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Cardiac Involvement (cardiac + involvement)
Kinds of Cardiac Involvement Selected AbstractsDetection of Subclinical Cardiac Involvement in Systemic Sclerosis by Echocardiographic Strain ImagingECHOCARDIOGRAPHY, Issue 2 2008Alper Kepez M.D. Background: Cardiac involvement is one of the major problems in systemic sclerosis (SSc). Subclinical cardiac involvement has a higher frequency than thought previously. In this study we investigated whether subclinical cardiac involvement can be detected by using echocardiographic strain imaging in SSc patients without pulmonary hypertension. Methods: Echocardiographic examinations were performed to 27 SSc patients and 26 healthy controls. Left ventricular strain parameters were obtained from apical views and average strain value was calculated from these measurements. Results: There were no significant differences between patients and controls regarding two-dimensional (2D), conventional Doppler and tissue Doppler velocity measurements. Strain was reduced in 6 of 12 segments of the left ventricle (LV) and in 1 of 2 segments of the right ventricle (RV). Strain rate (SR) was reduced in 2 of 12 segments of the LV and 1 of 2 segments of the RV in SSc patients as compared to controls (P < 0.05 for all). These involvements did not match any particular coronary artery distribution. More important differences were detected by average strain and SR values of the LV between patients and controls (19.78 ± 3.00% vs 23.41 ± 2.73%, P < 0.001; 2.01 ± 0.41 vs 2.23 ± 0.27/sec, P = 0.026, respectively). Furthermore, carbon monoxide diffusion capacity (DLCO) in scleroderma patients significantly correlated with LV average strain (r = 0.59; P = 0.001). Conclusion: Evaluation of ventricular function by using echocardiographic strain imaging appears to be useful to detect subclinical cardiac involvement in SSc patients with normal standard echocardiographic and tissue Doppler velocity findings. [source] Evaluation of Cardiac Involvement in Hypereosinophilic Syndrome: Complementary Roles of Transthoracic, Transesophageal, and Contrast EchocardiographyECHOCARDIOGRAPHY, Issue 8 2006Rajesh Shah M.D. Hypereosinophilic syndrome is a rare but important systemic disease with multiple clinical presentations. Approximately 40% of these cases have cardiac involvement. Echocardiography is the most easily available and versatile imaging modality in assessing cardiac involvement in this disease process. As described and reviewed in this case, it may be the first imaging modality to raise suspicion of this disease entity. Hence, clinicians interpreting echocardiograms and caring for patients need to be aware of the manifestations and complementary roles of various echo techniques in delineating cardiac involvement. Furthermore, the importance of a thorough history and laboratory review prior to echocardiography may provide valuable clues which may otherwise be missed. [source] Scleredema of Buschke in a Child with Cardiac InvolvementPEDIATRIC DERMATOLOGY, Issue 3 2010Adriana Isaac M.D. We report a case of an 11-year-old boy with diffuse nonpitting edema of the trunk and face associated with cardiac involvement. [source] Hypereosinophilic Syndrome Presenting with Biventricular Cardiac ThrombiECHOCARDIOGRAPHY, Issue 6 2010Ankur Lodha M.D. Hypereosinophilic syndrome is a rare condition characterized by idiopathic eosinophilia with organ system involvement. Cardiac involvement portends a less favorable prognosis as it can be complicated by development of heart failure, valvular dysfunction, and restrictive cardiomyopathy. We present a rare case of hypereosinophilic syndrome with FIP1L1/PDGFRA fusion in a 50-year-old male associated with thrombus in left and right ventricle. (Echocardiography 2010;27:E57-E59) [source] Detection of Subclinical Cardiac Involvement in Systemic Sclerosis by Echocardiographic Strain ImagingECHOCARDIOGRAPHY, Issue 2 2008Alper Kepez M.D. Background: Cardiac involvement is one of the major problems in systemic sclerosis (SSc). Subclinical cardiac involvement has a higher frequency than thought previously. In this study we investigated whether subclinical cardiac involvement can be detected by using echocardiographic strain imaging in SSc patients without pulmonary hypertension. Methods: Echocardiographic examinations were performed to 27 SSc patients and 26 healthy controls. Left ventricular strain parameters were obtained from apical views and average strain value was calculated from these measurements. Results: There were no significant differences between patients and controls regarding two-dimensional (2D), conventional Doppler and tissue Doppler velocity measurements. Strain was reduced in 6 of 12 segments of the left ventricle (LV) and in 1 of 2 segments of the right ventricle (RV). Strain rate (SR) was reduced in 2 of 12 segments of the LV and 1 of 2 segments of the RV in SSc patients as compared to controls (P < 0.05 for all). These involvements did not match any particular coronary artery distribution. More important differences were detected by average strain and SR values of the LV between patients and controls (19.78 ± 3.00% vs 23.41 ± 2.73%, P < 0.001; 2.01 ± 0.41 vs 2.23 ± 0.27/sec, P = 0.026, respectively). Furthermore, carbon monoxide diffusion capacity (DLCO) in scleroderma patients significantly correlated with LV average strain (r = 0.59; P = 0.001). Conclusion: Evaluation of ventricular function by using echocardiographic strain imaging appears to be useful to detect subclinical cardiac involvement in SSc patients with normal standard echocardiographic and tissue Doppler velocity findings. [source] Necrotizing Vasculitis: A Cause of Aortic Insufficiency and Conduction System DisturbanceECHOCARDIOGRAPHY, Issue 7 2003Miquel Gómez Pérez M.D. Cardiac involvement in vasculitis syndromes is uncommon. We describe a 50-year-old male who presented with progressive dyspnea and myalgies. Echocardiogram revealed significant thickening of aortic root, aortic cusps, and anterior mitral valve leaflet, with severe aortic regurgitation that required aortic valve replacement. Furthermore, this patient suffered progressive atrioventricular block that needed implantation of a pacemaker. The study performed disclosed the presence of necrotizing vasculitis positive for perinuclear antineutrophil cytoplasmic antibody. (ECHOCARDIOGRAPHY, Volume 20, October 2003) [source] Cardiac magnetic resonance imaging in the evaluation of cardiac sarcoidosis: an Australian single-centre experienceINTERNAL MEDICINE JOURNAL, Issue 2 2009V. Manins Abstract Background:, Cardiac involvement in systemic sarcoidosis is common; however, current diagnostic tools are imprecise. Recognition of cardiac sarcoidosis (CS) is important as it has a relatively poor prognosis. Gadolinium-enhanced cardiac magnetic resonance imaging (Gad-CMR) is emerging as an excellent technique in determining the presence of and extent to which cardiac muscle is affected by sarcoidosis. Methods:, A retrospective analysis was performed on all patients with biopsy-proven systemic sarcoidosis referred for Gad-CMR scanning to evaluate potential cardiac involvement. All patients also underwent an electrocardiogram, Holter monitor and echocardiography. Gallium-67 radionuclide investigation, positron emission tomography and cardiac biopsy were ordered at the discretion of the treating physician. Results:, Eleven of the 20 patients had Gad-CMR images supportive of the diagnosis of CS. Eight of these 11 patients met the Japanese Ministry of Health and Welfare (JMHW) criteria for the diagnosis of CS; three abnormal Gad-CME scans consistent with diagnosis of CS were seen in patients who did not meet JMHW criteria. No patients with normal Gad-CMR scan met JMHW criteria for CS. Conclusion:, These findings suggest that Gad-CMR is potentially superior to the JMHW criteria in the diagnosis of cardiac sarcoidosis. [source] Cardiovascular magnetic resonance reveals similar damage to the heart of patients with becker and limb-girdle muscular dystrophy but no cardiac symptomsJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 4 2009Ali Yilmaz MD Abstract Cardiac involvement in patients with a sarcoglycanopathy (limb-girdle muscular dystrophy) has been described previously; however, this is the first cardiovascular magnetic resonance (CMR) study in such a patient demonstrating an interesting pattern of myocardial damage using late gadolinium enhancement (LGE) imaging. Moreover, the wall motion abnormality and the subepicardial pattern of LGE in this patient with a sarcoglycanopathy is in agreement with the findings in another patient with Becker muscular dystrophy. The predominance of LGE in the subepicardial layers of the left ventricular inferolateral wall suggests that such a myocardial damage pattern represents a nonspecific cardiac phenotype in response to exaggerated mechanical stress in this region, at least in patients with a sarcoglycanopathy or dystrophinopathy. J. Magn. Reson. Imaging 2009;30:876,877. © 2009 Wiley-Liss, Inc. [source] Cardiac involvement in infantile Sandhoff diseaseJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 1 2002P Venugopalan Abstract: An 18-month-old boy with enzyme assay-confirmed infantile Sandhoff disease (MIM 268800) is reported. Besides the classical neurological features, this patient exhibited severe mitral regurgitation secondary to mitral valve prolapse and mild aortic regurgitation from aortic valve prolapse. He also had asymmetric hypertrophy of the interventricular septum without left ventricular outflow tract obstruction. [source] Cardiac involvement in Churg-Strauss syndromeARTHRITIS & RHEUMATISM, Issue 2 2010Robert M. Dennert Objective Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients. Methods Thirty-two consecutive patients with CSS in remission (mean ± SD duration of disease between diagnosis and enrollment 6.1 ± 5.8 years, mean ± SD age 61 ± 10 years) who were previously unaware of cardiac involvement were compared with 32 randomly selected age- and sex-matched control subjects, using clinical evaluation, electrocardiography (EKG), echocardiography, and cardiac magnetic resonance imaging (MRI). Results Detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in CSS patients compared with 3% in controls (P < 0.001), with clinical symptoms in 26% and 3%, respectively (P = 0.009), EKG abnormalities in 66% and 3%, respectively (P < 0.001), and echocardiographic defects in 50% and 3%, respectively (P < 0.001). Cardiac MRI detected cardiac manifestations in 62% of CSS patients. In the presence of cardiac MRI abnormalities, echocardiography could detect cardiac involvement with a sensitivity of 83% and a specificity of 80%. The absence of symptoms or EKG abnormalities did not exclude cardiac involvement, because abnormalities could still be detected in 38% of these patients at the time of echocardiography or cardiac MRI. Conclusion These results demonstrate a high incidence of cardiac involvement in CSS patients. Systematic cardiac evaluation including detailed imaging is required to properly identify CSS patients with cardiac involvement. [source] Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: A controlled study of 100 consecutive patientsARTHRITIS & RHEUMATISM, Issue 6 2008Christophe Meune Objective To assess the prevalence of primary cardiac complications in a large population of patients with systemic sclerosis (SSc), using recently developed echocardiographic techniques. Methods We prospectively studied 100 consecutive patients (mean ± SD age 54 ± 14 years; 86 women) presenting with SSc without pulmonary arterial hypertension or clinical manifestations of heart failure. All patients underwent standard echocardiography, along with measurements of longitudinal velocities by tissue Doppler imaging (TDI) to assess left ventricular (LV) and right ventricular (RV) contractility and LV diastolic function. Results were compared with those in 26 age- and sex-matched healthy controls. Results Patients with SSc had a wider mean left atrial diameter and impaired relaxation compared with the controls. A trend was observed toward a smaller LV ejection fraction (EF) in the patients (mean ± SD 64.9 ± 0.6%) than in the controls (67.2 ± 0.7%), as well as higher pulmonary artery pressure (mean ± SD 33.3 ± 0.6 mm Hg versus 30.8 ± 1.0 mm Hg). LVEF was <55% in 7 patients versus none of the controls. Peak systolic mitral annular velocity as measured by TDI was <7.5 cm/second in 14 patients versus none of the controls (P = 0.040). Mitral annulus early diastolic velocity was <10 cm/second in 30 patients versus 2 of the controls (P = 0.022). Fifteen patients and none of the controls had reduced peak systolic tricuspid annular velocity (P = 0.039). The TDI results correlated with each other, but not with lung abnormalities or other disease characteristics. Conclusion Depression of LV and RV systolic and LV diastolic function is common in patients with SSc and is due to primary myocardial involvement. Considering the major contributions of TDI, the addition of this simple technique to standard measurements may improve the detection of heart involvement in patients with SSc. [source] Evaluation of Biventricular Functions With Tissue Doppler Imaging in Patients With Myotonic DystrophyCLINICAL CARDIOLOGY, Issue 3 2010Tolga Ozyigit MD Background: Myotonic dystrophy (MD) is characterized by myotonia with dystrophic involvement of the muscles. Cardiac involvement is usually not evident in the early stages of MD. Hypothesis: We investigated biventricular functions by tissue Doppler imaging (TDI) in MD patients with no overt cardiac involvement to explore the value of TDI in the early detection of myocardial dysfunction. Methods: A total of 21 MD patients (15 male, age: 32.2 ± 12.3 yrs) and 21 healthy controls (13 male, age: 32.2 ± 7.8 yrs) were included. In addition to conventional echocardiography, pulsed Doppler and TDI were performed including measurement of myocardial performance index (MPI); peak systolic (Sm) and early (Em) and atrial (Am) diastolic myocardial velocities at the basal mitral and tricuspid annulus. Results: All patients and controls had normal ejection fraction. Transmitral E peak velocity was significantly lower while both deceleration time of E velocity and isovolumic relaxation time were significantly longer in MD patients (P = 0.007, P = 0.001, and P < 0.001, respectively). Sm, Em and Am peak velocities were significantly lower in MD patients in all segments except for Em of the mitral anterior annulus and Am of the tricuspid lateral annulus. Both left and right ventricular MPI were significantly higher in MD patients (P < 0.001 and P = 0.013, respectively). Conclusion: There are changes in myocardial systolic and diastolic functions in MD patients although they have no overt heart failure. Myocardial tissue velocities and MPI are useful in identifying subclinical biventricular involvement in these patients. Copyright © 2010 Wiley Periodicals, Inc. [source] Cardiac involvement in lightning strike injuryCLINICAL CARDIOLOGY, Issue 5 2000Johann Auer M.D. No abstract is available for this article. [source] Detection of Subclinical Cardiac Involvement in Systemic Sclerosis by Echocardiographic Strain ImagingECHOCARDIOGRAPHY, Issue 2 2008Alper Kepez M.D. Background: Cardiac involvement is one of the major problems in systemic sclerosis (SSc). Subclinical cardiac involvement has a higher frequency than thought previously. In this study we investigated whether subclinical cardiac involvement can be detected by using echocardiographic strain imaging in SSc patients without pulmonary hypertension. Methods: Echocardiographic examinations were performed to 27 SSc patients and 26 healthy controls. Left ventricular strain parameters were obtained from apical views and average strain value was calculated from these measurements. Results: There were no significant differences between patients and controls regarding two-dimensional (2D), conventional Doppler and tissue Doppler velocity measurements. Strain was reduced in 6 of 12 segments of the left ventricle (LV) and in 1 of 2 segments of the right ventricle (RV). Strain rate (SR) was reduced in 2 of 12 segments of the LV and 1 of 2 segments of the RV in SSc patients as compared to controls (P < 0.05 for all). These involvements did not match any particular coronary artery distribution. More important differences were detected by average strain and SR values of the LV between patients and controls (19.78 ± 3.00% vs 23.41 ± 2.73%, P < 0.001; 2.01 ± 0.41 vs 2.23 ± 0.27/sec, P = 0.026, respectively). Furthermore, carbon monoxide diffusion capacity (DLCO) in scleroderma patients significantly correlated with LV average strain (r = 0.59; P = 0.001). Conclusion: Evaluation of ventricular function by using echocardiographic strain imaging appears to be useful to detect subclinical cardiac involvement in SSc patients with normal standard echocardiographic and tissue Doppler velocity findings. [source] Left and Right Ventricular Function Is Impaired in Behçet's DiseaseECHOCARDIOGRAPHY, Issue 9 2006Bunyamin Yavuz M.D. Objectives: Subclinical cardiac involvement may occur in patients with Behçet's disease (BD). The purpose of our study was to assess the noninvasive parameters of biventricular function derived from Doppler tissue imaging (DTI) of the tricuspid and mitral annular motion in BD. Methods: Twenty-one patients with BD and 20 control subjects were enrolled in this study. All subjects were selected to exclude those with cardiovascular risk factors. Standard echocardiography and pulsed DTI were obtained in every patient. Results: Peak systolic (13.71 ± 2.09 vs 20.01 ± 1.57, P < 0.001), peak early diastolic (11.26 ± 2.52 vs 15.35 ± 2.06, P < 0.001) tricuspid annular velocities were significantly lower in patients than controls. Peak systolic (8.68 ± 1.4 vs 12.25 ± 1.7, P < 0.001), peak early diastolic (7.89 ± 1.07 vs 9.94 ± 1.12, P < 0.001), and peak end diastolic (8.30 ± 1.32 vs 9.23 ± 0.91, P = 0.013) lateral mitral annular velocities were significantly lower in patients than controls. Conclusions: We demonstrated that myocardial velocities, were affected in patients with BD. Therefore, we conclude that right and left ventricular function is impaired in patients with BD. [source] Evaluation of Cardiac Involvement in Hypereosinophilic Syndrome: Complementary Roles of Transthoracic, Transesophageal, and Contrast EchocardiographyECHOCARDIOGRAPHY, Issue 8 2006Rajesh Shah M.D. Hypereosinophilic syndrome is a rare but important systemic disease with multiple clinical presentations. Approximately 40% of these cases have cardiac involvement. Echocardiography is the most easily available and versatile imaging modality in assessing cardiac involvement in this disease process. As described and reviewed in this case, it may be the first imaging modality to raise suspicion of this disease entity. Hence, clinicians interpreting echocardiograms and caring for patients need to be aware of the manifestations and complementary roles of various echo techniques in delineating cardiac involvement. Furthermore, the importance of a thorough history and laboratory review prior to echocardiography may provide valuable clues which may otherwise be missed. [source] Impaired aortic elastic properties in patients with systemic sarcoidosisEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 2 2008I. Moyssakis Abstract Background, Systemic sarcoidosis (Sar) is a granulomatous disorder involving multiple organs. Widespread vascular involvement and microangiopathy are common in patients with Sar. In addition, subclinical cardiac involvement is increasingly recognized in patients with Sar. However, data on the effect of Sar on the elastic properties of the arteries and myocardial performance are limited. In this study we looked for differences in aortic distensibility (AoD) which is an index of aortic elasticity, and myocardial performance of the ventricles, between patients with Sar and healthy subjects. In addition, we examined potential associations between AoD and clinical, respiratory and echocardiographic findings in patients with Sar. Materials and methods, A total of 83 consecutive patients (26 male/57 female, mean age 51·1 ± 13·3 years) with Sar, without cardiac symptoms, were included. All patients underwent echocardiographic and respiratory evaluation including lung function tests. Additionally, 83 age- and sex-matched healthy subjects served as controls. AoD was determined non-invasively by ultrasonography. Results, AoD was lower in the Sar compared to the control group (2·29 ± 0·26 vs. 2·45 ± 0·20 ·10,6 cm2· dyn,1, P < 0·01), while left ventricular mass (LVM) was higher in the Sar group (221·3 ± 50·2 vs. 195·6 ± 31·3 g, P = 0·007). Furthermore, myocardial performance of both ventricles was impaired in the Sar group. Multivariate linear regression analysis in the total sample population demonstrated a significant and independent inverse relationship between AoD and the presence of Sar (P < 0·001). The same analysis in the Sar patients showed that AoD was associated significantly and independently with the stage of Sar, age, systolic blood pressure, LVM and myocardial performance of both ventricles. No significant relationship was found between AoD and disease duration, pulmonary artery pressure or lung function tests. Conclusions, Presence and severity of Sar are associated with reduced aortic distensibility, irrespective of the disease duration, pulmonary artery pressure and lung function. In addition, patients with Sar have increased LVM and impaired myocardial performance. [source] Update of the Pompe disease mutation database with 107 sequence variants and a format for severity rating,HUMAN MUTATION, Issue 6 2008Marian Kroos Abstract Pompe disease was named after the Dutch pathologist Dr JC Pompe who reported about a deceased infant with idiopathic hypertrophy of the heart. The clinical findings were failure to thrive, generalized muscle weakness and cardio-respiratory failure. The key pathologic finding was massive storage of glycogen in heart, skeletal muscle and many other tissues. The disease was classified as glycogen storage disease type II and decades later shown to be a lysosomal disorder caused by acid ,-glucosidase deficiency. The clinical spectrum of Pompe disease appeared much broader than originally recognized. Adults with the same enzyme deficiency, alternatively named acid maltase deficiency, were reported to have slowly progressive skeletal muscle weakness and respiratory problems, but no cardiac involvement. The clinical heterogeneity is largely explained by the kind and severity of mutations in the acid ,-glucosidase gene (GAA), but secondary factors, as yet unknown, have a substantial impact. The Pompe disease mutation database aims to list all GAA sequence variations and describe their effect. This update with 107 sequence variations (95 being novel) brings the number of published variations to 289, the number of non-pathogenic mutations to 67 and the number of proven pathogenic mutations to 197. Further, this article introduces a tool to rate the various mutations by severity, which will improve understanding of the genotype-phenotype correlation and facilitate the diagnosis and prognosis in Pompe disease. © 2008 Wiley-Liss, Inc. [source] Cardiac magnetic resonance imaging in the evaluation of cardiac sarcoidosis: an Australian single-centre experienceINTERNAL MEDICINE JOURNAL, Issue 2 2009V. Manins Abstract Background:, Cardiac involvement in systemic sarcoidosis is common; however, current diagnostic tools are imprecise. Recognition of cardiac sarcoidosis (CS) is important as it has a relatively poor prognosis. Gadolinium-enhanced cardiac magnetic resonance imaging (Gad-CMR) is emerging as an excellent technique in determining the presence of and extent to which cardiac muscle is affected by sarcoidosis. Methods:, A retrospective analysis was performed on all patients with biopsy-proven systemic sarcoidosis referred for Gad-CMR scanning to evaluate potential cardiac involvement. All patients also underwent an electrocardiogram, Holter monitor and echocardiography. Gallium-67 radionuclide investigation, positron emission tomography and cardiac biopsy were ordered at the discretion of the treating physician. Results:, Eleven of the 20 patients had Gad-CMR images supportive of the diagnosis of CS. Eight of these 11 patients met the Japanese Ministry of Health and Welfare (JMHW) criteria for the diagnosis of CS; three abnormal Gad-CME scans consistent with diagnosis of CS were seen in patients who did not meet JMHW criteria. No patients with normal Gad-CMR scan met JMHW criteria for CS. Conclusion:, These findings suggest that Gad-CMR is potentially superior to the JMHW criteria in the diagnosis of cardiac sarcoidosis. [source] Determination of cardiac involvement in sarcoidosis by magnetic resonance imaging and Doppler echocardiographyJOURNAL OF INTERNAL MEDICINE, Issue 5 2002C. M. Sköld Abstract. Sköld CM, Larsen FF, Rasmussen E, Pehrsson SK, Eklund AG (Karolinska Hospital and Institutet, Stockholm, Sweden). Determination of cardiac involvement in sarcoidosis by magnetic resonance imaging and Doppler echocardiography. J Intern Med 2002; 252: 465,471. Objectives. To elucidate whether cardiac magnetic resonance imaging (MRI) could be useful in disclosing structural changes in the myocardium in sarcoidosis patients and to relate echo-Doppler derived indices of left ventricular function to electrocardiogram (ECG) findings. Design. The MRI was performed in 18 consecutive patients with sarcoidosis. Left ventricular ejection fraction (LVEF), i.e. systolic function, was estimated echocardiographically by Simpson's two-dimensional method (n = 16). Diastolic function was estimated by age-corrected Doppler-derived indices: isovolumetric relaxation time (IVRT), deceleration time (DT) and early filling/atrial contraction ratio (E/A ratio). Results. Eleven patients had conduction defects or dysrhythmias (ECG+) whilst seven patients had a normal ECG (ECG,). In two patients, high signalling, contrast-enhanced, isolated regions, suggestive of deposits, were seen in the left ventricular myocardium on MRI. Both these patients had abnormal ECGs and signs of systolic and/or diastolic dysfunction on echocardiography. LVEF was subnormal in seven of 10 of the ECG+ patients and in two of six of the ECG,. Signs of diastolic dysfunction were found in 59% and 56% of the measurements in the ECG+ and ECG, patients, respectively. Conclusion. We conclude (i) that myocardial deposits on MRI in sarcoidosis patients have a high specificity for cardiac involvement but a rather low sensitivity; (ii) that a substantial proportion of sarcoidosis patients with abnormal ECGs have echocardiographic signs of systolic and/or diastolic dysfunction. [source] The diagnosis and management of Kawasaki diseaseJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 3 2005J Royle Abstract:, Kawasaki disease (KD) is a systemic vasculitis of childhood with a predilection for the coronary arteries. It is the predominant cause of paediatric acquired heart disease in developed countries. The aetiology of KD remains unknown and consequently there is no diagnostic test. The diagnosis is made using a constellation of clinical criteria that in isolation have poor sensitivity and specificity. Early treatment prevents overt coronary artery damage in the majority of children. The long-term effects of childhood KD on later cardiovascular health remain unknown. A recent study showed that treatment of KD in Australia is suboptimal, with late diagnosis occurring in approximately half of the cases and an unacceptably high incidence of acute cardiac involvement. These guidelines highlight the difficulties in the diagnosis of KD and offer some clues that may assist early recognition of this important paediatric disease. They also detail current treatment recommendations and the evidence on which they are based. Increased awareness of the epidemiology and spectrum of the clinical presentation of KD is essential for early recognition and optimal management. [source] Pacemaker Malfunctions in Danon's DiseasePACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 1 2008ELENA MARRAS M.D. We describe a case of a 30-year-old man with Danon's disease, an X-linked genetic disorder due to deficiency of lysosomal-associated membrane protein 2 with secondary intracytoplasmatic glycogen and autophagic material storage. This disease is characterized by skeletal muscle involvement, mental retardation, ophthalmic abnormalities, and cardiac disease. In this patient, cardiac involvement was characterized by hypertrophic cardiomyopathy in young age, preexcitation, and parossistic atrioventriular block. The patient underwent to an implantable cardioverter defibrillator implantation for conduction disorders and for primary prevention of sudden death, a frequent event in Danon's disease. This case report describes cardiac involvement with conduction disorders and multiple pacemaker malfunctions in Danon's disease. [source] Scleredema of Buschke in a Child with Cardiac InvolvementPEDIATRIC DERMATOLOGY, Issue 3 2010Adriana Isaac M.D. We report a case of an 11-year-old boy with diffuse nonpitting edema of the trunk and face associated with cardiac involvement. [source] Pediatric Churg-Strauss syndrome in MexicoPEDIATRIC PULMONOLOGY, Issue 4 2006Víctor Manuel Hernández-Bautista MD Abstract Churg-Strauss syndrome (CSS) is one of the rarest forms of vasculitis, and very rarely presents in the pediatric population. We present two cases of childhood CSS, both with hepatic and cardiac involvement. To our knowledge, these are the first two cases of CSS in the pediatric population described in Mexico. Pediatr Pulmonol. © 2006 Wiley-Liss, Inc. [source] Successful Long-Term Outcome of the First Combined Heart and Kidney Transplant in a Patient with Systemic AL AmyloidosisAMERICAN JOURNAL OF TRANSPLANTATION, Issue 1 2009V. Audard Simultaneous cardiac and renal involvement is associated with a particularly poor prognosis in patients with AL amyloidosis (AL-A). We report the first case of a successful long-term outcome of combined heart and kidney transplantation not followed by autologous stem cell transplantation in a patient with systemic AL-A. The recipient was a 46-year-old man with end-stage renal failure associated with serious cardiac involvement in the context of AL-A. Before transplantation, two courses of oral melphalan plus prednisone induced partial hematologic remission, as shown by the decrease in circulating free light chain with no improvement of renal or heart function. The patient underwent combined heart and kidney transplantation as a rescue treatment. During the follow-up period (36 months), plasma cell dyscrasia remains in complete remission, with normal free lambda light chain levels and no recurrence of amyloid deposition on heart and kidney grafts. This case report demonstrates that combined heart and kidney transplantation not systematically associated with stem cell transplantation may be considered an additional therapeutic option in AL-A patients with severe organ dysfunction and partial hematologic remission. [source] Cardiac and respiratory failure in limb-girdle muscular dystrophy 2IANNALS OF NEUROLOGY, Issue 5 2004Maja Poppe MD Mutations in the gene encoding fukutin-related protein cause limb-girdle muscular dystrophy 2I. In this multicenter retrospective analysis of 38 patients, 55.3% had cardiac abnormalities, of which 24% had developed cardiac failure. Heterozygotes for the common C826A mutation developed cardiac involvement earlier than homozygotes. All patients initially improved while receiving standard therapy. Independent of cardiac status, forced vital capacity was below 75% in 44.4% of the patients. There was no absolute correlation between skeletal muscle weakness and cardiomyopathy or respiratory insufficiency. These complications are a primary part of this specific type of limb-girdle muscular dystrophy, with important implications for management. Ann Neurol 2004;56:738,741 [source] Fragmented QRS Complexes on 12-Lead ECG: A Marker of Cardiac Sarcoidosis as Detected by Gadolinium Cardiac Magnetic Resonance ImagingANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 4 2009Mohamed Homsi M.D. Background: Fragmented QRS complexes (fQRS) on a 12-lead ECG are a marker of myocardial scar in patients with coronary artery disease. Cardiac sarcoidosis is also associated with myocardial granuloma formation and scarring. We evaluated the significance of fQRS on a 12-lead ECG compared to Gadolinium-delayed enhancement images (GDE) in cardiac magnetic resonance imaging (CMR). Method and results: The ECGs of patients (n = 17, mean age: 52 ± 11 years, male: 53%) with established diagnosis of sarcoidosis who underwent a CMR for evaluation of cardiac involvement were studied. ECG abnormalities included bundle branch block, Q wave, and fQRS. fQRS, Q wave, and bundle branch block were present in 9 (53%), 1 (6%), and 4 (24%) patients, respectively. The sensitivity and specificity of fQRS for detecting abnormal GDE were 100% and 80%, respectively. Sensitivity and specificity of Q waves were 11% and 100%, respectively. Conclusions: fQRS on a 12-lead ECG in patients with suspected cardiac sarcoidosis are associated with cardiac involvement as detected by GDE on CMR. [source] Electrocardiographic Evaluation in Patients with Systemic Scleroderma and without Clinically Evident Heart DiseaseANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 3 2009Anna Bie, Ph.D., ous-Wilk M.D. Background: In patients with systemic scleroderma (SSc), clinically evident cardiac involvement is recognized to be a poor prognostic factor. The aim of the study was to evaluate electrocardiographic changes, parameters of heart rate variability (HRV), and heart rate turbulence (HRT) in patients with SSc without evident symptoms of heart disease. Methods: A group of 27 patients with SSc were subjected to standard electrocardiography (ECG) examination and 24-hour Holter monitoring. Analysis of HRV in time and frequency domains, HRT, and echocardiography were also performed. Results: Holter monitoring revealed a larger number of premature supraventricular contractions (PSVCs), as well as premature ventricular contractions (PVCs) in the patients with systemic scleroderma, as compared with the control group. Moreover, the SSc patients showed decreased parameters of time and frequency domains, as referred to the controls, especially during night hours. In four patients, abnormal HRT values were present. On echocardiography, only slight changes were found, however in five patients left ventricle diastolic dysfunction was diagnosed. Conclusions: The noninvasive electrocardiographic methods seems to be useful for detecting early heart involvement in course of SSc and could be recommended for routine used in clinical practice. Significance of HRT analysis in patients with SSc needs further elucidation. [source] Cardiac involvement in Churg-Strauss syndromeARTHRITIS & RHEUMATISM, Issue 2 2010Robert M. Dennert Objective Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients. Methods Thirty-two consecutive patients with CSS in remission (mean ± SD duration of disease between diagnosis and enrollment 6.1 ± 5.8 years, mean ± SD age 61 ± 10 years) who were previously unaware of cardiac involvement were compared with 32 randomly selected age- and sex-matched control subjects, using clinical evaluation, electrocardiography (EKG), echocardiography, and cardiac magnetic resonance imaging (MRI). Results Detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in CSS patients compared with 3% in controls (P < 0.001), with clinical symptoms in 26% and 3%, respectively (P = 0.009), EKG abnormalities in 66% and 3%, respectively (P < 0.001), and echocardiographic defects in 50% and 3%, respectively (P < 0.001). Cardiac MRI detected cardiac manifestations in 62% of CSS patients. In the presence of cardiac MRI abnormalities, echocardiography could detect cardiac involvement with a sensitivity of 83% and a specificity of 80%. The absence of symptoms or EKG abnormalities did not exclude cardiac involvement, because abnormalities could still be detected in 38% of these patients at the time of echocardiography or cardiac MRI. Conclusion These results demonstrate a high incidence of cardiac involvement in CSS patients. Systematic cardiac evaluation including detailed imaging is required to properly identify CSS patients with cardiac involvement. [source] Anti,U3 RNP autoantibodies in systemic sclerosisARTHRITIS & RHEUMATISM, Issue 4 2009Rohit Aggarwal Objective To describe the classification, demographic and clinical features, and survival in anti,U3 RNP autoantibody,positive patients with systemic sclerosis (SSc). Methods Medical records of 108 anti,U3 RNP,positive and 2,471 anti,U3 RNP,negative SSc patients first evaluated during 1985,2003 were reviewed. Anti,U3 RNP antibody was detected by protein and RNA immunoprecipitation. Disease classification, demographic and clinical features, organ system involvement, and survival were compared between the 2 patient groups, by Student's t -test, chi-square analysis, and Mantel-Haenszel test. Results The anti,U3 RNP,positive group had a higher proportion of African American patients (27% versus 5%; P < 0.001) and male patients (29% versus 19%; P = 0.021), and was younger at the time of first physician diagnosis (mean age 42.8 years versus 47.4 years; P = 0.001). The 2 groups had similar proportions of patients with diffuse cutaneous involvement (47% and 45% in those with and those without anti,U3 RNP, respectively). However, among patients with diffuse cutaneous involvement, the mean maximum modified Rodnan skin score was significantly lower in the anti,U3 RNP group (22.3 versus 27.9; P < 0.001). Skeletal muscle involvement was more frequent in anti,U3 RNP,positive patients (25% versus 14%; P = 0.002), as was "intrinsic" pulmonary arterial hypertension (PAH) (31% versus 13%; P < 0.001). The frequency of gastrointestinal involvement, cardiac involvement, pulmonary fibrosis, and "renal crisis" did not differ significantly between the 2 groups. Survival was worse in the anti,U3 RNP,positive group (hazard ratio 1.38 [95% confidence interval 1.05,1.82]). PAH was the most common known cause of death in patients with anti,U3 RNP (30%, versus 10% in the anti,U3 RNP,negative group; P < 0.001). Conclusion The present findings demonstrate that the frequencies of African American race and male sex are greater among SSc patients with anti,U3 RNP antibody than those without, and the former group is younger at SSc diagnosis. Anti,U3 RNP,positive patients have more frequent skeletal muscle involvement and PAH, the latter being the most common cause of death. [source] | ||||||||||||||||||||||