Association between left atrial myopathy and sarcomere mutation in patients with hypertrophic cardiomyopathy: insights into left atrial strain by MRI feature tracking.

OBJECTIVES:Left atrial (LA) myopathy, characterized by LA enlargement and mechanical dysfunction, is associated with worse prognosis in hypertrophic cardiomyopathy (HCM) while the impact of sarcomere mutation on LA myopathy remains unclear. We aimed to assess the association between LA myopathy and sarcomere mutation and to explore the incremental utility of LA strain in mutation prediction.METHODS:A total of 105 consecutive HCM patients (mean age 47.8 ± 11.9 years, 71% male) who underwent HCM-related gene screening and cardiac MRI were retrospectively enrolled. LA volume, ejection fraction and strain indices in reservoir, conduit, and booster-pump phases were investigated respectively.RESULTS:Fifty mutation-positive patients showed higher LA maximal volume index (59.4 ± 28.2 vs 43.8 ± 18.1 mL/m2, p = 0.001), lower reservoir (21.3 ± 7.9 vs 26.2 ± 6.6%, p < 0.001), and booster-pump strain (12.1 ± 5.4 vs 17.1 ± 5.0%, p < 0.001) but similar conduit strain (9.2 ± 4.5 vs 9.1 ± 4.5%, p = 0.909) compared with mutation-negative patients. In multivariate logistic regression, LA booster-pump strain was associated with sarcomere mutation (odds ratio = 0.86, 95% confidence interval: 0.77-0.96, p = 0.010) independent of maximal wall thickness, late gadolinium enhancement, and LA volume. Furthermore, LA booster-pump strain showed incremental value for mutation prediction added to Mayo II score (AUC 0.798 vs 0.709, p = 0.024).CONCLUSIONS:In HCM, mutation-positive patients suffered worse LA enlargement and worse reservoir and booster-pump functions. LA booster-pump strain was a strong factor for sarcomere mutation prediction added to Mayo II score.CLINICAL RELEVANCE STATEMENT:The independent association between sarcomere mutation and left atrial mechanical dysfunction provide new insights into the pathogenesis of atrial myopathy and is helpful to understand the adverse prognosis regarding atrial fibrillation and stroke in mutation-positive patients.KEY POINTS:• In patients with hypertrophic cardiomyopathy, left atrial (LA) reservoir and booster-pump function, but not conduit function, were significantly impaired in mutation-positive patients compared with mutation-negative patients. • LA booster-pump strain measured by MRI-derived feature tracking is feasible to predict sarcomere mutation with high incremental value added to Mayo II score.

Adenosine Triphosphate Stress Myocardial Strain in Ischemic Heart Disease: An Animal Study with Histological Validation.

RATIONALE AND OBJECTIVES:It is still challenging for cardiac magnetic resonance (CMR) to detect ischemic heart disease (IHD) without the use of gadolinium contrast. We aimed to evaluate the potential value of adenosine triphosphate (ATP) stress myocardial strain derived from feature tracking (FT) as a novel method for detecting IHD in a swine model.MATERIALS AND METHODS:CMR cines, myocardial perfusion imaging at rest and during ATP stress, and late gadolinium enhancement were obtained in both control and IHD swine. Normal, remote, ischemic, and infarcted myocardium were analyzed. The diagnostic accuracy of myocardial strain for infarction and ischemia was assessed using coronary angiography and pathology as reference.RESULTS:Eleven IHD swine and five healthy control swine were enrolled in this study. Strain parameters, even at rest, were associated with myocardial ischemia and infarction(all p < 0.05). The area under receiver operating characteristic curve (AUC) values of all strain parameters for detecting infarcted myocardium exceeded 0.900 (all p < 0.05). The AUC values for detecting ischemic myocardium were as follows: 0.906 and 0.847 for stress and rest radial strain, 0.763 and 0.716 for stress and rest circumferential strain, 0.758 and 0.663 for stress and rest longitudinal strain (all p < 0.001). Heat maps demonstrated that all strain parameters showed mild to moderate correlations with the stress myocardial blood flow and myocardial perfusion reserve (all p < 0.05).CONCLUSION:CMR-FT-derived ATP stress myocardial strain shows promise as a noninvasive method for detecting myocardial ischemia and infarction in an IHD swine model, with rest strain parameters offering potential as a needle-free diagnostic option.

Developing and evaluating a chronic ischemic cardiomyopathy in swine model by rest and stress CMR.

A large animal model of chronic coronary artery disease (CAD) is crucial for the understanding the underlying pathophysiological processes of chronic CAD and consequences for cardiac structure and function. The goal of this study was to develop a chronic model of CAD in a swine model and to evaluate the changes of myocardial structure, myocardial motility, and myocardial viability during coronary stenosis. A total of 30 swine (including 24 experimental animals and 6 controls) were enrolled. The chronic ischemia model was constructed by using Ameroid constrictor in experimental group. The 24 experimental animals were further divided into 4 groups (6 animals in each group) and were sacrificed at 1, 2, 3 and 4 weeks after operation for pathological examination, respectively. Cardiac magnetic resonance (CMR) was performed preoperatively and weekly postoperatively until sacrificed both in experimental and control group. CMR cine images, rest/adenosine triphosphate (ATP) stress myocardial contrast perfusion and LGE were performed and analyzed. The rest wall thickening (WT) score was calculated from rest cine images. The MPRI (myocardial perfusion reserve index) and MPR (myocardial perfusion reserve) were calculated based on rest and stress perfusion images. Pathology staining including triphenyltetrazolium chloride, HE and picrosirus red staining were performed after swine were sacrificed and collagen volume fraction (CVF) was calculated. The time to formation of ischemic, hibernating, and infarcted myocardium was recorded. In experimental group, from 1w to 4w after surgery, the rest WT score decreased gradually from 35.2 ± 2.0%, 32.0 ± 2.9% to 30.5 ± 3.0% and finally 29.06 ± 1.78%, p < 0.001. Left ventricular ejection fraction was gradually impaired after modeling (58.9 ± 12.6%, 56.3 ± 10.1%, 55.3 ± 9.0%, 53.8 ± 9.9%, respectively). And the MPR and MPRI also decreased stepwise with extent of surgery time (MPRI dropped from 2.1 ± 0.4, 2.0 ± 0.2 to 1.8 ± 0.3 and finally 1.7 ± 0.1, p = 0.004; MPR dropped from 2.3 ± 0.4, 2.1 ± 0.2 to 1.9 ± 0.4 and finally 1.8 ± 0.1, p < 0.001). Stronger associations between MPR, MPRI and CVF were paralleled lower wall thickening scores in fibrosis-affected areas. The ischemic myocardium was first appeared in the first week after surgery (involving ten segments), hibernated myocardium was first appeared in the second week after surgery (involving seventeen segments). LGE was first appeared in eight swine in the third weeks after surgery (16 segments). At 4w after surgery, average 9.6 g scar tissue was found among 6 swine. At the same time, histological analysis established the presence of fibrosis and ongoing apoptosis in the infarcted area. In conclusion, our study provided valuable insights into the pathophysiological processes of chronic CAD and its consequences for cardiac structure and function in a large animal model through combining myocardial motion and stress perfusion.

Apical aneurysm formation in apical hypertrophic cardiomyopathy: Pilot study with cardiac magnetic resonance.

BACKGROUND:The dynamic changes and apical aneurysm formation in apical hypertrophic cardiomyopathy (HCM) have not been specifically described. This study aimed to describe these changes to better understand the progression of apical HCM.METHODS AND RESULTS:Seventy-two patients with apical HCM who underwent at least two cardiac magnetic resonance (CMR) examinations were retrospectively included in this study. The mean interval between the first and last CMR examinations was 50.1 ± 26.8 months (ranging from 4 to 118 months). Compared with the initial values, the left atrial diameter, maximum left ventricular wall thickness and late gadolinium enhancement extent significantly increased (all P < 0.05), while the left ventricular ejection fraction significantly decreased (P < 0.05), at the latest CMR examination. More importantly, the dynamic process of apical aneurysm formation in apical HCM was observed in a subset of patients, which may follow these four stages: starting with systolic apical cavity obliteration, then broadening of the apical slit in systole, further developing into an apical outpouching, and finally forming an apical aneurysm. Eleven patients experienced adverse cardiovascular events, including new-onset or progressive atrial fibrillation (n = 7), hospitalization with heart failure (n = 3) and implantable cardioverter defibrillator intervention (n = 1), at the time of the latest CMR examination.CONCLUSIONS:In the progression of apical HCM, cardiac structure and function will change accordingly. Apical aneurysm formation in apical HCM is a chronic and continuous dynamic process that may follow a 4-step pathway of disease progression.

[Hypertrophic cardiomyopathy with restrictive phenotype: a case report].

Detection of haemodynamic obstruction in hypertrophic cardiomyopathy using the sub-aortic complex: a cardiac MRI and Doppler study.

AIM:To investigate the "sub-aortic complex (SAC)", a new cardiac magnetic resonance imaging (CMRI)-derived parameter, for the evaluation of left ventricular (LV) outflow tract (LVOT) obstruction in patients with hypertrophic cardiomyopathy (HCM), compared with conventional CMRI parameters and Doppler echocardiography.MATERIALS AND METHODS:A total of 157 consecutive patients with HCM were recruited retrospectively. The patients were divided into two groups, 87 with LVOT obstruction and 70 without obstruction. The SAC was defined as a specific anatomical SAC affecting the LVOT, which were measured on the LV three-chamber steady-state free precession (SSFP) cine image at the end-systolic phase. The relations between the existence and severity of obstruction and SAC index (SACi) were evaluated using Pearson's correlation coefficient, receiver operating characteristic (ROC) curves, and logistic regression.RESULTS:The SACs were significantly different between the obstructive and non-obstructive groups. The ROC curves indicated that the SACi was able to discriminate obstructive and non-obstructive patients with the best predictive accuracy (AUC = 0.949, p<0.001). The SACi was an independent predictor of LVOT obstruction and there was a significant negative correlation between resting LVOT pressure gradient and SACi (r=0.72 p<0.001). In the subgroup of patients with or without severe basal septal hypertrophy, the SACi was still able to predict LVOT obstruction with excellent diagnostic accuracy (AUC = 0.944 and 0.948, p<0.001, respectively).CONCLUSION:The SAC is a reliable and straightforward CMRI marker for assessing LVOT obstruction. It is more effective than CMRI two-dimensional flow in diagnosing the severity of obstruction in patients with HCM.

Assessment of late gadolinium enhancement in hypertrophic cardiomyopathy improves risk stratification based on current guidelines.

BACKGROUND AND AIMS:Identifying patients with hypertrophic cardiomyopathy (HCM) who are candidates for implantable cardioverter defibrillator (ICD) implantation in primary prevention for sudden cardiac death (SCD) is crucial. The aim of this study was to externally validate the 2022 European Society of Cardiology (ESC) model and other guideline-based ICD class of recommendation (ICD-COR) models and explore the utility of late gadolinium enhancement (LGE) in further risk stratification.METHODS:Seven hundred and seventy-four consecutive patients who underwent cardiac magnetic resonance imaging were retrospectively enrolled.RESULTS:Forty-six (5.9%) patients reached the SCD-related endpoint during 7.4 ± 2.5 years of follow-up. Patients suffering from SCD had higher ESC Risk-SCD score (4.3 ± 2.4% vs. 2.8 ± 2.1%, P < .001) and LGE extent (13.7 ± 9.4% vs. 4.9 ± 6.6%, P < .001). Compared with the 2014 ESC model, the 2022 ESC model showed increased area under the curve (.76 vs. .63), sensitivity (76.1% vs. 43.5%), positive predictive value (16.8% vs. 13.6%), and negative predictive value (98.1% vs. 95.9%). The C-statistics for SCD prediction of 2011 American College of Cardiology (ACC)/American Heart Association (AHA), 2014 ESC, 2020 AHA/ACC, and 2022 ESC models were .68, .64, .76 and .78, respectively. Furthermore, in patients without extensive LGE, LGE ≥5% was responsible for seven-fold SCD risk after multivariable adjustment. Whether in ICD-COR II or ICD-COR III, patients with LGE ≥5% and <15% showed significantly worse prognosis than those with LGE <5% (all P < .001).CONCLUSIONS:The 2022 ESC model performed better than the 2014 ESC model with especially improved sensitivity. LGE enabled further risk stratification based on current guidelines.

Detection of Myocardial Ischemia Using Cardiovascular MRI Stress T1 Mapping: A Miniature-Swine Validation Study.

Purpose:To assess the efficacy of cardiac MRI stress T1 mapping in detecting ischemic and infarcted myocardium in a miniature-swine model, using pathologic findings as the reference standard.Materials and Methods:Ten adult male Chinese miniature swine, with coronary artery stenosis induced by an ameroid constrictor, and two healthy control swine were studied. Cardiac 3-T MRI rest and adenosine triphosphate stress T1 mapping and perfusion images, along with resting and late gadolinium enhancement images, were acquired at baseline and weekly up to 4 weeks after surgery or until humanely killed. A receiver operating characteristic analysis was used to analyze the performance of T1 mapping in the detection of myocardial ischemia.Results:In the experimental group, both the infarcted myocardium (ΔT1 = 10 msec ± 2 [SD]; ΔT1 percentage = 0.7% ± 0.1) and ischemic myocardium (ΔT1 = 10 msec ± 2; ΔT1 percentage = 0.9% ± 0.2) exhibited reduced T1 reactivity compared with the remote myocardium (ΔT1 = 53 msec ± 7; ΔT1 percentage = 4.7% ± 0.6) and normal myocardium (ΔT1 = 56 msec ± 11; ΔT1 percentage = 4.9% ± 1.1). Receiver operating characteristic analysis demonstrated high diagnostic performance of ΔT1 in detecting ischemic myocardium, with an area under the curve (AUC) of 0.84 (P < .001). Rest T1 displayed high diagnostic performance in detecting infarcted myocardium (AUC = 0.95; P < .001). When rest T1 and ΔT1 were combined, the diagnostic performance for both ischemic and infarcted myocardium were improved (AUCs, 0.89 and 0.97, respectively; all P < .001). The collagen volume fraction correlated with ΔT1, ΔT1 percentage, and Δ extracellular volume percentage (r = -0.70, -0.70, and -0.50, respectively; P = .001, .001, and .03, respectively).Conclusion:Using histopathologic validation in a swine model, noninvasive cardiac MRI stress T1 mapping demonstrated high performance in detecting ischemic and infarcted myocardium without the need for contrast agents.Keywords: Coronary Artery Disease, MRI, Myocardial Ischemia, Rest T1 Mapping, Stress T1 Mapping, Swine Model Supplemental material is available for this article. © RSNA, 2023See also commentary by Burrage and Ferreira in this issue.

Cardiac MRI-derived Myocardial Fibrosis and Ventricular Dyssynchrony Predict Response to Cardiac Resynchronization Therapy in Patients with Nonischemic Dilated Cardiomyopathy.

Purpose:To determine the association of myocardial fibrosis and left ventricular (LV) dyssynchrony measured using cardiac MRI with late gadolinium enhancement (LGE) and feature tracking (FT), respectively, with response to cardiac resynchronization therapy (CRT) for nonischemic dilated cardiomyopathy (DCM).Materials and Methods:This retrospective study included 98 patients (mean age, 59 years ± 10 [SD]; 54 men) who had nonischemic DCM, as assessed with LGE cardiac MRI before CRT. Cardiac MRI FT-derived dyssynchrony was defined as the SD of the time-to-peak strain (TTP-SD) of the LV segments in three directions (longitudinal, radial, and circumferential). CRT response was defined as a 15% increase in LV ejection fraction (LVEF) at echocardiography at 6-month follow-up, and then, long-term cardiovascular events were assessed. The likelihood ratio test was used to evaluate the incremental prognostic value of LGE and dyssynchrony parameters.Results:Seventy-one (72%) patients showed a favorable LVEF response following CRT. LGE presence (odds ratio: 0.14 [95% CI: 0.04, 0.47], P = .002; and hazard ratio: 3.52 [95% CI: 1.37, 9.07], P = .01) and lower circumferential TTP-SD (odds ratio: 1.04 [95% CI: 1.02, 1.07], P = .002; and hazard ratio: 0.98 [95% CI: 0.96, 1.00], P = .03) were independently associated with LVEF nonresponse and long-term outcomes. Combined LGE and circumferential TTP-SD provided the highest discrimination for LVEF nonresponse (area under the receiver operating characteristic curve [AUC]: 0.89 [95% CI: 0.81, 0.94], sensitivity: 84.5% [95% CI: 74.0%, 92.0%], specificity: 85.2% [95% CI: 66.3%, 95.8%]) and long-term outcomes (AUC: 0.84 [95% CI: 0.75, 0.91], sensitivity: 76.9% [95% CI: 56.4%, 91.0%], specificity: 87.0% [95% CI: 76.7%, 93.9%]).Conclusion:Myocardial fibrosis and lower circumferential dyssynchrony assessed with pretherapy cardiac MRI were independently associated with unfavorable LVEF response and long-term events following CRT in patients with nonischemic DCM and may provide incremental value in predicting prognosis.Keywords: MR Imaging, Cardiac, Outcomes Analysis Supplemental material is available for this article. © RSNA, 2023.

[The value of cardiac MRI in the risk stratification in patients with hypertrophic cardiomyopathy].

Objective: To explore the value of cardiac magnetic resonance imaging (CMR) in the risk stratification of hypertrophic cardiomyopathy (HCM). Methods: HCM patients who underwent CMR examination in Fuwai Hospital between March 2012 and May 2013 were retrospectively enrolled. Baseline clinical and CMR data were collected and patient follow-up was performed using telephone contact and medical record. The primary composite endpoint was sudden cardiac death (SCD) or and equivalent event. The secondary composite endpoint was all-cause death and heart transplant. Patients were divided into SCD and non-SCD groups. Cox regression was used to explore risk factors of adverse events. Receiver operating characteristic (ROC) curve analysis was used to assess the performance and the optimal cut-off of late gadolinium enhancement percentage (LGE%) for the prediction of endpoints. Kaplan-Meier and log-rank tests were used to compare survival differences between groups. Results: A total of 442 patients were enrolled. Mean age was (48.5±12.4) years and 143(32.4%) were female. At (7.6±2.5) years of follow-up, 30 (6.8%) patients met the primary endpoint including 23 SCD and 7 SCD equivalent events, and 36 (8.1%) patients met the secondary endpoint including 33 all-cause death and 3 heart transplant. In multivariate Cox regression, syncope(HR=4.531, 95%CI 2.033-10.099, P<0.001), LGE% (HR=1.075, 95%CI 1.032-1.120, P=0.001) and left ventricular ejection fraction (LVEF) (HR=0.956, 95%CI 0.923-0.991, P=0.013) were independent risk factors for primary endpoint; Age (HR=1.032, 95%CI 1.001-1.064, P=0.046), atrial fibrillation (HR=2.977, 95%CI 1.446-6.131, P=0.003),LGE% (HR=1.075, 95%CI 1.035-1.116, P<0.001) and LVEF (HR=0.968, 95%CI 0.937-1.000, P=0.047) were independent risk factors for secondary endpoint. ROC curve showed the optimal LGE% cut-offs were 5.1% and 5.8% for the prediction of primary and secondary endpoint, respectively. Patients were further divided into LGE%=0, 0

Association between left atrial myopathy and sarcomere mutation in patients with hypertrophic cardiomyopathy: insights into left atrial strain by MRI feature tracking.

OBJECTIVES:Left atrial (LA) myopathy, characterized by LA enlargement and mechanical dysfunction, is associated with worse prognosis in hypertrophic cardiomyopathy (HCM) while the impact of sarcomere mutation on LA myopathy remains unclear. We aimed to assess the association between LA myopathy and sarcomere mutation and to explore the incremental utility of LA strain in mutation prediction.METHODS:A total of 105 consecutive HCM patients (mean age 47.8 ± 11.9 years, 71% male) who underwent HCM-related gene screening and cardiac MRI were retrospectively enrolled. LA volume, ejection fraction and strain indices in reservoir, conduit, and booster-pump phases were investigated respectively.RESULTS:Fifty mutation-positive patients showed higher LA maximal volume index (59.4 ± 28.2 vs 43.8 ± 18.1 mL/m2, p = 0.001), lower reservoir (21.3 ± 7.9 vs 26.2 ± 6.6%, p < 0.001), and booster-pump strain (12.1 ± 5.4 vs 17.1 ± 5.0%, p < 0.001) but similar conduit strain (9.2 ± 4.5 vs 9.1 ± 4.5%, p = 0.909) compared with mutation-negative patients. In multivariate logistic regression, LA booster-pump strain was associated with sarcomere mutation (odds ratio = 0.86, 95% confidence interval: 0.77-0.96, p = 0.010) independent of maximal wall thickness, late gadolinium enhancement, and LA volume. Furthermore, LA booster-pump strain showed incremental value for mutation prediction added to Mayo II score (AUC 0.798 vs 0.709, p = 0.024).CONCLUSIONS:In HCM, mutation-positive patients suffered worse LA enlargement and worse reservoir and booster-pump functions. LA booster-pump strain was a strong factor for sarcomere mutation prediction added to Mayo II score.CLINICAL RELEVANCE STATEMENT:The independent association between sarcomere mutation and left atrial mechanical dysfunction provide new insights into the pathogenesis of atrial myopathy and is helpful to understand the adverse prognosis regarding atrial fibrillation and stroke in mutation-positive patients.KEY POINTS:• In patients with hypertrophic cardiomyopathy, left atrial (LA) reservoir and booster-pump function, but not conduit function, were significantly impaired in mutation-positive patients compared with mutation-negative patients. • LA booster-pump strain measured by MRI-derived feature tracking is feasible to predict sarcomere mutation with high incremental value added to Mayo II score.

Adenosine Triphosphate Stress Myocardial Strain in Ischemic Heart Disease: An Animal Study with Histological Validation.

RATIONALE AND OBJECTIVES:It is still challenging for cardiac magnetic resonance (CMR) to detect ischemic heart disease (IHD) without the use of gadolinium contrast. We aimed to evaluate the potential value of adenosine triphosphate (ATP) stress myocardial strain derived from feature tracking (FT) as a novel method for detecting IHD in a swine model.MATERIALS AND METHODS:CMR cines, myocardial perfusion imaging at rest and during ATP stress, and late gadolinium enhancement were obtained in both control and IHD swine. Normal, remote, ischemic, and infarcted myocardium were analyzed. The diagnostic accuracy of myocardial strain for infarction and ischemia was assessed using coronary angiography and pathology as reference.RESULTS:Eleven IHD swine and five healthy control swine were enrolled in this study. Strain parameters, even at rest, were associated with myocardial ischemia and infarction(all p < 0.05). The area under receiver operating characteristic curve (AUC) values of all strain parameters for detecting infarcted myocardium exceeded 0.900 (all p < 0.05). The AUC values for detecting ischemic myocardium were as follows: 0.906 and 0.847 for stress and rest radial strain, 0.763 and 0.716 for stress and rest circumferential strain, 0.758 and 0.663 for stress and rest longitudinal strain (all p < 0.001). Heat maps demonstrated that all strain parameters showed mild to moderate correlations with the stress myocardial blood flow and myocardial perfusion reserve (all p < 0.05).CONCLUSION:CMR-FT-derived ATP stress myocardial strain shows promise as a noninvasive method for detecting myocardial ischemia and infarction in an IHD swine model, with rest strain parameters offering potential as a needle-free diagnostic option.

Developing and evaluating a chronic ischemic cardiomyopathy in swine model by rest and stress CMR.

A large animal model of chronic coronary artery disease (CAD) is crucial for the understanding the underlying pathophysiological processes of chronic CAD and consequences for cardiac structure and function. The goal of this study was to develop a chronic model of CAD in a swine model and to evaluate the changes of myocardial structure, myocardial motility, and myocardial viability during coronary stenosis. A total of 30 swine (including 24 experimental animals and 6 controls) were enrolled. The chronic ischemia model was constructed by using Ameroid constrictor in experimental group. The 24 experimental animals were further divided into 4 groups (6 animals in each group) and were sacrificed at 1, 2, 3 and 4 weeks after operation for pathological examination, respectively. Cardiac magnetic resonance (CMR) was performed preoperatively and weekly postoperatively until sacrificed both in experimental and control group. CMR cine images, rest/adenosine triphosphate (ATP) stress myocardial contrast perfusion and LGE were performed and analyzed. The rest wall thickening (WT) score was calculated from rest cine images. The MPRI (myocardial perfusion reserve index) and MPR (myocardial perfusion reserve) were calculated based on rest and stress perfusion images. Pathology staining including triphenyltetrazolium chloride, HE and picrosirus red staining were performed after swine were sacrificed and collagen volume fraction (CVF) was calculated. The time to formation of ischemic, hibernating, and infarcted myocardium was recorded. In experimental group, from 1w to 4w after surgery, the rest WT score decreased gradually from 35.2 ± 2.0%, 32.0 ± 2.9% to 30.5 ± 3.0% and finally 29.06 ± 1.78%, p < 0.001. Left ventricular ejection fraction was gradually impaired after modeling (58.9 ± 12.6%, 56.3 ± 10.1%, 55.3 ± 9.0%, 53.8 ± 9.9%, respectively). And the MPR and MPRI also decreased stepwise with extent of surgery time (MPRI dropped from 2.1 ± 0.4, 2.0 ± 0.2 to 1.8 ± 0.3 and finally 1.7 ± 0.1, p = 0.004; MPR dropped from 2.3 ± 0.4, 2.1 ± 0.2 to 1.9 ± 0.4 and finally 1.8 ± 0.1, p < 0.001). Stronger associations between MPR, MPRI and CVF were paralleled lower wall thickening scores in fibrosis-affected areas. The ischemic myocardium was first appeared in the first week after surgery (involving ten segments), hibernated myocardium was first appeared in the second week after surgery (involving seventeen segments). LGE was first appeared in eight swine in the third weeks after surgery (16 segments). At 4w after surgery, average 9.6 g scar tissue was found among 6 swine. At the same time, histological analysis established the presence of fibrosis and ongoing apoptosis in the infarcted area. In conclusion, our study provided valuable insights into the pathophysiological processes of chronic CAD and its consequences for cardiac structure and function in a large animal model through combining myocardial motion and stress perfusion.

Apical aneurysm formation in apical hypertrophic cardiomyopathy: Pilot study with cardiac magnetic resonance.

BACKGROUND:The dynamic changes and apical aneurysm formation in apical hypertrophic cardiomyopathy (HCM) have not been specifically described. This study aimed to describe these changes to better understand the progression of apical HCM.METHODS AND RESULTS:Seventy-two patients with apical HCM who underwent at least two cardiac magnetic resonance (CMR) examinations were retrospectively included in this study. The mean interval between the first and last CMR examinations was 50.1 ± 26.8 months (ranging from 4 to 118 months). Compared with the initial values, the left atrial diameter, maximum left ventricular wall thickness and late gadolinium enhancement extent significantly increased (all P < 0.05), while the left ventricular ejection fraction significantly decreased (P < 0.05), at the latest CMR examination. More importantly, the dynamic process of apical aneurysm formation in apical HCM was observed in a subset of patients, which may follow these four stages: starting with systolic apical cavity obliteration, then broadening of the apical slit in systole, further developing into an apical outpouching, and finally forming an apical aneurysm. Eleven patients experienced adverse cardiovascular events, including new-onset or progressive atrial fibrillation (n = 7), hospitalization with heart failure (n = 3) and implantable cardioverter defibrillator intervention (n = 1), at the time of the latest CMR examination.CONCLUSIONS:In the progression of apical HCM, cardiac structure and function will change accordingly. Apical aneurysm formation in apical HCM is a chronic and continuous dynamic process that may follow a 4-step pathway of disease progression.

[Hypertrophic cardiomyopathy with restrictive phenotype: a case report].

Detection of haemodynamic obstruction in hypertrophic cardiomyopathy using the sub-aortic complex: a cardiac MRI and Doppler study.

AIM:To investigate the "sub-aortic complex (SAC)", a new cardiac magnetic resonance imaging (CMRI)-derived parameter, for the evaluation of left ventricular (LV) outflow tract (LVOT) obstruction in patients with hypertrophic cardiomyopathy (HCM), compared with conventional CMRI parameters and Doppler echocardiography.MATERIALS AND METHODS:A total of 157 consecutive patients with HCM were recruited retrospectively. The patients were divided into two groups, 87 with LVOT obstruction and 70 without obstruction. The SAC was defined as a specific anatomical SAC affecting the LVOT, which were measured on the LV three-chamber steady-state free precession (SSFP) cine image at the end-systolic phase. The relations between the existence and severity of obstruction and SAC index (SACi) were evaluated using Pearson's correlation coefficient, receiver operating characteristic (ROC) curves, and logistic regression.RESULTS:The SACs were significantly different between the obstructive and non-obstructive groups. The ROC curves indicated that the SACi was able to discriminate obstructive and non-obstructive patients with the best predictive accuracy (AUC = 0.949, p<0.001). The SACi was an independent predictor of LVOT obstruction and there was a significant negative correlation between resting LVOT pressure gradient and SACi (r=0.72 p<0.001). In the subgroup of patients with or without severe basal septal hypertrophy, the SACi was still able to predict LVOT obstruction with excellent diagnostic accuracy (AUC = 0.944 and 0.948, p<0.001, respectively).CONCLUSION:The SAC is a reliable and straightforward CMRI marker for assessing LVOT obstruction. It is more effective than CMRI two-dimensional flow in diagnosing the severity of obstruction in patients with HCM.

Assessment of late gadolinium enhancement in hypertrophic cardiomyopathy improves risk stratification based on current guidelines.

BACKGROUND AND AIMS:Identifying patients with hypertrophic cardiomyopathy (HCM) who are candidates for implantable cardioverter defibrillator (ICD) implantation in primary prevention for sudden cardiac death (SCD) is crucial. The aim of this study was to externally validate the 2022 European Society of Cardiology (ESC) model and other guideline-based ICD class of recommendation (ICD-COR) models and explore the utility of late gadolinium enhancement (LGE) in further risk stratification.METHODS:Seven hundred and seventy-four consecutive patients who underwent cardiac magnetic resonance imaging were retrospectively enrolled.RESULTS:Forty-six (5.9%) patients reached the SCD-related endpoint during 7.4 ± 2.5 years of follow-up. Patients suffering from SCD had higher ESC Risk-SCD score (4.3 ± 2.4% vs. 2.8 ± 2.1%, P < .001) and LGE extent (13.7 ± 9.4% vs. 4.9 ± 6.6%, P < .001). Compared with the 2014 ESC model, the 2022 ESC model showed increased area under the curve (.76 vs. .63), sensitivity (76.1% vs. 43.5%), positive predictive value (16.8% vs. 13.6%), and negative predictive value (98.1% vs. 95.9%). The C-statistics for SCD prediction of 2011 American College of Cardiology (ACC)/American Heart Association (AHA), 2014 ESC, 2020 AHA/ACC, and 2022 ESC models were .68, .64, .76 and .78, respectively. Furthermore, in patients without extensive LGE, LGE ≥5% was responsible for seven-fold SCD risk after multivariable adjustment. Whether in ICD-COR II or ICD-COR III, patients with LGE ≥5% and <15% showed significantly worse prognosis than those with LGE <5% (all P < .001).CONCLUSIONS:The 2022 ESC model performed better than the 2014 ESC model with especially improved sensitivity. LGE enabled further risk stratification based on current guidelines.

Detection of Myocardial Ischemia Using Cardiovascular MRI Stress T1 Mapping: A Miniature-Swine Validation Study.

Purpose:To assess the efficacy of cardiac MRI stress T1 mapping in detecting ischemic and infarcted myocardium in a miniature-swine model, using pathologic findings as the reference standard.Materials and Methods:Ten adult male Chinese miniature swine, with coronary artery stenosis induced by an ameroid constrictor, and two healthy control swine were studied. Cardiac 3-T MRI rest and adenosine triphosphate stress T1 mapping and perfusion images, along with resting and late gadolinium enhancement images, were acquired at baseline and weekly up to 4 weeks after surgery or until humanely killed. A receiver operating characteristic analysis was used to analyze the performance of T1 mapping in the detection of myocardial ischemia.Results:In the experimental group, both the infarcted myocardium (ΔT1 = 10 msec ± 2 [SD]; ΔT1 percentage = 0.7% ± 0.1) and ischemic myocardium (ΔT1 = 10 msec ± 2; ΔT1 percentage = 0.9% ± 0.2) exhibited reduced T1 reactivity compared with the remote myocardium (ΔT1 = 53 msec ± 7; ΔT1 percentage = 4.7% ± 0.6) and normal myocardium (ΔT1 = 56 msec ± 11; ΔT1 percentage = 4.9% ± 1.1). Receiver operating characteristic analysis demonstrated high diagnostic performance of ΔT1 in detecting ischemic myocardium, with an area under the curve (AUC) of 0.84 (P < .001). Rest T1 displayed high diagnostic performance in detecting infarcted myocardium (AUC = 0.95; P < .001). When rest T1 and ΔT1 were combined, the diagnostic performance for both ischemic and infarcted myocardium were improved (AUCs, 0.89 and 0.97, respectively; all P < .001). The collagen volume fraction correlated with ΔT1, ΔT1 percentage, and Δ extracellular volume percentage (r = -0.70, -0.70, and -0.50, respectively; P = .001, .001, and .03, respectively).Conclusion:Using histopathologic validation in a swine model, noninvasive cardiac MRI stress T1 mapping demonstrated high performance in detecting ischemic and infarcted myocardium without the need for contrast agents.Keywords: Coronary Artery Disease, MRI, Myocardial Ischemia, Rest T1 Mapping, Stress T1 Mapping, Swine Model Supplemental material is available for this article. © RSNA, 2023See also commentary by Burrage and Ferreira in this issue.

Cardiac MRI-derived Myocardial Fibrosis and Ventricular Dyssynchrony Predict Response to Cardiac Resynchronization Therapy in Patients with Nonischemic Dilated Cardiomyopathy.

Purpose:To determine the association of myocardial fibrosis and left ventricular (LV) dyssynchrony measured using cardiac MRI with late gadolinium enhancement (LGE) and feature tracking (FT), respectively, with response to cardiac resynchronization therapy (CRT) for nonischemic dilated cardiomyopathy (DCM).Materials and Methods:This retrospective study included 98 patients (mean age, 59 years ± 10 [SD]; 54 men) who had nonischemic DCM, as assessed with LGE cardiac MRI before CRT. Cardiac MRI FT-derived dyssynchrony was defined as the SD of the time-to-peak strain (TTP-SD) of the LV segments in three directions (longitudinal, radial, and circumferential). CRT response was defined as a 15% increase in LV ejection fraction (LVEF) at echocardiography at 6-month follow-up, and then, long-term cardiovascular events were assessed. The likelihood ratio test was used to evaluate the incremental prognostic value of LGE and dyssynchrony parameters.Results:Seventy-one (72%) patients showed a favorable LVEF response following CRT. LGE presence (odds ratio: 0.14 [95% CI: 0.04, 0.47], P = .002; and hazard ratio: 3.52 [95% CI: 1.37, 9.07], P = .01) and lower circumferential TTP-SD (odds ratio: 1.04 [95% CI: 1.02, 1.07], P = .002; and hazard ratio: 0.98 [95% CI: 0.96, 1.00], P = .03) were independently associated with LVEF nonresponse and long-term outcomes. Combined LGE and circumferential TTP-SD provided the highest discrimination for LVEF nonresponse (area under the receiver operating characteristic curve [AUC]: 0.89 [95% CI: 0.81, 0.94], sensitivity: 84.5% [95% CI: 74.0%, 92.0%], specificity: 85.2% [95% CI: 66.3%, 95.8%]) and long-term outcomes (AUC: 0.84 [95% CI: 0.75, 0.91], sensitivity: 76.9% [95% CI: 56.4%, 91.0%], specificity: 87.0% [95% CI: 76.7%, 93.9%]).Conclusion:Myocardial fibrosis and lower circumferential dyssynchrony assessed with pretherapy cardiac MRI were independently associated with unfavorable LVEF response and long-term events following CRT in patients with nonischemic DCM and may provide incremental value in predicting prognosis.Keywords: MR Imaging, Cardiac, Outcomes Analysis Supplemental material is available for this article. © RSNA, 2023.

[The value of cardiac MRI in the risk stratification in patients with hypertrophic cardiomyopathy].

Objective: To explore the value of cardiac magnetic resonance imaging (CMR) in the risk stratification of hypertrophic cardiomyopathy (HCM). Methods: HCM patients who underwent CMR examination in Fuwai Hospital between March 2012 and May 2013 were retrospectively enrolled. Baseline clinical and CMR data were collected and patient follow-up was performed using telephone contact and medical record. The primary composite endpoint was sudden cardiac death (SCD) or and equivalent event. The secondary composite endpoint was all-cause death and heart transplant. Patients were divided into SCD and non-SCD groups. Cox regression was used to explore risk factors of adverse events. Receiver operating characteristic (ROC) curve analysis was used to assess the performance and the optimal cut-off of late gadolinium enhancement percentage (LGE%) for the prediction of endpoints. Kaplan-Meier and log-rank tests were used to compare survival differences between groups. Results: A total of 442 patients were enrolled. Mean age was (48.5±12.4) years and 143(32.4%) were female. At (7.6±2.5) years of follow-up, 30 (6.8%) patients met the primary endpoint including 23 SCD and 7 SCD equivalent events, and 36 (8.1%) patients met the secondary endpoint including 33 all-cause death and 3 heart transplant. In multivariate Cox regression, syncope(HR=4.531, 95%CI 2.033-10.099, P<0.001), LGE% (HR=1.075, 95%CI 1.032-1.120, P=0.001) and left ventricular ejection fraction (LVEF) (HR=0.956, 95%CI 0.923-0.991, P=0.013) were independent risk factors for primary endpoint; Age (HR=1.032, 95%CI 1.001-1.064, P=0.046), atrial fibrillation (HR=2.977, 95%CI 1.446-6.131, P=0.003),LGE% (HR=1.075, 95%CI 1.035-1.116, P<0.001) and LVEF (HR=0.968, 95%CI 0.937-1.000, P=0.047) were independent risk factors for secondary endpoint. ROC curve showed the optimal LGE% cut-offs were 5.1% and 5.8% for the prediction of primary and secondary endpoint, respectively. Patients were further divided into LGE%=0, 0