Myocardial stress perfusion magnetic resonance in children with hypertrophic cardiomyopathy

2018 ◽  
Vol 28 (5) ◽  
pp. 702-708
Author(s):  
Lazaro E. Hernandez

AbstractBackgroundMicrovascular dysfunction in hypertrophic cardiomyopathy has been associated with poor clinical outcome. Several studies have demonstrated a reduced perfusion reserve proportional to the magnitude of the hypertrophy. We investigated the utility of stress perfusion cardiac MRI to detect microvascular dysfunction in children with hypertrophic cardiomyopathy.MethodsFrom January 2016 to January 2017, 13 patients, with a mean age of 15.3 years, with hypertrophic cardiomyopathy underwent regadenoson stress perfusion cardiac MRI (1.5-T Siemens Aera). A single-shot, T1-weighted saturation recovery gradient echo was used for first-pass perfusion in a multiple-slices group, including three short-axis slices and one four-chamber slice. Coronary vasodilatory stress was achieved using bolus injection of regadenoson (lexiscan 0.4 mg/5 ml) and dynamic perfusion during rest and stress performed by administering 0.05 mmol/kg of gadolinium contrast agent (gadoteridol) injected at a rate of 3.5 ml/s, followed by assessment of viability using two-dimensional phase-sensitive inversion recovery of the entire myocardium.ResultsAll patients completed protocols with no interruptions. In all, seven patients developed perfusion defects after the administration of regadenoson. Asymmetric septal hypertrophy was the most common pattern of hypertrophic cardiomyopathy (n=4) in those with abnormal perfusion. A total of four patients with perfusion defects had a maximum wall thickness <30 mm. The finding of perfusion defects in areas without late gadolinium enhancement in some of our patients indicates that gadolinium enhancement by itself could underestimate the true extension of microvascular disease. Out of seven patients, five patients with positive stress cardiac MRI have undergone implantable cardioverter defibrillator placement based on current guidelines.ConclusionsRegadenoson stress cardiac MRI is feasible and clinically valuable in paediatric patients. It is particularly effective in unmasking abnormal myocardial perfusion in the presence of microvascular dysfunction in children with hypertrophic cardiomyopathy.

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
R Franks ◽  
R Holtackers ◽  
M Nazir ◽  
S Plein ◽  
A Chiribiri

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): British Heart Foundation Background In patients with coronary artery disease (CAD), increasing myocardial ischaemic burden (MIB) is a strong predictor of adverse events. When measured by cardiovascular magnetic resonance (CMR), a MIB ≥12.5% is considered significant and often used as a threshold to guide revascularisation. Ischaemic scar can cause stress perfusion defects which do not represent ischaemia and should be excluded from the MIB calculation. Conventional bright-blood late gadolinium enhancement (LGE) is able to identify ischaemic scar but can suffer from poor scar-to-blood contrast, making accurate assessment of scar volume difficult. Dark-blood LGE methods increase scar-to-blood contrast and improve scar conspicuity which may impact the calculated scar burden and consequently the estimation of MIB when read in conjunction with perfusion images. Purpose To evaluate the impact of dark-blood LGE versus conventional bright-blood LGE on the estimation of MIB in patients with CAD. Methods 37 patients with suspected or known CAD who had evidence of CMR stress perfusion defects and ischaemic scar on LGE imaging were recruited. Patients underwent adenosine stress perfusion imaging followed by dark-blood LGE then conventional bright-blood LGE imaging at 3T. For dark-blood LGE, phase sensitive inversion recovery imaging with a shorter inversion time to null the LV blood-pool was used without any additional magnetization preparation. For each patient, three short-axis LGE slices were selected to match the three perfusion slice locations. Images were anonymised and analysed in random order. Ischaemic scar burden (ISB) was quantified for both LGE methods using a threshold &gt;5 standard deviations above remote myocardium. Perfusion defect burden (PDB) was quantified by manual contouring of perfusion defects. MIB was calculated by subtracting the ISB from the PDB. Results MIB calculated using dark-blood LGE was 19% less compared to bright-blood LGE (15.7 ± 15.2% vs 19.4 ± 15.2%, p &lt; 0.001). There was a strong positive correlation between the two LGE methods (rs = 0.960, p &lt; 0.001, Figure 1A). Bland-Altman analysis revealed a significant fixed bias (mean bias = -3.6%, bias 95% CI: -2.6 to -4.7%, 95% limits of agreement: -9.8 to 2.5%) with no proportional bias (Figure 1B). MIB was calculated ≥12.5% and &lt;12.5% by both LGE methods in 19 (51%) and 12 (32%) patients respectively. In 6 patients (16%), MIB was ≥12.5% using bright-blood LGE and &lt;12.5% using dark-blood LGE (Figure 1A – orange data points). Overall, when used to classify MIB as &lt;12.5% or ≥12.5%, there was only substantial agreement between the two LGE methods (κ=0.67, 95% CI: 0.45 to 0.90). Conclusions The use of dark-blood LGE in conjunction with perfusion imaging results in a lower estimate of MIB compared to conventional bright-blood LGE. This can cause disagreement around the threshold of clinically significant ischaemia which could impact clinical management in patients being considered for coronary revascularisation. Abstract Figure. Linear regression with corresponding B&A


2009 ◽  
Vol 36 (1) ◽  
pp. 106-112 ◽  
Author(s):  
HITOMI KOBAYASHI ◽  
ISAMU YOKOE ◽  
MASAHARU HIRANO ◽  
TETSUYA NAKAMURA ◽  
YASUO NAKAJIMA ◽  
...  

Objective.To assess cardiac involvement in asymptomatic patients with systemic sclerosis (SSc) by cardiac magnetic resonance imaging (MRI).Methods.Ten asymptomatic patients with SSc (all female; mean age 59.5 ± 9.4 yrs) underwent contrast enhanced cardiac MRI on a 1.5 T MRI device. Adenosine triphosphate was used for stress and rest perfusion to assess perfusion defects due to microvascular impairment or ischemia, and delayed enhanced (DE) imaging was obtained for the assessment of myocardial necrosis and fibrosis. We evaluated the pathophysiological associations of stress perfusion combined with DE imaging with SSc disease severity measures.Results.Stress perfusion defects were seen in 5 out of 9 patients (56%): 4 had nonsegmental subendocardial perfusion defects and one had a segmental subendocardial perfusion defect. Three patients were found to have DE. DE was not observed in any patient without perfusion defect; and among the 5 patients with perfusion defects, 3 (60%) had DE. Two of the 3 had DE in segments not matching the region of nonsegmental perfusion defects. The remaining one had a segmental subendocardial DE matching the region of a segmental perfusion defect. Perfusion defects were seen in 75% of patients with a history of digital ulceration compared to only 20% of those without history of ulceration.Conclusion.Subclinical myocardial involvement, as detected by cardiac MRI, was frequent in asymptomatic patients with SSc. Cardiac MRI may aid in understanding the pathophysiological mechanism of SSc.


2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
G Thornton ◽  
A Shetye ◽  
K Knott ◽  
Y Razvi ◽  
K Vimalesvaran ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background  Acute myocardial damage is common in hospitalized patients with severe COVID-19, with evidence of myocardial infarction and myocarditis demonstrated on cardiovascular magnetic resonance (CMR). Post-mortem studies have also implicated microvascular thrombosis, which may cause persistent microvascular disease.  Purpose  To determine the long-term coronary sequelae in recovered COVID-19 using multiparametric CMR including state-of-the-art inline quantitative stress myocardial blood flow (sMBF) mapping to assess global and regional sMBF. Methods  Prospective, multicentre observational study of recovered COVID-19 patients scanned at three London CMR units. Results were compared to a propensity-matched, pre-COVID chest pain cohort (104 patients referred for perfusion CMR, with subsequently demonstrated unobstructed coronary arteries) and 27 healthy volunteers (HV). Perfusion image analysis was performed using a novel artificial intelligence approach deriving global and regional stress and rest MBF with a cut-off of &gt;2.25mL/g/min signifying normal sMBF and &lt;1.82mL/g/min abnormal sMBF (Kotecha JCVI 2019).  Results  104 recovered, post-COVID patients (median age 62 years, 76% male; 89[87%] hospitalised, 41/89[46%] requiring ICU) underwent adenosine-stress perfusion CMR at a median 131(IQR 43-179) days from COVID-19 diagnosis. Median LVEF was 67% (IQR 60-71%; 12 (11.5%) with impaired LVEF), 51 patients (49%) had late gadolinium enhancement (LGE); 18% infarct-pattern and 33% non-ischaemic LGE.  Global stress MBF in post-COVID patients was no different to age-, sex- and co-morbidities-matched controls (2.57 ± 0.77 vs. 2.40 ± 0.75 ml/g/min, p = 0.11, Figure 1), though lower than HV (3.00 ± 0.76 ml/g/min, p = 0.001). Post-COVID, multivariate predictors of low sMBF were male sex (OR 0.57, 95%CI 0.41-0.80, p = 0.001) and hypertension (OR 0.67, 95%CI 0.51-0.88, p = 0.004), but not COVID-19 disease severity (ICU admission) or presence of scar (ischemic/non-ischemic).  21/42 with reduced sMBF (&lt;2.25mL/g/min) had regional perfusion defects consistent with epicardial coronary disease. Conclusions   COVID-19 survivors do not demonstrate evidence of reduced global MBF by CMR compared to risk factor matched controls. Stress perfusion CMR identifies etiology of acute myocardial damage (infarction/myocarditis) and presence of occult coronary ischemia.


Open Heart ◽  
2014 ◽  
Vol 1 (1) ◽  
pp. e000101 ◽  
Author(s):  
Sylvain Grall ◽  
Loïc Biere ◽  
Guillaume Clerfond ◽  
Victor Mateus ◽  
Fabrice Prunier ◽  
...  

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