Early Detection Of Cardiac Involvement Research Articles

Unveiling Cardiac Involvement in Juvenile Dermatomyositis Through Speckle-Tracking Echocardiography.

Early detection of cardiac involvement in Juvenile Dermatomyositis (JDM) is difficult due to the absence of clinical signs and symptoms, with systolic dysfunction often emerging in late stages and associated with a poor prognosis. This study aimed to employ two-dimensional speckle-tracking echocardiography (STE) for subclinical assessment of left ventricular (LV) systolic failure in JDM and explore potential associations between impaired LV systolic function (LV-GLS) and disease activity. A prospective study enrolled 20 healthy volunteers and 26 JDM patients (< 18years old) without cardiac symptoms. Clinical data were collected from medical records, and echocardiograms were conducted by a pediatric cardiologist. Our study cohort demonstrated similar age to controls (13.5 ± .6 vs. 13.8 ± 4.7; p = 0.465). Median illness duration at echocardiography was 5 (1.5-17.5) years, and conventional echocardiography indicated normal LV ejection fraction (> 55%) in all participants. However, STE revealed lowered LV GLS in JDM patients (-22.2 ± 4.1% vs. -26.5 ± 5.3% p = 0.022). Pulse steroid users displayed lower GLS average values compared to non-users (β = 4.99, 95% CI 1.34-8.64, p = 0.009). Negative correlations existed between LV-GLS and age at diagnosis (r = -0.499; p = 0.011), diastolic parameters (E/E' ratio) and age at diagnosis (r = -0.469; p = 0.018), as well as RV global strain and age at diagnosis (r = -0.443; p = 0.024). Employing STE in JDM patients facilitated the identification of preclinical cardiac dysfunction. Given JDM patients' younger age, early myocardial damage detection through STE may impact treatment decisions and long-term cardiovascular prognosis.

Clinical study of left ventricular structure and function in patients with Anderson-Fabry disease before and after enzyme replacement therapy.

Cardiac left ventricular hypertrophy (LVH) is the most common manifestation of heart involvement in Anderson-Fabry disease (AFD). Conventional cardiac imaging is not sensitive enough to detect early signs of LVH in AFD. It remains uncertain whether enzyme replacement therapy (ERT) can prevent LVH progression and improve myocardial function. This study aimed to assess the effectiveness of two-dimensional speckle tracking echocardiography (2D-STE) in early detection of cardiac involvement in AFD and monitoring the efficacy of agalsidase alfa and agalsidase beta therapy. Thirteen consecutive AFD patients and 12 healthy controls underwent standard transthoracic 2D, color Doppler, tissue Doppler echocardiography, and 2D strain analysis. Global longitudinal strain (GLS) and global circumferential strain (GCS) were measured. Diastolic strain rate (SR) was extracted. Compared to healthy subjects, AFD patients without LVH showed lower levels of GLS (p < 0.001) and SR (p = 0.01), while there was no difference in GCS (p = 0.82). Following treatment, apical circumferential strain (ACS) showed improvement (p = 0.01). In AFD patients without LVH, there was a decrease in global and segmental LS. Higher plasma Lyso-GL-3 concentrations were associated with elevated ACS values after ERT, indicating that ACS in AFD patients without LVH, albeit normal, is involved in early LV dysfunction.

Electrocardiogram analysis in Anderson-Fabry disease: a valuable tool for progressive phenotypic expression tracking.

Electrocardiogram (ECG) has proven to be useful for early detection of cardiac involvement in Anderson-Fabry disease (AFD); however, little evidence is available on the association between ECG alterations and the progression of the disease. To perform a cross sectional comparison of ECG abnormalities throughout different left ventricular hypertrophy (LVH) severity subgroups, providing ECG patterns specific of the progressive AFD stages. 189 AFD patients from a multicenter cohort underwent comprehensive ECG analysis, echocardiography, and clinical evaluation. The study cohort (39% males, median age 47 years, 68% classical AFD) was divided into 4 groups according to different degree of left ventricular (LV) thickness: group A ≤ 9 mm (n = 52, 28%); group B 10-14 mm (n = 76, 40%); group C 15-19 mm (n = 46, 24%); group D ≥ 20 mm (n = 15, 8%). The most frequent conduction delay was right bundle branch block (RBBB), incomplete in groups B and C (20%,22%) and complete RBBB in group D (54%, p < 0.001); none of the patients had left bundle branch block (LBBB). Left anterior fascicular block, LVH criteria, negative T waves, ST depression were more common in the advanced stages of the disease (p < 0.001). Summarizing our results, we suggested ECG patterns representative of the different AFD stages as assessed by the increases in LV thickness over time (Central Figure). Patients from group A showed mostly a normal ECG (77%) or minor anomalies like LVH criteria (8%) and delta wave/slurred QR onset + borderline PR (8%). Differently, patients from groups B and C exhibited more heterogeneous ECG patterns: LVH (17%; 7% respectively); LVH + LV strain (9%; 17%); incomplete RBBB + repolarization abnormalities (8%; 9%), more frequently associated with LVH criteria in group C than B (8%; 15%). Finally, patients from group D showed very peculiar ECG patterns, represented by complete RBBB + LVH and repolarization abnormalities (40%), sometimes associated with QRS fragmentation (13%). ECG is a sensitive tool for early identification and long-term monitoring of cardiac involvement in patients with AFD, providing "instantaneous pictures" along the natural history of AFD. Whether ECG changes may be associated with clinical events remains to be determined.

Myocardial strain analysis by cardiac magnetic resonance 3D feature-tracking identifies subclinical abnormalities in patients with neuromuscular disease and no overt cardiac involvement.

Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich's ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne's muscular dystrophy (BMD/DMD, n = 15), Duchenne's carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.

Prevalence and factors associated with impaired left ventricular global longitudinal strain in patients with Chagas disease: SaMi-Trop cohort study.

Cardiomyopathy is a major cause of death in Chagas disease and early detection of cardiac involvement is essential. Myocardial strain is a reliable technique for assessment of subtle left ventricular (LV) contractility alterations. This study assessed LV global longitudinal strain (GLS) in a large Chagas disease population living in remote areas. Between 2015 and 2016, Chagas disease patients were selected in the northern of the Minas Gerais state. All patients underwent T. cruzi antibodies tests and those who had positive tests were included. A resting 12-lead ECG was recorded and classified using the Minnesota Code criteria. Echocardiography was performed at public health primary care units and speckle-tracking strain was analyzed offline. LV dysfunction was defined as ejection fraction (LVEF < 50%) and reduced GLS was defined as < 16% (absolute value). A total of 1387 patients were included, mean age of 60.0 ± 12.5years, 68% were women, and 14% had LV dysfunction. Among patients with normal LVEF, 59% had impaired LV GLS. Overall, patients with impaired GLS were older, had more comorbidities and ECG abnormalities than those with normal GLS. The independent factors associated with reduced GLS were ST-T abnormalities (OR 1.954; 95% CI 1.027-3.718), QRS duration (OR 1.009; 95% CI 1.002-1.016), LVEF (OR 0.947; 95% CI 0.923-0.972), and E/e' ratio (OR 1.059; 95% CI 1.009-1.112). In a cohort of Chagas disease from endemic areas, impaired LV GLS was detected in a significant proportion of patients, despite normal ECG and preserved LVEF. The main determinants of reduced LV GLS were ST-T abnormalities, QRS duration, LVEF and E/e' ratio, adjusting for demographical and clinical data.

Early detection of cardiac involvement of desminopathy by cardiovascular magnetic resonance.

Early detection of cardiac involvement of desminopathy by cardiovascular magnetic resonance.

Cardiac Magnetic Resonance Features of Fabry Disease: From Early Diagnosis to Prognostic Stratification

In the past few years, the wide application of cardiac magnetic resonance (CMR) significantly changed the approach to the study of cardiac involvement in Fabry Disease (FD). The possibility to perform non-invasive tissue characterization, including new sequences such as T1/T2 mapping, offered a powerful tool for differential diagnosis with other forms of left ventricular hypertrophy. In patients with confirmed diagnosis of FD, CMR is the most sensitive non-invasive technique for early detection of cardiac involvement and it provides new insight into the evolution of cardiac damage, including gender-specific features. Finally, CMR multiparametric detection of subtle changes in cardiac morphology, function and tissue composition is potentially useful for monitoring the efficacy of specific treatment over time. This paper aims to provide a comprehensive review of current knowledge regarding the application of CMR in FD cardiac involvement and its clinical implication.

Role of endothelial cell specific molecule-1 (endocan) and cardiac imaging in early detection of cardiac involvement in psoriatic patients with or without arthritis

Aim of the workMeasuring serum endocan level to determine its potential role in detecting subclinical cardiovascular involvement in psoriatic patients with or without arthritis. Patients and methodsThis work included 14 psoriatic arthritis (PsA) patients, 14 psoriasis only (PsO) patients, and 14 age and sex matched controls. The psoriasis area severity index was evaluated. Serum endocan level was measured, subclinical atherosclerosis was assessed using brachial artery flow-mediated vasodilation (FMD), and echocardiography: standard and tissue Doppler imaging (TDI) was performed. ResultsThe mean age of PsA patients was 38.3 ± 9.9 years and for PsO was 37.9 ± 8 years. They were 3 males and 11 females in both groups with a comparable psoriasis duration (11 ± 4.9 vs 8 ± 6.3 years; p = 0.17). PsA patients had significantly increased endocan level (618 ± 227.8 ng/L) compared to those with PsO or controls (359 ± 185.7 and 130.6 ± 38.2 ng/L respectively; p < 0.001). 4 (28.6%) PsA patients, 1 (7.1%) PsO patient and none of the controls had FMD abnormality. TDI revealed early diastolic mitral annular motion velocity (E') abnormality in 5 (35.7%) PsA patients. In PsA patients, endocan level was significantly elevated in patients with FMD or E' abnormality compared to those without (p = 0.01 and p = 0.001, respectively). Serum endocan significantly negatively correlated with FMD and E' in psoriatic patients. Serum endocan significantly detected FMD and E' abnormalities in psoriatic patients (p = 0.002 and p = 0.001, respectively). ConclusionSubclinical cardiovascular involvement was evident among psoriatic patients, particularly those with arthritis. Serum endocan is a promising endothelial biomarker for detecting subclinical atherosclerosis and preclinical cardiac dysfunction in psoriatic patients.

Detection of cardiac involvement in pulmonary sarcoidosis using high-resolution Holter electrocardiogram.

BackgroundEarly detection of cardiac involvement in patients with sarcoidosis is important but currently unresolved. The aim of this study was to elucidate the utility of frequency domain microvolt T‐wave alternans (TWA), signal‐averaged ECG (SAECG), and heart rate turbulence (HRT) using 24‐hour Holter ECG for detecting cardiac involvement in patients with pulmonary sarcoidosis.MethodsThis study consisted of consecutive 40 pulmonary sarcoidosis patients (11 males, 62 ± 13 years) who underwent 24‐hour Holter monitoring with and without cardiac involvement. All patients underwent frequency domain TWA, SAECG, and HRT using 24‐hour Holter monitoring. Patients with atrial fibrillation pacing or wide QRS electrocardiogram were excluded.ResultsAfter 14 patients were excluded, a total of 26 patients (six males, 59 ± 14 years) were evaluated. Seven patients had cardiac involvement (cardiac sarcoidosis [CS] group). On the Holter SAECG, duration of low‐amplitude signals <40 μV in the terminal filtered QRS complex (LAS40) was significantly higher, and root mean square voltage of the terminal 40 ms of the filtered QRS complex (RMS40) was significantly lower in the CS group compared with the non‐CS group (LAS40: 61.4 ± 35.9 vs 37.6 ± 9.2 ms; P = .018, RMS40: 11.4 ± 10.3 vs 23.6 ± 13.2 ms; P = .023). Prevalence of positive late potential (LP) was also significantly higher in the CS group than that in the non‐CS group (85.7% vs 31.5%; P = .026). The sensitivity, specificity, positive, and negative predictive values of LP for identifying patients with cardiac involvement were 85.7%, 68.4%, 50.0%, and 92.8%, respectively.ConclusionHolter SAECG may be useful for detecting cardiac involvement in patients with pulmonary sarcoidosis.

Cardiac Magnetic Resonance in the Assessment of Chagas Disease and its Complications

Abstract The well-known occurrence of Chagas disease in endemic areas has become a worldwide problem, and cardiac magnetic resonance allows the early detection of cardiac involvement and complications of this disease. Cardiac magnetic resonance is a useful tool in all phases of Chagas disease, and new promising techniques using T1 mapping and extracellular volume measurements are able to detect cardiac involvement even earlier than conventional techniques. Cardiac Magnetic Resonance in the Assessment of Chagas Disease and its Complications

The prevalence and natural progress of myocardial fibrosis in Duchenne muscular dystrophy patients

Abstract Purpose The cardiac disease is now as the leading cause of morbidity and mortality in DMD patients. The early detection of cardiac involvement is challenging and the frequency and presence of the cardiac involvement in the first decade is uncertain. The aim of the current study was to characterize the frequency, pattern and extent of LGE in the first decade in DMD patients, and identify a set of prognosis factors that portend the presence and extent of LGE in those patients. Methods There are 93 DMD patients (mean age: 8.31±2.30 years, rang: 3–13 years) with a biopsy showing absent dystrophin and/or DNA analysis demonstrating a characteristic dystrophin mutation enrolled. All patients underwent a complete cine imaging and LGE imaging. The left ventricular function and tissue characteristic were analyzed and statistic. Results There were 42% patients presented LGE-positive. Six years old was the youngest age onset myocardial fibrosis, and after 7 years old, the frequency of LGE positive rising fast. Moreover, all LGE positive patients showed complex patterns with non-ischaemic pattern, including septum, papillary muscle and right ventricular involvement. The free wall, inferior, apical and septum were the most common position. The papillary and right ventricular were rarely common. And all patients presented right ventricular involvement associated with septum involvement. Moreover, compared with younger patients (&amp;lt;7 years), the presence and extent of LGE in older patients (≥7 years) were significant higher. And the logistic analysis showed that the age is a strong predictor of presence of LGE (OR: 1.61, 95% CI: 1.26–2.05, P&amp;lt;0.000). Conclusion The myocardial fibrosis could onset in DMD patients in the first decade with a rapid rising and presented with complex patterns. The early CMR examination is necessary for the assessment of cardiac involvement and should consider to complete in younger age in DMD patients. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): The National Natural Science Foundation

Progressive electrocardiographic changes in parallel with cardiac magnetic resonance findings in fabry disease

Abstract Background Cardiac Magnetic Resonance (CMR) allows to detect progressive stages of cardiac involvement in Fabry Disease (FD). A systematic description of electrocardiographic (ECG) alterations occurring in FD is currently missing. Purpose To explore ECG changes in progressive stages of FD cardiomyopathy. Methods 71 FD patients and 17 healthy controls underwent CMR with T1 mapping and 12-lead ECG. ECG analysis included the duration of the P-wave and the interval between the end of P-wave and the beginning of QRS (PendQ). FD patients in the test cohort were divided into 3 groups with increasing severity of cardiac involvement: A) normal T1, no LVH; B) low T1, no LVH; C) low T1, LVH. Results An increase of Pwave/PendQ ratio was observed in Group A compared to Controls (1.08 vs. 0.75, p&amp;lt;0.0001). Higher Pwave/PendQ ratio (1.50 vs. 1.08, p&amp;lt;0.0001), shorter PQc interval (127.9 vs. 159.5, p=0.0007), increased Sokolow-Lyon Index (SLI) (3.2 vs. 2.4, p&amp;lt;0,001) and T wave amplitude (0.6 vs. 0.4 mV, p=0.002) characterized Group B in comparison with Group A. A higher prevalence of left bundle branch blocks (13.6% vs. 0%, p=0.03) and repolarization abnormalities (77.3% vs. 5.7%), wider QRS (120 vs 95 msec, p&amp;lt;0.0001) and QT (460 vs 400 msec, p=0.003) intervals were found in Group C compared to Group B. SLI (AUC 0.769), Pwave/PendQ (AUC 0.778), QRS (AUC 0.703) and QT (AUC 0.769) durations resulted to be independent predictors of low T1 values on CMR at stepwise multivariate analysis. Conclusion FD is characterized by progressive ECG changes. The identification of ECG parameters able to predict a lowering of myocardial T1 values on CMR may promote early detection of cardiac involvement, helping to target the therapeutic approach. Progressive ECG and CMR changes in FD Funding Acknowledgement Type of funding source: Other. Main funding source(s): This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.

Fragmented QRS complexes are associated with subclinical left ventricular dysfunction in patients with Behcet's disease: Four-dimensional speckle tracking echocardiography.

Behcet's disease (BD) is a chronic systemic inflammatory disease in which early detection of cardiac involvement is essential. The aim of this study was to assess the left ventricular (LV) functions in BD patients using four-dimensional (4D) speckle tracking echocardiography (STE) and to test the correlation between LV dysfunction and the presence of QRS fragmentation. This cross-sectional study included 64 Behcet's patients and 48 healthy volunteers. The BD group was divided into two subgroups depending on the presence (fQRS+) or absence (fQRS-) of fragmented QRS (fQRS). In both groups, left ventricular global area strain (LV-GAS), global radial strain (GRS), global longitudinal strain (GLS), and global circumferential strain (GCS) were obtained with 4D echocardiography. GAS, GRS, GLS, and GCS values were significantly different in Behcet's patients and in healthy volunteers. GLS and GAS values were lower in the fQRS+ than in the fQRS- group (-15.8 ± 1.8 and -17.9 ± 1.6, P = .001 vs -25.0 ± 3.1 and -29.2 ± 4.2, P < .001, respectively). The duration of disease was longer in fQRS+ than in fQRS- patients (120.8 ± 67.4 vs 71.0 ± 40.5, P < .001). Multiple linear regression analysis showed that fQRS and disease duration were independent predictors of LV-GAS. Four-dimensional STE may be helpful for the prediction of early cardiac dysfunction in patients with BD. The presence of fQRS may be an indicator of subclinical LV dysfunction.

Early detection of ventricular dysfunction by tissue Doppler echocardiography related to cardiac iron overload in patients with thalassemia.

Cardiac T2* MRI is used as a gold standard for cardiac iron quantification in patients with transfusion-dependent thalassemia (TDT). We hypothesized that left ventricular (LV) diastolic dysfunction would reflect the severity of iron overload and can serve as an early detection of cardiac iron deposits. A study was conducted on all patients with TDT. Hemoglobin, serum ferritin and non-transferrin bound iron, together with a complete echocardiography and cardiac T2* MRI, were performed on all patients. Seventy-seven patients with TDT were enrolled (median age 14years). In the patient group with a mean serum ferritin of > 2500ng/mL during the past 12 months, there were more patients with severe cardiac iron deposits than in the group with a mean serum ferritin of ≤ 2500ng/mL. Diastolic dysfunction was absent in all patients with a serum ferritin of < 1000ng/mL. All patients with cardiac T2* ≤ 20ms had grade III LV diastolic dysfunction. However, twenty-one percent of patients with cardiac T2* >20ms had LV diastolic dysfunction. The differences observed in pulmonary vein atrial reversal duration and mitral A-wave (PVAR-MVA) duration ≥ - 1ms and an E/E' ratio ≥ 11 were proven to be the associated factors with the cardiac T2* ≤ 20ms. Increased PVAR-MVA duration and increased E/E'ratio reliably reflected a severe iron overload, according to a cardiac T2* in patients with TDT. LV diastolic dysfunction can occur prior to severe cardiac iron deposition. Tissue Doppler echocardiography has the potential for the early detection of cardiac involvement in patients with TDT .

Cardiac Magnetic Resonance in Chagas Disease—an Update

The purpose of this paper is to review the current role of cardiac magnetic resonance (CMR) in Chagas disease. CMR is crucial to detect the cardiac involvement in patients with Chagas disease given its ability to assess ventricular function and allow non-invasive tissue characterization. Chagas disease is an emerging health problem in non-endemic areas because of growing population movements. Among other imaging modalities, CMR aids in the early detection of cardiac involvement in Chagas disease, allows stage patients in the course of the cardiomyopathy, stratify patients’ prognosis, and subsequently guides management. In addition to its superior ability to assess global and regional biventricular function, CMR allows the detection and quantification of edema and myocardial fibrosis. In addition to conventional tissue characterization sequences, novel quantitative mapping techniques have emerged and are robust parameters in other inflammatory conditions. Despite its promising role to detect and graduate the inflammatory stage of the disease, no specific data in Chagas heart disease is available yet.

Early detection of cardiac involvement in hemodialysis patients

Cardiac involvement in hemodialysis patients is a serious condition with poor prognosis. The impact of chronic kidney disease and hemodialysis on heart function is not well understood. The transthoracic echocardiography (TTE) is performed when cardiac involvement is suspected. Recently, the development of (2D) strain imaging facilitated the simple and independent measurement of left ventricular (LV) deformation parameters in the longitudinal, circumferential and radial directions. To analyse LV systolic function in hemodialysis patients in two-dimensional (2D) TTE and with 2D speckle tracking (2D-STI) Twenty-eight chronic hemodialysis patients were included prospectively from November 2017 to December 2017 at the Nephrology department. Twenty-one patients comprised the control group. Conventional 2D-TTE was performed followed by two-dimensional strain imaging (2D-STI). A complete TTE was conducted, including LVEF by Simpson biplane method, calculation of indexed left ventricular mass, analysis of Diastolic function with evaluation of filling pressures and finally the study of the longitudinal deformation of the LV (GLS) by the speckle tracking technique. The average age of patients in our sample was 49.7 ± 9.7 years with a sex ratio of 2,5. All patients were dialyzed 3 times a week with a mean duration of hemodiaylyse of 60 months. All patients had a conserved LVEF with a mean value of 60.3 ± 7%. The prevalence of LVH was 60.7%. Segmental kinetic disorders were found in 3 patients. The average value of GLS was −14.9 ± 3.2, which was impaired compared to that of the control group. GLS is decreased in 40% of the population. The evaluation of longitudinal function by 2D strain gives a new insight into myocardial function in hemodialysis patients, which could improve pathophysiological understanding and identify patients at high risk of heart failure who can benefit from preventive strategies.

Cardiac Biomarkers for Early Detection of Cardiac Involvement in Children with Kawasaki Disease: A Cross-Sectional Study

Background: Kawasaki disease (KD) is one of the most prevalent vasculitis diseases in children and can bring about serious cardiovascular complications. Early detection of cardiac involvement in KD can play an essential role in managing and preventing the cardiac sequels. We aimed to evaluate the applicability and diagnostic accuracy of cardiac biomarkers including cardiac troponin I (cTnI), and N-terminal pro-brain natriuretic (NT-proBNP) for early detection of cardiac involvement in children with KD. Materials and Methods In this cross sectional study, 32 children with KD who were admitted to the Children's Hospital of Tabriz University of Medical Sciences, Tabriz, Iran, in a three-year period, were consecutively included. Transthoracic echocardiography was performed to evaluate cardiac involvement. Also, the serum levels of NT-proBNP and cTnI were measured to evaluate their diagnostic accuracy. ROC curve analysis was conducted to evaluate the discriminatory power of NT-ProBNP for the diagnosis of cardiac involvement in KD and to determine the best cut-off point at which the sensitivity and specificity were optimal. Results: Of 32 enrolled patients, 4 (12.5%) had cardiac involvement including 3 patients with perivascular brightness of coronary arteries and one patient with small aneurysm of the coronary arteries. In all study patients, the cTnI levels were lower than 0.35 ng/ml and the NT-proBNP mesurment revealed an average of 678.5 pg/ml. Children with cardiac involvement had significantly higher NT-proBNP (p= 0.001). Both sensitivity and specificity of NT-proBNP at the optimum cut-off point of 1354 pg/ml were 100 percent (AUC=1.000, p=0.001). Conclusion: Unlike the cTnI, our results support the applicability of NT-proBNP as an excellent objective test for early detection of cardiac involvement in children with KD.

The consequence of the first attack of acute rheumatic fever with subclinical carditis

Background: Immune mediated damage of RF occurs most prominently in the heart, joints, brain, skin and subcutaneous tissues, however RF leaves no lasting damage to the brain, joint or skin, it is the leading cause of acquired heart disease in children and adolescents worldwide, therefore carditis is the most important manifestation of RF. Echocardiography along with color; Doppler imaging is now worldwide used for early detection of cardiac involvement even in the absence of clinical evidence, which in this case is called subclinical carditis (SCC) and was reported to cause similar consequences to clinically evident carditis.Methods: A retrospective study carried out on 35 patients already diagnosed to have RF and subclinical RHD and attending the cardiology clinic at Alexandria University Children’s Hospital (AUCH) from December 2015 to November 2016. Data collected from patients' files and then appropriately updated during subsequent follow-up visits.Results: 35 children already diagnosed to have RF and subclinical RHD were included in the current study, 22 patients (66.6%) improved completely with no residual cardiac lesion, 10 patients (30.3%) had persistent lesion and one patient (3.1%) had worsened condition.Conclusions: The most important predictors of poor prognosis according to the current study were recurrence and lack of regular compliance to long acting penicillin (LAP). Furthermore the outcome was not influenced by the treatment given in the initial attack.

Nonlinear characterization of ECGs in patients with Chagas’ disease**Partially supported by Consejo Nacional de Investigaciones Científicas y Tecnológicas, CONICIT under the grant G-97000675 and Vice-Rectorado de Investigación, Universidad Nacional San Agustin de Arequipa-Perú grant contract 15-2017-UNSA.

According to the World-Wide Organization of the Health, the number of people infected with the Tripanosoma Cruzi is considered between 6 and 8 million, causal agent of the Chagas’ disease , and in 550000 the people are expected to be exposed to the affectation risk. When concluding in 1983 a longitudinal epidemiologist study in patients with the disease evaluated every 3 years, the cardiac affectation: chronic Chagasic myocarditis (MCHC) increased from a 17% at the beginning of the study to a 49, 4% after 15 years. Previous studies of the variability of cardiac frequency (HRV) in patients with the Chagas’ disease, show alterations in the spectral indices of the HRV. A nonlinear modeling technique allows the definition of early risk markers in patients with Chagas’s disease and without any evidence of cardiac involvement evaluated by standard diagnostic test (CH1). We analyze ECGs by Holter recordings in patients with ECG alterations (CH2), patients without ECG alterations (CH1) who had positive serological findings for Chagas’ disease and healthy (Control) matched for sex and age. Two ECGs were digitized for each volunteer, one begins at supine position and the other at orthostatic position. The technique applied is based upon quantitative comparison of the evolution from similar ECGs Sub segments, and a measure of the predictability decay rate were calculated for each register. We did find significant differences among Control and CH2 during supine and also standing with high sensibility and specificity in the early detection of cardiac involvement. It is suggested that these alterations are very early autonomic disturbances in patients without ECG alterations (CH1) that could be useful to enhance risk stratification.

Regional amyloid distribution and impact on mortality in light-chain amyloidosis: a T1 mapping cardiac magnetic resonance study

Background: T1 mapping allows quantitative assessment of “diffuse” deposition of amyloid protein in the myocardium. Early detection of cardiac involvement and potential prognostic improvement could benefit patients with AL amyloidosis.Objectives: This study aims to evaluate the regional variation of amyloid infiltration in the left ventricle and the prognostic value of T1 mapping in patients with AL amyloidosis.Methods: We prospectively enrolled 77 patients with AL amyloidosis who underwent cardiac magnetic resonance on a 3.0-T scanner. Native T1 and extracellular volume (ECV) were quantitated on the basal, mid, and apical levels of the left ventricle. Late gadolinium enhancement (LGE) pattern (no or non-specific LGE, sub-endocardial LGE, and transmural LGE) was also assessed. Forty healthy subjects served as controls. The primary end point was all-cause mortality.Results: Basal ECV (26.9 ± 2.8% versus 31.1 ± 4.9%, p < .001) were lower than apical ECV in the healthy controls; however, basal ECV (60.6 ± 11.5% versus 53.0 ± 9.6%, p = .003) were significantly higher than apical ECV in patients with transmural LGE. During the follow-up period (median duration, 28 months; 25th–75th percentile, 13.5–38.0 months), 46 patients died. Basal ECV has the largest area under the curve of 0.845 (95% CI, 0.747–0.917) to predict all-cause mortality. Multivariable Cox analysis indicated that basal ECV was an independent prognostic factor and showed incremental prognostic value beyond NYHA class, Mayo stage, and LGE pattern.Conclusion: We demonstrated that T1 mapping may have the potential to detect a characteristic amyloid deposition with a decreasing gradient from base to apex. Furthermore, myocardial ECV indicated that basal amyloid infiltration provided robust and incremental prognostic value in patients with AL amyloidosis.