mavrogeni ◽  
Dimitris Apostolou ◽  
Panayiotis Argyriou ◽  
Stella Velitsista ◽  
Lilika Papa ◽  
...  

The increasing use of cardiovascular magnetic resonance (CMR) is based on its capability to perform biventricular function assessment and tissue characterization without radiation and with high reproducibility. The use of late gadolinium enhancement (LGE) gave the potential of non-invasive biopsy for fibrosis quantification. However, LGE is unable to detect diffuse myocardial disease. Native T1 mapping and extracellular volume fraction (ECV) provide knowledge about pathologies affecting both the myocardium and interstitium that is otherwise difficult to identify. Changes of myocardial native T1 reflect cardiac diseases (acute coronary syndromes, infarction, myocarditis, and diffuse fibrosis, all with high T1) and systemic diseases such as cardiac amyloid (high T1), Anderson-Fabry disease (low T1), and siderosis (low T1). The ECV, an index generated by native and post-contrast T1 mapping, measures the cellular and extracellular interstitial matrix (ECM) compartments. This myocyte-ECM dichotomy has important implications for identifying specific therapeutic targets of great value for heart failure treatment. On the other hand, T2 mapping is superior compared with myocardial T1 and ECM for assessing the activity of myocarditis in recent-onset heart failure. Although these indices can significantly affect the clinical decision making, multicentre studies and a community-wide approach (including MRI vendors, funding, software, contrast agent manufacturers, and clinicians) are still missing.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Kjellstad Larsen ◽  
J Duchenne ◽  
E Galli ◽  
J M Aalen ◽  
E Kongsgaard ◽  
...  

Abstract Funding Acknowledgements The study was supported by Center for Cardiological Innovation Background Myocardial scar burden (focal fibrosis) is associated with poor response to cardiac resynchronization therapy (CRT), and should preferably be detected prior to device implantation. Late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is considered reference standard for scar detection, but is not available in renal failure. Diffuse fibrosis is assessed by T1 mapping CMR with or without calculation of extracellular volume fraction (ECV). The method is vulnerable to partial volume effects, thus subendocardial tissue is most often not included in mapping analyses. Whether the contrast-free native T1mapping could replace LGE in the preoperative evaluation of patients referred for CRT is unknown. Purpose To investigate if native T1 mapping and calculation of ECV can adequately detect scar in patients referred for CRT. Methods Scar was quantified as percentage segmental LGE in 45 patients (age 65 ± 10 years, 71% male, QRS-width 165 ± 17ms) referred for CRT. In total 720 segments were analyzed, and LGE≥50% was considered transmural scar. T1-mapping before and after contrast agent injection was performed in all patients. ECV was calculated based on the ratio between tissue T1 relaxation change and blood T1 relaxation change after contrast agent injection, corrected for the haematocrit level. The agreement between native T1/ECV and scar was evaluated with receiver operating characteristic (ROC) curves with calculation of area under the curve (AUC) and 95% confidence interval (CI). Results LGE was present in 255 segments, 465 segments were without LGE. Average native T1 in segments with LGE was 1028 ± 88 ms, and 1040 ± 60 ms in segments without LGE (p = 0.16). The corresponding numbers for ECV were 38.7 ± 10.9% and 30.0 ± 4.7%, p &lt; 0.001. Native T1 showed poor agreement to scar independent of scar size (AUC = 0.532, 95% CI 0.485-0.578 for scars of all sizes, and AUC = 0.572, 95% CI 0.495-0.650 for transmural scars). ECV, on the other hand, showed reasonable agreement with scar of all sizes (AUC = 0.777, 95% CI 0.739-0.815), and good agreement with transmural scars (AUC = 0.856, 95% CI 0.811-0.902). (Figure) Conclusion The contrast-free CMR technique T1 mapping does not adequately detect scars in patients referred for CRT. Adding post contrast T1 measurements and calculating ECV improves accuracy, especially for transmural scars. Future studies should investigate if diffuse fibrosis could be predictive of CRT response. Abstract P1585 Figure. Detection of transmural scars


2020 ◽  
Vol 311 ◽  
pp. 28-34
Author(s):  
Heiner Latus ◽  
Pia Kruppa ◽  
Lucas Hofmann ◽  
Bettina Reich ◽  
Christian Jux ◽  
...  

Heart ◽  
2021 ◽  
pp. heartjnl-2021-319926
Author(s):  
Trisha Singh ◽  
Thomas A Kite ◽  
Shruti S Joshi ◽  
Nick B Spath ◽  
Lucy Kershaw ◽  
...  

ObjectivesTo determine the contribution of comorbidities on the reported widespread myocardial abnormalities in patients with recent COVID-19.MethodsIn a prospective two-centre observational study, patients hospitalised with confirmed COVID-19 underwent gadolinium and manganese-enhanced MRI and CT coronary angiography (CTCA). They were compared with healthy and comorbidity-matched volunteers after blinded analysis.ResultsIn 52 patients (median age: 54 (IQR 51–57) years, 39 males) who recovered from COVID-19, one-third (n=15, 29%) were admitted to intensive care and a fifth (n=11, 21%) were ventilated. Twenty-three patients underwent CTCA, with one-third having underlying coronary artery disease (n=8, 35%). Compared with younger healthy volunteers (n=10), patients demonstrated reduced left (ejection fraction (EF): 57.4±11.1 (95% CI 54.0 to 60.1) versus 66.3±5 (95 CI 62.4 to 69.8)%; p=0.02) and right (EF: 51.7±9.1 (95% CI 53.9 to 60.1) vs 60.5±4.9 (95% CI 57.1 to 63.2)%; p≤0.0001) ventricular systolic function with elevated native T1 values (1225±46 (95% CI 1205 to 1240) vs 1197±30 (95% CI 1178 to 1216) ms;p=0.04) and extracellular volume fraction (ECV) (31±4 (95% CI 29.6 to 32.1) vs 24±3 (95% CI 22.4 to 26.4)%; p<0.0003) but reduced myocardial manganese uptake (6.9±0.9 (95% CI 6.5 to 7.3) vs 7.9±1.2 (95% CI 7.4 to 8.5) mL/100 g/min; p=0.01). Compared with comorbidity-matched volunteers (n=26), patients had preserved left ventricular function but reduced right ventricular systolic function (EF: 51.7±9.1 (95% CI 53.9 to 60.1) vs 59.3±4.9 (95% CI 51.0 to 66.5)%; p=0.0005) with comparable native T1 values (1225±46 (95% CI 1205 to 1240) vs 1227±51 (95% CI 1208 to 1246) ms; p=0.99), ECV (31±4 (95% CI 29.6 to 32.1) vs 29±5 (95% CI 27.0 to 31.2)%; p=0.35), presence of late gadolinium enhancement and manganese uptake. These findings remained irrespective of COVID-19 disease severity, presence of myocardial injury or ongoing symptoms.ConclusionsPatients demonstrate right but not left ventricular dysfunction. Previous reports of left ventricular myocardial abnormalities following COVID-19 may reflect pre-existing comorbidities.Trial registration numberNCT04625075.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Mavrogeni ◽  
L Gargani ◽  
A Pepe ◽  
L Monti ◽  
G Markousis-Mavrogenis ◽  
...  

Abstract Introduction Cardiac involvement in systemic sclerosis (SSc) accounts for 26–36% of deaths. This most frequently manifests as ventricular rhythm disturbances (VRDs), eventually culminating in sudden cardiac death. However, no specific guidelines exist for implantation of cardioverter defibrillators (ICD) in SSc patients. Parametric cardiovascular magnetic resonance (CMR) indices of myocardial oedema and fibrosis like native T1/T2 mapping have been shown to be associated with prognosis in SSc patients with acute cardiac events and normal echocardiograms. However, their relationship with arrhythmogenicity per se has not been previously investigated in SSc. Objectives To investigate the relationship between parametric CMR indices and arrhythmogenicity in SSc patients. Methods 84 consecutive SSc patients (80% diffuse-cutaneous SSc) from eight European centers presenting with cardiac symptoms were examined using a 1.5 T CMR system. 24h Holter recordings were obtained within a month of the CMR scan. The presence of VRDs was defined as any type of premature ventricular contraction (PVC) in couples, triplets, bigeminism, trigeminism, quadrigeminism and non-sustained ventricular tachycardia, as well as having &gt;30 PVCs per hour. Logistic regression analysis was used to evaluate the relationship between VRD occurrence and native T1/T2 mapping as well as myocardial extracellular volume fraction (ECV). Results Mean age in the cohort was 55 (13) years and 78 (93%) patients were female. Of these, 67 (80%) experienced at least one type of VRDs. Each 10 ms increase of native T1-mapping was associated with a higher occurrence of VRDs [odds ratio (95% confidence interval): 1.21 (1.08–1.36), p=0.001]. Similarly, a 1% increase in ECV conferred an increased probability of experiencing VRDs [1.25 (1.01–1.53), p=0.037]. Lastly, a 1ms unit increase in T2-mapping also led to increased probability of having experienced VRDs [1.09 (1.01–1.19), p=0.035]. Conclusion Parametric CMR indices are associated with arrhythmogenicity in SSc patients with cardiac symptoms and should be investigated further in larger studies for their clinical utility in selecting high-risk SSc patients for ICD implantation. Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Rawiwan Thongsongsang ◽  
Thammarak Songsangjinda ◽  
Prajak Tanapibunpon ◽  
Rungroj Krittayaphong

Abstract Background This study aimed to determine native T1 and extracellular volume fraction (ECV) in distinct types of myocardial disease, including amyloidosis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), myocarditis and coronary artery disease (CAD), compared to controls. Methods We retrospectively enrolled patients with distinct types of myocardial disease, CAD patients, and control group (no known heart disease and negative CMR study) who underwent 3.0 Tesla CMR with routine T1 mapping. The region of interest (ROI) was drawn in the myocardium of the mid left ventricular (LV) short axis slice and at the interventricular septum of mid LV slice. ECV was calculated by actual hematocrit (Hct) and synthetic Hct. T1 mapping and ECV was compared between myocardial disease and controls, and between CAD and controls. Diagnostic yield and cut-off values were assessed. Results A total of 1188 patients were enrolled. The average T1 values in the control group were 1304 ± 42 ms at septum, and 1294 ± 37 ms at mid LV slice. The average T1 values in patients with myocardial disease and CAD were significantly higher than in controls (1441 ± 72, 1349 ± 59, 1345 ± 59, 1355 ± 56, and 1328 ± 54 ms for septum of amyloidosis, DCM, HCM, myocarditis, and CAD). Native T1 of the mid LV level and ECV at septum and mid LV with actual and synthetic Hct of patients with myocardial disease or CAD were significantly higher than in controls. Conclusions Although native T1 and ECV of patients with cardiomyopathy and CAD were significantly higher than controls, the values overlapped. The greatest clinical utilization was found for the amyloidosis group.


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