Degree Of Myocardial Involvement Research Articles

Diagnosis of Cardiac Amyloidosis Using a Radiomics Approach Applied to Late Gadolinium-Enhanced Cardiac Magnetic Resonance Images: A Retrospective, Multicohort, Diagnostic Study.

ObjectivesTo assess the potential of a radiomics approach of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) in the diagnosis of cardiac amyloidosis (CA).Materials and MethodsThis retrospective study included 200 patients with biopsy-proven light-chain (AL) amyloidosis. CA was diagnosed on the basis of systemic amyloidosis confirmed with evidence of cardiac involvement by imaging and clinical biomarkers. A total of 139 patients [54 ± 8 years, 75 (54%) men] in our institution were divided into training cohort [n = 97, mean age of 53 ± 8 years, 54 (56%) men] and internal validation cohort [n = 42, mean age: 56 ± 8 years, 21 (50%) men] with a ratio of 7:3, while 61 patients [mean age: 60 ± 9 years, 42 (69%) men] from the other two institutions were enrolled for external validation. Radiomics features were extracted from global (all short-axis images from base-to-apex) left ventricular (LV) myocardium and three different segments (basal, midventricular, and apex) on short-axis LGE images using the phase-sensitive reconstruction (PSIR) sequence. The Boruta algorithm was used to select the radiomics features. This model was built using the XGBoost algorithm. The two readers performed qualitative and semiquantitative assessment of the LGE images based on the visual LGE patterns, while the quantitative assessment was measured using a dedicated semi-automatic CMR software. The diagnostic performance of the radiomics and other qualitative and quantitative parameters were compared by a receiver operating characteristic (ROC) curve analysis. A correlation between radiomics and the degree of myocardial involvement by amyloidosis was tested.ResultsA total of 1,906 radiomics features were extracted for each LV section. No statistical significance was indicated between any two slices for diagnosing CA, and the highest area under the curve (AUC) was found in basal section {0.92 [95% confidence interval (CI), 0.86–0.97] in the LGE images in the training set, 0.89 (95% CI, 0.79–1.00) in the internal validation set, and 0.92 (95% CI, 0.85–0.99) in the external validation set}, which was superior to the visual assessment and quantitative LGE parameters. Moderate correlations between global or basal radiomics scores (Rad-scores) and Mayo stage in all patients were reported (Spearman’s Rho = 0.61, 0.62; all p < 0.01).ConclusionA radiomics analysis of the LGE images provides incremental information compared with the visual assessment and quantitative parameters on CMR to diagnose CA. Radiomics was moderately correlated with the severity of CA. Further studies are needed to assess the prognostic significance of radiomics in patients with CA.

Sex-Related Risk of Cardiac Involvement in Hereditary Transthyretin Amyloidosis: Insights From THAOS

ObjectivesBecause patients with ATTRv cardiomyopathy are more likely to be male, this analysis aimed to increase information on associations between sex and genotype, phenotype, and degree of myocardial involvement in ATTRv amyloidosis. BackgroundTransthyretin amyloid cardiomyopathy is a progressive, fatal disease that occurs due to accumulation of wild-type or variant (ATTRv) transthyretin amyloid fibrils in the myocardium. MethodsThe Transthyretin Amyloidosis Outcomes Survey (THAOS) is an ongoing global longitudinal observational survey of patients with ATTR amyloidosis and asymptomatic carriers with TTR mutations. Data from THAOS (data cutoff: January 6, 2020) were analyzed to determine any sex-based differences in genotype, phenotype, and presence of cardiac and neurological symptoms in patients with ATTRv amyloidosis and in patients with ATTRv amyloidosis and cardiomyopathy. ResultsThere were 2,790 patients with ATTRv amyloidosis enrolled in THAOS, with male patients more likely to have symptoms of cardiac involvement and a cardiac phenotype. Male prevalence was greater in patients with more severe cardiac manifestations of disease, as assessed with N-terminal pro–B-type natriuretic peptide, left-ventricular (LV) ejection fraction, mean LV wall thickness divided by height, and LV mass index divided by height. Sex, age at disease onset, and genotype category were identified by multivariate analyses as risk factors for the development of cardiomyopathy (defined as increased LV septum thickness divided by height). ConclusionsIn this analysis, myocardial involvement was more frequent and pronounced in male patients with ATTRv amyloidosis, suggesting that there may be biological characteristics that inhibit myocardial amyloid infiltration in females or facilitate it in males.

Myocardial Fibrosis and Inflammation in Liver Cirrhosis: MRI Study of the Liver-Heart Axis.

Background Cardiac involvement in liver cirrhosis in the absence of underlying cardiac disease is termed cirrhotic cardiomyopathy. The pathophysiology of this condition is still poorly understood. Purpose To investigate the extent of subclinical imaging changes in terms of fibrosis and inflammation and to explore the relationship between the severity of liver disease and the degree of myocardial involvement. Materials and Methods In this prospective study from November 2018 to December 2019, participants with liver cirrhosis and healthy control participants underwent hepatic and cardiac MRI. The multiparametric scan protocol assessed hepatic (T1 and T2 relaxation times, extracellular volume [ECV], and MR elastography-based liver stiffness) and cardiac (T1 and T2 relaxation times, ECV, myocardial edema, late gadolinium enhancement [LGE], and myocardial strain) parameters. Student t tests, one-way analysis of variance, Pearson correlation, and multivariable binary regression analysis were used for statistical analyses. Results A total of 42 participants with liver cirrhosis (mean age ± standard deviation, 57 years ± 11; 23 men) and 18 control participants (mean age, 54 years ± 19; 11 men) were evaluated. Compared with control participants, the participants with liver cirrhosis displayed reduced longitudinal strain and elevated markers of myocardial disease (T1 and T2 relaxation times, ECV, and qualitative and quantitative LGE). Myocardial T1 (978 msec ± 23 vs 1006 msec ± 29 vs 1044 msec ± 14; P < .001) and T2 relaxation times (56 msec ± 4 vs 59 msec ± 3 vs 62 msec ± 8; P = .04) and ECV (30% ± 5 vs 33% ± 5 vs 38% ± 7; P = .009) were higher depending on Child-Pugh class (A vs B vs C). Positive LGE lesions (three of 11 [27%] vs 10 of 19 [53%] vs nine of 11 [82%]; P = .04) were more prevalent in advanced Child-Pugh classes. MR elastography-based liver stiffness was an independent predictor for LGE (odds ratio, 1.6; 95% confidence interval: 1.2%, 2.1%; P = .004) and correlated with quantitative LGE (r = 0.67; P < .001), myocardial T1 relaxation times (r = 0.55; P < .001), and ECV (r = 0.39; P = .01). Conclusion In participants with liver cirrhosis, systolic dysfunction and elevated parameters of myocardial edema and fibrosis were observed at MRI, which were more abnormal with greater severity of liver disease. © RSNA, 2020 Online supplemental material is available for this article. See also the editorial by de Roos and Lamb in this issue.

Clinical analysis of 155 patients with Duchenne muscular dystrophy

Objective To investigate the clinical manifestations and laboratory examinations of Duchenne muscular dystrophy (DMD) patients and evaluate the principle of intermittent intravenous combined with oral glucocorticoid therapy. Methods The clinical features, laboratory examinations andfollow-up data of 155 DMD patients were collected. These patients were given dexamethasone 5-10 mg/d by intravenous infusion for 10-15 d and oral prednisone acetate 0.50-0.75 mg/(kg·d) for one month. After treatment, the motor ability of lower limbs, the level of serum creatine kinase (CK) and 99mTc-MIBI gated myocardial perfusion imaging (GMPI) findings were compared with those before glucocorticoid therapy by statistical analysis. Results 1) The motor ability was improved in 70 follow-up cases of DMD patients with long-term oral prednisone (squat and rise: Z = 207.000, P = 0.034; climbing stairs: Z = 237.000, P =0.008). 2) The level of serum CK of 155 first diagnosed patients reached the peak at 3 years old, and declined after the age of 8 (P < 0.05, for all). The serum CK of 70 follow-up cases was significantly decreased after 10-15 d dexamethasone intravenous infusion (P = 0.000), and increased again after one-month oral administration of prednisone acetate (P = 0.000), but was still lower than that before treatment (P = 0.008). 3) The 99mTc-MIBI GMPI of 77 patients showed different degrees of myocardial involvement and significantly uneven left ventricular radionuclide distribution, especially in apex cordis (55 cases), inferior wall (45 cases) and anterior wall (30 cases) of apex. Conclusions There exists increased level of serum CK in the sub⁃clinical stage of DMD. The level of serum CK declined year by year after the age of 8. Intermittent intravenous combined with oral glucocorticoid therapy has an important effect on protecting motor and cardiac functions, extending walking time and reducing serum CK level and muscle cell damage. Early glucocorticoid therapy is recommended. DOI : 10.3969/j.issn.1672-6731.2015.05.008

Myopericarditis in Children: Elevated Troponin I Level Does Not Predict Outcome

Myopericarditis is primarily a pericarditic syndrome with some degree of myocardial involvement, as evident by elevated cardiac enzymes. Differentiating myopericarditis from acute coronary syndromes can be challenging and may require coronary angiography or perfusion studies. Data on myopericarditis and its outcome for children are scarce. This study delineates the demography, clinical presentation, and outcomes of myopericarditis for children and evaluates the prognostic value of elevated troponin I. The authors retrospectively reviewed 880 patients younger than 18 years of age who were admitted with diagnoses of chest pain, myocarditis, or pericarditis between 2000 and 2010 at their institution. Myopericarditis was defined as a clinical presentation of pericarditis in the presence of elevated levels of cardiac enzymes. Medical records were reviewed to abstract the demographic data, clinical presentation, evaluation, treatment, and follow-up outcomes. A total of 12 patients (1.4%) with myopericarditis were identified. All the patients were male, 8 (67%) of whom were Caucasian, and their median age was 16 years (range, 11-17 years). Two of the patients (17%) had recently used illicit drugs, and two (17%) had recently smoked cigarettes. At presentation, symptoms included chest pain in 12 patients (100%, 12/12), upper respiratory symptoms in 3 patients (25%, 3/12), and shortness of breath in 3 patients (25%, 3/12). No cardiac murmur or gallop was noted in any patient. Electrocardiographic (ECG) changes included diffuse ST-T changes (5 patients), localized ST-T changes (6 patients), and no ST-T changes (1 patient). All the patients had elevated levels of cardiac enzymes, with a median Troponin I level of 21.4 ng/ml (range, 5.0-134.4 ng/ml) and a median CK-MB level of 50.2 ng/ml (range, 7-135 ng/ml). Echocardiography showed normal left ventricular systolic function in all the patients (median ejection fraction, 61%; range, 56-69%). None had pericardial effusion during the first echocardiographic evaluation. Coronary angiography showed normal coronary arteries in all nine subjects for whom it was performed. Treatment of myopericarditis consisted of ibuprofen, acetaminophen, and/or aspirin. During a median follow-up period of 2 months (range, 2 weeks to 3 years), all the patients were asymptomatic with echocardiography showing normal left ventricular size and function. Myopericarditis was exclusively seen in male adolescents. Despite markedly elevated levels of cardiac enzymes, the clinical evolution of myopericarditis seems benign without any myocardial dysfunction. The inflammatory involvement of the myocardium appears to be self-limited without short-term, overt sequelae. An elevated troponin I level in myopericarditis, unlike acute coronary syndromes, does not seem to carry an adverse prognosis. Further studies are needed to evaluate the long-term prognosis for such patients.

Biochemical markers of neonatal myocardial dysfunction

Introduction. Cardiac ultrasounds (US) are not always available at the bedside. Cardiac Troponin I (cTnI), CK-MB and NT-proBNP may be an alternative or complementary to influence evaluation and treatment.Objectives. To determine reference ranges of biochemical markers cTnI, CK-MB and NT-proBNP in normal neonates.Methodology. Cord and blood samples were collected from neonates and the above biochemical markers were determined. Ultrasounds were performed blindly.Results. CK-MB remains constant from cord blood to the first day, declining thereafter to almost half the values (81.5 vs 52.0 U/l); cTnI increases from 0.004 to 0.058 ng/ml by 72 h falling to 0.030 by day 10; NT-proBNP peaks by 24 h (5085.5 pg/ml), subsiding to 3388.5 pg/ml by day 3, falling to 1316.0 pg/ml by day 10.Conclusions. CK-MB, mostly of muscle origin and reflecting labor stress or injury, is not to recommend as a measure of myocardial damage in the neonate. The rise in cTnI may be explained by a degree of myocardial involvement, albeit physiological. The initial rise and subsequent fall of NT-proBNP represents the physiological ventricular overload of transient birth adaptation.

Biochemical markers of neonatal myocardial dysfunction

Introduction. Cardiac ultrasounds (US) are not always available at the bedside. Cardiac Troponin I (cTnI), CK-MB and NT-proBNP may be an alternative or complementary to influence evaluation and treatment. Objectives. To determine reference ranges of biochemical markers cTnI, CK-MB and NT-proBNP in normal neonates. Methodology. Cord and blood samples were collected from neonates and the above biochemical markers were determined. Ultrasounds were performed blindly. Results. CK-MB remains constant from cord blood to the first day, declining thereafter to almost half the values (81.5 vs 52.0 U/l); cTnI increases from 0.004 to 0.058 ng/ml by 72 h falling to 0.030 by day 10; NT-proBNP peaks by 24 h (5085.5 pg/ml), subsiding to 3388.5 pg/ml by day 3, falling to 1316.0 pg/ml by day 10. Conclusions. CK-MB, mostly of muscle origin and reflecting labor stress or injury, is not to recommend as a measure of myocardial damage in the neonate. The rise in cTnI may be explained by a degree of myocardial involvement, albeit physiological. The initial rise and subsequent fall of NT-proBNP represents the physiological ventricular overload of transient birth adaptation.

Valsalva maneuver: a test of the functional state of cardiac innervation in Chagasic myocarditis

The ratio between maximal and minimal R-R intervals measured during the Valsalva maneuver is an adequate means of studying the functional state of the cardiac parasympathetic nervous system. We studied the changes in heart rate evoked during the Valsalva maneuver in 49 asymptomatic Chagasic patients with different degrees of myocardial involvement. The Chagasic patients were divided in three groups. Twelve patients had no evidence of heart disease except for an abnormal myocardial biopsy. Sixteen patients had a normal electrocardiogram but revealed evidence of localized myocardial damage as shown by left ventricular cineangiography. Twenty-one patients had abnormal electrocardiograms and revealed multiple areas of abnormal left ventricular wall motion. The Valsalva ratio (mean ± standard error) was 1.62 ± 0.12 for controls, 1.52 ± 0.10 for those with an abnormal biopsy, 1.48 ± 0.06 for those with localized myocardial damage, and 1.31 ± 0.04 for those with an abnormal electrocardiogram. There were no statistically significant differences between the control group (normal sero-negative subjects) and those patients with normal electrocardiograms. Results in those with an abnormal electrocardiogram, however, were statistically different ( P < 0.05) from the other groups. These results are in discordance with the commonly accepted hypothesis that cardiac parasympathetic denervation causes a dilated myocardiopathy in patients with chronic Chagasic myocarditis.

Systemic nodular panniculitis with cardiac involvement.

A case of systemic nodular panniculitis is described in which the myocardium was found at necropsy to be extensively involved with focal interstitial carditis, identical histologically with nodules of panniculitis biopsied from the skin. This degree of myocardial involvement, which was not apparent during life and was not confined to pericardial or myocardial adipose tissue, has not previously been reported. The literature relating to nodular panniculitis is briefly reviewed and the concept of Weber-Christian disease critically re-appraised.

Asymptomatic giant cell granulomatous myocarditis

A 76-year-old man who was known to be an epileptic died from a massive subdural hematoma and a rapidly progressive renal insufficiency. Incidental findings at necropsy were the histopathologic changes of giant cell granulomatous myocarditis and granulomatous nodules in the lungs. The patient had never had symptoms relating to his underlying cardiac pathology. It is postulated that the degree of myocardial involvement was not extensive enough to interfere with cardiac function, and an intercurrent fatal episode precluded the usual progression of this disease. Chronic giant cell granulomatous myocarditis is part of a systemic disease of unknown etiology. However, the renal lesions of acute interstitial nephritis seen in our patient lend support for a hypersensitivity mechanism. We think that this is the oldest patient to be reported on, and the only one in whom the cardiac pathology was not the cause of death.