Left ventricular deformation and myocardial fibrosis in pediatric patients with Duchenne muscular dystrophy

Background: Left ventricular (LV) strain and rotation are emerging functional markers for early detection of LV dysfunction and have been associated with the burden of myocardial fibrosis in several disease states. This study examined the association between LV deformation (i.e., LV strain and rotation) and extent and location of LV myocardial fibrosis in pediatric patients with Duchenne muscular dystrophy (DMD). Methods and results: 34 pediatric patients with DMD underwent cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) to assess LV myocardial fibrosis. Offline CMR feature-tracking analysis was used to assess global and segmental longitudinal and circumferential LV strain, and LV rotation. Patients with fibrosis (n = 18, 52.9%) were older than those without fibrosis (14 ± 3 years (yrs) vs 11 ± 2 yrs., p = 0.01). There was no significant difference in LV ejection fraction (LVEF) between subjects with and without fibrosis (54 ± 6% vs 56 ± 4%, p = 0.18). However, lower endocardial global circumferential strain (GCS), but not LV rotation, was associated with presence of fibrosis (adjusted Odds Ratio 1.25 [95% CI 1.01–1.56], p = 0.04). Both GCS and global longitudinal strain correlated with the extent of fibrosis (r =.52, p = 0.03 and r =.75, p &lt; 0.01, respectively). Importantly, segmental strain did not seem to correspond to location of fibrosis. Conclusion: A lower global, but not segmental, strain is associated with presence and extent of LV myocardial fibrosis in pediatric DMD patients. Therefore, strain parameters might detect structural myocardial alterations, however currently more research is needed to evaluate its value (e.g., prognostic) in clinical practice.</p

Left ventricular strain-volume loops in bicuspid aortic valve disease:new insights in cardiomechanics

AIMS: By combining temporal changes in left ventricular (LV) global longitudinal strain (GLS) with LV volume, LV strain-volume loops can assess cardiac function across the cardiac cycle. This study compared LV strain-volume loops between bicuspid aortic valve (BAV) patients and controls, and investigated the loop’s prognostic value for clinical events.METHODS AND RESULTS: From a prospective cohort of congenital heart disease patients, BAV patients were selected and compared with healthy volunteers, who were matched for age and sex at group level. GLS7 analysis from apical views were used to construct strain-volume loops. Associations with clinical events, i.e. a composite of all-cause mortality, heart failure, arrhythmias and aortic valve replacement, were assessed by Cox regression. 113 BAV patients were included (median age 32 years, 40% female). BAV patients demonstrated lower Sslope (0.21%/mL, [Q1-Q3: 0.17-0.28] vs. 0.27%/mL [0.24-0.34], p&lt;0.001) and ESslope (0.19%/mL [0.12-0.25] vs. 0.29%/mL [0.21-0.43], p&lt;0.001) compared to controls, but also greater uncoupling during early (0.48±1.29 vs. 0.06±1.2, p=0.018) and late diastole (0.66±1.01 vs -0.06±1.09, p&lt;0.001). Median follow-up duration was 9.9 [9.3-10.4] years. Peak aortic jet velocity (HR 1.22, p=0.03), enlarged left atrium (HR 3.16, p=0.003), E/e’ ratio (HR 1.17, p=0.002), GLS (HR 1.16, p=0.008) and ESslope (HR 0.66, p=0.04) were associated with the occurrence of clinical events.CONCLUSION: Greater uncoupling and lower systolic and diastolic slopes were observed in BAV patients compared to healthy controls, suggesting presence of altered LV cardiomechanics. Moreover, lower ESslope was associated with clinical events, highlighting the strain-volume loop’s potential as prognostic marker

Left ventricular strain-volume loops in bicuspid aortic valve disease:new insights in cardiomechanics

AIMS: By combining temporal changes in left ventricular (LV) global longitudinal strain (GLS) with LV volume, LV strain-volume loops can assess cardiac function across the cardiac cycle. This study compared LV strain-volume loops between bicuspid aortic valve (BAV) patients and controls, and investigated the loop’s prognostic value for clinical events.METHODS AND RESULTS: From a prospective cohort of congenital heart disease patients, BAV patients were selected and compared with healthy volunteers, who were matched for age and sex at group level. GLS7 analysis from apical views were used to construct strain-volume loops. Associations with clinical events, i.e. a composite of all-cause mortality, heart failure, arrhythmias and aortic valve replacement, were assessed by Cox regression. 113 BAV patients were included (median age 32 years, 40% female). BAV patients demonstrated lower Sslope (0.21%/mL, [Q1-Q3: 0.17-0.28] vs. 0.27%/mL [0.24-0.34], p&lt;0.001) and ESslope (0.19%/mL [0.12-0.25] vs. 0.29%/mL [0.21-0.43], p&lt;0.001) compared to controls, but also greater uncoupling during early (0.48±1.29 vs. 0.06±1.2, p=0.018) and late diastole (0.66±1.01 vs -0.06±1.09, p&lt;0.001). Median follow-up duration was 9.9 [9.3-10.4] years. Peak aortic jet velocity (HR 1.22, p=0.03), enlarged left atrium (HR 3.16, p=0.003), E/e’ ratio (HR 1.17, p=0.002), GLS (HR 1.16, p=0.008) and ESslope (HR 0.66, p=0.04) were associated with the occurrence of clinical events.CONCLUSION: Greater uncoupling and lower systolic and diastolic slopes were observed in BAV patients compared to healthy controls, suggesting presence of altered LV cardiomechanics. Moreover, lower ESslope was associated with clinical events, highlighting the strain-volume loop’s potential as prognostic marker

Similarity between carotid and coronary artery responses to sympathetic stimulation and the role of alpha-1 receptors in humans.

BACKGROUND: Carotid artery (CCA) dilation occurs in healthy subjects during cold pressor test (CPT), whilst the magnitude of dilation relates to cardiovascular risk. To further explore this phenomena and mechanism, we examined carotid artery responses to different sympathetic tests, with and without α1-receptor blockade, and assessed similarity to these responses between carotid and coronary arteries. METHODS: In randomised order, 10 healthy participants (25{plus minus}3yrs) underwent sympathetic stimulation using CPT (3-minutes left hand immersion in ice-slush) and lower-body negative pressure (LBNP). Before and during sympathetic tests, CCA diameter and velocity (Doppler ultrasound) and left anterior descending (LAD) coronary artery velocity (echocardiography) were recorded across 3-min. Measures were repeated 90-min following selective α1-receptor blockade via oral Prazosin (0.05mg-per-kg bodyweight). RESULTS: CPT significantly increased CCA diameter, LAD maximal velocity and velocity-time integral area-under-the-curve (all P<0.05). In contrast, LBNP resulted in a decrease in CCA diameter, LAD maximal velocity and velocity time integral (VTI, all P<0.05). Following α1-receptor blockade, CCA and LAD velocity responses to CPT were diminished. In contrast, during LBNP (-30mmHg), α1-receptor blockade did not alter CCA or LAD responses. Finally, changes in CCA diameter and LAD VTI-responses to sympathetic stimulation were positively correlated (r=0.66, P<0.01). CONCLUSION: We found distinct carotid artery responses to different tests of sympathetic stimulation, where α1-receptors partly contribute to CPT-induced responses. Finally, we found agreement between carotid and coronary artery responses. These data indicate similarity between carotid and coronary responses to sympathetic tests and the role of α1-receptors that is dependent on the nature of the sympathetic challenge

Cardiac rehabilitation and adverse events among adult patients with simple congenital heart disease and heart failure

Aims: Improved care has resulted in prolonged survival of patients with congenital heart disease (ConHD), increasing age-related cardiovascular comorbidities. Although cardiovascular rehabilitation (CR) represents evidence-based care for heart failure (HF), the clinical impact of CR in patients with ConHD who developed HF during adulthood is unclear. We investigated 12-month mortality and morbidity in patients with simple ConHD diagnosed with HF with CR versus without CR. Methods: A retrospective cohort study was conducted for the time period February 2004 - February 2024. Utilizing TriNetX, a global federated health research network, a real-world dataset of simple ConHD patients was acquired to compare patients with vs. without (controls) prescription for exercise-based CR. Patients were propensity-score matched for age, sex, ethnicity, comorbidities, procedures, and medication. The primary outcome was a composite of all-cause mortality, ischemic stroke, and acute coronary syndrome (major adverse cardiovascular events; MACE) within 12 months. Results: Following propensity score matching, the total cohort consisted of 6,866 simple ConHD patients with HF. CR was associated with significantly lower odds for MACE (odds ratio (OR) 0.61 [95 % confidence interval (CI): 0.54–0.69]) and its individual components all-cause mortality (OR 0.40 [95 % CI 0.33–0.47]) and ischemic stroke (OR 0.75 [95 % CI 0.64–0.88]), but not acute coronary syndrome (OR 1.24 [95 % CI 0.91–1.69]). Conclusion: CR was associated with significantly lower 12-month MACE in patients with simple ConHD with concomitant HF compared to usual care