Fabry Disease Cardiac Involvement: Strengthen the Understanding of Early Diagnosis and Treatment / 罕见病研究

Fabry disease is an X-linked inherited lysosomal storage disorder caused by mutations of the GLA gene, resulting in the decreased a-galactosidase A activity and the accumulation of its substrate globotriasylceramide (GL-3) in the heart, kidney and other organs. The main clinical manifestations of cardiac involvement in Fabry disease are left ventricular hypertrophy (LVH), myocardial fibrosis, heart failure and arrhythmia, which limit quality of life and represent the most common causes of death. Following the development of enzyme activity and genetics testing, diagnosis of Fabry disease is no longer difficult. The application of enzyme replacement therapy (ERT) has also significantly slow disease progression. Therefore, early diagnosis and treatment have become essential in the management of Fabry disease cardiac involvement. Electrocardiogram, echocardiography and cardiac magnetic resonance(CMR) allow early detection of suspected patients. In addition, with the approval of oral chaperone therapy and substrate reduction therapy, the Fabry disease specific treatment landscape is evolving. This article will review the general features, pathophysiology, diagnosis and treatment of Fabry disease with cardiac involvement.

Advances in cardiac involvement in children with Duchenne muscular dystrophy / 国际儿科学杂志

Duchenne muscular dystrophy(DMD)is an X-linked recessive muscular disorder that affects mainly males.With its low incidence, insidious onset, and rapid progression, DMD is characterized by proximal muscle weakness, gastrocnemius hypertrophy, and markedly elevated serum creatine kinase.In addition to severe motor dysfunction, it also causes cardiac involvement in children, mainly manifested as dilated cardiomyopathy and arrhythmias.The mutations of DMD gene lead to the absence of dystrophin, which results in cytoskeletal defects and the impairment of the integrity of myocardial cell membrane.Meanwhile, calcium overload makes the myocytes more susceptible to damage.Exon deletion is the most common type of gene mutations in children with DMD, followed by point mutations, duplications and small insertion or deletion.The relationship among the clinical manifestations, pathogenesis, evaluation of cardiac damage in DMD and its genotype has not been clarified, which still needs further research and exploration, although some advances have been made recently.

The use of high-sensitivity cardiac troponin I in assessing cardiac involvement and Disease prognosis in idiopathic inflammatory myopathy

Abstract Objectives Cardiac involvement is one of the most serious complications of idiopathic inflammatory myopathy (IIM) that indicates poor prognosis. However, there is a lack of effective biomarkers for the identification of cardiac involvement and the prediction of prognosis in IIM. Here, we aimed to explore the value of different cardiac biomarkers in IIM patients. Methods A total of 142 IIM patients in the Department of Rheumatology and Immunology, Ruijin Hospital from July 2019 to October 2022 were included in this study. The clinical characteristics, laboratory tests, treatments and prognosis were recorded. The disease activity was assessed according to the core set measures. The correlations of the serum cardiac biomarkers levels with disease activity were analyzed by the Spearman correlation test. Risk factors for cardiac involvement were evaluated by multivariate logistic regression analysis. Results Higher high-sensitivity cardiac troponin I (hs-cTnI) levels were associated with cardiac involvement (n = 41) in IIM patients [adjusted OR 7.810 (95% CI: 1.962-31.097); p = 0.004], independent of other serum cardiac biomarkers. The abnormal hs-cTnI had the highest AUC for distinguishing of cardiac involvement in IIM patients (AUC = 0.848, 95% CI: 0.772,0.924; p < 0.001). Besides, we found that high serum levels of hs-cTnI were significantly correlated with disease activity. Moreover, patients with higher serum levels of hs-cTnI tended to suffer from poor prognosis. Conclusions Serum hs-cTnI testing may play a role in screening for cardiac involvement in IIM patients. Abnormal levels of serum hs-cTnI were associated with increased disease activity and poor prognosis. Key Points Among all the cardiac biomarkers, the serum levels of hs-cTnI were independently associated with cardiac involvement in IIM patients. The serum levels of hs-cTnI were significantly correlated with disease activity in IIM patients. The abnormal hs-cTnI levels were correlated with poor prognosis in IIM patients.

Distrofia muscular de Duchenne, una causa infrecuente de miocardiopatía dilatada / Duchenne muscular dystrophy, a rare cause of dilated cardiomyopathy

Neuromuscular diseases represent a rare cause of dilated myocardiopathy, among them Duchenne muscular dystrophy is the most common. Transthoracic echocardiography and cardiac magnetic resonance imaging can assess cardiac involvement early. The case of a patient diagnosed with Duchenne muscular dystrophy who develops cardiac involvement during cardiology follow-up is presented below.

Kawasaki disease in Colombia: A systematic review and contrast with multisystem inflammatory syndrome in children associated with COVID-19

Introduction: Kawasaki disease (KD) is an acute vasculitis with multisystem involvement. Recently, the increasing incidence of a condition that closely resembles KD in many cases, named multisystem inflammatory syndrome in children (MIS-C), has set off alarms amid the current worldwide coronavirus disease-19 (COVID-19) pandemic. Hence, the aim is to conduct a systematic review of the literature about KD in Colombia and contrast it with COVID-19-related MIS-C. Materials and methods: A search was carried out in both international and Latin American electronic databases for publications concerning patients with KD in the Colombian population. Records were then screened by titles and/or abstracts, assessed for eligibility, and reviewed. Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were followed. The search included studies reporting MIS-C associated with COVID-19, and compared these patients with our findings of KD in Colombia. Results: Out of 36 publications retrieved, 17 were included, representing 120 individuals. Male to female ratio was 1.6, and most patients (90.4%) were aged 5 years or less. Among the main features of KD, fever was the most frequent (96.2% of the patients), while cervical lymphadenopathy was present in only 40.6%. Intravenous immunoglobulin was administered in 91.4% cases and 6.2% were resistant. Cardiac involvement was found in around 30%, and 20% had coronary artery lesions. Comparison between MIS-C associated with COVID-19 and KD in Colombia indicates that patients affected by MIS-C were older (72.2% of MIS-C patients > 5 years), had higher rates of cardiac involvement, and required critical care more often. Conclusions: Our findings of KD in Colombia are consistent with the available descriptions of KD in the scientific literature. Given the increasing rate of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in Colombia and Latin America, our study raises awareness about MIS-C in pediatric patients with COVID-19 and its relationship with KD.

Introducción: La enfermedad de Kawasaki (EK) es una vasculitis aguda con compromiso multisistémico. Recientemente, la incidencia creciente de una condición que se asemeja en forma considerable a la EK en muchos casos, denominada síndrome inflamatorio multisistémico (SIMS) en niños, ha encendido las alarmas en medio de la actual pandemia mundial de la enfermedad COVID-19. Por consiguiente, nos propusimos realizar una revisión sistemática de la literatura acerca de la EK en Colombia y contrastarla con el SIMS relacionado con COVID-19 en niños. Materiales y métodos: Buscamos publicaciones respecto a pacientes con EK en población colombiana, en bases de datos electrónicas tanto internacionales como latinoamericanas. Los registros hallados fueron tamizados por títulos o resúmenes, evaluados para elegibilidad y revisados. Se siguieron las guías Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA). Posteriormente, buscamos estudios que reportaran SIMS temporalmente asociado con COVID-19 en niños y comparamos estos pacientes con nuestros hallazgos de EK en Colombia. Resultados: De 36 publicaciones encontradas se incluyeron 17, las cuales representaron 120 individuos. La razón hombre a mujer fue de 1,6 y la mayoría de los pacientes (90,4%) tenía 5 anos o menos. Entre las principales características de EK, la fiebre fue la más frecuente (96,2%), mientras que la linfadenopatía cervical estuvo presente solo en el 40,6%. La inmunoglobulina intravenosa se administró en el 91,4% de los casos y 6,2% presentaron resistencia. Se encontró compromiso cardiaco en alrededor del 30% de los pacientes, en tanto que el 20% tuvo lesiones de arterias coronarias. La comparación entre las características clínicas de la EK y el SIMS asociado a COVID-19 mostró que los individuos afectados por el SIMS eran mayores (72,2% con SIMS tenían más de cinco anos), tuvieron mayores índices de compromiso cardiaco y requirieron cuidado crítico con mayor frecuencia. Conclusiones: Nuestros hallazgos de EK en Colombia son consistentes con las descripciones disponibles de esta enfermedad en la literatura científica. Debido al aumento de infección por SARS-CoV-2 en Colombia y Latinoamérica, nuestro estudio busca crear conciencia sobre el SIMS en pacientes pediátricos con COVID-19 y su relación con la EK.

Lesión miocárdica en una paciente con lupus eritematoso sistémico: Un reto diagnóstico / Myocardial lesion in a patient with systemic lupus erythematosus: A diagnostic challenge

Resumen Mujer de 37 años con antecedentes de lupus eritematoso sistémico (LES), tromboembolia pulmonar y trombosis venosa profunda, quien consultó por dolor torácico, con hallazgos de elevación de la troponina, disfunción sistólica del ventrículo izquierdo y alteraciones de la contractilidad segmentaria, además de anti-DNA elevado y complemento consumido. La angiografía coronaria mostró estenosis grave en el tercio medio de la arteria descendente anterior, que fue intervenida con angioplastia más stent medicado. La resonancia magnética cardiaca reveló infarto agudo de miocardio extenso en el territorio de la arteria descendente anterior, sin miocarditis. Se descartó compromiso cardiaco por LES, se continuó igual terapia inmunosupresora, se añadió manejo para enfermedad coronaria y egresó por buena evolución. El enfoque de las causas de lesión miocárdica en un paciente con LES supone un reto, dadas las múltiples posibilidades en el espectro de compromiso cardiaco, ya que varias estructuras se pueden ver afectadas. La miocarditis y la pericarditis se consideran las más frecuentes, pero el compromiso coronario no puede descartarse, independientemente de la edad y de la presencia de factores de riesgo tradicionales, puesto que el componente inflamatorio de la patología autoinmunitaria confiere un desarrollo acelerado de ateroesclerosis. La multimodalidad diagnóstica y el enfoque interdisciplinario son necesarios para aclarar el mecanismo de la lesión y así brindar un tratamiento dirigido.

Abstract A 37-year-old woman with a history of systemic lupus erythematosus (SLE), pulmonary thromboembolism and deep vein thrombosis consulted for chest pain, with findings of elevated troponin, left ventricular systolic dysfunction, and segmental contractility abnormalities, as well as elevated anti-DNA and complement consumption. Coronary angiography showed severe stenosis of the middle third of the anterior descending artery, which was treated with angioplasty plus a medicated stent. Cardiac magnetic resonance imaging revealed an extensive acute myocardial infarction in the area supplied by the anterior descending artery, without myocarditis. Cardiac involvement in SLE was ruled out, immunosuppressive treatment was maintained, coronary disease treatment was added, and she was discharged due to improvement. Identifying the causes of myocardial insult in a patient with SLE is challenging, given the multiple possibilities across the cardiac involvement spectrum, in which several structures may be affected. Myocarditis and pericarditis are considered to be the most frequent, but coronary involvement cannot be ruled out, regardless of age or the presence of traditional risk factors, since the inflammatory component of the autoimmune disease accelerates the development of atherosclerosis. Multimodal diagnostics and an interdisciplinary approach are necessary to clarify the mechanism of injury and provide targeted treatment.

Review of cardiovascular involvements in COVID-19

Background: A spectrum of cardiovascular pathologies occurs in patients with COVID-19 and increases the risk of mortality. Risk of mortality is also heightened in cardiovascular disease patients who contact COVID-19. Methodology: Online search for the keywords in PubMed, Medline, Embase, Google scholar was done. Relevant research articles yielded from the searches were reviewed. Results: the searches yielded a total of 172 results, out of which 111 were reviewed. Cardiac involvement was found in 70.6% COVID-19 patients: tachycardia (19%), electrocardiography abnormalities (22%), echocardiography abnormalities (57%), elevated myocardial enzymes (53%), and acute cardiac injury (9%). Eight percent of patients with acute cardiac injury were aged >60 years; 87.5% of them had ≥2 underlying comorbidities (hypertension, diabetes mellitus, cardiovascular diseases, chronic obstructive pulmonary disease, and chronic kidney disease). Novel coronavirus pneumonia was much more severe in the patients with acute cardiac injury than in patients with non-definite acute cardiac injury (P<0.001). Multivariate analyses showed that C-reactive protein (CRP) levels, old age, novel coronavirus pneumonia severity, and underlying comorbidities were the risk factors for cardiac abnormalities in patients with COVID-19. Conclusion: Besides its prominent expression at the level of the respiratory apparatus, COVID-19 is also characterized by a substantial degree of cardiovascular involvement, both in terms of deterioration of pre-existing conditions, and as the effect of inflammation-facilitated acute events. They include ischemic and inflammatory heart disease, ventricular arrhythmias, conduction disturbances, thrombotic events at the level of the lungs, systemic activation of the coagulation cascade and disseminated intravascular coagulation.

Systemic Scleroderma with Complete Heart Block

For patients with Systemic Sclerosis (SSc), cardiac involvement (CI) is directly caused by myocardial fibrosisor ischemia or is secondary to pulmonary arterial hypertension1 Here we present a similar case of a femalepatient aged 45 yr old female who presented with complaint of recurrent syncopal attacks, and difficulty inswallowing food and liquids. She had typical CREST Syndrome manifestations with complete heart block(CHB). For which pacemaker implantation was done.

Compromiso cardiaco en distrofias musculares: a propósito de un caso / Cardiac involvement in muscular dystrophies: a clinical case

Resumen Para las distrofias musculares no miotónicas como la Distrofia de Duchenne y la Distrofia de Becker, la cardiopatía forma parte inherente de su espectro clínico. La expresión clínica de éstas se puede manifestar con insuficiencia cardíaca des- compensada, arritmias o muerte súbita; gran parte de ellos cursan asintomáticos en el transcurso del tiempo, pero cuando se manifiestan constituyen una de las principales causas de muerte en estos pacientes, por lo que su detección temprana y tratamiento óptimo influyen en gran medida en el pronóstico clínico de estos pacientes. A continuación, se presenta el caso de un paciente en quien se encontró de forma incidental el compromiso cardíaco de uno de estos desórdenes neuromusculares.

Abstract Cardiac involvement in muscular dystrophies: a clinical case For non-myotonic muscular dystrophies such as Duchenne Dystrophy and Becker Dystrophy, heart disease is an inherent part of its clinical spectrum. The clinical expression of these diseases can be manifested with decompensated heart failure, arrhythmias or sudden death; A large part of them are asymptomatic over time, but when they manifest they constitute one of the main causes of death in these patients, so their early detection and optimal treatment greatly influence the clinical prognosis of these patients. The following is the case of a patient in whom the cardiac involvement of one of these neuromuscular disorders was found incidentally.

Cardiovascular manifestations of people living with HIV/AIDS: Report from a hot spot in eastern India

Objective To study the profile and characteristics of cardiovascular abnormalities among patients living with human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) at a tertiary care hospital in India. The association of cardiovascular abnormalities with the CD4 count and disease stages, according to the World Health Organization (WHO) classification, was also analyzed. Methods A total of 200 patients with HIV/AIDS were compared with 50 healthy controls. All patients underwent blood investigations, chest X-ray, electrocardiography, and echocardiography. Results The mean age of the patients was 38.66 ± 9.22 years, with a male-to-female ratio of 3.25:1. Echocardiographic abnormalities were found in 52% of the patients and 12% of the controls, with the most common abnormality being left ventricular diastolic dysfunction. Echocardiographic abnormalities were markedly more common in patients with a CD4 count of <200/μL. The advanced stage of the disease, according to the WHO classification, was also associated with an increased incidence of echocardiographic abnormalities. Conclusions Cardiovascular abnormalities in the form of electrocardiogram and ECHO findings were present in 54.5% and 52% of patients, respectively. Echocardiographic findings showed significant correlation with CD4 count and WHO disease stage.

Inferior vena cava and intra-cardiac invasion of hepatocellular carcinoma: a rare but catastrophic complication

Intra-cardiac extension of hepatocellular carcinoma (HCC) is an uncommon but serious condition related to poor prognosis. We report a 57-year-old male diagnosed with HCC with intra-cardiac extension into the right atrium at presentation. There were no symptoms related to cardiac involvement and intra-cardiac extension was incidentally noted on radiological imaging. He was offered palliative treatment and succumbed to his disease within 50 days of first diagnosis.

Cardiac Manifestation of Fabry Disease: From Hypertrophic Cardiomyopathy to Early Diagnosis and Treatment in Patients Without Left Ventricular Hypertrophy

Abstract Although Fabry disease was identified a century ago, it is still a challenging condition to diagnose and treat. Registries data suggest that at least 10% of patients may first present with a cardiac event and that cardiac disease is 1 of the 3 major causes of morbidity and mortality in affected males and females. Cardiac involvement in Fabry disease may be expressed as left ventricular hypertrophy (LVH), coronary disease, atrioventricular conduction disturbances, arrhythmias, and valvular involvement. The exact mechanism by which hypertrophy and fibrosis in the heart occur is not fully understood. Lysosomal globotriaosylceramide accumulation in the myocardium is responsible for only 3% of the mass in the hypertrophic heart, indicating that the LVH is not a direct result of substrate infiltration. One of the most important contributions that cardiologists can make is to consider the diagnosis of Fabry disease in patients with cardiac manifestations preceding the development of LVH and conduct family screening to identify patients with early cardiac involvement which will benefit more from enzyme replacement therapy (ERT). Fabry patients without cardiac manifestations of the disease should be evaluated annually by a cardiologist specialized in Fabry disease, regardless of the indication for ERT.

Unusual Cardiac Infiltration in Diffuse Large B-Cell Lymphoma

No abstract available.

Compromiso cardíaco en pacientes con enfermedad de Fabry / Cardiac involvement in patients with fabry's disease

Antecedentes: La enfermedad de Fabry (EF) es un desorden lisosomal de transmisión ligada al cromosoma X debido al déficit de la enzima alfa galactosidasa A, con acumulación multisistémica de globotriaosilceramida (GB3). La afectación cardíaca reduce expectativa y calidad de vida. Objetivo: Describir compromiso cardiológico de 38 pacientes con EF, diagnosticados y estudiados multidisciplinariamente. Destacar alta prevalencia en esta región. Método: A partir de caso índice, se aplica encuesta y elabora familiograma de 5 familias. Estudio genético y enzimático de 65 sospechosos confirma 38 afectados (25 Mujeres y 13 hombres) evaluados multidisciplina-riamente con Electrocardiograma, Ecocardiograma y exámenes de laboratorio. Resultado: Compromiso cardiológico en 66 por ciento de adultos, no presente en niños. Cardiopatía hipertrófica (CH) fue el hallazgo cardiológico más frecuente (56 por ciento de adultos) presente en 63 por ciento de los hombres y en 52 por ciento de las mujeres. En mayores de 40 años, 100 por ciento de hombres y 82 por ciento de mujeres están afectados. La edad promedio fue 38 en hombres y 57 en mujeres. Cardiopatía dilatada 26 por ciento en su mayoría asociado a CH. Insuficiencia valvular mitral leve en 47 por ciento, con predominio femenino, PR corto en 7 mujeres, fibrilación auricular 2 mujeres y 1 hombre, TPSV 1 mujer, BAV completo 1 hombre. Sin eventos coronarios. Conclusión: La afectación cardíaca en nuestro grupo es similar a la reportada internacionalmente. Dada la alta prevalencia que tiene esta patología en nuestro medio, frente a un paciente no hipertenso con hipertrofia ventricular debería descartarse EF. Evaluación dermatológica y oftalmológica apoyaría diagnóstico presuntivo antes de confirmación enzimática o estudio genético.

Aim: Fabry's disease (FD) is a lysosomal disorder with an X chromosome linkage. It is related to a deficiency of alphagalactosidase A, leading to multisystemic accumulation of globotriasocyl ceramyde (GB3). Cardiac compromise reduces life expectancy. Herein we describe cardiac and systemic findings in 38 patients with FD. Methods: A genetic and enzymatic characterization was obtained in all patients. FD was identified in 38 out of 65 screened subjects (25 females and 13 males), which belonged to 5 families identified from index cases. ECG and echocardiography was used to evaluate cardiac involvement. Results: Cardiac involvement was present in 66 per cent of adults and absent in all children. HVI was the most frequent abnormality observed in 56 of adults (63 per cent in males, 52 per cent in females). The prevalence of HVI increased with age reaching 100 per cent in males and 82 per cent in females aged 40 and older. Among other findings, cardiac dilatation was observed in 26 per cent, mild mitral insufficiency in 47 per cent and atrial fibrillation in 3 cases. No case of coronary artery disease was identified Conclusion: Fabry's disease is more prevalent than usually suspected. Cardiac involvement is frequent, especially the presence of HVI. It should be investigated in all subjects with unexplained LVH, especially when associated to characteristic dermatologic and ophtalmologic findings. Confirmation of the diagnosis may be obtained through enzymatic and genetic studies.

Changes of Th17 cells in systemic lupus erythematosus with cardiac involvement / 中华风湿病学杂志

ObjectiveTo investigate the role of Thl7 cells in the pathogenesis of SLE patients with cardiac involvement,and to understand the value of cardiac markers in SLE patients with cardiac involvement.MethodsSerum IL-17A levels were measured by enzyme-linked immunosorbent assay in 47 SLE patients with cardiac involvement (group Ⅰ ),55 SLE patients without cardiac involvement (group Ⅱ ) and 38 healthy controls(group Ⅲ ).The ADVIA Centaur(R)-XP immunoassay analysis system and Olympus AU2700 automatic biochemical system were used to measure cardiac markers.Then real time-quantitative polymerase chain reaction was used to measureRORγt mRNA in 13 SLE patients with cardiac involvement,14 SLE patients without cardiac involvement and 13 healthy controls.Kruskal-Wallis test,Mann-Whitney U test,F test and Spearman correlation were used for statistical analysis.Results① Serum levels of IL-17A were markedly increased in group Ⅰ than group Ⅱ and Ⅲ [27.98 (8.44-138.81) vs 11.12 (3.64-22.30) vs 5.77 (2.22-9.60) pg/ml,both P＜0.05].② Serum levels of BNP were significantly higher in group Ⅰ than group Ⅱ and Ⅲ [49(13.50-107.50) vs 17(9-26) vs 7.50(4.75-13) pg/ml,both P＜0.01 ].③ Age,course,SLEDAI were significantly higher in group Ⅰ SLE patients than group Ⅱ (P＜0.01 or P＜0.05).④ The level of RORγt mRNA were significantly elevated in group Ⅰ compared to group Ⅱ and Ⅲ [2.2(0.79-2.83) vs 0.72(0.39-1.14) vs 0.19(0.15-0.75),P＜0.05].Conclusion① Th17 cells may contribute to the inflammation of heart in SLE.② The older age,longer course and higher disease activity of SLE patients are risk factors for cardiac involvement in SLE.③ Serum BNP may be a useful indicator in SLE patients with heart involvement.

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior / Electrocardiographic markers for the early detection of cardiac disease in patients with beta-thalassemia major

OBJETIVO: Analisar comparativamente a dispersão da onda P (DOP) em pacientes com talassemia beta maior (β-TM) e indivíduos saudáveis (controles) para a detecção precoce do risco de arritmias. MÉTODOS: Oitenta e uma crianças com β-TM, com idades entre 4 e 19 anos, e 74 crianças saudáveis (grupo controle) foram submetidas a exame eletrocardiográfico e ecocardiograma transtorácico de rotina para avaliação cardíaca. A DOP foi calculada como a diferença entre as durações máxima e mínima da onda P. RESULTADOS: Houve uma diferença estatisticamente significativa entre o grupo de estudo e o grupo controle no pico de velocidade do fluxo transmitral no início da diástole (E) e na razão E/fluxo transmitral tardio (A). A duração máxima da onda P e a DOP foram significativamente maiores nos pacientes com β-TM do que nos indivíduos controles. CONCLUSÕES: O aumento da DOP em nossos pacientes com β-TM pode estar relacionado à depressão na condução intra-atrial, devido à dilatação atrial, e ao aumento da atividade simpática. Estes pacientes devem ser acompanhados atentamente devido à possibilidade de ocorrência de arritmias com risco de vida.

OBJECTIVE: To comparatively evaluate P-wave dispersion (PWD) in patients with β-thalassemia major (TM) and healthy control subjects for the early prediction of arrhythmia risk. METHODS: Eighty-one children with β-TM, aged 4-19 years, and 74 healthy children (control group) underwent routine electrocardiography and transthoracic echocardiography for cardiac evaluation. PWD was calculated as the difference between the maximum and the minimum P-wave duration. RESULTS: There was a statistically significant difference between study and control groups in peak early (E) mitral inflow velocity and E/late (A) velocity ratio. Maximum P-wave duration and PWD were found to be significantly higher in β-TM patients than in control subjects. CONCLUSIONS: Increased PWD in our β-TM patients might be related to depression of intra-atrial conduction due to atrial dilatation and increased sympathetic activity. These patients should be closely followed up for risk of life-threatening arrhythmias.

Clinical analysis of cardiac involvement in patients with rheumatoid arthritis / 中国综合临床

Objective To explore the relationship between cardiac involvement and laboratory indicators in patients with rheumatoid arthritis(RA). Methods Cardiac echocardiography and ECG performance of 184 patients with RA were analyzed retrospectively. Results Among the 184 patients with RA, the pulmonary hypertension detection rate was 8. 3%, valvular disease 38. 9%, arteriosclerosis 27. 8%, wall to reduce the exercise 13.9%, myocarditis 5.6% and pericardial effusion 5.6%, according to the echocardiography examinations;Sinus tachycardia was evidenced in 15. 22% patients, ST-T changes in 39. 13%, electric axis left side in 8. 70%, branch block in 13.04%, left ventricular hypertrophy in 4. 35%, atrial fibrillation in 4. 35%, premature in 8.70%, early repolarization syndrome in 2. 17% and electric-axis right side in 4. 35% patients by ECG examinations. The serum level of CRP (46. 77 ±5. 87) mg/L was significantly higher in RA patients with cardiac involvement than that in the non-cardiac involvement patientsm (28. 45 ±3. 21) mg/L (P ＜0.05) ;While the serum level of ESR,RF,IgG,IgA,IgM, PLT showed no statistically significant differences between the two groups (P ＞ 0.05); Within RA patients withcardiac involvement, the serum level of CRP showed no significant difference among different sub-groups , which were classified according to the echocardiography performance (P ＞ 0.05). Conclusions Cardiac involvement occurred frequently in patients with rheumatoid arthritis. The valvular disease, arteriosclerosis, reducing of the wall motion and pericardial effusion are the main manifestations by echocardiography examination; Sinus tachycardia, ST-T changes,branch block and premature beats are the main ECG abnormalities. The serum level of CRP is significantly higher in RA patients with cardiac involvement than that with non-cardiac involvement patients. The higher level of CRP in patients with RA may indicate the cardiac involvement presence.

Cardiac Involvement in Patients with Duchenne Muscular Dystrophy

BACKGROUND: Cardiac involvement in Duchenne muscular dystrophy (DMD) is common, but usually latent without symptoms or signs in the initial period of disease. This study investigated the incidence and predictor of cardiac involvement in DMD patients. METHOD: From January 2000 to June 2005, we enrolled 45 patients with DMD (aged 20.2+/-3.0 years) who admitted to the Yongdong Severance Hospital. Electrocardiography and transthoracic echocardiography was done to evaluate the cardiac function. RESULT: Electrocardiographic abnormalities were present in 80.1% of patients. Sinus tachycardia was most common (50%). LVEF was decreased (46.7+/-13.8%), and 56% of the patients had diastolic dysfunction. Patients with pulmonary involvement were older (20.7+/-3.8 vs 17.6+/-2.8 years, p=0.028), and patients with reduced LVEF (<50%) had longer duration of disease (11.4+/-4.4 vs 14.3+/-2.4 years, p=0.04). However, on multivariate analysis, age, duration of disease, pulmonary involvement, dyspnea symptom, electrocardiographic abnormality was not an independent predictor for LV systolic dysfunction in adolescent and adult patients with DMD. CONCLUSION: Cardiac involvement in adolescent and adult patients with DMD was frequently observed independent of age, duration of disease, pulmonary involvement, and dyspnea symptom. Therefore, more active cardiac investigation is required in patients with DMD, even without clinical suspicion.

Cardiac involvement in systemic lupus erythematosus:a retrospective analysis of 272 systemic lupus erythematosus patients / 中华风湿病学杂志

0 05),but they had significantly longer course of disease ( P