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Sample records for non-ischaemic cardiomyopathy implanted

  1. Adding the implantable cardioverter-defibrillator to cardiac resynchronization therapy is associated with improved long-term survival in ischaemic, but not in non-ischaemic cardiomyopathy

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    Witt, Christoffer Tobias; Kronborg, Mads Brix; Nohr, Ellen Aagaard

    2016-01-01

    , and the multivariate analyses were adjusted for a priori selected variables. We included 917 HF patients, 427 with NICM, and 490 with ICM. Median follow-up was 4.0 years. Adjusted hazard ratio (aHR) for all-cause mortality was 0.76 [95% confidence interval (95% CI), 0.60-0.97; P = 0.03] in all patients; 0.96 (95% CI...... to ischaemic cardiomyopathy (ICM) or non-ischaemic cardiomyopathy (NICM) treated with a CRT device with or without defibrillator backup. METHODS AND RESULTS: In this observational study, consecutive patients with an ejection fraction ≤35% and QRS width ≥120 ms receiving a CRT device at Aarhus University......, 0.60-1.51; P = 0.85) in patients with NICM, and 0.74 (95% CI, 0.56-0.97; P = 0.03) in patients with ICM. In patients with NICM, ICD backup seemed to be associated with improved survival among non-responders to CRT (P = 0.08), but not among responders (P = 0.61). CONCLUSION: Adding an ICD backup...

  2. Clinical impact of implantable cardioverter-defibrillator in primary prevention of total mortality in non-ischaemic cardiomyopathy: results from a meta-analysis of prospective randomized clinical trials.

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    Romero, Jorge; Díaz, Juan Carlos; Grushko, Michael; Quispe, Renato; Briceno, David; Avendano, Ricardo; Golive, Anjani; Pina, Ileana; Natale, Andrea; Garcia, Mario J; Jorde, Ulrich P; Di Biase, Luigi

    2017-11-17

    Primary prophylactic implantable cardioverter defibrillators (ICDs) have demonstrated a clear all-cause mortality benefit in patients with ischaemic cardiomyopathy, with less compelling evidence supporting its use in patients with non-ischaemic cardiomyopathy (NICM). We performed a meta-analysis of randomized controlled trials (RCTs) evaluating the role of ICD for reduction in total mortality in NICM patients. An electronic search on PubMed, the Cochrane Library, and EMBASE databases was performed to identify the RCTs evaluating the role of prophylactic ICD placement in NICM patients. Mantel-Haenszel risk ratio (RR) fixed-effects model was used to summarize data across treatment arms. Random-effects model was used if heterogeneity (I2) ≥ 25. Patients with cardiac resynchronization therapy pacemaker (CRT-P) were included in the control group. Six RCTs, with a total of 3128 patients and a mean follow-up period of 48 ± 22 months comparing ICD with medical therapy in NICM were included in this analysis. There was a significant reduction in all-cause mortality in the ICD group compared with the medical therapy group [RR 0.79, 95% confidence interval (95% CI) 0.68-0.92; P = 0.002]. No publication bias was noted. Currently available evidence demonstrates that the use of ICD provides a clear and significant reduction in all-cause mortality among patients with NICM. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

  3. The value of the 12-lead electrocardiogram in localizing the scar in non-ischaemic cardiomyopathy.

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    Oloriz, Teresa; Wellens, Hein J J; Santagostino, Giulia; Trevisi, Nicola; Silberbauer, John; Peretto, Giovanni; Maccabelli, Giuseppe; Della Bella, Paolo

    2016-12-01

    Patients with non-ischaemic cardiomyopathy (NICM) and ventricular tachycardia can be categorized as anteroseptal (AS) or inferolateral (IL) scar sub-types based on imaging and voltage mapping studies. The aim of this study was to correlate the baseline electrocardiogram (ECG) with endo-epicardial voltage maps created during ablation procedures and identify the ECG characteristics that may help to distinguish the scar as AS or IL. We assessed 108 baseline ECGs; 72 patients fulfilled criteria for dilated cardiomyopathy whereas 36 showed minimal structural abnormalities. Based on the unipolar low-voltage distribution, the scar pattern was classified as predominantly AS (n = 59) or IL (n = 49). Three ECG criteria (PR interval 230 ms or QRS > 170 ms or an r ≤ 0.3 mV in V3 having 92 and 81% of sensitivity and specificity, respectively, in predicting AS scar pattern. A significant negative correlation was found between the extension of the endocardial unipolar low voltage area and left ventricular EF (r s = -0.719, P < 0.001). The extent of endocardial AS unipolar low voltage was correlated with PR interval and QRS duration (r s = 0.583 and r s = 0.680, P < 0.001, respectively) and the IL epicardial unipolar low voltage with the mean voltage of the limb leads (r s = -0.639, P < 0.001). Baseline ECG features are well correlated with the distribution of unipolar voltage abnormalities in NICM and may help to predict the location of scar in this population. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

  4. Screening and risk evaluation for sudden cardiac death in ischaemic and non-ischaemic cardiomyopathy

    DEFF Research Database (Denmark)

    Proclemer, Alessandro; Lewalter, Thorsten; Bongiorni, Maria Grazia

    2013-01-01

    centres (32.2%), and in patients with permanent dialysis in 8 centres (33.3%). Signal-averaged electrocardiography and heart rate variability were never considered as risk stratification tools in 23 centres (74.2%). Implantation of a loop recorder was performed in patients with borderline indications...... on the clinical risk stratification and not on non-invasive and invasive diagnostic tests or implantable loop recorder use....

  5. The prognostic value of right ventricular long axis strain in non-ischaemic dilated cardiomyopathies using standard cardiac magnetic resonance imaging

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    Arenja, Nisha [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); Kantonsspital Olten, Department of Cardiology, Solothurner Spitaeler AG, Olten (Switzerland); Riffel, Johannes H.; Halder, Manuel; Djiokou, Charly N.; Fritz, Thomas; Andre, Florian; Siepen, Fabian aus dem; Zelniker, Thomas; Meder, Benjamin; Kayvanpour, Elham; Korosoglou, Grigorios [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); Katus, Hugo A. [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); DZHK (German Centre for Cardiovascular Research), partner site Heidelberg, Heidelberg (Germany); Buss, Sebastian J. [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); Das Radiologische Zentrum - Radiology Center, Sinsheim-Eberbach-Erbach-Walldorf-Heidelberg (Germany)

    2017-09-15

    To investigate the association of right ventricular long axis strain (RV-LAS), a parameter of longitudinal function, with outcome in patients with non-ischaemic dilated cardiomyopathy (NIDCM). In 441 patients with NIDCM, RV-LAS was analysed retrospectively by measuring the length between the epicardial border of the left ventricular apex and the middle of a line connecting the origins of the tricuspidal valve leaflets in end-diastole and end-systole on non-contrast standard cine sequences. The primary endpoint (cardiac death or heart transplantation) occurred in 41 patients, whereas 95 reached the combined endpoint (including cardiac decompensation and sustained ventricular arrhythmias) during a median follow-up of 4.2 years. Kaplan-Meier survival curves showed a poor outcome in patients with RV-LAS values below -10% (log-rank, p < 0.0001). In a risk stratification model RV-LAS improved prediction of outcome in addition to RV ejection fraction (RVEF) and presence of late gadolinium enhancement. Assessment of RV-LAS offered incremental information compared to clinical symptoms, biomarkers and RVEF. Even in the subgroup with normal RVEF (>45%, n = 213) reduced RV-LAS was still associated with poor outcome. Assessment of RV-LAS is an independent indicator of outcome in patients with NIDCM and offers incremental information beyond clinical and cardiac MR parameters. (orig.)

  6. Cardiomyopathy

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    ... often in middle-aged people and is more likely to affect men. The most common cause is coronary artery disease or heart attack. Hypertrophic cardiomyopathy. This type involves abnormal thickening of ...

  7. Current Indications for Implantable Cardioverter Defibrillators in Non-Ischemic Cardiomyopathies and Channelopathies.

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    González-Torrecilla, Esteban; Arenal, Angel; Atienza, Felipe; Datino, Tomás; Bravo, Loreto; Ruiz, Pablo; Ávila, Pablo; Fernández-Avilés, Francisco

    2015-01-01

    Current indications for implantable cardioverter defibrillators (ICDs) in patients with channelopathies and cardiomyopathies of non-ischemic origin are mainly based on non-randomized evidence. In patients with nonischemic dilated cardiomyopathy (NIDCM), there is a tendency towards a beneficial effect on total mortality of ICD therapy in patients with significant left ventricular (LV) dysfunction. Although an important reduction in sudden cardiac death (SCD) seems to be clearly demonstrated in these patients, a net beneficial effect on total mortality is unclear mostly in cases with good functional status. Risk stratification has been changing over the last two decades in patients with hypertrophic cardiomyopathy (HCM). Its risk profile has been delineated in parallel with the beneficial effect of ICD in high risk patients. Observational results based on "appropriate" ICD interventions do support its usefulness both in primary and secondary SCD prevention in these patients. Novel risk models quantify the rate of sudden cardiac death in these patients on individual basis. Less clear risk stratification is available for cases of arrhythmogenic right ventricular cardiomyopathy (ARVC) and in other uncommon familiar cardiomyopathies. Main features of risk stratification vary among the different channelopathies (long QT syndrome -LQTS-, Brugada syndrome, etc) with great debate on the management of asymptomatic patients. For most familiar cardiomyopathies, ICD therapy is the only accepted strategy in the prevention of SCD. So far, genetic testing has a limited role in risk evaluation and management of the individual patient. This review aims to summarize these criticisms and to refine the current indications of ICD implantation in patients with cardiomyopathies and major channelopathies.

  8. Implantable cardioverter defibrillator therapy in young patients with cardiomyopathies and channelopathies: a single Italian centre experience.

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    Migliore, Federico; Silvano, Maria; Zorzi, Alessandro; Bertaglia, Emanuele; Siciliano, Mariachiara; Leoni, Loira; De Franceschi, Pietro; Iliceto, Sabino; Corrado, Domenico

    2016-07-01

    This study was designed to prospectively evaluate the risk-benefit ratio of implantable cardioverter defibrillator (ICD) therapy in young patients with cardiomyopathies and channelopathies. The study population included 96 consecutive patients [68 men, median age 27 (22-32) years] with cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (n = 35), dilated cardiomyopathy (n = 17), hypertrophic cardiomyopathy (n = 15), Brugada syndrome (n = 14), idiopathic ventricular fibrillation (n = 5), left ventricular noncompaction (n = 4), long-QT syndrome (n = 4) and short-QT syndrome (n = 2), who were 18-35 years old at the time of ICD implantation. During a mean follow-up of 72.6 ± 53.3 months, one patient with end-stage hypertrophic cardiomyopathy died because of acute heart failure, and 11 patients underwent orthotopic heart transplantation. Twenty patients (20.8%) had a total of 38 appropriate ICD interventions (4%/year), and 26 patients (27.1%) experienced a total of 49 adverse ICD-related events (5.4%/year), including 23 inappropriate ICD interventions occurring in nine patients (9.4%) and 26 device-related complications requiring surgical revision occurring in 20 patients (20.8%). Lead failure/fracture requiring lead extraction was the most common complication (n = 9). A threshold for ICD therapy less than 300 ms was associated with a borderline significant lower probability of inappropriate ICD interventions (hazard ratio = 0.2; 95% confidence interval 0.02-1.2; P = 0.07), whereas underweight status was an independent predictor of device-related complications (hazard ratio = 5.4; 95% confidence interval 1.5-19.4; P = 0.01). In young patients with cardiomyopathies and channelopathies, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. However, because ICD-related adverse events are common, the risk/benefit ratio should be

  9. Higher defibrillation threshold in methamphetamine cardiomyopathy patients with implantable cardioverter-defibrillator

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    Rakesh Malhotra

    2017-11-01

    Full Text Available Introduction: Identification of patients with an increased risk of high defibrillation thresholds (DFTs is important in planning implantable cardioverter-defibrillator (ICD procedures. Clinical observations have suggested that patients with methamphetamine cardiomyopathy (MACMP have significantly elevated defibrillation thresholds. We hypothesized that MACMP patients would have higher DFT thresholds than controls and would require procedural changes during ICD implantation to accommodate higher thresholds. Methods: We identified consecutive patients with MACMP undergoing ICD implantation at the academic center from 2003 to 2007. We then compared DFTs against age-and sex-matched controls. Results: The MACMP (n = 10 group showed significantly increased DFT thresholds (23.7 ± 6.7 J compared with age and sex-matched controls (14.5 ± 4.6 J, p < 0.005. Additionally, patients with MACMP had evidence of more severe congestive heart failure, with increased B-type natrieutic protein (BNP levels (1173 ± 784 vs 260 ± 349, p = 0.02 and decreased left ventricular ejection fraction (LVEF (17.8 ± 9.4 vs 35.9 ± 15.2, p = 0.02. MACMP patients required high output devices than controls (50% versus 0%, p = 0.03. Differences between groups remained significant despite adjusting for LVEF. Conclusions: Planning for ICD implantation should take into consideration a history of methamphetamine abuse, mandating DFT testing and empiric consideration of high output devices for such patients. Keywords: Methamphetamine cardiomyopathy, Implantable cardioverter-defibrillatior, Defibrillation threshold testing, B-type natriuretic peptide, Ejection fraction

  10. Sudden death mechanisms in nonischemic cardiomyopathies: Insights gleaned from clinical implantable cardioverter-defibrillator trials.

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    Steinberg, Benjamin A; Mulpuru, Siva K; Fang, James C; Gersh, Bernard J

    2017-12-01

    Sudden cardiac death (SCD) represents a major cause of death among patients with heart failure. Although scar-based, macroreentrant ventricular tachycardia/ventricular fibrillation is the primary etiology for SCD among patients with ischemic cardiomyopathy, a more diverse set of mechanisms and substrates is likely at play for the diverse group of patients characterized by nonischemic dilated cardiomyopathy (NICM). These causes may include scar-based reentry, but also neurohormonal stimulation (sympathetic, parasympathetic, renin-angiotensin-aldosterone), inflammation, and nonarrhythmic processes occurring in the context of a genetic predisposition. In addition to basic and translational science, observations from large randomized clinical trials of implantable cardioverter-defibrillators (ICDs) can also offer insight and support for specific mechanisms of SCD in these patients. This review will discuss the background of SCD in NICM, its potential mechanisms based on experimental and theoretical models, and the evidence for these mechanisms that can be derived from clinical trials of ICD therapy. Copyright © 2017 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  11. Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia.

    LENUS (Irish Health Repository)

    Corrado, Domenico

    2010-09-21

    The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy\\/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue.

  12. Implantable Cardioverter-Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications.

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    Orgeron, Gabriela M; James, Cynthia A; Te Riele, Anneline; Tichnell, Crystal; Murray, Brittney; Bhonsale, Aditya; Kamel, Ihab R; Zimmerman, Stephan L; Judge, Daniel P; Crosson, Jane; Tandri, Harikrishna; Calkins, Hugh

    2017-06-06

    Arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by ventricular arrhythmias and sudden cardiac death. Once the diagnosis is established, risk stratification to determine whether implantable cardioverter-defibrillator (ICD) placement is warranted is critical. The cohort included 312 patients (163 men, age at presentation 33.6±13.9 years) with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy who received an ICD. Over 8.8±7.33 years, 186 participants (60%) had appropriate ICD therapy and 58 (19%) had an intervention for ventricular fibrillation/flutter. Ventricular tachycardia at presentation (hazard ratio [HR]: 1.86; 95% confidence interval [CI], 1.38-2.49; P right ventricular dysplasia/cardiomyopathy. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  13. Implantable Cardioverter-defibrillator Therapy for Hypertrophic Cardiomyopathy: Usefulness in Primary and Secondary Prevention.

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    Sarrias, Axel; Galve, Enrique; Sabaté, Xavier; Moya, Àngel; Anguera, Ignacio; Núñez, Elaine; Villuendas, Roger; Alcalde, Óscar; García-Dorado, David

    2015-06-01

    Hypertrophic cardiomyopathy is a frequent cause of sudden death. Clinical practice guidelines indicate defibrillator implantation for primary prevention in patients with 1 or more risk factors and for secondary prevention in patients with a history of aborted sudden death or sustained ventricular arrhythmias. The aim of the present study was to analyze the follow-up of patients who received an implantable defibrillator following the current guidelines in nonreferral centers for this disease. This retrospective observational study included all patients who underwent defibrillator implantation between January 1996 and December 2012 in 3 centers in the province of Barcelona. The study included 69 patients (mean age [standard deviation], 44.8 [17] years; 79.3% men), 48 in primary prevention and 21 in secondary prevention. The mean number of risk factors per patient was 1.8 in the primary prevention group and 0.5 in the secondary prevention group (P=.029). The median follow-up duration was 40.5 months. The appropriate therapy rate was 32.7/100 patient-years in secondary prevention and 1.7/100 patient-years in primary prevention (P<.001). Overall mortality was 10.1%. Implant-related complications were experienced by 8.7% of patients, and 13% had inappropriate defibrillator discharges. In patients with a defibrillator for primary prevention, the appropriate therapy rate is extremely low, indicating the low predictive power of the current risk stratification criteria. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  14. Embodying a New Meaning of Being At Risk: Living With an Implantable Cardioverter Defibrillator for Arrhythmogenic Right Ventricular Cardiomyopathy.

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    Manuel, April; Brunger, Fern

    2016-01-01

    Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is a genetic condition that can cause fatal arrhythmias. The implantable cardioverter defibrillation (ICD) is a primary treatment for ARVC/D. Using a grounded theory approach, this study examines the experiences of 15 individuals living with an ICD. The ability to cope with and adjust to having an ICD is influenced by the acceptance of the ICD as something needed to survive, an understanding of the ICD's function, existing support networks, and ones' ability to manage everyday challenges. Coping well requires reshaping ideas about the meaning of being at risk and understanding how the ICD fits into that changing personal risk narrative. A thorough understanding of the unique needs of individuals with ARVC/D and of the specific factors contributing to the psychosocial distress related to having an ICD (vs. having the disease itself) is needed. Nurses must be prepared to provide ongoing support and education to this population.

  15. Complications Associated With Implantable Cardioverter Defibrillators in Adults With Congenital Heart Disease or Left Ventricular Noncompaction Cardiomyopathy (From the NCDR®Implantable Cardioverter-Defibrillator Registry).

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    Gleva, Marye J; Wang, Yongfei; Curtis, Jeptha P; Berul, Charles I; Huddleston, Charles B; Poole, Jeanne E

    2017-11-15

    Patients with childhood heart disease are living longer and entering adulthood, and may undergo implantable cardioverter-defibrillator (ICD) implantation to reduce the risk of sudden death. We evaluated the characteristics of adult patients with congenital heart disease or left ventricular noncompaction cardiomyopathy (LVNC) in the National Cardiovascular Disease Registry ICD Registry and determined ICD-related in-hospital complications. Patients with LVNC or transposition of the great arteries, tetralogy of Fallot, Ebstein's anomaly, atrial septal defect, ventricular septal defect, or common ventricle were identified in the registry. In-hospital complications were compared among different diagnoses using the chi-square test for categorical variables and the F-test in analyses of variance for continuous variables. A total of 3,077 patients were identified. The mean age was 48.0 ± 16.0 years, and 39.9% were female. Single-chamber ICDs were implanted in 25.2%, dual chamber in 41.9%, and cardiac resynchronization in 30.8%. Intraprocedural or postprocedural complications occurred in 70 patients (2.3%); there were 6 in-hospital deaths (0.2%). The most frequent complications were acute lead dislodgments, pneumothorax, and hematomas. Patients with Ebstein's anomaly had the greatest complication rate (8.3%, p = 0.03). The complication rate was 1.55% in single-chamber devices, 1.86% in dual chamber, and 3.5% in cardiac resynchronization (p < 0.001). For initial implants, the complication rate was 2.55%, 1.62% in generator replacements, and 8.77% in lead revisions (p = 0.001). In conclusion, in this large contemporary adult cohort of congenital heart disease and LVNC patients who underwent ICD implant procedures, periprocedural complication rates were low. Lead-related risks predominated. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Impact of shocks on mortality in patients with ischemic or dilated cardiomyopathy and defibrillators implanted for primary prevention.

    Directory of Open Access Journals (Sweden)

    Florian Streitner

    Full Text Available BACKGROUND: Emerging interest is seen in the paradox of defibrillator shocks for ventricular tachyarrhythmia and increased mortality risk. Particularly in patients with dilated cardiomyopathy (DCM, the prognostic importance of shocks is unclear. The purpose of this study was to compare the outcome after shocks in patients with ischemic cardiomyopathy (ICM or DCM and defibrillators (ICD implanted for primary prevention. METHODS AND RESULTS: Data of 561 patients were analyzed (mean age 68.6±10.6 years, mean left ventricular ejection fraction 28.6±7.3%. During a median follow-up of 49.3 months, occurrence of device therapies and all-cause mortality were recorded. 74 out of 561 patients (13.2% experienced ≥1 appropriate and 51 out of 561 patients (9.1% ≥1 inappropriate shock. All-cause mortality was 24.2% (136 out of 561 subjects. Appropriate shock was associated with a trend to higher mortality in the overall patient population (HR 1.48, 95% CI 0.96-2.28, log rank p = 0.072. The effect was significant in ICM patients (HR 1.61, 95% CI 1.00-2.59, log rank p = 0.049 but not in DCM patients (HR 1.03, 95% CI 0.36-2.96, log rank p = 0.96. Appropriate shocks occurring before the median follow-up revealed a much stronger impact on mortality (HR for the overall patient population 2.12, 95% CI 1.24-3.63, p = 0.005. The effect was driven by ICM patients (HR 2.48, 95% CI 1.41-4.37, p = 0.001, as appropriate shocks again did not influence survival of DCM patients (HR 0.63, 95% CI 0.083-4.75, p = 0.65. Appropriate shocks occurring after the median follow-up and inappropriate shocks occurring at any time revealed no impact on survival in any of the groups (p = ns. CONCLUSION: Appropriate shocks are associated with reduced survival in patients with ICM but not in patients with DCM and ICDs implanted for primary prevention. Furthermore, the negative effect of appropriate shocks on survival in ICM patients is only evident within the

  17. A Pregnancy with Severe Hypertrophic Obstructive Cardiomyopathy after Surgery for an Implantable Cardioverter Defibrillator: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Takashi Mitsui

    2016-01-01

    Full Text Available Hypertrophic obstructive cardiomyopathy (HOCM is cardiac hypertrophy of ventricular myocardium with left ventricular outflow tract obstruction. We report a pregnancy with HOCM after defibrillator implantation surgery. The patient was a 33-year-old nulligravida and was categorized as New York Heart Association class II. Her brain natriuretic peptide (BNP level was 724.6 pg/dL at preconception. She received careful pregnancy management. However, because frequent uterine contractions were observed at 25 weeks and 6 days of pregnancy, she was hospitalized, and magnesium sulfate was started as a tocolytic agent. At 27 weeks and 5 days of pregnancy, she had respiratory discomfort and orthopnea with a sudden decrease in peripheral oxygen saturation. Cardiac ultrasonography showed a worsened condition of HOCM and her BNP level was 1418.0 pg/mL. We performed an emergent cesarean section and she delivered a boy weighing 999 g. The Apgar score was 8 and 9 points at 1 and 5 minutes, respectively. The mother’s heart failure quickly improved after birth and she was discharged at 10 days postoperatively. Fluctuations in circulatory dynamics during pregnancy may sometimes exacerbate heart disease. Therefore, the risks should be fully explained and careful assessment of cardiac function should be performed during pregnancy in patients with severe HOCM.

  18. A case of “non-compact myocardium” cardiomyopathy diagnosed and treated by means of implantable cardioverter-defibrillators

    Directory of Open Access Journals (Sweden)

    И. Е. Доровских

    2016-11-01

    Full Text Available The article presents a case of non-compact myocardium primary diagnosis of a woman of 45 years, who has complained of general weakness, pain in the heart and interruptions in its work, shortness of breath and dizziness from her youth. A peculiarity of this case is that the patient was initially examined locally and diagnosed with rheumatic heart disease and then with ischemic heart disease. However, the age at which the disease made its debut, the characteristics of pain in the heart, the severity of symptoms of arrhythmia and heart failure, no changes of blood lipid spectrum and other signs of atherosclerosis raised doubts about the presence of angina. Since the clinical syndromes showed a marked violation of all basic functions of the heart muscle, differential diagnostics with diffuse myocarditis was performed, which led to the latter’s exclusion. During ECHO CT followed by transesophageal ECHO at the Academy’s Cardiac Surgery Department, primary “non-compact myocardium” cardiomyopathy was diagnosed. Provided the patient’s complex arrhythmias, a cardioverter-defibrillator with a pacemaker function was implanted and her health significantly improved.Received 9 August 2016. Accepted 28 September 2016.Funding: The study has not been sponsored or awarded a grant.Conflict of interest:The authors declare no conflict of interest.

  19. Performance of the 2015 International Task Force Consensus Statement Risk Stratification Algorithm for Implantable Cardioverter-Defibrillator Placement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy.

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    Orgeron, Gabriela M; Te Riele, Anneline; Tichnell, Crystal; Wang, Weijia; Murray, Brittney; Bhonsale, Aditya; Judge, Daniel P; Kamel, Ihab R; Zimmerman, Stephan L; Tandri, Harikrishna; Calkins, Hugh; James, Cynthia A

    2018-02-01

    Ventricular arrhythmias are a feared complication of arrhythmogenic right ventricular dysplasia/cardiomyopathy. In 2015, an International Task Force Consensus Statement proposed a risk stratification algorithm for implantable cardioverter-defibrillator placement in arrhythmogenic right ventricular dysplasia/cardiomyopathy. To evaluate performance of the algorithm, 365 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients were classified as having a Class I, IIa, IIb, or III indication per the algorithm at baseline. Survival free from sustained ventricular arrhythmia (VT/VF) in follow-up was the primary outcome. Incidence of ventricular fibrillation/flutter cycle length 10%/year Class I; 15.5 [confidence interval 11.1-21.6] versus 1% to 10%/year Class IIa). In addition, the algorithm did not differentiate survival free from ventricular fibrillation/flutter between Class I and IIa patients ( P =0.97) or for VT/VF in Class I and IIa primary prevention patients ( P =0.22). Adding Holter results (International Task Force Consensus classification differentiated risks. While the algorithm differentiates arrhythmic risk well overall, it did not distinguish ventricular fibrillation/flutter risks of patients with Class I and IIa implantable cardioverter-defibrillator indications. Limited differentiation was seen for primary prevention cases. As these are vital uncertainties in clinical decision-making, refinements to the algorithm are suggested prior to implementation. © 2018 American Heart Association, Inc.

  20. Prediction of Appropriate Shocks Using 24-Hour Holter Variables and T-Wave Alternans After First Implantable Cardioverter-Defibrillator Implantation in Patients With Ischemic or Nonischemic Cardiomyopathy.

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    Seegers, Joachim; Bergau, Leonard; Expósito, Pascal Muñoz; Bauer, Axel; Fischer, Thomas H; Lüthje, Lars; Hasenfuß, Gerd; Friede, Tim; Zabel, Markus

    2016-07-01

    In patients treated with implantable cardioverter defibrillator (ICD), prediction of both overall survival and occurrence of shocks is important if improved patient selection is desired. We prospectively studied the predictive value of biomarkers and indexes of cardiac and renal function and spectral microvolt T-wave alternans testing and 24-hour Holter variables in a population who underwent first ICD implantation. Consecutive patients in sinus rhythm with ischemic or dilated cardiomyopathy scheduled for primary or secondary prophylactic ICD implantation were enrolled. Exercise microvolt T-wave alternans and 24-hour Holter for number of ventricular premature contractions (VPCs), deceleration capacity, heart rate variability, and heart rate turbulence were done. Death of any cause and first appropriate ICD shock were defined as end points. Over 33 ± 15 months of follow-up, 36 of 253 patients (14%) received appropriate shocks and 39 of 253 patients (15%) died. Only 3 of 253 patients (1%) died after receiving at least 1 appropriate shock. In univariate analyses, New York Heart Association class, ejection fraction, N-terminal pro brain-type natriuretic peptide (NT-proBNP), renal function, ICD indication, deceleration capacity, heart rate variability, and heart rate turbulence were predictive of all-cause mortality and VPC number and deceleration capacity predicted first appropriate shock. NT-proBNP (≥1,600 pg/ml) was identified as the only independent predictor of all-cause mortality (hazard ratio 3.0, confidence interval 1.3 to 7.3, p = 0.014). In contrast, VPC number predicted appropriate shocks (hazard ratio 2.3, confidence interval 1.0 to 5.5, p = 0.047) as the only independent risk marker. In conclusion, NT-proBNP is a strong independent predictor of mortality in a typical prospective cohort of newly implanted patients with ICD, among many electrocardiographic and clinical variables studied. Number of VPCs was identified as a predictor of appropriate shocks

  1. Introducing ICD-resistant mortality as an end point to evaluate the clinical efficacy of an implantable cardioverter-defibrillator in ischaemic cardiomyopathy.

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    Floré, Vincent; Vandenberk, Bert; Belmans, Ann; Garweg, Christophe; Ector, Joris; Willems, Rik

    2018-02-01

    A new end point called ICD-resistant mortality was evaluated to assess the clinical efficacy of ICD implantations. In 302 ICD patients with ischaemic cardiomyopathy, we investigated which clinical parameters predicted useful ICD implantations using cumulative incidence competing risk analysis. Implantation was deemed clinically useful when the ICD provided appropriate therapy and the patient survived implantation by 1 year and the first shock by 30 days. ICD-resistant mortality (ICDRM) was defined as death within 30 days after the first shock, within 1 year of implantation or without previous appropriate ICD therapy. After 5 years, ICDRM occurred in 23% of implantations, while 36% were clinically useful. After multivariable analysis, ICDRM was associated with LVEF <35% (HR: 2.63; p = .005), beta-blocker dose <50% (HR: 2.0; p = .01) and anterior or diffuse infarct location (HR: 3.61; p = .001 and HR: 2.89; p = .02). Useful ICD implantations were associated with beta-blocker dose <50% (HR: 1.64; p = .02) and non-anterior infarct location (HR: 3.22 vs anterior and 1.59 vs diffuse; combined p<.001). Five years after implantation, an ICD could be classified as useful in 1 out of 3, while ICDRM occurred in one out of four patients. At 10 years, up to 80% of implantations could be categorized. Lower LVEF was related with significantly higher incidence of ICDRM. Anterior infarcts were associated with more ICDRM and less useful implantations than non-anterior infarcts. Future risk stratification for ICD should focus more on the discrimination between arrhythmic risk, probably preventable by ICDs and ICD-resistant mortality risk.

  2. Role of implantable cardioverter defibrillator in non-ischemic cardiomyopathy: a systematic review and meta-analysis of prospective randomized clinical trials.

    Science.gov (United States)

    Romero, Jorge; Chaudhary, Rahul; Garg, Jalaj; Lupercio, Florentino; Shah, Neeraj; Gupta, Rahul; Nazir, Talha; Bozorgnia, Babak; Natale, Andrea; Di Biase, Luigi

    2017-09-01

    A mortality benefit in patients with implantable cardioverter defibrillator (ICD) in ischemic cardiomyopathy is well established. However, the benefit of ICD implantation in non-ischemic cardiomyopathy (NICM) on total mortality remains uncertain. We performed a systematic review and meta-analysis of randomized controlled trials (RCT) evaluating the role of primary prevention ICD in NICM patients. We performed a systematic review on PubMed, The Cochrane Library, EMBASE, EBSCO, Web of Science, and CINAHL databases from the inception through February 2017 to identify RCT evaluating the role of ICD in NICM patients. Mantel-Haenszel risk ratio (RR) fixed effects model was used to summarize data across treatment arms. If heterogeneity (I 2 ) ≥25, random effects model was used instead. We analyzed a total of 2573 patients from five RCTs comparing ICD with medical therapy in patients with NICM. The mean follow up for the trials was 48 ± 22 months. There was a significant reduction in (a) all-cause mortality (RR 0.84, 95% CI 0.71-0.99, p = 0.03) and (b) sudden cardiac death (RR 0.47, 95% CI 0.30-0.73, p < 0.001) in ICD group versus medical therapy. Our analysis demonstrates that the use of ICD for primary prevention is associated with a reduction in all-cause mortality and SCD in patients with NICM.

  3. The clinical use of stress echocardiography in non-ischaemic heart disease: recommendations from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

    Science.gov (United States)

    Lancellotti, Patrizio; Pellikka, Patricia A; Budts, Werner; Chaudhry, Farooq A; Donal, Erwan; Dulgheru, Raluca; Edvardsen, Thor; Garbi, Madalina; Ha, Jong-Won; Kane, Garvan C; Kreeger, Joe; Mertens, Luc; Pibarot, Philippe; Picano, Eugenio; Ryan, Thomas; Tsutsui, Jeane M; Varga, Albert

    2016-11-01

    A unique and highly versatile technique, stress echocardiography (SE) is increasingly recognized for its utility in the evaluation of non-ischaemic heart disease. SE allows for simultaneous assessment of myocardial function and haemodynamics under physiological or pharmacological conditions. Due to its diagnostic and prognostic value, SE has become widely implemented to assess various conditions other than ischaemic heart disease. It has thus become essential to establish guidance for its applications and performance in the area of non-ischaemic heart disease. This paper summarizes these recommendations. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.

  4. Prognostic value of cardiovascular magnetic resonance imaging for life-threatening arrhythmia detected by implantable cardioverter-defibrillator in Japanese patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Hen, Yasuki; Tsugu-Yagawa, Mayuko; Iguchi, Nobuo; Utanohara, Yuko; Takada, Kaori; Machida, Haruhiko; Takara, Ayako; Teraoka, Kunihiko; Inoue, Kanki; Takamisawa, Itaru; Takayama, Morimasa; Yoshikawa, Tsutomu

    2018-01-01

    Implantable cardioverter-defibrillator (ICD) is effective to prevent sudden death in HCM patients. We reviewed ICD records to analyze the relation between life-threatening arrhythmia and late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) in Japanese hypertrophic cardiomyopathy (HCM) patients. In 102 consecutive patients (median age 63 years, 63 males) implanted with an ICD after CMR with gadolinium enhancement (median follow-up 2.8 years), the outcome of life-threatening arrhythmic events (appropriate ICD interventions for ventricular tachycardia or ventricular fibrillation) was examined. Appropriate interventions rate were 10.3% per year for secondary prevention and 7.4% per year for primary prevention. The annualized ICD-related complication rate was 3.7%. 43/91 patients (47%) implanted ICD for primary prevention had maximum wall thickness ≥20 mm plus LGE in ≥4 of 17 left ventricular segments (cut-off value obtained from ROC curve); the appropriate ICD intervention rate was significantly higher in this group than in other patients group (annualized event rate, 11.1 vs. 4.6%; log-rank P = 0.038). A combination of myocardial hypertrophy and LGE is a useful outcome predictive factor for life-threatening ventricular arrhythmia in Japanese HCM patients.

  5. Functional recovery of patients with ischemic cardiomyopathy treated with coronary artery bypass surgery and concomitant intramyocardial bone marrow mononuclear cell implantation: A long term follow-up study

    Directory of Open Access Journals (Sweden)

    Trifunović Zoran

    2015-01-01

    Full Text Available Background/Aim. Intramyocardial bone marrow mononuclear cells (BMMNC implantation concomitant to coronary artery bypass grafting (CABG surgery as an option for regenerative therapy in chronic ischemic heart failure was tested in a very few number of studies, with not consistent conclusions regarding improvement in left ventricular function, and with a follow-up period between 6 months and 1 year. This study was focused on testing of the hypothesis that intramyocardial BMMNC implantation, concomitant to CABG surgery in ischemic cardiomyopathy patients, leads to better postoperative long-term results regarding the primary endpoint of conditional status-functional capacity and the secondary endpoint of mortality than CABG surgery alone in a median follow-up period of 5 years. Methods. A total of 30 patients with ischemic cardiomyopathy and the median left ventricular ejection fraction (LVEF of 35.9 ± 4.7% were prospectively and randomly enrolled in a single center interventional, open labeled clinical trial as two groups: group I of 15 patients designated as the study group to receive CABG surgery and intramyocardial implantation of BMMNC and group II of 15 patients as the control group to receive only the CABG procedure. All the patients in both groups received the average of 3.4 ± 0.7 implanted coronary grafts, and all of them received the left internal mammary artery (LIMA to the left anterior descending (LAD and autovenous to other coronaries. Results. The group with BMMNC and CABG had the average of 17.5 ± 3.8 injections of BMMNC suspension with the average number of injected bone marrow mononuclear cells of 70.7 ± 32.4 × 106 in the total average volume of 5.7 ± 1.5 mL. In this volume the average count of CD34+ and CD133+ cells was 3.96 ± 2.77 × 106 and 2.65 ± 1.71 × 106, respectively. All the patients were followed up in 2.5 to 7.5 years (median, 5 years. At the end of the follow-up period, significantly more patients from the group

  6. Dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Salvatore, M.; Cuocolo, A.

    1988-01-01

    Radionuclide techniques are easily obtainable, noninvasive examinations that provide useful information in the evaluation, diagnosis and management of patients with dilated cardiomyopathy. The gated blood pool scan allows the assessment of ventricular size, configuration, and wall and septal thickness. These data allow the functional class of the cardiomyopathy (congestive, restrictive or hypertrophic) to be defined. Often THallium-201 myocardial perfusion imaging adds further information and is particularly useful in distinguishing congestive cardiomyopathy from severe coronary artery disease and in depicting septal abnormalities in hipertrophic cardiomyopathy. Useful as these techniques are, they are not substitutes for conventional approaches to diagnosis. Careful history taking and physical examination, as well as scrutiny of the electrocardiogram, chest X-ray and echocardiogram should be standard practice for the evaluation of patients with suspected cardiomyopathy. Judicious use of noninvasive techniques may obviate the need for cardiac catheterization in many patients

  7. Microarray analysis for delineating the gene expression in biopsies of gastrocnemius muscle of patients with chronic critical limb ischaemia compared with non-ischaemic controls.

    Science.gov (United States)

    Freund, Daniel; Brilloff, Silke; Ghazy, Tamer; Kirschner, Stephan; Gäbel, Gabor; Hinterseher, Irene; Weiss, Norbert; Mahlmann, Adrian

    2018-03-20

    Microarray analysis has been carried out in this pilot study to compare delineated gene expression profiles in the biopsies of skeletal muscle taken from patients with chronic critical limb ischaemia (CLI) and non-ischaemic control subjects. Biopsy of gastrocnemius muscle was obtained from six patients with unreconstructed CLI referred for surgical major amputation. As control, biopsies of six patients undergoing elective knee arthroplasty without evidence of peripheral arterial occlusive disease were taken. The differences in gene expression associated with angiogenic processes in specimens obtained from ischaemic and non-ischaemic skeletal muscle were confirmed by quantitative real-time polymerase chain reaction (PCR) analysis. Compared with non-ischaemic skeletal muscle biopsy of chronic-ischaemic skeletal muscle contained 55 significantly up-regulated and 45 down-regulated genes, out of which 64 genes had a known genetic product ((Author, please revise sentence)). Tissue samples of ischaemic muscle were characterized by increased expression of cell survival factors (e. g. tissue factor pathway inhibitor 2) in combination with reduced expression of cell proliferation effectors (e. g. microfibrillar-associated protein 5 and transferrin receptor). The expression of growth factors (e. g. early growth response 3 and chemokine receptor chemokine C-X-C motif ligand 4) which play a central role in arterial and angiogenic processes and anti-angiogenetic factors (e. g. pentraxin 3) were increased in chronic ischaemic skeletal muscle. An increased expression of extracellular matrix proteins (e. g. cysteine-rich angiogenic inducer 61) was also observed. Gene expression profiles in biopsies of gastrocnemius muscle in patients with chronic critical limb ischaemia showed an increase in pro-survival factors, extracellular matrix protein deposition, and impaired proliferation, compared with non-ischaemic controls. Further studies are required to analyse the endogenous repair

  8. Implante de cardio-desfibrilador em gestantes com cardiomiopatia hipertrófica Implantable cardioverter-defibrillator in pregnant women with hypertrophic cardiomyopathy

    OpenAIRE

    Leonardo Jorge Cordeiro de Paula; Henrique Barbosa Ribeiro; Roberto Márcio de Oliveira Júnior; Kátia Regina da Silva

    2010-01-01

    Descrevemos os casos de duas gestantes portadoras de cardiomiopatia hipertrófica com alto risco de morte súbita arrítmica, que foram submetidas a implante de cardioversor-desfibrilador automático (CDI) no intercurso da gestação. O momento para a realização do procedimento e os cuidados necessários para o implante do CDI durante a gestação são discutidos e foram os principais objetivos deste relato.We describe the successful implantation of a cardioverter-defibrillator (ICD) in two pregnant wo...

  9. Peripartum Cardiomyopathy

    African Journals Online (AJOL)

    pregnant women. The pathophysiology is poorly understood. Echocardiography is central to diagnosis of PPCM and effective treatment monitoring in patients of PPCM. The outcome ... Keywords: Cardiomyopathy, Echocardiography, Peripartum, Pregnancy ... complicated by pre‑eclampsia, gestational hypertension,.

  10. Dilated cardiomyopathy

    Science.gov (United States)

    ... Valve replacement or repair For advanced cardiomyopathy: A heart transplant may be recommended if standard treatments have not ... accountability. A.D.A.M. is among the first to achieve this important distinction for online health ...

  11. Restrictive Cardiomyopathy

    Science.gov (United States)

    ... can be mistaken for a condition called constrictive pericarditis. This condition causes the sac-like membrane around ... inflamed and thickened. Surgery can usually correct constrictive pericarditis. On the other hand, restrictive cardiomyopathy cannot be ...

  12. Mitochondrial cardiomyopathies

    Directory of Open Access Journals (Sweden)

    Ayman W. El-Hattab

    2016-07-01

    Full Text Available Mitochondria are found in all nucleated human cells and perform a variety of essential functions, including the generation of cellular energy. Mitochondria are under dual genome control. Only a small fraction of their proteins are encoded by mitochondrial DNA (mtDNA while more than 99% of them are encoded by nuclear DNA (nDNA. Mutations in mtDNA or mitochondria-related nDNA genes result in mitochondrial dysfunction leading to insufficient energy production required to meet the needs of various organs, particularly those with high energy requirements, including the central nervous system, skeletal and cardiac muscles, kidneys, liver, and endocrine system. Because cardiac muscles are one of the high energy demanding tissues, cardiac involvement occurs in mitochondrial diseases with cardiomyopathies being one of the most frequent cardiac manifestations found in these disorders. Cardiomyopathy is estimated to occur in 20-40% of children with mitochondrial diseases. Mitochondrial cardiomyopathies can vary in severity from asymptomatic status to severe manifestations including heart failure, arrhythmias, and sudden cardiac death. Hypertrophic cardiomyopathy is the most common type; however, mitochondrial cardiomyopathies might also present as dilated, restrictive, left ventricular noncompaction, and histiocytoid cardiomyopathies. Cardiomyopathies are frequent manifestations of mitochondrial diseases associated with defects in electron transport chain (ETC complexes subunits and their assembly factors, mitochondrial tRNAs, rRNAs, ribosomal proteins, and translation factors, mtDNA maintenance, and coenzyme Q10 synthesis. Other mitochondrial diseases with cardiomyopathies include Barth syndrome, Sengers syndrome, TMEM70-related mitochondrial complex V deficiency, and Friedreich ataxia.

  13. Orage rythmique chez un patient porteur d’une cardiomyopathie dilatée et un défibrillateur automatique implantable (DAI)

    Science.gov (United States)

    Ikama, Stéphane Méo; Makani, Jospin; Ellenga-Mbolla, Bertrand; Ondze-Kafata, Louis Igor; Gombet, Thierry Raoul; Kimbally-Kaky, Gisèle

    2017-01-01

    Les arythmies ventriculaires graves sont fréquentes au cours de l’insuffisance cardiaque, mettant en jeu le pronostic vital du fait du risque accru de mort subite. Leur prise en charge efficace reste limitée en Afrique Subsaharienne, du fait des moyens limités ou non disponibles comme le défibrillateur automatique implantable (DAI). Nous rapportons l’observation d’un patient de 56 ans, porteur d’une cardiomyopathie dilatée non ischémique à fraction d’éjection du ventricule gauche (FEVG) très abaissée, et qui a bénéficié en 2012 de l’implantation d’un DAI en prévention primaire de mort subite pour des arythmies ventriculaires. Le traitement d’entretien associait un diurétique, un IEC, et un anti-vitamine K. Le patient a présenté au mois de novembre 2014 des épisodes itératifs de décharges électriques délivrées par le DAI, sans sensation de palpitations suggestives d’épisodes d’arythmies. L’examen clinique est pauvre, en particulier pas de signes d’insuffisance cardiaque. L’interrogation du DAI a objectivé de nombreux épisodes de tachycardie et fibrillation ventriculaires ayant justifié le traitement par ATP ou par chocs de 15 joules. Le patient est mis sous amiodarone et bêtabloquant. L’évolution a été favorable avec un recul de trois mois, marquée par la reprise d’une vie normale, sans nouvel épisode de choc. Les anti-arythmiques gardent une importance capitale en cas d’arythmies ventriculaires graves, même en présence d’un DAI. PMID:28761607

  14. Implantable cardioverter-defibrillator elective generator replacement: a procedure for all?

    Science.gov (United States)

    Barra, Sergio; Goonewardene, Manoj; Heck, Patrick; Begley, David; Virdee, Munmohan; Fynn, Simon; Grace, Andrew; Agarwal, Sharad

    2016-03-01

    Six risk stratification scores have been developed to estimate mortality risk in patients receiving an implantable cardioverter-defibrillator (ICD). This study aims at validating and comparing these risk scores in patients having elective ICD generator replacement (GR) and assessing the outcome of patients submitted to this procedure. Two hundred twenty three consecutive patients with ischaemic or non-ischaemic dilated cardiomyopathy submitted to elective ICD GR and followed-up for 44 ± 19 months were included. We evaluated which of six previously developed risk scores could predict post-discharge all-cause mortality risk in this context with the highest efficacy. Comparisons between these scores were made using receiver-operating characteristic curves and the integrated discrimination improvement (IDI) index. We further assessed risk of appropriate ICD therapies and all-cause mortality following ICD GR. The prognostic utility of the six scores was assessed by calculating the AUC for follow-up all-cause mortality prediction: Goldenberg - 0.758 ± 0.042, p gender (OR 6.6, CI 95% 0.8-55, p = 0.082) and history of atrial fibrillation (OR 2.28, CI 95% 1.1-4.5, p = 0.019). Current prediction scores are useful in predicting mortality risk of patients considered for ICD generator replacement and can potentially help identify patients who may not benefit from continuous ICD treatment due to high mortality rates regardless of the ICD.

  15. Syncope in high-risk cardiomyopathy patients with implantable defibrillators: frequency, risk factors, mechanisms, and association with mortality: results from the multicenter automatic defibrillator implantation trial-reduce inappropriate therapy (MADIT-RIT) study.

    Science.gov (United States)

    Ruwald, Martin H; Okumura, Ken; Kimura, Takeshi; Aonuma, Kazutaka; Shoda, Morio; Kutyifa, Valentina; Ruwald, Anne-Christine H; McNitt, Scott; Zareba, Wojciech; Moss, Arthur J

    2014-02-04

    There is a relative paucity of studies investigating the mechanisms of syncope among heart failure patients with implantable cardioverter-defibrillators, and it is controversial whether nonarrhythmogenic syncope is associated with increased mortality. The Multicenter Automatic Defibrillator Implantation Trial-Reduce Inappropriate Therapy (MADIT-RIT) randomized 1500 patients to 3 different implantable cardioverter-defibrillator programming arms: (1) Conventional programming with therapy for ventricular tachycardia ≥170 bpm; (2) high-rate cutoff with therapy for ventricular tachycardia ≥200 bpm and a monitoring zone at 170 to 199 bpm, and (3) prolonged 60-second delay with a monitoring zone before therapy. Syncope was a prespecified safety end point that was adjudicated independently. Multivariable Cox models were used to identify risk factors associated with syncope and to analyze subsequent risk of mortality. During follow-up, 64 of 1500 patients (4.3%) had syncope. The incidence of syncope was similar across the 3 treatment arms. Prognostic factors for all-cause syncope included the presence of ischemic cardiomyopathy (hazard ratio [HR], 2.48; 95% confidence interval [CI], 1.42-4.34; P=0.002), previous ventricular arrhythmias (HR, 2.99; 95% CI, 1.18-7.59; P=0.021), left ventricular ejection fraction ≤25% (HR, 1.65; 95% CI, 0.98-2.77; P=0.059), and younger age (by 10 years; HR, 1.25; 95% CI, 1.00-1.52; P=0.046). Syncope was associated with increased risk of death regardless of its cause (arrhythmogenic syncope: HR, 4.51; 95% CI, 1.39-14.64, P=0.012; nonarrhythmogenic syncope: HR, 2.97; 95% CI, 1.07-8.28, P=0.038). Innovative programming of implantable cardioverter-defibrillators with therapy for ventricular tachycardia ≥200 bpm or a long delay is not associated with increased risk of arrhythmogenic or all-cause syncope, and syncope caused by slow ventricular tachycardias (<200 bpm) is a rare event. The clinical risk factors associated with syncope are

  16. Cardiomyopathy in neurological disorders.

    Science.gov (United States)

    Finsterer, Josef; Stöllberger, Claudia; Wahbi, Karim

    2013-01-01

    According to the American Heart Association, cardiomyopathies are classified as primary (solely or predominantly confined to heart muscle), secondary (those showing pathological myocardial involvement as part of a neuromuscular disorder) and those in which cardiomyopathy is the first/predominant manifestation of a neuromuscular disorder. Cardiomyopathies may be further classified as hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, or unclassified cardiomyopathy (noncompaction, Takotsubo-cardiomyopathy). This review focuses on secondary cardiomyopathies and those in which cardiomyopathy is the predominant manifestation of a myopathy. Any of them may cause neurological disease, and any of them may be a manifestation of a neurological disorder. Neurological disease most frequently caused by cardiomyopathies is ischemic stroke, followed by transitory ischemic attack, syncope, or vertigo. Neurological disease, which most frequently manifests with cardiomyopathies are the neuromuscular disorders. Most commonly associated with cardiomyopathies are muscular dystrophies, myofibrillar myopathies, congenital myopathies and metabolic myopathies. Management of neurological disease caused by cardiomyopathies is not at variance from the same neurological disorders due to other causes. Management of secondary cardiomyopathies is not different from that of cardiomyopathies due to other causes either. Patients with neuromuscular disorders require early cardiologic investigations and close follow-ups, patients with cardiomyopathies require neurological investigation and avoidance of muscle toxic medication if a neuromuscular disorder is diagnosed. Which patients with cardiomyopathy profit most from primary stroke prevention is unsolved and requires further investigations. Copyright © 2013 Elsevier Inc. All rights reserved.

  17. Cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Wiese, Signe; Hove, Jens D; Bendtsen, Flemming

    2014-01-01

    Cirrhosis is known to cause alterations in the systemic haemodynamic system. Cirrhotic cardiomyopathy designates a cardiac dysfunction that includes impaired cardiac contractility with systolic and diastolic dysfunction, as well as electromechanical abnormalities in the absence of other known...... causes of cardiac disease. This condition is primarily revealed by inducing physical or pharmacological stress, but echocardiography is excellent at revealing diastolic dysfunction and might also be used to detect systolic dysfunction at rest. Furthermore, measurement of circulating levels of cardiac...

  18. Hypertrophic cardiomyopathy presenting with 3rd-degree atrioventricular block.

    OpenAIRE

    Rosen, K L; Cameron, R W; Bigham, P J; Neish, S R

    1997-01-01

    A 15-year-old boy presented with exercise intolerance and near syncope. Electrocardiography showed 3rd-degree atrioventricular block. He was treated emergently with temporary transvenous ventricular pacing. Two-dimensional echocardiography revealed hypertrophic cardiomyopathy. Subsequently, a permanent, transvenous, dual-chamber pacemaker was implanted. Hypertrophic cardiomyopathy rarely causes complete atrioventricular block. It is particularly unusual for the presenting symptoms of hypertro...

  19. [Ventricular tachyarrhythmias in patients with cardiomyopathy

    DEFF Research Database (Denmark)

    Henningsen, K.; Christensen, A.H.; Svendsen, Jesper Hastrup

    2008-01-01

    INTRODUCTION: The purpose of this study was to determine the number and distribution of cardiomyopathies as the aetiology of ventricular tachyarrhythmias among patients discharged from the Department of Cardiology, Rigshospitalet. MATERIALS AND METHODS: The study was a retrospective review......), 57 (45%) patients had arrhythmogenic right ventricular cardiomyopathy (ARVC) and 13 (10%) had hypertrophic cardiomyopathy (HCM). The average age was 44 years for HCM, 41 years for ARVC and 58 years for DCM. The majority of the patients were male. ICD treatment was used in 95% of the patients...... cardiology department. Implantation of an ICD device has a central position in the treatment of patients with cardiomyopathy and ventricular tachyarrythmias and is primarily used as a prophylactic treatment Udgivelsesdato: 2008/6/16...

  20. Hypertrophic cardiomyopathy: a review.

    Science.gov (United States)

    Hensley, Nadia; Dietrich, Jennifer; Nyhan, Daniel; Mitter, Nanhi; Yee, May-Sann; Brady, MaryBeth

    2015-03-01

    Hypertrophic cardiomyopathy (HCM) is a relatively common disorder that anesthesiologists encounter among patients in the perioperative period. Fifty years ago, HCM was thought to be an obscure disease. Today, however, our understanding and ability to diagnose patients with HCM have improved dramatically. Patients with HCM have genotypic and phenotypic variability. Indeed, a subgroup of these patients exhibits the HCM genotype but not the phenotype (left ventricular hypertrophy). There are a number of treatment modalities for these patients, including pharmacotherapy to control symptoms, implantable cardiac defibrillators to manage malignant arrhythmias, and surgical myectomy and septal ablation to decrease the left ventricular outflow obstruction. Accurate diagnosis is vital for the perioperative management of these patients. Diagnosis is most often made using echocardiographic assessment of left ventricular hypertrophy, left ventricular outflow tract gradients, systolic and diastolic function, and mitral valve anatomy and function. Cardiac magnetic resonance imaging also has a diagnostic role by determining the extent and location of left ventricular hypertrophy and the anatomic abnormalities of the mitral valve and papillary muscles. In this review on hypertrophic cardiomyopathy for the noncardiac anesthesiologist, we discuss the clinical presentation and genetic mutations associated with HCM, the critical role of echocardiography in the diagnosis and the assessment of surgical interventions, and the perioperative management of patients with HCM undergoing noncardiac surgery and management of the parturient with HCM.

  1. Risk stratification for implantable cardioverter defibrillator therapy: the role of the wearable cardioverter-defibrillator.

    Science.gov (United States)

    Klein, Helmut U; Goldenberg, Ilan; Moss, Arthur J

    2013-08-01

    The benefit of implantable cardioverter-defibrillator (ICD) therapy depends upon appropriate evaluation of a persisting risk of sudden death and estimation of the patient's overall survival. Assessment of a stable and unchangeable arrhythmogenic substrate is often difficult. Structural abnormality and ventricular dysfunction, the two major risk parameters, may recover, and heart failure symptoms can improve so that ICD therapy may not be indicated. Risk stratification can take time while the patient continues to be at high risk of arrhythmic death, and patients may need temporary bridging by a defibrillator in cases of interrupted ICD therapy. The wearable cardioverter-defibrillator (WCD) combines a long-term electrocardiogram (ECG)-monitoring system with an external automatic defibrillator. The LIfeVest® (ZOLL, Pittsburgh, PA, USA) is composed of a garment, containing two defibrillation patch electrodes on the back, and an elastic belt with a front-defibrillation patch electrode and four non-adhesive ECG electrodes, connected to a monitoring and defibrillation unit. The WCD is a safe and effective tool to terminate ventricular tachycardia/ventricular fibrillation events, unless a conscious patient withholds shock delivery. It may be used in patients in the early phase after acute myocardial infarction with poor left ventricular function, after acute coronary revascularization procedures (percutaneous coronary intervention or coronary artery bypass grafting) and reduced left ventricular ejection fraction (≤35%), in patients with acute heart failure in non-ischaemic cardiomyopathy of uncertain aetiology and prognosis. The WCD may be helpful in subjects with syncope of assumed tachyarrhythmia origin or in patients with inherited arrhythmia syndromes. The WCD may replace ICD implantation in patients waiting for heart transplantation or who need a ventricular-assist device. This review describes the technical details and characteristics of the WCD, discusses its

  2. Cardiomyopathies and anaesthesia

    Directory of Open Access Journals (Sweden)

    Rajiv Juneja

    2017-01-01

    Full Text Available Cardiomyopathy is considered as a heart muscle disease of multiple aetiologies, unlike other cardiac diseases related to a definitive pathophysiology. With more and more research and with the advent of genetic analysis pin pointing the disease causing mutations, causative factors have been defined and classifications and definitions have changed over time. Patients with these conditions present to anaesthesiologists in elective and emergency situations, placement of automated internal cardioverter defibrillator (AICD devices or biventricular pacing but may also be diagnosed at anaesthetic pre-assessment. We describe cardiomyopathies such as dilated cardiomyopathy, hypertrophic cardiomyopathy, post-partum cardiomyopathy and Takotsubo cardiomyopathy in brief and their anaesthetic management.

  3. MR imaging in cardiomyopathies

    International Nuclear Information System (INIS)

    Miller, S.; Riessen, R.

    2005-01-01

    According to the WHO classification, cardiomyopathies are a group of diseases which are associated with myocardial dysfunction and can be classified either as primary or secondary cardiomyopathies. Genetic disorders have been identified in certain primary cardiomyopathies, however often the etiology remains unknown. The term ''secondary cardiomyopathy'' is used to specify diseases with the clinical indications of a cardiomyopathy, but can be attributed to a certain pathophysiological mechanism such as exposure to toxic substances, metabolic syndromes or systemic diseases. Based on morphological and functional criteria, primary cardiomyopathies are divided into dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and restrictive cardiomyopathy (RCM). During the last two decades MR imaging has emerged to a well established diagnostic tool for the understanding and treatment of cardiomyopathies. Morphological and functional information can be achieved with a high level of accuracy and reproducibility. Tissue alteration of the myocardium can be detected assessing regional contrast enhancement, T1- and T2-signal intensities and chemical shift phenomena. This article describes characteristic aspects of MR imaging for the diagnosis of primary and secondary cardiomyopathies. (orig.)

  4. Peripartum cardiomyopathy

    International Nuclear Information System (INIS)

    Velasquez V, Jorge E; Duque R, Mauricio

    2008-01-01

    Peripartum cardiomyopathy is a clinical entity with a variable frequency according to the zone of the study. It is characterized by a systolic dysfunction of the left ventricle and posterior appearance of heart failure symptoms that occur during the last month of pregnancy and the first post-partum months. Its etiology isn't still clear, but different theories are proposed based on inflammatory, infectious and autoimmune processes. Alterations related to oxidative stress that could largely explain this pathology were recently described. Its clinical presentation has a big similitude with all other causes of heart failure although atypical presentations have been described. Its diagnosis requires a high suspicion level and must be considered in any woman with symptoms of heart failure during the peripartum. The conventional treatment of chronic heart failure that includes beta-blockers, angiotensin converting enzyme inhibitors and diuretics, in addition to the advances in diagnosis and management of acute heart failure, allowed changing the history of the disease by lowering mortality and recovering systolic function of the left ventricle. Gestations posterior to the development of this entity will depend on the complete recovery of heart function without lowering the risk of recurrence. There still remain many questions to answer in areas like etiology, risk factors, treatment and prognosis markers that may allow to prevent and to manage in an appropriate and safe way both the mother and her son.

  5. Assessment of chest pain in hypertrophic cardiomyopathy using exercise thallium-201 myocardial scintigraphy

    International Nuclear Information System (INIS)

    Pitcher, D.; Wainwright, R.; Maisey, M.; Curry, P.; Sowton, E.

    1980-01-01

    Exercise thallium-201 myocardial scintigraphy was performed in 23 patients with hypertrophic cardiomyopathy. Eighteen patients presented with chest pain which was a persistent symptom in 11. Selective coronary arteriography was performed in 16 patients and showed normal coronary arteries in 15 and insignificant luminal irregularities in one patient. Eighteen patients had abnormal scintigrams. Three had an abnormal distribution of tracer entirely attributable to asymmetric septal hypertrophy, whereas 15 had discrete tracer uptake defects which could not be explained solely by myocardial hypertrophy. In this latter group of patients three scintigraphic patterns were identified: (1) in 10 patients defects were seen in scintigrams immediately after exercise but not in delayed images obtained four to six hours later. Eight of these patients had chest pain. (2) Four patients had uptake defects seen in both initial and delayed images. One patient had chest pain. (3) In three patients, one of whom had chest pain, tracer defects were seen only in delayed images and were not apparent in the initial scintigrams. Chest pain occurred in eight out of 10 patients with scintigraphic evidence of myocardial ischaemia but was present in only three out of 13 patients with non-ischaemic scintigrams. The value of exercise thallium-201 myocardial imaging as a diagnostic technique in hypertrophic cardiomyopathy appears limited. Scintigraphic evidence of regional myocardial ischaemia in the absence of significant coronary artery disease, however, contributes to an understanding of the mechanism of angina production in patients with hypertrophic cardiomyopathy. (author)

  6. Cardiomyopathy in Vici syndrome

    African Journals Online (AJOL)

    Solaf M. Elsayed

    2018-01-12

    Jan 12, 2018 ... Cardiomyopathy in Vici syndrome. Dear Editor,. A 7 month old boy was referred to our clinic with provisional diagnosis of Pompe disease because of his Echocardiographic find- ings of hypertrophic cardiomyopathy. Although the patient had severe hypotonia and increased CPK level characteristics of ...

  7. Genetics Home Reference: arrhythmogenic right ventricular cardiomyopathy

    Science.gov (United States)

    ... cardiomyopathy Merck Manual Consumer Version: Cardiomyopathy Merck Manual Consumer Version: Overview of Abnormal Heart Rhythms My46 Trait Profile Orphanet: Arrhythmogenic right ventricular cardiomyopathy Orphanet: Familial isolated ...

  8. Imaging and 1-day kinetics of intracoronary stem cell transplantation in patients with idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Lezaic, Luka; Socan, Aljaz; Peitl, Petra Kolenc; Poglajen, Gregor; Sever, Matjaz; Cukjati, Marko; Cernelc, Peter; Vrtovec, Bojan

    2016-01-01

    Background: Stem cell transplantation is an emerging method of treatment for patients with cardiovascular disease. There are few studies completed or ongoing on stem cell therapy in patients with idiopathic dilated cardiomyopathy (IDCM). Information on stem cell homing and distribution in the myocardium after transplantation might provide important insight into effectiveness of transplantation procedure. Aim: To assess early engraftment, retention and migration of intracoronarily transplanted stem cells in the myocardium of patients with advanced dilated cardiomyopathy of non-ischaemic origin using stem cell labeling with 99m Tc-exametazime (HMPAO). Materials, methods: Thirty-five patients with IDCM and advanced heart failure were included in the study. Autologous hematopoietic (CD34 +) stem cells were harvested by peripheral blood apheresis after bone marrow stimulation, labeled with 99m Tc-HMPAO, tested for viability and injected into coronary vessel supplying areas of myocardium selected by myocardial perfusion scintigraphy as dysfunctional yet viable. Imaging was performed 1 h and 18 h after transplantation. Results: Myocardial stem cell retention ranged from 0 to 1.44% on early and 0–0.97% on delayed imaging. Significant efflux of stem cells occurred from site of delivery in this time period (p < 0.001). Stem cell viability was not affected by labeling. Conclusion: Stem cell labeling with 99m Tc-HMPAO is a feasible method for stem cell tracking after transplantation in patients with IDCM.

  9. Sex differences in cardiomyopathies

    NARCIS (Netherlands)

    Meyer, Sven; van der Meer, Peter; van Tintelen, J. Peter; van den Berg, Maarten P.

    Cardiomyopathies are a heterogeneous group of heart muscle diseases with a variety of specific phenotypes. According to the contemporary European Society of Cardiology classification, they are classified into hypertrophic (HCM), dilated (DCM), arrhythmogenic right ventricular (ARVC), restrictive

  10. Takotsubo (Stress) Cardiomyopathy

    Science.gov (United States)

    ... the American Heart Association Cardiology Patient Page Takotsubo (Stress) Cardiomyopathy Scott W. Sharkey , John R. Lesser , Barry ... heart contraction has returned to normal. Importance of Stress In 85% of cases, takotsubo is triggered by ...

  11. New perspectives in Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel)

    1998-01-01

    textabstractHypertrophic cardiomyopathy is a primary cardiac disorder with a heterogeneous expression. Although relatively uncommon, the disease has been studied extensively as appears from the numerous studies that have explored specific facets of hypertrophic cardiomyopathy. This review will focus

  12. Danon’s disease as a cause of hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2015-01-01

    Full Text Available Hypertrophic cardiomyopathy is the most common inherited disease of the myocardium. The causes of the disease are heterogeneous; its primary form results from mutations in the genes encoding cardiac sarcomeric proteins; its secondary (metabolic and syndromic forms develop due to mutations in the genes encoding non-sarcomeric proteins. Glycogenosis is the most common cause of the metabolic ones of hypertrophic cardiomyopathy. Danon’s disease (lysosome-associated membrane protein 2 (LAMP2-cardiomyopathy is a form of glycogenosis and it is characterized by a typical triad: hypertrophic cardiomyopathy, mental retardation, and skeletal myopathy. The disease occurs with mutations in the LAMP2 gene; X-linked dominant inheritance. LAMP2-cardiomyopathy does not virtually differ in its clinical manifestations from the severe form of hypertrophic cardiomyopathy, which results from mutations in the sarcomeric protein genes. The disease is characterized by a poor progressive course with the high probability of causing sudden death or with the progression of severe heart failure. Implantation of a cardioverter defibrillator is a main method to prevent sudden cardiac death. 

  13. Exercise Rehabilitation in Pediatric Cardiomyopathy

    OpenAIRE

    Somarriba, Gabriel; Extein, Jason; Miller, Tracie L.

    2008-01-01

    Children with cardiomyopathy carry significant risk of morbidity and mortality. New research and technology have brought about significant advancements to the diagnosis and clinical management of children with cardiomyopathy. However, currently heart transplantation remains the standard of care for children with symptomatic and progressive cardiomyopathy. Cardiovascular rehabilitation programs have yielded success in improving cardiac function, overall physical activity, and quality of life i...

  14. HYPERTROPHIC CARDIOMYOPATHY IN MULTIMORBIDITY

    Directory of Open Access Journals (Sweden)

    A. I. Lakhonina

    2016-06-01

    Full Text Available Aspects of diagnosis, difficulties in the diagnosis and optimal therapeutic strategies in patient with hypertrophic cardiomyopathy and comorbid conditions such as arterial hypertension, ischemic heart disease, dyslipidemia, diabetes mellitus type 2, stenosis of the left renal artery, obesity are reviewed on the example of clinical case. Hypertrophic cardiomyopathy combined with multimorbidity conditions requires a high-quality medical management, where the main goal is to improve the quality and duration of patient's life. This goal is being achieved by optimizing patient's lifestyle and assigning only the minimum amount of medications. Necessity of careful diagnosis of hypertrophic cardiomyopathy, evaluation of the risk of sudden death and search of optimal treatment in patients with multimorbidity pathology are demonstrated in clinical case.

  15. Treatment of Chagas cardiomyopathy.

    Science.gov (United States)

    Botoni, Fernando A; Ribeiro, Antonio Luiz P; Marinho, Carolina Coimbra; Lima, Marcia Maria Oliveira; Nunes, Maria do Carmo Pereira; Rocha, Manoel Otávio C

    2013-01-01

    Chagas' disease (ChD), caused by the protozoa Trypanosoma cruzi (T. cruzi), was discovered and described by the Brazilian physician Carlos Chagas in 1909. After a century of original description, trypanosomiasis still brings much misery to humanity and is classified as a neglected tropical disease prevalent in underdeveloped countries, particularly in South America. It is an increasing worldwide problem due to the number of cases in endemic areas and the migration of infected subjects to more developed regions, mainly North America and Europe. Despite its importance, chronic chagas cardiomyopathy (CCC) pathophysiology is yet poorly understood, and independently of its social, clinical, and epidemiological importance, the therapeutic approach of CCC is still transposed from the knowledge acquired from other cardiomyopathies. Therefore, the objective of this review is to describe the treatment of Chagas cardiomyopathy with emphasis on its peculiarities.

  16. Cardiomyopathy induced by anthracycline

    International Nuclear Information System (INIS)

    Quiroz, Isabel; Espinoza, Gerson; Poveda, Maria; Flores, Walter

    2002-01-01

    Anthracycline cardiomyopathy is less frequently encountered nowadays, due to the well recognized dose limitations and cardiac monitoring protocols used by chemotherapy centers. However, it is a condition that will persist due to the sensitivity of some patients to these drugs and the necessity for large doses to be used for certain individuals. We have demonstrated the benefit of angiotensin converting enzyme inhibitor therapy and would consider introducing these compounds at the earliest opportunity. The use of probucol and vitamins as antioxidants capable of preventing the onset of cardiomyopathy in humans appears to require further investigation but may significantly reduce the incidence of this condition in the future. (The author)

  17. Cardiomyopathy in Vici syndrome

    African Journals Online (AJOL)

    Solaf M. Elsayed

    2018-01-12

    Jan 12, 2018 ... The syndrome was first described in 1988, in two brothers with ACC, bilateral cataracts, ... syndrome with brain anomalies, cardiomyopathy, and severe intellectual disability. Case Reports in Genetics 2011, Volume 2011. [6] Finocchi A, Angelino G, Cantarutti N, Corbari M, Bevivino E, Cascioli S, et al.

  18. Cardiomyopathy Following Latrodectus Envenomation

    Directory of Open Access Journals (Sweden)

    Levine, Michael

    2010-12-01

    Full Text Available Latrodectus envenomations are common throughout the United States and the world. While many envenomations can result in catecholamine release with resultant hypertension and tachycardia, myocarditis is very rare. We describe a case of a 22- year-old male who sustained a Latrodectus envenomation complicated by cardiomyopathy. [West J Emerg Med. 2010; 11(5:521-523.

  19. Hypertrophic Cardiomyopathy Association

    Science.gov (United States)

    ... be donated to Hypertrophic Cardiomyopathy Association. iGive.com - Online Shopping Joing iGive.com to earn money for the ... it works, check out the iGive website . AmazonSmile - Online Shopping Amazon donates 0.5% of the purchase price ...

  20. Leopard syndrome and hypertrophic cardiomyopathy: an association related to sudden death.

    Science.gov (United States)

    Antunes, Murillo de Oliveira; Arteaga, Edmundo; Matsumoto, Afonso Yoshikiro; Ianni, Barbara Maria

    2009-06-01

    We describe an uncommon association between Leopard syndrome and hypertrophic cardiomyopathy in a 27-year-old woman, who was little symptomatic and came for sudden death risk stratification and prevention. She has a rare syndrome, whose symptoms are maculae over the body and abnormalities in eyes, genital organs, heart and in growth. Association of hypertrophic cardiomyopathy with sudden death risk factors determined the implantation of cardioverter-defibrillator (ICD) for primary prevention.

  1. Left Ventricular Systolic Dysfunction Induced by Ventricular Ectopy: a Novel Model for PVC-induced Cardiomyopathy

    Science.gov (United States)

    Huizar, Jose F.; Kaszala, Karoly; Potfay, Jonathan; Minisi, Anthony J.; Lesnefsky, Edward J.; Abbate, Antonio; Mezzaroma, Eleonora; Chen, Qun; Kukreja, Rakesh C.; Hoke, Nicholas N.; Thacker, Leroy R.; Ellenbogen, Kenneth A.; Wood, Mark A.

    2011-01-01

    Background Premature ventricular contractions (PVCs) commonly coexist with cardiomyopathy. Recently, PVCs have been identified as possible cause of cardiomyopathy. We developed a PVC-induced cardiomyopathy animal model using a novel premature pacing algorithm to assess timeframe and reversibility of this cardiomyopathy and examine the associated histopathological abnormalities. Methods and Results Thirteen mongrel dogs were implanted with a specially programmed pacemaker capable of simulating ventricular extrasystoles. Animals were randomly assigned to either 12 weeks of bigeminal PVCs (n=7) or no PVCs (control, n=6). Continuous 24-hr Holter corroborated ventricular bigeminy in the PVC group (PVC 49.8% vs. control PVC group developed cardiomyopathy with a significant reduction in left ventricular (LV) ejection fraction (PVC 39.7±5.4% vs. control 60.7±3.8%, PPVC 33.3±3.5mm vs. control 23.7±3.6mm, PPVC group. PVC-induced cardiomyopathy was resolved within 2-4 weeks after discontinuation of PVCs. No inflammation, fibrosis, or changes in apoptosis and mitochondrial oxidative phosphorylation were observed with PVC-induced cardiomyopathy. Conclusions This novel PVC animal model demonstrates that frequent PVCs alone can induce a reversible form of cardiomyopathy in otherwise structurally normal hearts. PVC-induced CM lacks gross histopathological and mitochondrial abnormalities seen in other canine models of CM. PMID:21576277

  2. Takotsubo (Stress Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Justin J Hourmozdi

    2017-01-01

    Full Text Available History of present illness: A 59-year-old male presented to the emergency department in shock from pneumonia. The patient was initially afebrile, pulse rate 120 beats per minute, blood pressure 117/69 mmHg, respiratory rate 42 breaths per minute, pulse oximetry 94% on a non-rebreather mask and a lactate of 14 mmol/L. He became progressively more hypotensive despite fluid resuscitation and was started on norepinephrine. Shortly after, the patient developed torsades de pointes that was terminated with intravenous push magnesium. His initial ECG had shown sinus tachycardia; however, repeat ECG showed ST-segment elevation in the inferolateral leads and the patient had troponin I elevation that peaked at 16 ng/mL. Significant findings: Bedside echocardiography showed the findings consistent with Takotsubo cardiomyopathy. Echocardiographic images are shown in subxiphoid (A and apical four chamber (B views. Note the apical ballooning appearance (asterisk of the left ventricle (LV. Discussion: Formal echocardiography confirmed features classic for Takotsubo (stress cardiomyopathy, including globally depressed left ventricle (LV ejection fraction, systolic apical ballooning appearance of the LV, mid and apical segments of LV depression, and hyper kinesis of the basal walls. Takotsubo cardiomyopathy is a syndrome known to cause ST-segment elevation on ECG, transient LV dysfunction, and dysrhythmia in the absence of acute obstructive coronary disease. There is no consensus on diagnostic criteria; however, these criteria are commonly used: 1 transient hypokinesis, akinesis, or dyskinesis in the LV mid-segments with or without apical involvement; regional wall motion abnormalities that extend beyond a single epicardial vascular distribution; and frequently, but not always, a stressful trigger 2 the absence of acute coronary disease or angiographic evidence of acute plaque rupture 3 new ECG abnormalities (ST-segment elevation and/or T-wave inversion or modest

  3. Magnetic resonance imaging assessment of intraventricular dyssynchrony and delayed enhancement as predictors of response to cardiac resynchronization therapy in patients with heart failure of ischaemic and non-ischaemic etiologies

    Energy Technology Data Exchange (ETDEWEB)

    Petryka, Joanna, E-mail: joannapetryka@hotmail.com [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw (Poland); Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Miśko, Jolanta [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Department of Radiology, Institute of Cardiology, Warsaw (Poland); Przybylski, Andrzej [Department of Arrhythmia, Institute of Cardiology, Warsaw (Poland); Śpiewak, Mateusz [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw (Poland); Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Małek, Łukasz A. [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Department of Cardiology and Interventional Angiology, Institute of Cardiology, Warsaw (Poland); Werys, Konrad [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Mazurkiewicz, Łukasz [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Department of Cardiomyopathy, Institute of Cardiology, Warsaw (Poland); Gepner, Katarzyna [Department of Coronary Artery Disease, Institute of Cardiology, Warsaw (Poland); Croisille, Pierre [Creatis Laboratory, UMR CNRS 5515, INSERM, U1044, CHU Saint-Etienne, Universite de Lyon (France); Demkow, Marcin [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw (Poland); Rużyłło, Witold [Institute of Cardiology, Warsaw (Poland)

    2012-10-15

    Purpose: To assess the value of dyssynchrony and myocardial viability assessment by cardiac magnetic resonance (CMR) in prediction of response to cardiac resynchronization therapy (CRT) in patients with heart failure (HF) of both ischaemic and non-ischaemic etiologies. Materials and methods: Patients scheduled for CRT in NYHA class II–IV, left ventricular ejection fraction <35%, QRS ≥ 120 ms were included. Tagged cine and late gadolinium enhancement (LGE) images were performed. Dyssynchrony was assessed with inTag toolbox and LGE was quantified using cutoff value at half of maximal signal in the scar. Cardiopulmonary exercise test, echocardiography and blood testing for NT-proBNP levels were done at baseline and 6 months after CRT. Results: 52 patients (age 60.3 ± 13 years) were included. 26 patients (50%) met response criteria. The ischaemic etiology of HF was more frequent (69% vs. 31%, p = 0.002), the percent of LGE was higher (7.7% [0–13.5%] vs. 19.0% (0–31.9%], p = 0.013), regional vector of circumferential strain variance (RVV) was lower (0.27 ± 0.08 vs. 0.34 ± 0.09, p = 0.009) and uniformity of radial strain was higher (0.72 ± 0.25 vs. 0.56 ± 0.29, p = 0.046) in non-responders vs. responders. Multivariate logistic regression showed that RVV predicted response to CRT (HR 2.3, 95% CI 1.02–5.02, p = 0.0430) independently of LGE and the etiology of heart failure. In the subgroup of patients with ischaemic HF the extend of transmural scar within myocardium was higher in non-responders vs. responders (26.3% vs. 15.0% respectively, p = 0.01) and was a predictor of response to CRT in univariable analysis (HR 0.87, 95% CI 0.77–0.98, p = 0.025) providing the sensitivity of 76% and specificity of 75% at the cutoff point of 18% in the prediction of poor response to CRT. In patients with non-ischaemic HF QRS was wider (162 ms vs. 140 ms, p = 0.04), regional vector of strain variance (RVV) was higher (0.39 vs. 0.25, p = 0.002) and uniformity of radial

  4. Reversible ischaemia in hypertrophic cardiomyopathy.

    OpenAIRE

    Thomson, H.; Fong, W.; Stafford, W.; Frenneaux, M.

    1995-01-01

    Atypical and typical chest pains are common symptoms in patients with hypertrophic cardiomyopathy. Some of these chest pains seem to be caused by ischaemia. It is difficult to objectively demonstrate ischaemia in hypertrophic cardiomyopathy. The first line treatment for chest pain considered to be ischaemic in patients with hypertrophic cardiomyopathy is the use of either a beta blocker or calcium blocker. Septal myectomy can be effective in patients with symptoms refractory to conventional t...

  5. Intracoronary allogeneic cardiosphere-derived stem cells are safe for use in dogs with dilated cardiomyopathy.

    Science.gov (United States)

    Hensley, Michael Taylor; Tang, Junnan; Woodruff, Kathleen; Defrancesco, Teresa; Tou, Sandra; Williams, Christina M; Breen, Mathew; Meurs, Kathryn; Keene, Bruce; Cheng, Ke

    2017-08-01

    Cardiosphere-derived cells (CDCs) have been shown to reduce scar size and increase viable myocardium in human patients with mild/moderate myocardial infarction. Studies in rodent models suggest that CDC therapy may confer therapeutic benefits in patients with non-ischaemic dilated cardiomyopathy (DCM). We sought to determine the safety and efficacy of allogeneic CDC in a large animal (canine) model of spontaneous DCM. Canine CDCs (cCDCs) were grown from a donor dog heart. Similar to human CDCs, cCDCs express CD105 and are slightly positive for c-kit and CD90. Thirty million of allogeneic cCDCs was infused into the coronary vessels of Doberman pinscher dogs with spontaneous DCM. Adverse events were closely monitored, and cardiac functions were measured by echocardiography. No adverse events occurred during and after cell infusion. Histology on dog hearts (after natural death) revealed no sign of immune rejection from the transplanted cells. © 2017 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  6. Hypertrophic Cardiomyopathy: A Review

    OpenAIRE

    Houston, Brian A; Stevens, Gerin R

    2015-01-01

    Hypertrophic cardiomyopathy (HCM) is a global disease with cases reported in all continents, affecting people of both genders and of various racial and ethnic origins. Widely accepted as a monogenic disease caused by a mutation in 1 of 13 or more sarcomeric genes, HCM can present catastrophically with sudden cardiac death (SCD) or ventricular arrhythmias or insidiously with symptoms of heart failure. Given the velocity of progress in both the fields of heart failure and HCM, we present a revi...

  7. MRI of the cardiomyopathies

    Energy Technology Data Exchange (ETDEWEB)

    Di Cesare, Ernesto E-mail: ernesto.dicesare@cc.univaq.it

    2001-06-01

    We examined the potentialities of Magnetic resonance imaging (MRI) in the evaluation of the main cardiomyopathies: hypertrophic, dilated, restrictive and arrhythmogenic right ventricular. The hypertrophic cardiomyopathy is generally adequately investigated by echocardiography, that well defines the myocardial thickening and the obstruction of the left ventricular output. However, by echocardiography we still have difficulties in the evaluation of the apex of the left ventricle and the right ventricle involvement. MRI provides a complete evaluation of the heart with a clear evidence also of the echocardiographic dark zones by means of a clear evidence of the apex of the right ventricle. The dilated form is also well investigated by MRI that provides a clear evaluation of the volumes, mass and ejection fraction by means of the 3D analysis including conditions of the ventricular remodelling. Moreover, this technique helps in the differential diagnosis of acute myocarditis. In the acute phase of myocarditis (first 2 weeks), in fact, the myocardium produces high signal intensity on the T2 weighted sequences due to the presence of oedema. The third form of cardiomyopathy is the restrictive one, characterised by reduced diastolic filling and diastolic volume, normality of the systolic function and parietal thickness, interstitial fibrosis and enlargement of both atria. The mean potentiality of MRI is related to the differential diagnosis with constrictive pericarditis. Only in the former, the pericardium appears irregularly thickened with areas exceeding 4 mm of pericardial thickness. Finally, the right ventricular arrhythmogenic cardiomyopathy represents the main indication to MRI evaluation. With this imaging modality we are can obtain a clear morpho-functional evaluation of the right ventricle and distinguish the intramyocardial adipose substitution characterised by areas of high signal in the myocardium.

  8. MRI of the cardiomyopathies

    International Nuclear Information System (INIS)

    Di Cesare, Ernesto

    2001-01-01

    We examined the potentialities of Magnetic resonance imaging (MRI) in the evaluation of the main cardiomyopathies: hypertrophic, dilated, restrictive and arrhythmogenic right ventricular. The hypertrophic cardiomyopathy is generally adequately investigated by echocardiography, that well defines the myocardial thickening and the obstruction of the left ventricular output. However, by echocardiography we still have difficulties in the evaluation of the apex of the left ventricle and the right ventricle involvement. MRI provides a complete evaluation of the heart with a clear evidence also of the echocardiographic dark zones by means of a clear evidence of the apex of the right ventricle. The dilated form is also well investigated by MRI that provides a clear evaluation of the volumes, mass and ejection fraction by means of the 3D analysis including conditions of the ventricular remodelling. Moreover, this technique helps in the differential diagnosis of acute myocarditis. In the acute phase of myocarditis (first 2 weeks), in fact, the myocardium produces high signal intensity on the T2 weighted sequences due to the presence of oedema. The third form of cardiomyopathy is the restrictive one, characterised by reduced diastolic filling and diastolic volume, normality of the systolic function and parietal thickness, interstitial fibrosis and enlargement of both atria. The mean potentiality of MRI is related to the differential diagnosis with constrictive pericarditis. Only in the former, the pericardium appears irregularly thickened with areas exceeding 4 mm of pericardial thickness. Finally, the right ventricular arrhythmogenic cardiomyopathy represents the main indication to MRI evaluation. With this imaging modality we are can obtain a clear morpho-functional evaluation of the right ventricle and distinguish the intramyocardial adipose substitution characterised by areas of high signal in the myocardium

  9. The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporary management of adult patients with cardiomyopathies.

    Science.gov (United States)

    Charron, Philippe; Elliott, Perry M; Gimeno, Juan R; Caforio, Alida L P; Kaski, Juan Pablo; Tavazzi, Luigi; Tendera, Michal; Maupain, Carole; Laroche, Cécile; Rubis, Pawel; Jurcut, Ruxandra; Calò, Leonardo; Heliö, Tiina M; Sinagra, Gianfranco; Zdravkovic, Marija; Kavoliuniene, Aušra; Felix, Stephan B; Grzybowski, Jacek; Losi, Maria-Angela; Asselbergs, Folkert W; García-Pinilla, José Manuel; Salazar-Mendiguchia, Joel; Mizia-Stec, Katarzyna; Maggioni, Aldo P

    2018-01-24

    The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001). By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe. Published on behalf of the European Society of Cardiology. All rights reserved.

  10. Medical therapy versus interventional therapy in hypertropic obstructive cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Seggewiss Hubert

    2000-10-01

    Full Text Available Abstract Medical treatment in symptomatic patients with hypertrophic obstructive cardiomyopathy aims to reduce the outflow tract gradients, and to improve diastolic dysfunction and rhythm disorders. Surgical myectomy is the standard treatment in patients with drug refractory symptoms. Since the early 1990s, dual-chamber (DDD-pacemaker implantation and percutaneous transluminal septal myocardial ablation by alcohol-induced septal branch occlusion have widened treatment options in this subgroup of patients. An overview of medical and interventional treatment is presented.

  11. New perspectives in Hypertrophic Cardiomyopathy

    OpenAIRE

    Kofflard, Marcel

    1998-01-01

    textabstractHypertrophic cardiomyopathy is a primary cardiac disorder with a heterogeneous expression. Although relatively uncommon, the disease has been studied extensively as appears from the numerous studies that have explored specific facets of hypertrophic cardiomyopathy. This review will focus on the anatomic abnormalities, the prevalence, symptoms and clinical outcome, therapeutic interventions and genetic mutations responsible for the disease.

  12. Cardiomyopathy in the pediatric patients

    Directory of Open Access Journals (Sweden)

    Shi-Min Yuan

    2018-04-01

    Full Text Available Pediatric cardiomyopathies are a group of myocardial diseases with complex taxonomies. Cardiomyopathy can occur in children at any age, and it is a common cause of heart failure and heart transplantation in children. The incidence of pediatric cardiomyopathy is increasing with time. They may be associated with variable comorbidities, which are most often arrhythmia, heart failure, and sudden death. Medical imaging technologies, including echocardiography, cardiac magnetic resonance, and nuclear cardiology, are helpful in reaching a diagnosis of cardiomyopathy. Nevertheless, endomyocardial biopsy is the final diagnostic method of diagnosis. Patients warrant surgical operations, such as palliative operations, bridging operations, ventricular septal maneuvers, and heart transplantation, if pharmaceutical therapies are ineffective. Individual therapeutic regimens due to pediatric characteristics, genetic factors, and pathogenesis may improve the effects of treatment and patients' survival. Key Words: cardiomyopathy, classification, pediatrics

  13. An International External Validation Study of the 2014 European Society of Cardiology Guideline on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (Evidence from HCM)

    NARCIS (Netherlands)

    O'Mahony, Constantinos; Jichi, Fatima; Ommen, Steve R.; Christiaans, Imke; Arbustini, Eloisa; Garcia-Pavia, Pablo; Cecchi, Franco; Olivotto, Iacopo; Kitaoka, Hiroaki; Gotsman, Israel; Carr-White, Gerald; Mogensen, Jens; Antoniades, Loizos; Mohiddin, Saidi; Maurer, Mathew S.; Tang, Hak Chiaw; Geske, Jeffrey B.; Siontis, Konstantinos C.; Mahmoud, Karim; Vermeer, Alexa; Wilde, Arthur; Favalli, Valentina; Guttmann, Oliver; Gallego-Delgado, Maria; Dominguez, Fernando; Tanini, Ilaria; Kubo, Toru; Keren, Andre; Bueser, Teofila; Waters, Sarah; Issa, Issa F.; Malcolmson, James; Burns, Thomas; Sekhri, Neha; Hoeger, Christopher W.; Omar, Rumana Z.; Elliott, Perry M.

    2017-01-01

    Background -Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require prophylactic implantable cardioverter defibrillator (ICD) is challenging. In 2014, the European Society of Cardiology (ESC) proposed a new risk stratification method

  14. An International External Validation Study of the 2014 European Society of Cardiology Guideline on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (Evidence from HCM)

    DEFF Research Database (Denmark)

    O'Mahony, Constantinos; Jichi, Fatima; Ommen, Steve R

    2018-01-01

    Background -Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require prophylactic implantable cardioverter defibrillator (ICD) is challenging. In 2014, the European Society of Cardiology (ESC) proposed a new risk stratification method...

  15. ACE I/D polymorphism in Indian patients with hypertrophic cardiomyopathy and dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Rai, Taranjit Singh; Dhandapany, Perundurai Subramaniam; Ahluwalia, Tarun Veer Singh

    2008-01-01

    The study was carried to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) polymorphism with the risk of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM)....

  16. Peripartum cardiomyopathy in Denmark

    DEFF Research Database (Denmark)

    Ersbøll, Anne S; Johansen, Marianne; Damm, Peter

    2017-01-01

    and searched for cardiomyopathy and heart failure ICD-10 diagnoses in a period of nine months before to 12 months after a delivery from 1 January 2005 through 31 December 2014. Diagnoses were validated and additional data were extracted from patient charts. A total of 61 women met the inclusion criteria...... equalling 1 in 10 149 deliveries. The majority recovered left ventricular systolic function within one year, but 14.8% suffered a major adverse event with 3.3% mortality, 8.2% mechanical circulatory support requirement and/or heart transplantation and 4.9% persistent severe heart failure. Half of the women...

  17. Microfibrillar cardiomyopathy: A rare case

    Directory of Open Access Journals (Sweden)

    Narender Kumar

    2011-01-01

    Full Text Available Microfibrillar cardiomyopathy is a very rare cause of restrictive cardiomyopathy (RCM. The index case was a male patient who presented with shortness of breath and pedal edema. Further clinical investigations favored a clinical diagnosis of RCM. An endomyocardial biopsy revealed subendocardial and interstitial hyaline eosinophillic material resembling amyloid that did not stain with Congo red. An electron microscopic examination showed that this material was composed of twisted linear and bundles of tangled microfibrils. The etiology of the microfibrillar deposition is currently unknown. The pathologists should entertain the diagnosis of microfibrillar cardiomyopathy in suspected cases of amyloidosis that are negative for Congo red.

  18. Genetics of cardiomyopathies in children

    Directory of Open Access Journals (Sweden)

    Matteo Vatta

    2011-08-01

    Full Text Available Cardiomyopathies are diseases of the heart muscle leading to heart failure and/or an increased risk of arrhythmogenic sudden cardiac death. These disorders represent a major cause of morbidity and mortality in children. In childhood forms of cardiomyopathy, genetic etiologies are frequent, but non-genetic or acquired causes, such viral infection, also play a significant role. In the last twenty years, the genetic causes of cardiomyopathies have been increasingly identified and clinical correlations are beginning to be defined. Here we present an overview of the recent advances in our understanding of the genetics of cardiomyopathies in children and what is known about the pathophysiological mechanisms underlying these gene-related forms of disease.

  19. New insights into cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Søren; Hove, Jens D; Dixen, Ulrik

    2013-01-01

    Cirrhotic cardiomyopathy designates a cardiac dysfunction, which includes reduced cardiac contractility with systolic and diastolic dysfunction, and presence of electrophysiological abnormalities in particular prolongation of the QT interval. Several pathophysiological mechanisms including reduced...... beta-receptor function seem involved in the autonomic and cardiac dysfunction. Cirrhotic cardiomyopathy can be revealed by tissue Doppler imaging but is best demasked by physical or pharmacological stress. Liver transplantation may revert cardiac dysfunction but surgery and shunt insertion may also...

  20. Arrhythmogenic right ventricular cardiomyopathy.

    Science.gov (United States)

    Soni, Roopali; Oade, Yvette

    2011-08-24

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease of the heart muscle that causes ventricular tachyarrhythmias and sudden death in young people and athletes. It results in fibrofatty replacement of the right ventricle, and the subepicardial region of the left ventricle. It is the most common cause of sudden cardiac death in young people after hypertrophic heart disease. Diagnosis can be difficult and at present there is no cure for ARVC. Prevention of sudden death is the most important management strategy. Paediatricians need to be aware of the possibility of ARVC in adolescents and young adults presenting with palpitations, fatigue, syncope or cardiac arrest. The authors present two cases of apparently healthy teenage boys who died suddenly and unexpectedly. Postmortem examination of the myocardium was strongly suggestive of ARVC in both cases.

  1. [Peripartum cardiomyopathy: a case report].

    Science.gov (United States)

    Karchmer-Krivitzky, S; Espinosa-Fernández, R; Sánchez-Aranda, A; LópezRioja, M J; Monzalbo-Núñez, D

    2016-08-01

    Peripartum cardiomyopathy also known as cardiomyopathy associated with pregnancy, is rarely a cause of heart failure, it affects pregnant or puerperal women in the first 5 months. Although the first case reported was in 1849, it was recognized until 1930. In 2010 the European Society of Cardiology Working Group on peripartum cardiomyopathy, defined this pathology as an idiopathic cardiomyopathy that affects pregnant women between the third trimester and five months after delivery. Characterized by a left ventricular failure with an ejection fraction of ≤45% and an end-diastolic dimension ≥2.7 cm/m2 , in absence of an identifiable cause of heart failure. We report a case of a 39-year-old patient, diagnosed with a peripartum cardiomyopathy in the early puerperium, characterized by hypertension, tachycardia, dyspnea and oxygen desaturation. The transesophageal echocardiogram reported heart failure, a hypokinetic left ventricle and a ventricular failure with an ejection fraction <40%. We could not identify an other cause to justify heart failure. Multidisciplinary management was administered successfully. The importance of this article relies in the fact that eripartum cardiomyopathy has a high morbidity and mortality. The impact of this pathology is unknow in our country. Here we establish and discuss the multidisciplinary management held in our hospital with this specific patient in order to improve the prognosis on future occasions.

  2. DIFFERENTIAL DIAGNOSIS OF HYPERTROPHIC CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2017-01-01

    Full Text Available Hypertrophic cardiomyopathy is the most common form of cardiomyopathy, occurring in childhood, occurring when a gene is mutated that encodes proteins of sarcomeric and non-sarcomeric complexes. The diagnosis of the disease is based on the data of echocardiography, revealing structural changes in the heart muscle according to the type of hypertrophy, while the genesis of these changes remains unclear. The causes of hypertrophic cardiomyopathy in childhood are diverse. Of great importance is the early diagnosis of metabolic forms of hypertrophic cardiomyopathy, so in some cases regress of hypertrophy is possible against the background of enzyme-substitution or other drug therapy. The article presents a clinical (cardiac and extracardiac symptoms and laboratory markers of hypertrophic cardiomyopathy with mutations of genes of proteins of the sarcomeric complex, congenital metabolic disorders (glycogenoses, lysosomal pathology, fatty acid metabolism disorders, and mitochondrial diseases, genetic syndromes (Noonan, LEOPARD, Costello, cardio-fascial-cutaneous, neuromuscular diseases. The criteria for differential diagnosis of genetic forms of hypertrophic cardiomyopathy and myocardial hypertrophy in athletes are presented. 

  3. Sequential Atrioventricular Pacing in Patients With Hypertrophic Cardiomyopathy: An 18-year Experience.

    Science.gov (United States)

    Jurado Román, Alfonso; Montero Cabezas, José M; Rubio Alonso, Belén; García Tejada, Julio; Hernández Hernández, Felipe; Albarrán González-Trevilla, Agustín; Velázquez Martín, María T; Coma Samartín, Raúl; Rodríguez García, Jesús; Tascón Pérez, Juan C

    2016-04-01

    Controversy persists regarding the role of sequential atrioventricular pacing in patients with obstructive hypertrophic cardiomyopathy and disabling symptoms. The aim of this study was to evaluate the effect of pacing on symptoms, dynamic gradient, and left ventricular function in patients with hypertrophic cardiomyopathy. From 1991 to 2009, dual-chamber pacemakers were implanted in 82 patients with obstructive hypertrophic cardiomyopathy and disabling symptoms despite optimal medical therapy. Sequential pacing was performed with a short atrioventricular delay. Clinical and echocardiographic parameters were measured before and immediately after implantation and after a long follow-up (median, 8.5 years [range, 1-18 years]). The New York Heart Association functional class was immediately reduced after pacemaker implantation in 95% of patients (P hypertrophic cardiomyopathy improves functional class and reduces dynamic gradient and mitral regurgitation immediately after pacemaker implantation and at final follow-up. Prolonged ventricular pacing has no negative effects on systolic or diastolic function in these patients. Copyright © 2015 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  4. Congenital complete atrioventricular block and dilated cardiomyopathy: new light for an old disease.

    Science.gov (United States)

    Paiva, Mariana; Ribeiro, Vania; Garcia, Raquel; Amorim, Sandra; Campelo, Manuel; Martins, Elisabete; Moura, Brenda; Cardoso, Jose; Maciel, M Júlia

    2012-01-01

    We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

  5. Congenital Complete Atrioventricular Block and Dilated Cardiomyopathy: New Light for an Old Disease

    Directory of Open Access Journals (Sweden)

    Mariana Paiva

    2012-01-01

    Full Text Available We present a case of a patient with known complete congenital atrioventricular block (CAVB since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

  6. Congenital Complete Atrioventricular Block and Dilated Cardiomyopathy: New Light for an Old Disease

    OpenAIRE

    Paiva, Mariana; Ribeiro, Vania; Garcia, Raquel; Amorim, Sandra; Campelo, Manuel; Martins, Elisabete; Moura, Brenda; Cardoso, Jose; Maciel, M. Júlia

    2012-01-01

    We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

  7. Atrial myxoma in a patient with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Abdou, Mahmoud; Hayek, Salim; Williams, Byron R

    2013-01-01

    Atrial myxoma is the most common primary cardiac tumor. Patients with atrial myxoma typically present with obstructive, embolic, or systemic symptoms; asymptomatic presentation is very rare. To our knowledge, isolated association of atrial myxoma with hypertrophic cardiomyopathy has been reported only once in the English-language medical literature. We report the case of an asymptomatic 71-year-old woman with known hypertrophic cardiomyopathy in whom a left atrial mass was incidentally identified on cardiac magnetic resonance images. After surgical excision of the mass and partial excision of the left atrial septum, histopathologic analysis confirmed the diagnosis of atrial myxoma. The patient was placed on preventive implantable cardioverter-defibrillator therapy and remained asymptomatic. The management of asymptomatic cardiac myxoma is a topic of debate, because no reports definitively favor either conservative or surgical measures.

  8. Implantable cardioverter-defibrillators in children.

    Science.gov (United States)

    Werner, Bozena; Przybylski, Andrzej; Kucińska, Beata; Lewandowski, Michał; Szwed, Hanna; Wróblewska-Kałuzewska, Maria

    2004-03-01

    Implantable cardioverter-defibrillators (ICD) have been increasingly used in adult patients for the prevention of sudden cardiac death (SCD). The usefulness and feasibility of ICD implantation in children have been less well established. To analyse indications, results and safety of ICD therapy in children. ICDs were implanted in seven children, aged from 6 to 17 years. All patients underwent cardiological evaluation which included analysis of medical history, physical examination, chest X-ray, standard ECG, 24-hour Holter ECG monitoring and echocardiography. In five children devices were implanted due to aborted sudden death (ventricular fibrillation) whereas in the remaining two - as a primary prevention of SCD. Three children had hypertrophic cardiomyopathy, one - dilated cardiomyopathy, one - mitral valve prolapse and QT prolongation, one - congenital long QT syndrome and the remaining patient - idiopathic ventricular tachycardia. Single-chamber devices were implanted in six children, and dual-chamber system - in one patient. In all patients endocardial leads were implanted and ICD pocket was formed under the greater pectoral muscle. During follow-up ranging between four months to 5.4 years, four children developed ventricular fibrillation or ventricular tachycardia which were terminated by appropriate ICD discharges. 1. ICD implantation in children is effective in the prevention of SCD. 2. In our population, the most frequent indications for device implantation were life-threatening ventricular arrhythmias occurring in patients with cardiomyopathy. 3. Cardiac arrest due to ventricular fibrillation may occur in children without a history of aborted SCD. 4. ICD implantation in children is feasible and safe.

  9. Risk of Cardiomyopathy in Younger Persons With a Family History of Death from Cardiomyopathy

    DEFF Research Database (Denmark)

    Ranthe, Mattis F; Carstensen, Lisbeth; Øyen, Nina

    2015-01-01

    BACKGROUND: Recommendations for presymptomatic screening of relatives of cardiomyopathy patients are based on findings from tertiary centers. Cardiomyopathy inheritance patterns are fairly well understood, but how cardiomyopathy in younger persons (<50 years) aggregates in families at the populat......BACKGROUND: Recommendations for presymptomatic screening of relatives of cardiomyopathy patients are based on findings from tertiary centers. Cardiomyopathy inheritance patterns are fairly well understood, but how cardiomyopathy in younger persons (families...... at the population level is unclear. In a nationwide cohort, we examined the risk of cardiomyopathy by family history of premature death (... ascertained family history of premature (

  10. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    A. G. Osiev

    2015-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCMP is a relatively common disease with genetic predisposition, that is widely spread irrespective of gender, race or ethnicity. The cause of this pathology are mutations of genes encoding synthesis of contracting proteins. Degree and type of mutations define clinical manifestation of the disease and its prognosis. HCMP is classified according to four main criteria: depending on morphology, presence of left ventricular outlet obstruction, pressure gradient and hemodynamic parameters. Its prevalence amounts to 1:500, and in the recent years mortality has decreased significantly to 1%. Main symptoms of HCMP include dyspnoea, dizziness, syncope, angina, and heart arrhythmias. HCMP does not manifest obligatorily with all above mentioned signs and symptoms. Presence and severity of any symptoms depend on morphological particulars of the disease. Particular attention should be paid to arrhythmias, with atrial fibrillation among them, that may cause hazardous and occasionally lethal complications. Electrocardiography and echocardiography are recognized as the “golden standard” of HCMP diagnostics, while in the recent years, magnetic resonance imaging has become a highly informative diagnostic tool. Radionuclide diagnostics is used less frequently, while physical examination and assessments have been moving backwards. At present, main strategies in HCMP include medical treatment with β-blockers, calcium antagonists, angiotensin-converting enzyme inhibitors and anti-arrhythmics. There are two techniques for surgical treatment, i.e. myectomy by Morrow and alcohol septal ablation that is becoming increasingly popular. The article reviews literature on state-of-the-art diagnostics and treatment of HCMP patients.

  11. Peripartum cardiomyopathy - case series.

    Science.gov (United States)

    Prasad, Gowri Sayi; Bhupali, Ashok; Prasad, Sayi; Patil, Ajit N; Deka, Yashodhan

    2014-01-01

    To study the pattern of presentation, course of disease and outcome of pregnancy in Peripartum Cardiomyopathy. A prospective study of sixteen cases of PPCM was conducted at Apple Saraswati Multispecialty Hospital and Dr. D.Y. Patil Medical College and Hospital, Kolhapur, Maharashtra, India from January 2006 to December 2012. Data included age distribution, parity, gestational age, symptoms and risk factors. Medical management and pregnancy outcome were documented. Serial echocardiography data was compiled for a period of one year. In our study 9/16 (56%) were primigravidae, 4/16 (25%) had pre-eclamsia and 6/16 (35%) had co-existing hypertension. The difference in Echocardiography parameters observed between recovered and non-recovered patients was significant: Left Ventricular End diastolic dimension (5.6 cm vs 6.06 cm), Left Ventricular Ejection Fraction (28.7% vs 22.4%) and Left Ventricular fractional shortening (17.5% vs 13.4%). Thirteen out of sixteen patients were followed up for a period of one year out of which 61% (8/13) patients recovered completely. There was one mortality. PPCM is a diagnosis of exclusion. Majority were young primigravidae presenting postnatally. Pre-eclampsia and hypertension were risk factors. ECHO parameters were reliable predictors of recovery. Future pregnancies are better avoided. Copyright © 2014 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  12. Heart transplantation for Chagas cardiomyopathy.

    Science.gov (United States)

    Ramalho, Ana Rita; Prieto, David; Antunes, Pedro; Franco, Fátima; Antunes, Manuel J

    2017-11-01

    Chagas disease is an endemic disease in Latin America that is increasingly found in non-endemic areas all over the world due to the flow of migrants from Central and South America. We present the case of a Brazilian immigrant in Portugal who underwent orthotopic heart transplantation for end-stage Chagas cardiomyopathy. Immunosuppressive therapy included prednisone, mycophenolate mofetil and tacrolimus. Twelve months after the procedure she is asymptomatic, with good graft function, and with no evidence of complications such as graft rejection, opportunistic infections, neoplasms or reactivation of Trypanosoma cruzi infection. By reporting the first case in Portugal of heart transplantation for Chagas cardiomyopathy, we aim to increase awareness of Chagas disease as an emerging global problem and of Chagas cardiomyopathy as a serious complication for which heart transplantation is a valuable therapeutic option. Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. Lysosome-associated hypertrophic cardiomyopathy (Danon's disease in two siblings

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2015-01-01

    Full Text Available The paper presents a clinical observation of two siblings with Danon's disease (lysosome-associated cardiomyopathy verified by genetic examination. Heart lesion in Danon's disease bears a phenotypic similarity to the primary forms of hypertrophic cardiomyopathy; in this connection the correct etiology of the disease has remained long unestablished. The presence of laboratory markers as the significantly raised levels of transaminases, creatine phosphokinase, and lactate dehydrogenase was as a guide for suspecting the metabolic origin of the disease. Two siblings with a similar LAMP gene mutation were observed to have a different clinical course: a severer clinical course of cardiomyopathy with extreme myocardial hypertrophy, myocardial electric instability, and mental development retardation in one case and a more favorable course in the other; although a 2-year follow-up also revealed negative changes. For the prevention of sudden cardiac death, a cardioverter defibrulator was implanted and continuous therapy with p-adrenoblockers was performed. The specific feature of the cases was no symptoms of skeletal myopathy, moderate mental retardation only in the elder brother, no evidence of an accessory atrioventricular junction despite the fact that there were ECG manifestations of Wolff-Parkinson-White syndrome

  14. Animal models of hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Maass, Alexander; Leinwand, Leslie A.

    Familial hypertrophic cardiomyopathy (FHC) is an autosomal-dominant disease that is both clinically and genetically heterogeneous. Disease-causing mutations have been found in eight genes encoding structural components of the thick and thin filament systems of the cardiac myocyte; it has therefore

  15. Improving Outcomes in Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    P.A. Vriesendorp (Pieter)

    2016-01-01

    markdownabstractImproving outcomes in hypertrophic cardiomyopathy (HCM) is focused on the improvement of the therapeutic strategies for patients with HCM. First it demonstrates that individual patient selection in patients with obstructive and symptomatic HCM can lead to near normal life-expectancy;

  16. Cor triatriatum and hypertrophic cardiomyopathy.

    Science.gov (United States)

    Pellaton, Cyril; O'Mahony, Costas; Ludman, Andrew J; Sekhri, Neha; Mohiddin, Saidi

    2016-12-01

    : Cor triatriatum is a rare congenital anomaly known to be associated with other inherited heart diseases. We present a nonrestrictive cor triatriatum sinistrum associated with hypertrophic cardiomyopathy to illustrate how different multimodality noninvasive imaging techniques complement each other and can help with the diagnosis. To the best of our knowledge, this coexistence has not been previously reported.

  17. Left Ventricular Assist Device Insertion in a Patient With Biventricular Noncompaction Cardiomyopathy, Ebstein Anomaly, and a Left Atrial Mass: A Case Report.

    Science.gov (United States)

    Kumar, Nikhil; Troianos, Christopher A; Baisden, Joshua S

    2016-12-15

    In this report, we present the case of a patient with biventricular noncompaction cardiomyopathy, Ebstein anomaly, and a left atrial mass who required emergent placement of a left ventricular assist device. The noncompaction cardiomyopathy complicated the left ventricular assist device implantation procedure because the thickened, trabeculated myocardium made it difficult to place the inflow cannula. We discuss our perioperative management strategy, in which transesophageal echocardiography was used, to help the surgical team identify the proper cannula placement and provide a bridge to transplantation.

  18. Left Ventricular Non-compaction Cardiomyopathy - A Case Report

    Directory of Open Access Journals (Sweden)

    Timea Szakacs Xantus

    2015-06-01

    Full Text Available Background: Left non-compaction cardiomyopathy (LVNC or “spongy myocardium” is a relatively rare primary genetic cardiomyopathy, characterized by prominent wall trabeculations and intertrabecular recesses which communicate with the ventricular cavity. It appears in isolated form or coexists with other congenital heart diseases and/or systemic abnormalities. Material and method: A 28-year-old woman was admitted with exertional dyspnoea, palpitations, non-specific chest pain and progressive fatigue on exertion. In her family history sudden cardiac-related deaths at young age are present. Cardiovascular system examination revealed tachycardia, intermittent extrabeats. The rest EKG showed sinusal tachycardia (105 bpm, negative T-waves in DII, DIII, aVF, V4-V6. Consecutive 24 hours Holter EKG monitoring revealed nonsustained ventricular tachycardia, paroxysmal atrial fibrillation, isolated ventricular extrasystoles. Echocardiography showed left ventricular systolic dysfunction (LVEF:30-35%, slight LV enlargement, normal right ventricle and small left ventricle (LV trabeculae in the apical area. Cardiac MRI demonstrated dilated LV and the presence of the trabeculations of LV walls suggestive for non-compaction cardiomyopathy. A combined treatment for heart failure and cardiac arrhythmias was initiated with good clinical results. Patient was scheduled for an implantable cardioverter defibrillator “life-saving”. Conclusions: The symptoms of heart failure and cardiac arrhythmias should be considered important in apparently healthy young patients. Besides intensive medical treatment is indicated the implantation of an ICD “life-saving” and in advanced cases heart transplantation. Even if the electrocardiographic findings are non specific for noncompaction, a complete diagnostic evaluation is important, including sophisticated imaging techniques, a screening of first-degree relatives, and an extensive clinical, and genetic appreciation by a

  19. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy and infiltrative cardiomyopathy

    OpenAIRE

    Schofield, R.; Manacho, K.; Castelletti, S.; Moon, J. C.

    2016-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. Cardiac imaging plays a key role in the diagnosis and management, with cardiovascular magnetic resonance (CMR) an important modality. CMR provides a number of different techniques in one examination: structure and function, flow imaging and tissue characterisation particularly with the late gadolinium enhancement (LGE) technique. Other techniques include vasodilator perfusion, mapping (especially T1 mapping and ex...

  20. Syncope in children with hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2014-01-01

    Full Text Available Seventy children aged 7 to 17 years with hypertrophic cardiomyopathy (HCM were examined; among them there were 11 syncope patients and 5 presyncope patients. The screening program included standard electrocardiography (ECG, Doppler echocardiogra-phy, 24-hour Holter ECG monitoring, and an incremental exercise testing (Bruce treadmill test. The markers of myocardial electrical instability were determined. In the children with HCM, syncope was established to be heterogeneous; it had an arrhythmogenic origin and, in most cases, occurred in the presence of tachyarrhythmia (44% or bradyarrythmia (25%; its vasovagal genesis was probable in one third of the examinees. The children with syncope were typified by the asymmetric, obstructive form of HCM, at the same tone there was most commonly left ventricular hypertrophy concurrent with left atrial enlargement. 24-hour Holter monitoring showed that bradycardia was prevalent in the patients, 3 patients were found to have more than 2-second cardiac rhythm pauses caused by second-degree atrioventricular block in 1 case or by sick sinus syndrome in 2. Nonsustained ventricular tachycardia was noted in two patients. The children with syncope were typified by the signs of myocardial electrical instability as a reduction in the early phase of heart rate turbulence and by impaired QT/RR interval adaptation as hyperadaptation. The paper presents the developed management tactics for children with syncope and indications for the implantation of a cardioverter defibrillator, a pacemaker, or an ECG loop recorder.

  1. Determinants of Thyrotoxic Cardiomyopathy Recovery

    Directory of Open Access Journals (Sweden)

    Lucia Oliveros-Ruiz

    2013-01-01

    Full Text Available The purpose was to evaluate the effect of the disease duration prior to treatment, thyroid hormones level, or both on the reversibility of dilated cardiomyopathy. Between January 2006 and December 2010, a longitudinal study with a 6 months follow-up was carried on. One hundred and seventy patients with hyperthyroidism were referred to the cardiologist, and 127 had a 6 months followup after antithyroid treatment and were evaluated by echocardiography. Dilated cardiomyopathy reversibility criteria were established according to echocardiographic parameters. Complete reversibility existed when all parameters were met, partial reversibility when LVEF was ≥55% plus two or three other parameters, and no reversibility when LVEF was ≤55% regardless of other parameters. The results showed that echocardiography parameters related to the regression of myocardial mass were associated with a disease duration shorter than 10.38 months. This was the main predictive variable for reversal of dilated cardiomyopathy, followed by β-blocker treatment, and the last predictive variable was the serum level of free triiodothyronine. This study showed that the effect on the myocardium related to thyrotoxicosis was associated with the disease duration before treatment.

  2. Clinical Spectrum, Therapeutic Options, and Outcome of Advanced Heart Failure in Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Pasqualucci, Daniele; Fornaro, Alessandra; Castelli, Gabriele; Rossi, Alessandra; Arretini, Anna; Chiriatti, Chiara; Targetti, Mattia; Girolami, Francesca; Corda, Marco; Orrù, Pierpaolo; Matta, Gildo; Stefàno, Pierluigi; Cecchi, Franco; Porcu, Maurizio; Olivotto, Iacopo

    2015-11-01

    The clinical course of patients with hypertrophic cardiomyopathy and advanced heart failure (HF) subtended by progressive left ventricular dysfunction has received limited attention. Our aim was to assess the outcome of HF and impact of treatment options including the implantable cardioverter-defibrillator and heart transplantation (HT) in patients with hypertrophic cardiomyopathy evaluated at 2 Italian referral centers >3 decades. All-cause mortality and a combined end point including death, HT, or appropriate implantable cardioverter-defibrillator shock were assessed in 71 consecutive patients with HF not related to outflow obstruction (7% of the entire hypertrophic cardiomyopathy cohort) followed up for 6.1±6.9 years after development of New York Heart Association class III to IV symptoms. At enrollment, left ventricular ejection fraction was 50% in 16; all had restrictive left ventricular filling. During follow-up, 35 patients died (49%%; 5-year rate, 49%) and 53 met the combined end point (75%; 5-year rate, 62%). Most events occurred in the 3 years after HF onset (17% per year compared with only 3% per year subsequently). Appropriate implantable cardioverter-defibrillator shocks occurred in 11 of 34 implanted patients. Of 37 patients evaluated for HT, 14 were transplanted, 10 listed, and 13 excluded; 2 early post-HT deaths occurred in patients with elevated pulmonary vascular resistance. Eleven of the 14 HT patients were alive at 10±8 years. In hypertrophic cardiomyopathy, advanced HF not associated with outflow obstruction portends a severely unfavorable prognosis, particularly in the first 3 years after onset of symptoms, despite frequently preserved systolic function in about one quarter of the patients. Outcome of HT is favorable but requires early consideration, as the window of opportunity may be short. © 2015 American Heart Association, Inc.

  3. Hypertrophic cardiomyopathy and Wolff-Parkinson-White Syndrome with complete auriculoventricular block. A strange association

    International Nuclear Information System (INIS)

    Vallejo, Franco J; Montana, Paula A; Vesga, Carlos; Miranda Antonio; Citelli Jose E; Negrete Alberto; Gil, Efrain

    2007-01-01

    A 22 years old male patient is admitted for a syncope episode. An electrocardiogram shows a Wolff-Parkinson-White pattern and signs of auricular overload with left ventricular hypertrophy and complete auriculoventricular block. The transthoracic echocardiogram is compatible with non-obstructive hypertrophic cardiomyopathy. An electrophysiological study is carried out, finding pre-excitation through an accessory way and infra-His auriculoventricular block. An ablation is performed and a bicameral pacemaker is implanted

  4. Apical Hypertrophic Cardiomyopathy Mimicking Acute Coronary Syndrome

    Directory of Open Access Journals (Sweden)

    Lütfü Bekar

    2013-03-01

    Full Text Available Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy and it can be mistaken for coronary artery disease due to the symptoms and electrocardiography findings. In this report, we aimed to present a patient referred to our clinic with complaints of chest pain and electrocardiography findings who had been misdiagnosed as non-ST elevation acute coronary syndrome.

  5. Peripartum Cardiomyopathy: Euro Observational Research Program

    NARCIS (Netherlands)

    M.F. Hoes; I.M. van Hagen (Iris); F. Russo; D.J. van Veldhuisen (Dirk); M.P. van den Berg (Maarten); J.W. Roos-Hesselink (Jolien); K.Y. van Spaendonck-Zwarts (Karin); P. van der Meer (Peter)

    2014-01-01

    textabstractPeripartum cardiomyopathy is a rare but potentially life-threatening form of heart failure affecting women late in pregnancy or in the first months after delivery. Peripartum cardiomyopathy is difficult to diagnose and its onset and progression are variable between individuals. The

  6. Peripartum cardiomyopathy : Euro Observational Research Program

    NARCIS (Netherlands)

    Hoes, M. F.; van Hagen, I.; Russo, F.; Van Veldhuisen, D. J.; Van den Berg, M. P.; Roos-Hesselink, J.; van Spaendonck-Zwarts, K. Y.; van der Meer, P.

    Peripartum cardiomyopathy is a rare but potentially life-threatening form of heart failure affecting women late in pregnancy or in the first months after delivery. Peripartum cardiomyopathy is difficult to diagnose and its onset and progression are variable between individuals. The pathophysiology

  7. Peripartum cardiomyopathy: Euro Observational Research Program

    NARCIS (Netherlands)

    Hoes, M. F.; van Hagen, I.; Russo, F.; van Veldhuisen, D. J.; van den Berg, M. P.; Roos-Hesselink, J.; van Spaendonck-Zwarts, K. Y.; van der Meer, P.

    2014-01-01

    Peripartum cardiomyopathy is a rare but potentially life-threatening form of heart failure affecting women late in pregnancy or in the first months after delivery. Peripartum cardiomyopathy is difficult to diagnose and its onset and progression are variable between individuals. The pathophysiology

  8. Tako-tsubo cardiomyopathy after a quarrel.

    African Journals Online (AJOL)

    Conclusion: We report a case of Tako-tsubo cardiomyopathy after a quarrel, implicating that severe emotional or physical stress could trigger myocardial stunning. Keywords: Tako-tsubo cardiomyopathy, ocular trauma, blindness, postmenopause. DOI: http://dx.doi.org/10.4314/ahs.v15i4.39. Cite as: Jiang D-M, Sun Z-W, Han ...

  9. Clinical and Prognostic Profiles of Cardiomyopathies Caused by Mutations in the Troponin T Gene.

    Science.gov (United States)

    Ripoll-Vera, Tomás; Gámez, José María; Govea, Nancy; Gómez, Yolanda; Núñez, Juana; Socías, Lorenzo; Escandell, Ángela; Rosell, Jorge

    2016-02-01

    Mutations in the troponin T gene (TTNT2) have been associated in small studies with the development of hypertrophic cardiomyopathy characterized by a high risk of sudden death and mild hypertrophy. We describe the clinical course of patients carrying mutations in this gene. We analyzed the clinical characteristics and prognosis of patients with mutations in the TNNT2 gene who were seen in an inherited cardiac disease unit. Of 180 families with genetically studied cardiomyopathies, 21 families (11.7%) were identified as having mutations in TNNT2: 10 families had Arg92Gln, 5 had Arg286His, 3 had Arg278Cys, 1 had Arg92Trp, 1 had Arg94His, and 1 had Ile221Thr. Thirty-three additional genetic carriers were identified through family assessment. The study included 54 genetic carriers: 56% were male, and the mean average age was 41 ± 17 years. There were 33 cases of hypertrophic cardiomyopathy, 9 of dilated cardiomyopathy, and 1 of noncompaction cardiomyopathy, and maximal myocardial thickness was 18.5 ± 6mm. Ventricular dysfunction was present in 30% of individuals and a history of sudden death in 62%. During follow-up, 4 patients died and 14 (33%) received a defibrillator (8 probands, 6 relatives). Mean survival was 54 years. Carriers of Arg92Gln had early disease development, high penetrance, a high risk of sudden death, a high rate of defibrillator implantation, and a high frequency of mixed phenotype. Mutations in the TNNT2 gene were more common in this series than in previous studies. The clinical and prognostic profiles depended on the mutation present. Carriers of the Arg92Gln mutation developed hypertrophic or dilated cardiomyopathy and had a significantly worse prognosis than those with other mutations in TNNT2 or other sarcomeric genes. Copyright © 2015 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  10. Supra-Hisian Conduction Block as an Unusual Presenting Feature of Takotsubo Cardiomyopathy.

    Science.gov (United States)

    Prabhu, Mukund Aravind; Pai, Praveen Gopalakrishna; Vupputuri, Anjith; Shekhar, Saritha; Harikrishnan, Madhavankutty Santhakumari; Kumaraswamy, Natarajan Umayammal

    2017-05-01

    Atrioventricular (AV) block is rare in Takotsubo cardiomyopathy (TC). A 66-year-old female presented with fatigue. Her electrocardiogram revealed 3:2 Mobitz Type II AV block, confirmed to be supra-Hisian by electrophysiological study. Echocardiogram and left ventricular angiogram showed moderate left ventricular dysfunction and apical ballooning, whereas coronary angiogram revealed mildly ectatic coronaries. At 2 weeks AV block persisted, needing permanent pacemaker implantation. At 1-month follow-up, she had normal ventricular function and no AV block. Delayed recovery of AV conduction is possible in TC, thus implying to wait for a longer period before implanting a pacemaker. © 2016 Wiley Periodicals, Inc.

  11. Adaptive cardiac resynchronization therapy for dilated cardiomyopathy with functional mitral regurgitation

    Directory of Open Access Journals (Sweden)

    Yoshiki Nagata, MD, PhD

    2017-10-01

    Full Text Available We report the case of a man in his 60s who had dilated cardiomyopathy with severe functional mitral regurgitation. Four years after a cardiac resynchronization therapy (CRT device with an implantable cardioverter defibrillator was implanted, this device was replaced with an adaptive CRT device because of battery consumption. Seven months after replacement of this device, the left ventricular pacing to right ventricular activation and the atrioventricular delay from automatic adjustments contributed to less functional mitral regurgitation. The findings from our case suggest that optimal CRT, by measuring intracardiac conduction parameters, is effective for functional mitral regurgitation.

  12. Breast Implants

    Science.gov (United States)

    ... Medical Procedures Implants and Prosthetics Breast Implants Breast Implants Share Tweet Linkedin Pin it More sharing options Linkedin Pin it Email Print Breast implants are medical devices that are implanted under the ...

  13. Desmoplakin truncations and arrhythmogenic left ventricular cardiomyopathy: characterizing a phenotype.

    Science.gov (United States)

    López-Ayala, Jose María; Gómez-Milanés, Ivan; Sánchez Muñoz, Juan José; Ruiz-Espejo, Francisco; Ortíz, Martín; González-Carrillo, Josefa; López-Cuenca, David; Oliva-Sandoval, M J; Monserrat, Lorenzo; Valdés, Mariano; Gimeno, Juan R

    2014-12-01

    Risk stratification for sudden death in arrhythmogenic right ventricular cardiomyopathy (ARVC) is challenging in clinical practice. We lack recommendations for the risk stratification of exclusive left-sided phenotypes. The aim of this study was to investigate genotype-phenotype correlations in patients carrying a novel DSP c.1339C>T, and to review the literature on the clinical expression and the outcomes in patients with DSP truncating mutations. Genetic screening of the DSP gene was performed in 47 consecutive patients with a phenotype of either an ARVC (n = 24) or an idiopathic dilated cardiomyopathy (DCM), who presented with ventricular arrhythmias or a family history of sudden death (n = 23) (aged 40 ± 19 years, 62% males). Three unrelated probands with DCM were found to be carriers of a novel mutation (c.1339C>T). Cascade family screening led to the identification of 15 relatives who are carriers. Penetrance in c.1339C>T carriers was 83%. Sustained ventricular tachycardia was the first clinical manifestation in six patients and nine patients were diagnosed with left ventricular impairment (two had overt severe disease and seven had a mild dysfunction). Cardiac magnetic resonance revealed left ventricular involvement in nine cases and biventricular disease in three patients. Extensive fibrotic patterns in six and non-compaction phenotype in five patients were the hallmark in imaging. DSP c.1339C>T is associated with an aggressive clinical phenotype of left-dominant arrhythmogenic cardiomyopathy and left ventricular non-compaction. Truncating mutations in desmoplakin are consistently associated with aggressive phenotypes and must be considered as a risk factor of sudden death. Since ventricular tachycardia occurs even in the absence of severe systolic dysfunction, an implantable cardioverter-defibrillator should be indicated promptly. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please

  14. Peripartum Cardiomyopathy: A Current Review

    Directory of Open Access Journals (Sweden)

    Katie M. Twomley

    2010-01-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a rare but potentially lethal complication of pregnancy occurring in approximately 1 : 3,000 live births in the United States although some series report a much higher incidence. African-American women are particularly at risk. Diagnosis requires symptoms of heart failure in the last month of pregnancy or within five months of delivery in the absence of recognized cardiac disease prior to pregnancy as well as objective evidence of left ventricular systolic dysfunction. This paper provides an updated, comprehensive review of PPCM, including emerging insights into the etiology of this disorder as well as current treatment options.

  15. Takotsubo cardiomyopathy: a case report.

    Science.gov (United States)

    Johar, Sandeep; Prasad, Heramba; Kozman, Hani

    2007-01-01

    A 57-year-old Caucasian woman presented to the emergency department with chest discomfort after testifying in a child custody battle in court. Electrocardiography (ECG) revealed ST-segment elevation in the lateral leads, and T-wave inversions in the inferior and lateral leads. The creatine kinase, creatine kinase-MB, and troponin I concentrations were elevated on presentation. Despite the ECG changes and elevated cardiac markers, coronary angiography demonstrated normal arteries. Left ventricular angiogram revealed an aneurysm at the apex. Takotsubo cardiomyopathy was diagnosed on the basis of these characteristic findings. The patient was discharged on her third hospital day without any complications from the coronary angiography.

  16. Apical hypertrophic cardiomyopathy with hemodynamically unstable ventricular arrhythmia - Atypical presentation.

    Science.gov (United States)

    Chaturvedi, Hemant; Pandey, Rudra Dev; Sharma, Krishna Kumar; Makkar, Jitendra Singh; Sharma, Sanjeev K

    2016-09-01

    We present a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM) who recently developed cardiac arrhythmias, and shortly discuss the diagnostic modalities, differential diagnosis, and treatment strategy for this condition. AHCM is a rare form of hypertrophic cardiomyopathy, which usually involves the apex of the left ventricle. AHCM can occur with varied presentations such as chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation, and congestive heart failure. The most peculiar electrocardiogram findings are giant T-waves inversion in the precordial leads with left ventricular (LV) hypertrophy. A transthoracic echocardiogram is the initial diagnostic modality in the evaluation of AHCM and shows hypertrophy of the LV apex. Other diagnostic modalities, including left ventriculography, multislice spiral computed tomography, and cardiac magnetic resonance imagings, are also valuable tools. Medications used to manage include verapamil, beta-blockers, and antiarrhythmic agents. An implantable cardioverter defibrillator (ICD) is recommended for high-risk patients. Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  17. [Diagnosis and therapy of arrhythmogenic right ventricular cardiomyopathy].

    Science.gov (United States)

    Zorzi, Alessandro; Rigato, Ilaria; Migliore, Federico; Perazzolo Marra, Martina; Basso, Cristina; Thiene, Gaetano; Bauce, Barbara; Corrado, Domenico

    2014-11-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease that predisposes to the occurrence of ventricular arrhythmias and sudden death, particularly in the young and athlete. The classic variant of the disease predominantly affects the right ventricle, but phenotypic variants with early and prevalent left ventricular involvement ("left-dominant" ARVC) have also been described, supporting the concept that arrhythmogenic cardiomyopathy is a disease of both ventricles. The diagnosis is multiparametric and is based on a series of criteria, including ECG abnormalities, arrhythmic manifestations, morpho-functional abnormalities and genetic defects. The main goal of therapy is sudden death prevention. Implant of a cardioverter-defibrillator is the most effective strategy for prevention of sudden death, but it should be reserved to selected patients after accurate risk stratification, in view of the high complication rate over a long-term follow-up, the costs and the significant psychological impact of such therapy, especially in the young individual. The other therapies (either pharmacological or not) are palliative and aimed at relieving symptoms and preventing disease progression. The definitive cure of ARVC will be based on the discovery of the molecular mechanisms that are involved in the etiology and pathogenesis of the disease.

  18. Arrhythmogenic right ventricular cardiomyopathy: contribution of different electrocardiographic techniques.

    Science.gov (United States)

    Moreira, Davide; Delgado, Anne; Marmelo, Bruno; Correia, Emanuel; Gama, Pedro; Pipa, João; Nunes, Luís; Santos, Oliveira

    2014-04-01

    Arrhythmogenic right ventricular cardiomyopathy, also known as arrhythmogenic right ventricular dysplasia, is a condition in which myocardium is replaced by fibrous or fibrofatty tissue, predominantly in the right ventricle. It is clinically characterized by potentially lethal ventricular arrhythmias, and is a leading cause of sudden cardiac death. Its prevalence is not known exactly but is estimated at approximately 1:5000 in the adult population. Diagnosis can be on the basis of structural and functional alterations of the right ventricle, electrocardiographic abnormalities (including depolarization and repolarization alterations and ventricular arrhythmias) and family history. Diagnostic criteria facilitate the recognition and interpretation of non-specific clinical features of this disease. The authors present a case in which the diagnosis of arrhythmogenic right ventricular cardiomyopathy was prompted by the suspicion of right ventricular disease on transthoracic echocardiography. This was confirmed by detection of epsilon waves on analysis of the ECG, which generally go unnoticed but in this case were the key to the diagnosis. Their presence was also shown by non-conventional ECG techniques such as modified Fontaine ECG. The course of the disease culminated in the occurrence of ventricular tachycardia, which prompted placement of an implantable cardioverter-defibrillator. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  19. The Treatment of Chronic Heart Failure Secondary to Chagas Cardiomyopathy in the Contemporary Era

    Directory of Open Access Journals (Sweden)

    Reinaldo B. Bestetti

    2016-01-01

    Full Text Available Chronic Heart Failure affects about half patients with Chagas cardiomyopathy. Poor outcomes of CHF secondary to Chagas cardiomyopathy are relentless with an annual mortality approaching 20%, which is higher than that observed in non-Chagas disease heart failure. The pathophysiology of Chagas disease is similar to that found in non-Chagas disease heart failure with a marked activation of the neurohormonal system. No randomized trial has been conducted in patients with Chagas cardiomyopathy with CHF to assess the effect of a drug on mortality of such patients. Therefore, the treatment of CHF secondary to Chagas cardiomyopathy relies on drugs prescribed to patients with non-Chagas disease heart failure. Patients with Chagas disease heart failure have been classified into stages A to D according to the American College of Cardiology/American Heart Association. Little can be done to patients in the stage A of CHF, except for treatment of comorbidities. In patients in the stage B of CHF, aldosterone receptor antagonist, angiotensin converting enzyme inhibitors (ACEI, and Betablockers (BB are indicated. In patients in the stage C of CHF, the same drugs are of value. In addition, diuretics, digoxin, angiotensin receptor blockers to patients intolerant to ACEI have also been used. Cardiac Resynchronization Therapy and Implantable Cardioverter Defibrillator may have indications similar to that of non-Chagas disease patients. In stage D of CHF, heart transplantation is a valid option for patients with this condition.

  20. Cardiomyopathy due to ingestion of Adderall.

    Science.gov (United States)

    Marks, Donald H

    2008-01-01

    A patient is described who developed cardiomyopathy after receiving a therapeutic course of dextroamphetamine/amphetamine. The patient's cardiac function deteriorated to the point of heart failure, necessitating a heart transplantation. Cardiomyopathy associated with amphetamine use is a serious and potentially lethal condition. With early diagnosis, identification of the cause, and treatment, cardiomyopathy may be reversible. The dangers of therapeutic use of amphetamines are discussed, as well as problems and assumptions associated with U.S. Food and Drug Administration monitoring and removal from the market of harmful substances.

  1. Does Body Mass Index Predict Premature Cardiomyopathy Onset for Duchenne Muscular Dystrophy?

    Science.gov (United States)

    McKane, Meghann; Soslow, Jonathan H; Xu, Meng; Saville, Benjamin R; Slaughter, James C; Burnette, W Bryan; Markham, Larry W

    2017-04-01

    Duchenne muscular dystrophy leads to cardiomyopathy. The objective of this study was to estimate the association of body mass index with cardiomyopathy onset. Cardiomyopathy was defined as left ventricular ejection fraction Duchenne muscular dystrophy subjects and age of cardiomyopathy onset.

  2. Restrictive cardiomyopathy and hypertrophic cardiomyopathy overlap: the importance of the phenotype

    Directory of Open Access Journals (Sweden)

    Juan Pablo Kaski

    2012-10-01

    Full Text Available Restrictive cardiomyopathy (RCM is defined on the basis of the haemodynamic finding of restrictive ventricular physiology. However, restrictive ventricular pathophysiology is also a feature of other subtypes of cardiomyopathy, including hypertrophic cardiomyopathy (HCM. Clinically and aetiologically, there is an overlap between RCM and HCM with restrictive physiology. However, the clinical distinction between these two entities can be an important pointer towards the underlying aetiology. This review highlights the importance of the recognition of the clinical phenotype as the first step in the classification of cardiomyopathies.

  3. Serious arrhythmias in patients with apical hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Okishige, Kaoru; Sasano, Tetsuo; Yano, Kei; Azegami, Kouji; Suzuki, Kou; Itoh, Kuniyasu [Yokohama Red Cross Hospital (Japan)

    2001-05-01

    We report cases of serious arrhythmias associated with apical hypertrophic cardiomyopathy (AHCM). Thirty-one patients were referred to our institute to undergo further assessment of their AHCM from 1988 to 1999. Three patients with nonsustained ventricular tachycardia demonstrated an {sup 123}I-MIBG regional reduction in the tracer uptake. In two patients with ventricular fibrillation (VF), the findings from {sup 123}I-MIBG imaging revealed regional sympathetic denervation in the inferior and lateral regions. Electrophysiologic study demonstrated reproducible induction of VF in aborted sudden death and presyncopal patients, resulting in the need for an implantable defibrillator device and amiodarone in each patient. Patients with refractory atrial fibrillation with a rapid ventricular response suffered from serious congestive heart failure. A prudent assessment and strategy in patients with this disease would be indispensable in avoiding a disastrous outcome. (author)

  4. Radiofrequency Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC).

    Science.gov (United States)

    Romero, Jorge; Grushko, Michael; Briceño, David F; Natale, Andrea; Di Biase, Luigi

    2017-09-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable form of cardiomyopathy, typically with autosomal dominant transmission, shown to be a defect in the cardiac desmosomes, with distinct regional and histopathological features. Clinically, this can ultimately result in bi-ventricular failure and/or malignant ventricular tachycardia (VT) via reentrant circuits created by patchy scar formation. We sought to review the current treatment for ventricular arrhythmias in the setting of ARVC, with particular attention to radiofrequency (RF) ablation and its varied techniques, along with potential therapies in the ablation spectrum. There is underwhelming data on the effectiveness of medical therapy for ARVC-related VT, including beta-blockers and antiarrhythmic medication. Primary and secondary prophylactic implantable-cardioverter defibrillator (ICD) implantation in higher-risk patients is recommended. More recently, RF ablation has been used for ARVC-related VT. Endocardial VT ablation in this setting can produce acute success, though recurrence rate is quite high, which may be explained by the more epicardial and patchy nature of the disease. Combined endocardial-epicardial ablation has since been shown to be feasible, safe, and with significantly better acute and long-term success, particularly when combined with scar dechanneling or homogenization of the scar. However, recurrence rates are not insignificant, and ablation does not eliminate the need for ICD placement. Medical therapy for ARVC-related VT is suboptimal. RF ablation techniques including endocardial and epicardial approaches appear to have the highest success rates for ARVC-related VT. Catheter ablation of VT in ARVC patients should be considered a potentially effective strategy for eliminating frequent VT episodes and ICD shocks rather than a curative therapeutic approach, until long-term efficacy has been consistently documented. Research into the optimal mapping and ablation techniques

  5. Genetics Home Reference: familial dilated cardiomyopathy

    Science.gov (United States)

    ... familial dilated cardiomyopathy can include an irregular heartbeat (arrhythmia), shortness of breath (dyspnea), extreme tiredness (fatigue), fainting ... Celebrates Its 15th Anniversary National DNA Day 2018 Newborn Screening Saves Lives Act Turns 10 All Bulletins ...

  6. Hypertrophic cardiomyopathy screening in young athletes

    Energy Technology Data Exchange (ETDEWEB)

    Rappoport, W.J. [Arizona Heart Inst., Phoenix, AZ (United States); Steingard, P.M. [Phoenix Suns, Phoenix, AZ (United States)

    2006-07-01

    Hypertrophic cardiomyopathy is the leading cause of sudden death during vigorous exercise. Early identification of this abnormality by ECG screening of high-school athletes before they participate in competitive sports helps save lives. (orig.)

  7. Tako-tsubo cardiomyopathy after a quarrel.

    African Journals Online (AJOL)

    com. Tel: +86571 85519933;. Fax:+8657187236569. It was first recognized in Japan ... coronary artery disease on cardiac catheterization8. The pathophysiological mechanism associated with. Tako-tsubo cardiomyopathy is higher prevalence in.

  8. Aborted Sudden Cardiac Death in a Female Patient Presenting with Takotsubo-Like Cardiomyopathy due to Epicardial Coronary Vasospasm

    Directory of Open Access Journals (Sweden)

    Sorin Giusca

    2017-01-01

    Full Text Available Takotsubo cardiomyopathy is characterized by apical ballooning of the left ventricle (LV in the absence of relevant coronary artery stenosis, which typically occurs in elderly women after emotional stress. Catecholamine cardiotoxicity, metabolic disturbance, and coronary microvascular impairment have previously been proposed as underlying pathophysiologic mechanisms of takotsubo cardiomyopathy, whereas myocardial stunning resulting from epicardial coronary artery vasospasm is not generally accepted as a cause of takotsubo cardiomyopathy. The prognosis of takotsubo cardiomyopathy is generally more favourable compared to myocardial infarction; however, severe complications such as rupture of the LV and life-threatening arrhythmias may occur. Herein, we describe a case of an 84-year-old female, who presented with aborted sudden cardiac death due to ventricular fibrillation. Echocardiography suggested LV apical ballooning with severely impaired LV-function, so that takotsubo cardiomyopathy was suspected. However, coronary angiography revealed epicardial spasm of the left anterior ascending, which resolved after intracoronary injection of 0.2 mg nitroglycerine. Cardiac magnetic resonance exhibited subendocardial late enhancement and echocardiography showed normalization of LV dysfunction during follow-up. The patient was put on conservative treatment with nitrates and calcium inhibitors and ICD implantation were deferred.

  9. Mechanical aberrations in hypetrophic cardiomyopathy: emerging concepts.

    Directory of Open Access Journals (Sweden)

    Dimitrios eNtelios

    2015-08-01

    Full Text Available Hypertrophic cardiomyopathy is the most common monogenic disorder in cardiology. Despite important advances in understanding disease pathogenesis, it is not clear how flaws in individual sarcomere components are responsible for the observed phenotype. The aim of this article is to provide a brief interpretative analysis of some currently proposed pathophysiological mechanisms of hypertrophic cardiomyopathy, with a special emphasis on alterations in the cardiac mechanical properties.

  10. RATIONAL PHARMACOTHERAPY IN TAKOTSUBO CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    S. Marchev

    2012-01-01

    Full Text Available Rational pharmacotherapy in Takotsubo cardiomyopathy is based on clinical picture and data of functional and laboratory investigations of concrete patient. In patients with hypotension and moderate-to-severe left ventricle outflow tract obstruction inotropic agents must not to be used because they can worsen the degree of obstruction. In these patients beta blockers can improve hemodynamics by causing resolution of the obstruction. If intraventricular thrombus is detected, anticoagulation for at least 3 months is recommended. The duration of anticoagulant therapy may be modified depending on the extent of cardiac function recovery and thrombus resolution. For patients without thrombus but with severe left ventricular dysfunction, anticoagulation is recommended until the akinesis or dyskinesis has resolved but not more than 3 months.

  11. Stimulant-related Takotsubo cardiomyopathy.

    Science.gov (United States)

    Butterfield, Mike; Riguzzi, Christine; Frenkel, Oron; Nagdev, Arun

    2015-03-01

    Takotsubo cardiomyopathy (TC) is a rare but increasingly recognized mimic of acute coronary syndrome. Patients present with angina,ST-segment changes on electrocardiogram (both elevations and depressions),and rapid rises in cardiac biomarkers. Many kinds of stressful events have been associated with TC, but only a handful of drug-related cases have previously been reported. We describe the case of a 58-year-old woman who developed TC 2 days after crack cocaine use, a diagnosis first suggested as bedside echocardiography in the emergency department.Recognition of the classic echocardiographic appearance of TC—apical hypokinesis causing “ballooning” of the left ventricle during systole—may greatly assist providers in the early identification of this condition.

  12. Penile Implants

    Science.gov (United States)

    ... the discussion with your doctor. Types of penile implants There are two main types of penile implants: ... might help reduce the risk of infection. Comparing implant types When choosing which type of penile implant ...

  13. Diabetic Cardiomyopathy: An Immunometabolic Perspective

    Directory of Open Access Journals (Sweden)

    Paras K. Mishra

    2017-04-01

    Full Text Available The heart possesses a remarkable inherent capability to adapt itself to a wide array of genetic and extrinsic factors to maintain contractile function. Failure to sustain its compensatory responses results in cardiac dysfunction, leading to cardiomyopathy. Diabetic cardiomyopathy (DCM is characterized by left ventricular hypertrophy and reduced diastolic function, with or without concurrent systolic dysfunction in the absence of hypertension and coronary artery disease. Changes in substrate metabolism, oxidative stress, endoplasmic reticulum stress, formation of extracellular matrix proteins, and advanced glycation end products constitute the early stage in DCM. These early events are followed by steatosis (accumulation of lipid droplets in cardiomyocytes, which is followed by apoptosis, changes in immune responses with a consequent increase in fibrosis, remodeling of cardiomyocytes, and the resultant decrease in cardiac function. The heart is an omnivore, metabolically flexible, and consumes the highest amount of ATP in the body. Altered myocardial substrate and energy metabolism initiate the development of DCM. Diabetic hearts shift away from the utilization of glucose, rely almost completely on fatty acids (FAs as the energy source, and become metabolically inflexible. Oxidation of FAs is metabolically inefficient as it consumes more energy. In addition to metabolic inflexibility and energy inefficiency, the diabetic heart suffers from impaired calcium handling with consequent alteration of relaxation–contraction dynamics leading to diastolic and systolic dysfunction. Sarcoplasmic reticulum (SR plays a key role in excitation–contraction coupling as Ca2+ is transported into the SR by the SERCA2a (sarcoplasmic/endoplasmic reticulum calcium-ATPase 2a during cardiac relaxation. Diabetic cardiomyocytes display decreased SERCA2a activity and leaky Ca2+ release channel resulting in reduced SR calcium load. The diabetic heart also suffers from

  14. Cardiomyopathy

    Science.gov (United States)

    ... Diagnosis & treatment Doctors & departments Care at Mayo Clinic Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  15. Cardiomyopathy

    Science.gov (United States)

    ... flow to the damaged or weakened heart muscle Heart transplant that may be tried when all other treatments ... accountability. A.D.A.M. is among the first to achieve this important distinction for online health ...

  16. Apical hypertrophic cardiomyopathy with hemodynamically unstable ventricular arrhythmia – Atypical presentation

    Directory of Open Access Journals (Sweden)

    Hemant Chaturvedi

    2016-09-01

    Full Text Available We present a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM who recently developed cardiac arrhythmias, and shortly discuss the diagnostic modalities, differential diagnosis, and treatment strategy for this condition. AHCM is a rare form of hypertrophic cardiomyopathy, which usually involves the apex of the left ventricle. AHCM can occur with varied presentations such as chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation, and congestive heart failure. The most peculiar electrocardiogram findings are giant T-waves inversion in the precordial leads with left ventricular (LV hypertrophy. A transthoracic echocardiogram is the initial diagnostic modality in the evaluation of AHCM and shows hypertrophy of the LV apex. Other diagnostic modalities, including left ventriculography, multislice spiral computed tomography, and cardiac magnetic resonance imagings, are also valuable tools. Medications used to manage include verapamil, beta-blockers, and antiarrhythmic agents. An implantable cardioverter defibrillator (ICD is recommended for high-risk patients.

  17. Spanish implantable cardioverter-defibrillator registry. 5th official report of the spanish society of cardiology working group on implantable cardioverter-defibrillators (2008).

    Science.gov (United States)

    Peinado, Rafael; Torrecilla, Esteban G; Ormaetxe, José; Alvarez, Miguel; Cózar, Rocío; Alzueta, Javier

    2009-12-01

    To summarize the findings of the Spanish Implantable Cardioverter-Defibrillator (ICD) Registry for 2008 compiled by the Spanish Society of Cardiology Working Group on Implantable Cardioverter-Defibrillators. Prospective data recorded voluntarily on single-page questionnaires were sent to the Spanish Society of Cardiology by each implantation team. Overall, 3486 device implantations were reported, which is 84.7% of the estimated total number of implantations. The reported implantation rate was 76 per million population and the estimated total implantation rate was 90 per million. The proportion of first implantations was 78.1%. There continued to be substantial regional variations within Spain. The majority of ICD implantations took place in men (mean age 62+/-12 years) who had severe or moderate-to-severe ventricular dysfunction and were in New York Heart Association functional class II. Ischemic heart disease was the most frequent underlying cardiac condition, followed by dilated cardiomyopathy. The number of indications for primary prevention increased relative to the previous year, especially in patients with ischemic cardiomyopathy, and now account for 57% of first implantations. The types of ICD implanted were unchanged from 2007. Overall, 73.6% of ICDs were implanted by cardiac electrophysiologists. The 2008 Spanish ICD Registry includes data on almost 85% of all ICD implantations performed in Spain. Although the number has continued to increase, it still remains far from the European average. There was a significant increase in indications for primary prevention. Substantial regional variations continue to exist within Spain.

  18. Arrhythmogenic Right Ventricular Cardiomyopathy: Risk Stratification and Indications for Defibrillator Therapy.

    Science.gov (United States)

    Zorzi, Alessandro; Rigato, Ilaria; Bauce, Barbara; Pilichou, Kalliopi; Basso, Cristina; Thiene, Gaetano; Iliceto, Sabino; Corrado, Domenico

    2016-06-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined disease which predisposes to life-threatening ventricular arrhythmias. The main goal of ARVC therapy is prevention of sudden cardiac death (SCD). Implantable cardioverter defibrillator (ICD) is the most effective therapy for interruption of potentially lethal ventricular tachyarrhythmias. Despite its life-saving potential, ICD implantation is associated with a high rate of complications and significant impact on quality of life. Accurate risk stratification is needed to identify individuals who most benefit from the therapy. While there is general agreement that patients with a history of cardiac arrest or hemodynamically unstable ventricular tachycardia are at high risk of SCD and needs an ICD, indications for primary prevention remain a matter of debate. The article reviews the available scientific evidence and guidelines that may help to stratify the arrhythmic risk of ARVC patients and guide ICD implantation. Other therapeutic strategies, either alternative or additional to ICD, will be also addressed.

  19. Athlete's heart or hypertrophic cardiomyopathy?

    Science.gov (United States)

    Lauschke, Jörg; Maisch, Bernhard

    2009-02-01

    Intensive endurance training is able to cause a distinct pattern of functional and structural changes of the cardiovascular system. In an unknown proportion of athletes a so called "athlete's heart" develops. There is an overlap between this type of physiologic cardiac hypertrophy and mild forms of hypertrophic cardiomyopathy (HCM), the most common genetic disorder of the cardiovascular system with a prevalence of 0.2%. HCM is caused by mutations in 14 genes coding for sarcomere proteins. In the literature up to 50% of cases of sudden cardiac death (SCD) in younger sportsmen were connected to hypertrophic cardiomyopathy. It is therefore the most common cause of SCD in highly trained young athletes. Because of this data a great interest in distinguishing these two diagnoses exists. Apart from clinical examination and some non-specific ECG-changes, Echocardiography is the method of choice. The athlete's heart shows an eccentric biventricular hypertrophy with wall thicknesses under 15 mm and a moderately dilated left ventricle (LVEDD up to 58 mm). HCM is commonly characterized by asymmetric left ventricular hypertrophy with a reduced LV-diameter. In up to 70% of cases left ventricular outflow tract obstruction is evident during stress echocardiography. Systolic function is normal in highly trained athletes and the majority of HCM patients as well. There are important differences regarding diastolic filling patterns. Physiological hypertrophy is consistent with a normal diastolic function with even increased early diastolic filling. In case of HCM diastolic dysfunction (mostly relaxation disturbances) occurs in the majority of patients and is therefore inconsistent with an athlete's heart. If the diagnosis could not be stated using echocardiography, methods like cardiac-MRI, metabolic exercise testing, histological studies of endomyocardial biopsies and genetic testing can provide further information. A correct diagnosis may on the one hand prevent some athletes from

  20. MR imaging in cardiomyopathies; MR-tomographische Diagnostik von Kardiomyopathien

    Energy Technology Data Exchange (ETDEWEB)

    Miller, S. [Radiologische Universitaetsklinik Tuebingen (Germany); Riessen, R. [Tuebingen Univ. (Germany). Medizinische Klinik

    2005-11-15

    According to the WHO classification, cardiomyopathies are a group of diseases which are associated with myocardial dysfunction and can be classified either as primary or secondary cardiomyopathies. Genetic disorders have been identified in certain primary cardiomyopathies, however often the etiology remains unknown. The term ''secondary cardiomyopathy'' is used to specify diseases with the clinical indications of a cardiomyopathy, but can be attributed to a certain pathophysiological mechanism such as exposure to toxic substances, metabolic syndromes or systemic diseases. Based on morphological and functional criteria, primary cardiomyopathies are divided into dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and restrictive cardiomyopathy (RCM). During the last two decades MR imaging has emerged to a well established diagnostic tool for the understanding and treatment of cardiomyopathies. Morphological and functional information can be achieved with a high level of accuracy and reproducibility. Tissue alteration of the myocardium can be detected assessing regional contrast enhancement, T1- and T2-signal intensities and chemical shift phenomena. This article describes characteristic aspects of MR imaging for the diagnosis of primary and secondary cardiomyopathies. (orig.)

  1. Apical Hypertrophic Cardiomyopathy in Association with PulmonaryArtery Hypertension

    Directory of Open Access Journals (Sweden)

    Mehdi Peighambari

    2012-09-01

    Full Text Available Apical Hypertrophic Cardiomyopathy is an uncommon condition constituting 1% -2% of the cases with Hypertrophic Cardiomyopathy (HCM diagnosis. We interestingly report two patients with apical hypertrophic cardiomyopathy in association with significant pulmonary artery hypertension without any other underlying reason for pulmonary hypertension. The patients were assessed by echocardiography, cardiac catheterization and pulmonary function parameters study.

  2. Metabolic imaging of patients with cardiomyopathy

    International Nuclear Information System (INIS)

    Geltman, E.M.

    1991-01-01

    The cardiomyopathies comprise a diverse group of illnesses that can be characterized functionally by several techniques. However, the delineation of derangements of regional perfusion and metabolism have been accomplished only relatively recently with positron emission tomography (PET). Regional myocardial accumulation and clearance of 11C-palmitate, the primary myocardial substrate under most conditions, demonstrate marked spatial heterogeneity when studied under fasting conditions or with glucose loading. PET with 11C-palmitate permits the noninvasive differentiation of patients with nonischemic from ischemic dilated cardiomyopathy, since patients with ischemic cardiomyopathy demonstrate large zones of intensely depressed accumulation of 11C-palmitate, probably reflecting prior infarction. Patients with hypertrophic cardiomyopathy and Duchenne's muscular dystrophy demonstrate relatively unique patterns of myocardial abnormalities of perfusion and metabolism. The availability of new tracers and techniques for the evaluation of myocardial metabolism (11C-acetate), perfusion (H2(15)O), and autonomic tone (11-C-hydroxyephedrine) should facilitate further understanding of the pathogenesis of the cardiomyopathies

  3. Cardiac MRI in restrictive cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, A. [Department of Cardiovascular Radiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India); Singh Gulati, G., E-mail: gulatigurpreet@rediffmail.com [Department of Cardiovascular Radiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India); Seth, S. [Department of Cardiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India); Sharma, S. [Department of Cardiovascular Radiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India)

    2012-02-15

    Restrictive cardiomyopathy (RCM) is a specific group of heart muscle disorders characterized by inadequate ventricular relaxation during diastole. This leads to diastolic dysfunction with relative preservation of systolic function. Although short axis systolic function is usually preserved in RCM, the long axis systolic function may be severely impaired. Confirmation of diagnosis and information regarding aetiology, extent of myocardial damage, and response to treatment requires imaging. Importantly, differentiation from constrictive pericarditis (CCP) is needed, as only the latter is managed surgically. Echocardiography is the initial cardiac imaging technique but cannot reliably suggest a tissue diagnosis; although recent advances, especially tissue Doppler imaging and spectral tracking, have improved its ability to differentiate RCM from CCP. Cardiac catheterization is the reference standard, but is invasive, two-dimensional, and does not aid myocardial characterization. Cardiac magnetic resonance (CMR) is a versatile technique providing anatomical, morphological and functional information. In recent years, it has been shown to provide important information regarding disease mechanisms, and also been found useful to guide treatment, assess its outcome and predict patient prognosis. This review describes the CMR features of RCM, appearances in various diseases, its overall role in patient management, and how it compares with other imaging techniques.

  4. Peripartum cardiomyopathy – case series

    Science.gov (United States)

    Prasad, Gowri Sayi; Bhupali, Ashok; Prasad, Sayi; Patil, Ajit N.; Deka, Yashodhan

    2014-01-01

    Objectives To study the pattern of presentation, course of disease and outcome of pregnancy in Peripartum Cardiomyopathy. Methods A prospective study of sixteen cases of PPCM was conducted at Apple Saraswati Multispecialty Hospital and Dr. D.Y. Patil Medical College and Hospital, Kolhapur, Maharashtra, India from January 2006 to December 2012. Data included age distribution, parity, gestational age, symptoms and risk factors. Medical management and pregnancy outcome were documented. Serial echocardiography data was compiled for a period of one year. Results In our study 9/16 (56%) were primigravidae, 4/16 (25%) had pre-eclamsia and 6/16 (35%) had co-existing hypertension. The difference in Echocardiography parameters observed between recovered and non-recovered patients was significant: Left Ventricular End diastolic dimension (5.6 cm vs 6.06 cm), Left Ventricular Ejection Fraction (28.7% vs 22.4%) and Left Ventricular fractional shortening (17.5% vs 13.4%). Thirteen out of sixteen patients were followed up for a period of one year out of which 61% (8/13) patients recovered completely. There was one mortality. Conclusion PPCM is a diagnosis of exclusion. Majority were young primigravidae presenting postnatally. Pre-eclampsia and hypertension were risk factors. ECHO parameters were reliable predictors of recovery. Future pregnancies are better avoided. PMID:24814122

  5. Cardiac sarcoid: a chameleon masquerading as hypertrophic cardiomyopathy and dilated cardiomyopathy in the same patient.

    Science.gov (United States)

    Agarwal, Anushree; Sulemanjee, Nasir Z; Cheema, Omar; Downey, Francis X; Tajik, A Jamil

    2014-05-01

    Sarcoidosis is a multisystem, granulomatous disease of unknown etiology often seen in young adults, with cardiac involvement in more than one-quarter of sarcoid patients. The clinical presentation of cardiac sarcoid depends upon the location and extent of myocardium involved. Although cardiac sarcoid may produce asymmetrical septal hypertrophy, it is most commonly considered in the differential diagnosis of dilated cardiomyopathy. The hypertrophic stage of cardiac sarcoid is rarely seen. We describe a case of cardiac sarcoid in a young patient wherein a distinctive appearance of the cardiac sarcoid spectrum from "hypertrophic" stage to thinned/scarred stage, masquerading as hypertrophic cardiomyopathy followed by dilated cardiomyopathy, is demonstrated. © 2014, Wiley Periodicals, Inc.

  6. Recurrent Syncope, a Clue in Amyloid Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Julian A. Marin-Acevedo

    2018-01-01

    Full Text Available Infiltrative cardiomyopathies include a variety of disorders that lead to myocardial thickening resulting in a constellation of clinical manifestations and eventually heart failure that could be the first clue to reach the diagnosis. Among the more described infiltrative diseases of the heart is amyloid cardiomyopathy. The disease usually presents with subtle, nonspecific symptoms. Herein, we illustrate a case of recurrent syncope as the initial presenting symptom for systemic amyloid with polyneuropathy and cardiomyopathy as a cause of syncope. The article illustrates the role of advanced cardiac imaging in the diagnosis of the disease with a focused literature review. We also highlight the role of early, shared decision-making between patient, family, and medical team in the management of cardiac amyloidosis.

  7. Cardiomyopathie hypertrophique neonatale de diagnostic etiologique difficile

    Directory of Open Access Journals (Sweden)

    Rania Hammami

    2011-12-01

    Full Text Available La cardiomyopathie hypertrophique neonatale est une entite rare, heterogene regroupant plusieurs formes cliniques et donc de diagnostic etiologique difficile. Nous rapportons l�observation d�un nouveau ne issu d�une grossesse gemellaire, ayant presente a la naissance un tableau d�insuffisance cardiaque, l�echocardiographie avait conclut a une cardiomyopathie hypertrophique obstructive. Le bilan etiologique etait negatif notamment une mere non diabetique. L�evolution etait favorable avec regression de l�hypertrophie 2 semaines apres la naissance. L�etiologie finalement suggeree etait une cardiomyopathie secondaire a l�injection antenatale de corticoides dans le but d�accelerer la maturation pulmonaire. L�etablissement par les societes savantes d�un consensus de bilan etiologique minimal standard selon une chronologie bien determinee serait d�un grand apport dans la prise en charge de cette anomalie.

  8. Diagnostic work-up in cardiomyopathies

    DEFF Research Database (Denmark)

    Rapezzi, Claudio; Arbustini, Eloisa; Caforio, Alida L P

    2013-01-01

    In 2008, The ESC Working Group on Myocardial and Pericardial Diseases proposed an updated classification of cardiomyopathies based on morphological and functional phenotypes and subcategories of familial/genetic and non-familial/non-genetic disease. In this position statement, we propose a framew...... conventional cardiological assessment with non-cardiac and molecular parameters increases diagnostic accuracy and thus improves advice and treatment for patients and families....... a framework for the clinical approach to diagnosis in cardiomyopathies based on the recognition of diagnostic 'red flags' that can be used to guide rational selection of specialized tests including genetic analysis. The basic premise is that the adoption of a cardiomyopathy-specific mindset which combines...

  9. [Spanish Implantable Cardioverter-Defibrillator Registry. Fourth Official Report of the Spanish Society of Cardiology Working Group on Implantable Cardioverter-Defibrillators (2007)].

    Science.gov (United States)

    Peinado Peinado, Rafael; Torrecilla, Esteban G; Ormaetxe, José; Alvarez, Miguel

    2008-11-01

    This article presents the 2007 findings of the Spanish Implantable Cardioverter-Defibrillator (ICD) Registry, established by the Working Group on Implantable Cardioverter-Defibrillators, Electrophysiology and Arrhythmia Section, Spanish Society of Cardiology. The Spanish Society of Cardiology received prospective data recorded on a single-page questionnaire on 96.6% of device implantations. Overall, 3,291 implantations were reported (90.1% of the estimated total). The reported implantation rate was 72.8 per million inhabitants, and 77.1% were first implantations. The majority of ICDs were implanted in males (mean age, 61 [12] years) in functional class II with severe or moderate-to-severe left ventricular dysfunction. The most frequent form of heart disease was ischemic heart disease, followed by dilated cardiomyopathy. Indications for primary prevention remained unchanged relative to the previous year and now account for half of all first implantations, with an increasing number of patients with dilated cardiomyopathy. The number of ICDs incorporating cardiac resynchronization therapy has increased slightly and now comprises 30.1% of the total. Around 70% of ICD implantations were performed in an electrophysiology laboratory by a cardiac electrophysiologist. The incidence of complications was very low. The 2007 Spanish Implantable Cardioverter-Defibrillator Registry contains data on more than 90% of all ICD implantations performed in Spain, thereby confirming that it has become increasingly representative in recent years. The number of implantations has continued to grow, though the proportion carried out for primary prevention has stabilized at around 50%.

  10. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy and infiltrative cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Rebecca Schofield

    2016-11-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is the most common inherited cardiac disease. Cardiac imaging plays a key role in the diagnosis and management, with cardiovascular magnetic resonance (CMR an important modality. CMR provides a number of different techniques in one examination: structure and function, flow imaging and tissue characterisation particularly with the late gadolinium enhancement (LGE technique. Other techniques include vasodilator perfusion, mapping (especially T1 mapping and extracellular volume quantification [ECV] and diffusion-weighted imaging with its potential to detect disarray. Clinically, the uses of CMR are diverse. The imaging must be considered within the context of work-up, particularly the personal and family history, Electrocardiogram (ECG and echocardiogram findings. Subtle markers of possible HCM can be identified in genotype positive left ventricular hypertrophy (LVH-negative subjects. CMR has particular advantages for assessment of the left ventricle (LV apex and is able to detect both missed LVH (apical and basal antero-septum, when the echocardiography is normal but the ECG abnormal. CMR is important in distinguishing HCM from both common phenocopies (hypertensive heart disease, athletic adaptation, ageing related changes and rarer pheno and/or genocopies such as Fabry disease and amyloidosis. For these, in particular the LGE technique and T1 mapping are very useful with a low T1 in Fabry’s, and high T1 and very high ECV in amyloidosis. Moreover, the tissue characterisation that is possible using CMR offers a potential role in patient risk stratification, as scar is a very strong predictor of future heart failure. Scar may also play a role in the prediction of sudden death. CMR is helpful in follow-up assessment, especially after septal alcohol ablation and myomectomy.

  11. Randomized Trial of Benznidazole for Chronic Chagas' Cardiomyopathy.

    Science.gov (United States)

    Morillo, Carlos A; Marin-Neto, Jose Antonio; Avezum, Alvaro; Sosa-Estani, Sergio; Rassi, Anis; Rosas, Fernando; Villena, Erick; Quiroz, Roberto; Bonilla, Rina; Britto, Constança; Guhl, Felipe; Velazquez, Elsa; Bonilla, Laura; Meeks, Brandi; Rao-Melacini, Purnima; Pogue, Janice; Mattos, Antonio; Lazdins, Janis; Rassi, Anis; Connolly, Stuart J; Yusuf, Salim

    2015-10-01

    The role of trypanocidal therapy in patients with established Chagas' cardiomyopathy is unproven. We conducted a prospective, multicenter, randomized study involving 2854 patients with Chagas' cardiomyopathy who received benznidazole or placebo for up to 80 days and were followed for a mean of 5.4 years. The primary outcome in the time-to-event analysis was the first event of any of the components of the composite outcome of death, resuscitated cardiac arrest, sustained ventricular tachycardia, insertion of a pacemaker or implantable cardioverter-defibrillator, cardiac transplantation, new heart failure, stroke, or other thromboembolic event. The primary outcome occurred in 394 patients (27.5%) in the benznidazole group and in 414 (29.1%) in the placebo group (hazard ratio, 0.93; 95% confidence interval [CI], 0.81 to 1.07; P=0.31). At baseline, a polymerase-chain-reaction (PCR) assay was performed on blood samples obtained from 1896 patients; 60.5% had positive results for Trypanosoma cruzi on PCR. The rates of conversion to negative PCR results (PCR conversion) were 66.2% in the benznidazole group and 33.5% in the placebo group at the end of treatment, 55.4% and 35.3%, respectively, at 2 years, and 46.7% and 33.1%, respectively, at 5 years or more (P<0.001 for all comparisons). The effect of treatment on PCR conversion varied according to geographic region: in Brazil, the odds ratio for PCR conversion was 3.03 (95% CI, 2.12 to 4.34) at 2 years and 1.87 (95% CI, 1.33 to 2.63) at 5 or more years; in Colombia and El Salvador, the odds ratio was 1.33 (95% CI, 0.90 to 1.98) at 2 years and 0.96 (95% CI, 0.63 to 1.45) at 5 or more years; and in Argentina and Bolivia, the odds ratio was 2.63 (95% CI, 1.89 to 3.66) at 2 years and 2.79 (95% CI, 1.99 to 3.92) at 5 or more years (P<0.001 for interaction). However, the rates of PCR conversion did not correspond to effects on clinical outcome (P=0.16 for interaction). Trypanocidal therapy with benznidazole in patients with

  12. Cardiac Pacing Suppressed Macroscopic T Wave Alternans in a Patient with Heart Failure Caused by Non-ischemic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Kotaro Miyaji, MD

    2008-01-01

    Full Text Available A 67-year-old male with dilated cardiomyopathy and chronic renal failure who received chronic hemodialysis for 9 years admitted with pulmonary edema. Three days after admission, electrocardiogram showed transient prolongation of QT interval which was followed by macroscopic T wave alternans (TWA and ventricular fibrillation (VF. Temporary pacing from right ventricular apex suppressed TWA and VF effectively. Combined cardiac resynchronization therapy and implantable cardioverter defibrillator device was implanted for the secondary prevention of VF. Both prolongation of QT interval and TWA disappeared for 10 days after therapy and no arrhythmic event occurred since then.

  13. X-linked cardiomyopathy is heterogeneous

    Energy Technology Data Exchange (ETDEWEB)

    Wilson, M.J.; Sillence, D.O.; Mulley, J.C. [and others

    1994-09-01

    Two major loci of X-linked cardiomyopathy have been mapped by linkage analysis. The gene for X-linked dilated cardiomyopathy (XLCM) is mapped to the dystrophin locus at Xp21, while Barth syndrome has been localised to distal Xq28. XLCM usually presents in juvenile males with no skeletal disease but decreased dystrophin in cardiac muscle. Barth syndrome most often presents in infants and is characterized by skeletal myopathy, short stature and neutropenia in association with cardiomyopathy of variable severity. Prior to carrier or prenatal diagnosis in a family, delineation of the cardiomyopathy locus involved is essential. We report the linkage mapping of a large kindred in which several male infants have died with hypertrophic cardiomyopathy. There is a family history of unexplained death of infant males less than 6 months old over 4 generations. Features of Barth syndrome such as short stature, skeletal myopathy and neutropenia have not been observed. Genotyping at 10 marker loci in Xq28 has revealed significant pairwise lod scores with the cardiomyopathy phenotype at DXS52 (Z=2.21 at {theta}=0.0), at markers p26 and p39 near DXS15 (Z=2.30 at {theta}=0.0) and at F8C (Z=2.24 at {theta}=0.0). A recombinant detected with DXS296 defines the proximal limit to the localization. No recombinants were detected at any of the loci distal to DXS296. The most distal marker in Xq28, DXS1108, is within 500 kb of the telomere. As the gene in this family is localized to Xq28, it is possible that this disorder is an allelic variant at the Barth syndrome locus.

  14. Cirrhotic cardiomyopathy: a pathophysiological review of circulatory dysfunction in liver disease

    DEFF Research Database (Denmark)

    Møller, Søren; Henriksen, Jens Henrik Sahl

    2002-01-01

    is an entity different from that seen in alcoholic heart muscle disease. Clinically, these patients present with sodium fluid retention and strain often unmasks the presence of latent heart failure. No specific treatment can yet be recommended but caution should be used with respect to procedures that may...... stress the heart such as shunt implantation and liver transplantation.......The systemic circulation in patients with cirrhosis is hyperdynamic with an increased cardiac output and heart rate and a reduced systemic vascular resistance as the most pronounced alterations. The concomitant cardiac dysfunction has recently been termed "cirrhotic cardiomyopathy", which...

  15. Rachitic hypocalcemic cardiomyopathy in an infant

    Directory of Open Access Journals (Sweden)

    Abdelwahab T.H. Elidrissy

    2017-04-01

    Full Text Available Cardiomyopathy in infants is characterized by heart failure in apparently normal children without previous organic cardiac lesions. Cardiomyopathy has been found to comprise four types. Rickets is common in Saudi Arabia, that is why I reviewed this subject. Recently this case with classical features of rickets being admitted in a serious state we thought of publishing it. The infant responded well to treatment and full recovery was achieved. Follow up biochemistry, radiology cardiac function completely recovered and bony abnormalities showed evidence of healing. This case might have been missed as respiratory infection. We recommend meticulous look for biochemical features of rickets in infants admitted with respiratory symptoms.

  16. Multimodality imaging in apical hypertrophic cardiomyopathy

    Science.gov (United States)

    Parisi, Rosario; Mirabella, Francesca; Secco, Gioel Gabrio; Fattori, Rossella

    2014-01-01

    Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the first-line imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease. PMID:25276293

  17. Predictors of appropriate ICD therapy in patients with arrhythmogenic right ventricular cardiomyopathy: long term experience of a tertiary care center.

    Directory of Open Access Journals (Sweden)

    Pia K Schuler

    Full Text Available INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC is a rare genetically transmitted disease prone to ventricular arrhythmias. We therefore investigated the clinical, echocardiographical and electrophysiological predictors of appropriate implantable cardioverter defibrillator (ICD therapy in patients with ARVC. METHODS: A retrospective analysis was performed in 26 patients (median age of 40 years at diagnosis, 21 males and 5 females with ARVC who underwent ICD implantation. RESULTS: Over a median (range follow-up period of 10 (2.7, 37 years, appropriate ICD therapy for ventricular arrhythmias was documented in 12 (46% out of 26 patients. In all patients with appropriate ICD therapy the ICD was originally inserted for secondary prevention. Median time from ICD implantation to ICD therapy was 9 months (range 3.6, 54 months. History of heart failure was a significant predictor of appropriate ICD therapy (p = 0.033. Left ventricular disease involvement (p = 0.059 and age at implantation (p = 0.063 were borderline significant predictors. Patients with syncope at time of diagnosis were significantly less likely to receive ICD therapy (p = 0.02. Invasive electrophysiological testing was not significantly associated with appropriate ICD therapy. CONCLUSION: In our cohort of patients with ARVC, history of heart failure was a significant predictor of appropriate ICD therapy, whereas left ventricular involvement and age at time of ICD implantation were of borderline significance. These predictors should be tested in larger prospective cohorts to optimize ICD therapy in this rare cardiomyopathy.

  18. Mitochondrial Cardiomyopathy Presenting as Dilated Phase of Hypertrophic Cardiomyopathy Diagnosed with Histological and Genetic Analyses

    Directory of Open Access Journals (Sweden)

    Toshiki Kuno

    2017-01-01

    Full Text Available We report a case with 46-year-old man diagnosed with mitochondrial cardiomyopathy in the dilated phase of hypertrophic cardiomyopathy. Since cardiac magnetic resonance imaging, beta-methyl-p-I123-iodophenyl-pentadecanoic myocardial scintigraphy, and positron emission tomography/computed tomography revealed no remarkable findings, we performed electron microscopic examination, which aided in diagnosing mitochondrial cardiomyopathy. Muscle biopsy was also compatible with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes and DNA analysis also concluded it. Since muscle biopsy is less invasive for patients compared to endomyocardial biopsy, cardiologists need to consider it. The diagnosis of mitochondrial cardiomyopathy is helpful because it is a genetic condition and also for consideration of device therapy, as well as management for acute crisis.

  19. Guidelines for the diagnosis and management of arrhythmogenic right ventricular cardiomyopathy.

    Science.gov (United States)

    Smith, Warren

    2011-12-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an uncommon inherited myocardial disorder characterised by fibro-fatty inflammation affecting the right and left ventricles. It most commonly presents with palpitations or syncope but sudden death may occur, especially in young males. Diagnosis is not possible with a single test and may be difficult. Task Force criteria agreed in 1994 comprise major and minor criteria spanning structural abnormalities, ECG appearances, arrhythmias, family history of premature death and myocardial histology. Modified criteria were introduced in 2010 to improve sensitivity. Arrhythmogenic right ventricular cardiomyopathy is a desmosomal disease. Mutations have been detected in five desmosomal genes, most frequently in plakophilin-2 (PKP2) and multiple mutations are also reported. Antiarrhythmic drugs such as sotalol and amiodarone may improve symptoms but are unproven to increase survival. An implantable defibrillator is appropriate in individuals surviving cardiac arrest or sustained ventricular tachycardia, but there is not yet consensus about prophylactic treatment of Task Force positive but asymptomatic individuals. Arrhythmogenic right ventricular cardiomyopathy is more common than previously believed. Preliminary evidence supports improved sensitivity without loss of specificity using the revised Task Force criteria. The genetics of the disease are complex but should ultimately advance diagnosis and management. Copyright © 2011 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier B.V. All rights reserved.

  20. Reversible dilated cardiomyopathy: into the thaumaturgy of the heart - Part 1

    Directory of Open Access Journals (Sweden)

    Giovanni Quarta

    2016-10-01

    Full Text Available Dilated cardiomyopathy (DCM is a genetic or acquired heart muscle disorder characterized by dilation and impaired contraction of one or both ventricles. In the acquired forms of the disease, if the pathogenic agent is persistent, undiagnosed or untreated, permanent ultrastructural and morphological changes may lead to irreversible dysfunction. Conversely, when DCM is promptly recognized and treated, the heart may show an extraordinary ability to recover from left ventricular (LV systolic dysfunction. While much research in heart failure has focused on morbidity and mortality associated with persistent LV systolic dysfunction, relatively little attention has been devoted to this remarkable potential for recovery. In this two-part review we will focus on the most common types of reversible DCM. The first part will deal with Tako-Tsubo cardiomyopathy, tachycardiainduced cardiomyopathy, metabolic DCM and recovery after Left ventricular assist device implantation. Although diverse in etiopathogenesis, genetic background, therapeutic options and outcome, the forms of DCM characterized by reversible LV dysfunction share similar challenges in diagnosis and clinical management. The identification of pathways to recovery may show the way for novel therapeutic targets ultimately benefitting all cardiac patients.

  1. Pathology of Chronic Chagas Cardiomyopathy in the United States:  A Detailed Review of 13 Cardiectomy Cases.

    Science.gov (United States)

    Kransdorf, Evan P; Fishbein, Mike C; Czer, Lawrence S C; Patel, Jignesh K; Velleca, Angela; Tazelaar, Henry D; Roy, R Raina; Steidley, D Eric; Kobashigawa, Jon A; Luthringer, Daniel J

    2016-08-01

    The pathologic features of chronic Chagas cardiomyopathy may not be widely appreciated in the United States. We sought to describe the gross, microscopic, immunohistochemical, and molecular pathology features useful to diagnose chronic Chagas cardiomyopathy. The features from a case series of cardiectomy specimens of patients undergoing heart transplantation (12 patients) or mechanical circulatory support device implantation (one patient) for chronic Chagas cardiomyopathy at three institutions in the United States are reported and analyzed. Gross findings included enlarged and dilated ventricles (100% of cases), mural thrombi (54%), epicardial plaques (42%), and left ventricular aneurysm (36%). Microscopic evaluation revealed myocarditis (100% of cases) characterized by mononuclear cell infiltration, fibrosis (100%), nonnecrotizing granulomas (62%), and giant cells (38%). Two specimens (15%) showed rare intracellular amastigotes. Immunohistochemical assays for Trypanosoma cruzi organisms were negative in all cardiectomy specimens, whereas tissue polymerase chain reaction was positive in six (54%) of 11 cases. The gross and microscopic features of chronic Chagas cardiomyopathy in the United States appear similar to those reported in endemic countries. Importantly, tissue polymerase chain reaction may be useful to confirm the diagnosis. © American Society for Clinical Pathology, 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  2. Fabry Disease in Families With Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Adalsteinsdottir, Berglind; Palsson, Runolfur; Desnick, Robert J

    2017-01-01

    BACKGROUND: The screening of Icelandic patients clinically diagnosed with hypertrophic cardiomyopathy resulted in identification of 8 individuals from 2 families with X-linked Fabry disease (FD) caused by GLA(α-galactosidase A gene) mutations encoding p.D322E (family A) or p.I232T (family B...

  3. Contractile Dysfunction in Sarcomeric Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    MacIver, David H; Clark, Andrew L

    2016-09-01

    The pathophysiological mechanisms underlying the clinical phenotype of sarcomeric hypertrophic cardiomyopathy are controversial. The development of cardiac hypertrophy in hypertension and aortic stenosis is usually described as a compensatory mechanism that normalizes wall stress. We suggest that an important abnormality in hypertrophic cardiomyopathy is reduced contractile stress (the force per unit area) generated by myocardial tissue secondary to abnormalities such as cardiomyocyte disarray. In turn, a progressive deterioration in contractile stress provokes worsening hypertrophy and disarray. A maintained or even exaggerated ejection fraction is explained by the increased end-diastolic wall thickness producing augmented thickening. We propose that the nature of the hemodynamic load in an individual with hypertrophic cardiomyopathy could determine its phenotype. Hypertensive patients with hypertrophic cardiomyopathy are more likely to develop exaggerated concentric hypertrophy; athletic individuals an asymmetric pattern; and inactive individuals a more apical hypertrophy. The development of a left ventricular outflow tract gradient and mitral regurgitation may be explained by differential regional strain resulting in mitral annular rotation. Copyright © 2016. Published by Elsevier Inc.

  4. Hypertrophic Cardiomyopathy Update on Prognosis and Therapy

    NARCIS (Netherlands)

    C. van der Lee (Christiaan)

    2006-01-01

    textabstractHypertrophic cardiomyopathy (HCM) is an intriguing disease due to its heterogeneity in genetic, morphologic, and clinical spectrum. The insights in the prognosis and natural history of HCM have evolved tremendously over the past 40 years. The fi rst studies, which contained data

  5. Hypertrophic Cardiomyopathy in Athletes: Catching a Killer.

    Science.gov (United States)

    Maron, Barry J.

    1993-01-01

    A leading cause of sudden death among young athletes, hypertrophic cardiomyopathy (HCM) does not always present cardiac signs and symptoms. Echocardiography offers the most effective means for diagnosis. Some patients require pharmaceutical or surgical intervention. Patients with HCM should not engage in organized competitive sports or…

  6. Clinical and molecular classification of cardiomyopathies

    Directory of Open Access Journals (Sweden)

    Franco Cecchi

    2012-07-01

    Full Text Available The term “cardiomyopathies” was used for the first time 55 years ago, in 1957. Since then awareness and knowledge of this important and complex group of heart muscle diseases have improved substantially. Over these past five decades a large number of definitions, nomenclature and schemes, have been advanced by experts and consensus panel, which reflect the fast and continued advance of the scientific understanding in the field. Cardiomyopathies are a heterogeneous group of inherited myocardial diseases, which represent an important cause of disability and adverse outcome. Although considered rare diseases, the overall estimated prevalence of all cardiomyopathies is at least 3% in the general population worldwide. Furthermore, their recognition is increasing due to advances in imaging techniques and greater awareness in both the public and medical community. Cardiomyopathies represent an ideal translational model of integration between basic and clinical sciences. A multidisciplinary approach is therefore essential in order to ensure their correct diagnosis and management. In the present work, we aim to provide a concise overview of the historical background, genetic and phenotypic spectrum and evolving concepts leading to the various attempts of cardiomyopathy classifications produced over the decades.

  7. Functional effects of losartan in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Axelsson, Anna Karin Irene; Iversen, Kasper; Vejlstrup, Niels G.

    2016-01-01

    OBJECTIVE: There is a lack of disease-modifying treatments in hypertrophic cardiomyopathy (HCM). The aim of this randomised, placebo-controlled study was to assess if losartan could improve or ameliorate deterioration of cardiac function and exercise capacity. METHODS: Echocardiography, exercise...

  8. Pregnancy in women with hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Pieper, P. G.; Walker, F.

    Hypertrophic cardiomyopathy (HCM) is increasingly being diagnosed in pregnant women. Women with HCM generally tolerate pregnancy well. The risk is however higher in women who are symptomatic before pregnancy or in those with severe left ventricular outflow tract obstruction. The incidence of

  9. A Systematic Review of Phenotypic Features Associated With Cardiac Troponin I Mutations in Hereditary Cardiomyopathies

    DEFF Research Database (Denmark)

    Mogensen, Jens; Hey, Thomas; Lambrecht, Sascha

    2015-01-01

    BACKGROUND: Genetic investigations have established that mutations in proteins of the contractile unit of the myocardium, known as the sarcomere, may be associated with hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), and dilated cardiomyopathy (DCM). It has become clinical...

  10. Autologous transplantation of bone marrow adult stem cells for the treatment of idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Westphal, Ricardo João; Bueno, Ronaldo Rocha Loures; Galvão, Paulo Bezerra de Araújo; Zanis Neto, José; Souza, Juliano Mendes; Guérios, Ênio Eduardo; Senegaglia, Alexandra Cristina; Brofman, Paulo Roberto; Pasquini, Ricardo; Cunha, Claudio Leinig Pereira da

    2014-12-01

    Morbimortality in patients with dilated idiopathic cardiomyopathy is high, even under optimal medical treatment. Autologous infusion of bone marrow adult stem cells has shown promising preliminary results in these patients. Determine the effectiveness of autologous transplantation of bone marrow adult stem cells on systolic and diastolic left ventricular function, and on the degree of mitral regurgitation in patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. We administered 4,54 x 10(8) ± 0,89 x 10(8) bone marrow adult stem cells into the coronary arteries of 24 patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Changes in functional class, systolic and diastolic left ventricular function and degree of mitral regurgitation were assessed after 3 months, 6 months and 1 year. During follow-up, six patients (25%) improved functional class and eight (33.3%) kept stable. Left ventricular ejection fraction improved 8.9%, 9.7% e 13.6%, after 3, 6 and 12 months (p = 0.024; 0.017 and 0.018), respectively. There were no significant changes neither in diastolic left ventricular function nor in mitral regurgitation degree. A combined cardiac resynchronization and implantable cardioversion defibrillation was implanted in two patients (8.3%). Four patients (16.6%) had sudden death and four patients died due to terminal cardiac failure. Average survival of these eight patients was 2.6 years. Intracoronary infusion of bone marrow adult stem cells was associated with an improvement or stabilization of functional class and an improvement in left ventricular ejection fraction, suggesting the efficacy of this intervention. There were no significant changes neither in left ventricular diastolic function nor in the degree of mitral regurgitation.

  11. Autologous Transplantation of Bone Marrow Adult Stem Cells for the Treatment of Idiopathic Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ricardo João Westphal

    2014-12-01

    Full Text Available Background: Morbimortality in patients with dilated idiopathic cardiomyopathy is high, even under optimal medical treatment. Autologous infusion of bone marrow adult stem cells has shown promising preliminary results in these patients. Objective: Determine the effectiveness of autologous transplantation of bone marrow adult stem cells on systolic and diastolic left ventricular function, and on the degree of mitral regurgitation in patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Methods: We administered 4,54 x 108 ± 0,89 x 108 bone marrow adult stem cells into the coronary arteries of 24 patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Changes in functional class, systolic and diastolic left ventricular function and degree of mitral regurgitation were assessed after 3 months, 6 months and 1 year. Results: During follow-up, six patients (25% improved functional class and eight (33.3% kept stable. Left ventricular ejection fraction improved 8.9%, 9.7% e 13.6%, after 3, 6 and 12 months (p = 0.024; 0.017 and 0.018, respectively. There were no significant changes neither in diastolic left ventricular function nor in mitral regurgitation degree. A combined cardiac resynchronization and implantable cardioversion defibrillation was implanted in two patients (8.3%. Four patients (16.6% had sudden death and four patients died due to terminal cardiac failure. Average survival of these eight patients was 2.6 years. Conclusion: Intracoronary infusion of bone marrow adult stem cells was associated with an improvement or stabilization of functional class and an improvement in left ventricular ejection fraction, suggesting the efficacy of this intervention. There were no significant changes neither in left ventricular diastolic function nor in the degree of mitral regurgitation.

  12. Evaluation of left ventricular torsion in children with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Prinz, Christian; Faber, Lothar; Horstkotte, Dieter; Körperich, Hermann; Moysich, Axel; Haas, Nikolaus; Kececioglu, Deniz; Thorsten Laser, Kai

    2014-04-01

    To evaluate the role of torsion in hypertrophic cardiomyopathy in children. A total of 88 children with idiopathic hypertrophic cardiomyopathy (n = 24) and concentric hypertrophy (n = 20) were investigated with speckle-tracking echocardiography and compared with age- and gender-matched healthy controls (n = 44). In hypertrophic cardiomyopathy, we found increased torsion (2.8 ± 1.6 versus 1.9 ± 1.0°/cm [controls], p Hypertrophic cardiomyopathy patients demonstrated a negative correlation between left ventricular muscle mass and torsion (r = -0.7, p hypertrophic cardiomyopathy is characterised by predominantly enhanced systolic basal clockwise rotation. Diastolic untwisting is delayed in both groups. Torsion may be an interesting marker to guide patients with hypertrophic cardiomyopathy.

  13. Cushing's syndrome in pregnancy and neonatal hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Fayol, L; Masson, P; Millet, V; Simeoni, U

    2004-10-01

    Cushing's syndrome is rare in pregnancy but can cause spontaneous abortion, stillbirth or premature birth. We report a case of transient hypertrophic obstructive cardiomyopathy in a newborn whose mother had hypercortisolism due to a primary adrenal lesion. There was no family history of hypertrophic obstructive cardiomyopathy. Follow-up revealed complete resolution of the cardiac abnormalities in the infant. Cushing's syndrome in the mother resolved after delivery. Although maternal hypercortisolism seldom results in symptomatic hypercortisolism in the newborn, hypertrophic obstructive cardiomyopathy can occur.

  14. Arrhythmogenic right ventricular cardiomyopathy/dysplasia in Iraq.

    Science.gov (United States)

    Al-Hamdi, Amar; Al-Kinani, Tahseen Ali; Al-Khafaji, Adnan Taan; Hamed, Mouayed Basheer; Al-Mayahi, Mohammed Hashim; Al-Sudani, Nazar Hassan

    2010-01-01

    Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a disorder that involves replacement of the right ventricular myocardium with fibro-fatty tissue. Ventricular tachycardia is a main presenting feature. There are no known reports of this disease from the Arab countries in the Middle East. This is the first report of 34 patients from Iraq. Thirty four patients with ARVC/D diagnosed from January 2003 to May 2007 according to the International Task Force criteria were included in this study. All patients presented with ventricular tachycardia of left bundle branch block morphology. The following findings were seen on the 12-lead electrocardiography during sinus rhythm: T wave inversion V1-V3 or beyond in 80%, epsilon wave in 28%, and parietal block in 48%. Right ventricular enlargement by echocardiography was seen in 69%. Twenty two per cent had a family history of sudden cardiac death. All patients were treated with implanted cardioverter-defibrillators. ARVC/D is a disease seen in Iraq. It requires a high diagnostic suspicion with verification using the international task force criteria.

  15. Progress with genetic cardiomyopathies: Screening, counseling, and testing in dilated, hypertrophic and arrhythmogenic right ventricular dysplasia/cardiomyopathy

    Science.gov (United States)

    Hershberger, Ray E.; Cowan, Jason; Morales, Ana; Siegfried, Jill D.

    2010-01-01

    Summary This review focuses on the genetic cardiomyopathies: principally dilated cardiomyopathy (DCM), with salient features of hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), regarding genetic etiology, genetic testing, and genetic counseling. Enormous progress has recently been made in identifying genetic causes for each cardiomyopathy, and key phenotype and genotype information is reviewed. Clinical genetic testing is rapidly emerging with a principal rationale of identifying at-risk asymptomatic or disease-free relatives. Knowledge of a disease-causing mutation can guide clinical surveillance for disease onset, thereby enhancing preventive and treatment interventions. Genetic counseling is also indicated for patients and their family members regarding the symptoms of their cardiomyopathy, its inheritance pattern, family screening recommendations, and genetic testing options and possible results. PMID:19808347

  16. Hybrid approach of ventricular assist device and autologous bone marrow stem cells implantation in end-stage ischemic heart failure enhances myocardial reperfusion

    Directory of Open Access Journals (Sweden)

    Khayat Andre

    2011-01-01

    Full Text Available Abstract We challenge the hypothesis of enhanced myocardial reperfusion after implanting a left ventricular assist device together with bone marrow mononuclear stem cells in patients with end-stage ischemic cardiomyopathy. Irreversible myocardial loss observed in ischemic cardiomyopathy leads to progressive cardiac remodelling and dysfunction through a complex neurohormonal cascade. New generation assist devices promote myocardial recovery only in patients with dilated or peripartum cardiomyopathy. In the setting of diffuse myocardial ischemia not amenable to revascularization, native myocardial recovery has not been observed after implantation of an assist device as destination therapy. The hybrid approach of implanting autologous bone marrow stem cells during assist device implantation may eventually improve native cardiac function, which may be associated with a better prognosis eventually ameliorating the need for subsequent heart transplantation. The aforementioned hypothesis has to be tested with well-designed prospective multicentre studies.

  17. Magnetic resonance imaging in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Ichida, Fukiko; Hamamichi, Yuuji; Hashimoto, Ikuo; Tsubata, Shinichi; Miyazaki, Ayumi; Okada, Toshio; Futatsuya, Ryuusuke; Okada, Eikichi (Toyama Medical and Pharmaceutical Univ. (Japan))

    1994-02-01

    To evaluate the capability of magnetic resonance imaging (MRI) in the anatomical diagnosis and tissue characterization, 8 children with hypertrophic cardiomyopathy were studied comparing with echocardiography and [sup 201]Tl myocardial imaging. The severity and distribution of hypertrophy were comparable on echocardiography and MRI. MRI was superior to echocardiography to demonstrate the apical hypertrophy. In 4 patients with severe hypertrophy, heterogenous high signal intensity was demonstrated in the site of hypertrophy, which was enhanced by T[sub 2] weighted imaging. In the patient with decreased cardiac performance and progressed cardiac failure, the heterogeneity and high signal intensity progressed in one year interval. Simultaneously performed [sup 201]Tl myocardial imaging showed patchy perfusion defect. Histological findings of the left ventricle demonstrated hypertrophy, degeneration and marked dysarray of the myocytes and fibrosis. MRI has the potential ability for the evaluation and sequential monitoring of myocardial tissue characterization as well as cardiac anatomy in childhood hypertrophic cardiomyopathy. (author).

  18. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Shiozaki, Afonso Akio; Parga, Jose Rodrigues; Arteaga, Edmundo; Rochitte, Carlos Eduardo [Sao Paulo Univ. (USP), SP (Brazil). Instituto do Coracao. Setor de Tomografia Computarizada e Ressonancia Magnetica Cardiovascular]. E-mail: rochitte@incor.usp.br; Kim, Raymond J. [Duke Cardiovascular Magnetic Resonance Center, Durham, NC (United States); Tassi, Eduardo Marinho [Diagnosticos da America S.A., Rio de Janeiro, RJ (Brazil). Sector of Cardiovascular Magnetic Resonance and Computed Tomography

    2007-03-15

    Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiac disease that causes sudden death in young people, with an incidence of 1:500 adults. The routinely used criteria for worst prognosis have limited sensitivity and specificity. Thus, the estimated risk of evolving to dilated cardiomyopathy or sudden death is somewhat inaccurate, leading to management uncertainty of HCM patients. Therefore, an accurate noninvasive method for the diagnosis of HCM with prognostic value is of great importance. In the last years, Cardiovascular Magnetic Resonance (CMR) emerged not only as a diagnostic tool, but also as a study with prognostic values, by characterizing myocardial fibrosis with great accuracy in HCM patients. Additionally, CMR identifies the types of hypertrophy, analyses the ventricular function, estimates the intraventricular gradient and allows the determination of differential diagnosis. Moreover, CMR can uniquely access myocardial fibrosis in HCM. (author)

  19. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Shiozaki, Afonso Akio; Parga, Jose Rodrigues; Arteaga, Edmundo; Rochitte, Carlos Eduardo; Tassi, Eduardo Marinho

    2007-01-01

    Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiac disease that causes sudden death in young people, with an incidence of 1:500 adults. The routinely used criteria for worst prognosis have limited sensitivity and specificity. Thus, the estimated risk of evolving to dilated cardiomyopathy or sudden death is somewhat inaccurate, leading to management uncertainty of HCM patients. Therefore, an accurate noninvasive method for the diagnosis of HCM with prognostic value is of great importance. In the last years, Cardiovascular Magnetic Resonance (CMR) emerged not only as a diagnostic tool, but also as a study with prognostic values, by characterizing myocardial fibrosis with great accuracy in HCM patients. Additionally, CMR identifies the types of hypertrophy, analyses the ventricular function, estimates the intraventricular gradient and allows the determination of differential diagnosis. Moreover, CMR can uniquely access myocardial fibrosis in HCM. (author)

  20. Developments in the management of Chagas cardiomyopathy.

    Science.gov (United States)

    Tanowitz, Herbert B; Machado, Fabiana S; Spray, David C; Friedman, Joel M; Weiss, Oren S; Lora, Jose N; Nagajyothi, Jyothi; Moraes, Diego N; Garg, Nisha Jain; Nunes, Maria Carmo P; Ribeiro, Antonio Luiz P

    2015-12-01

    Over 100 years have elapsed since the discovery of Chagas disease and there is still much to learn regarding pathogenesis and treatment. Although there are antiparasitic drugs available, such as benznidazole and nifurtimox, they are not totally reliable and often toxic. A recently released negative clinical trial with benznidazole in patients with chronic Chagas cardiomyopathy further reinforces the concerns regarding its effectiveness. New drugs and new delivery systems, including those based on nanotechnology, are being sought. Although vaccine development is still in its infancy, the reality of a therapeutic vaccine remains a challenge. New ECG methods may help to recognize patients prone to developing malignant ventricular arrhythmias. The management of heart failure, stroke and arrhythmias also remains a challenge. Although animal experiments have suggested that stem cell based therapy may be therapeutic in the management of heart failure in Chagas cardiomyopathy, clinical trials have not been promising.

  1. Electrolyte Disorders as Triggers for Takotsubo Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Fabio Andreozzi

    2018-02-01

    Full Text Available A 56-year-old woman presented with cognitive impairment, confusion and slowed speech, muscle cramps and peripheral paraesthesia preceded by vomiting. Blood tests revealed severe hypokalaemia, hyponatremia, hypomagnesemia and hypocalcaemia. Following a diagnosis of Takotsubo cardiomyopathy based on ultrasonography, the patient was treated with electrolyte supplementation and recovered within 48h. When heart failure is suspected, electrolyte abnormalities should be carefully ruled out as they can affect cardiac function.

  2. Takotsubo cardiomyopathy in amiodarone-induced hyperthyroidism

    OpenAIRE

    Ismael Capel; Elisabet Tasa-Vinyals; Albert Cano-Palomares; Irene Bergés-Raso; Lara Albert; Mercedes Rigla; Assumpta Caixàs

    2017-01-01

    Summary Takotsubo cardiomyopathy (TC) is an atypical, severe but reversible form of acute heart insufficiency. It typically presents with left ventricular failure, transient apical and mid-segments hypokinesis, absence of significant coronary stenosis and new electrographic abnormalities and/or elevation in serum cardiac enzymes. Although TC (?broken heart syndrome?) has classically been associated with emotional trauma, evidence suggests that other precipitants might exist, including iatroge...

  3. Comparación de la eficacia y seguridad de la terapia combinada de cardiomioplastia celular con el factor estimulante de colonias de granulocitos en pacientes con cardiopatía isquémica en dos vías de implatación Comparison of efficacy and safety of combined therapy of cellular cardiomyoplasty and granulocyte colony stimulating factor in patients with ischemic cardiomyopathy in two routes of implantation

    Directory of Open Access Journals (Sweden)

    Juan M Senior

    2011-04-01

    ía sostenida de la fracción de eyección y los MET más allá de los beneficios que se logran con la revascularización y la terapia farmacológica óptima.The objective of this study is to assess efficacy and safety of combined therapy of cellular cardiomyoplasty and granulocyte colony stimulating factor in patients with ischemic cardiomyopathy and explore possible differences between the implantation routes. METHODOLOGY: we performed a before and after study for longitudinal data comparing echocardiographic variables and number of Met achieved in the stress test before and at two, six and twelve months after the procedure. Likewise, mortality and adverse therapy effects were evaluated. Differences in the results were analyzed according to the intracoronary vs. epicardiac route of implantation. RESULTS: eighteen patients were included; 62,3% men, with mean age 49.4 ± 11,7 years. Mean ejection fraction was 31% ± 0,04. In twelve patients implantation was performed by intracoronary route and in six by epicardiac route. Mean ejection fraction before cell implantation was 30% with an interquartil range (IQR of 28-35%, and MET average was 6 with an interquartil rage of 5-7. Both variables as well as end-systolic and end-diastolic volumes increased significantly after the procedure, with a tendency to greater increase in ejection fraction in the group of patients whose route was epicardial implantation compared with intracoronary route; however, the number of patients in each subgroup prevented to make a definitive analysis. One patient had surgical wound infection and three died two months after implantation (one of septic shock and two of cardiogenic shock. CONCLUSION: in our environment the performance of combination therapy with cellular cardiomyoplasty and granulocyte colony stimulating factor is feasible. This is a safe procedure that achieved a sustained improvement in ejection fraction and MET beyond benefits achieved with revascularization and optimal pharmacological

  4. Two different cardiomyopathies in a single patient : hypertrophic cardiomyopathy and left ventricular noncompaction.

    Science.gov (United States)

    Sunbul, M; Ozben, B; Mutlu, B

    2013-05-01

    Hypertrophic cardiomyopathy is a complex and relatively common genetic disorder characterized by left ventricular (LV) hypertrophy, usually associated with a nondilated and hyperdynamic chamber with heterogeneous phenotypic expression and clinical course. On the other hand, LV noncompaction is an uncommon cardiomyopathy characterized by the persistence of fetal myocardium with a pattern of prominent trabecular meshwork and deep intertrabecular recesses, systolic dysfunction, and LV dilatation. We report a 29-year-old man with these two different inherent conditions. Our case raises the possibility of a genetic mutation common to these two clinical entities or different gene mutations existing in the same individual.

  5. Cochlear Implants

    Science.gov (United States)

    A cochlear implant is a small, complex electronic device that can help to provide a sense of sound. People who are ... of-hearing can get help from them. The implant consists of two parts. One part sits on ...

  6. Goserelin Implant

    Science.gov (United States)

    Goserelin implant is used in combination with radiation therapy and other medications to treat localized prostate cancer and is ... treatment of abnormal bleeding of the uterus. Goserelin implant is in a class of medications called gonadotropin- ...

  7. Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.

    Science.gov (United States)

    Ware, James S; Li, Jian; Mazaika, Erica; Yasso, Christopher M; DeSouza, Tiffany; Cappola, Thomas P; Tsai, Emily J; Hilfiker-Kleiner, Denise; Kamiya, Chizuko A; Mazzarotto, Francesco; Cook, Stuart A; Halder, Indrani; Prasad, Sanjay K; Pisarcik, Jessica; Hanley-Yanez, Karen; Alharethi, Rami; Damp, Julie; Hsich, Eileen; Elkayam, Uri; Sheppard, Richard; Kealey, Angela; Alexis, Jeffrey; Ramani, Gautam; Safirstein, Jordan; Boehmer, John; Pauly, Daniel F; Wittstein, Ilan S; Thohan, Vinay; Zucker, Mark J; Liu, Peter; Gorcsan, John; McNamara, Dennis M; Seidman, Christine E; Seidman, Jonathan G; Arany, Zoltan

    2016-01-21

    Background Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin. Methods In 172 women with peripartum cardiomyopathy, we sequenced 43 genes with variants that have been associated with dilated cardiomyopathy. We compared the prevalence of different variant types (nonsense, frameshift, and splicing) in these women with the prevalence of such variants in persons with dilated cardiomyopathy and with population controls. Results We identified 26 distinct, rare truncating variants in eight genes among women with peripartum cardiomyopathy. The prevalence of truncating variants (26 in 172 [15%]) was significantly higher than that in a reference population of 60,706 persons (4.7%, P=1.3×10(-7)) but was similar to that in a cohort of patients with dilated cardiomyopathy (55 of 332 patients [17%], P=0.81). Two thirds of identified truncating variants were in TTN, as seen in 10% of the patients and in 1.4% of the reference population (P=2.7×10(-10)); almost all TTN variants were located in the titin A-band. Seven of the TTN truncating variants were previously reported in patients with idiopathic dilated cardiomyopathy. In a clinically well-characterized cohort of 83 women with peripartum cardiomyopathy, the presence of TTN truncating variants was significantly correlated with a lower ejection fraction at 1-year follow-up (P=0.005). Conclusions The distribution of truncating variants in a large series of women with peripartum cardiomyopathy was remarkably similar to that found in patients with idiopathic dilated cardiomyopathy. TTN truncating variants were the most prevalent genetic predisposition in each disorder.

  8. An Uncommon Association of Familial Partial Lipodystrophy, Dilated Cardiomyopathy, and Conduction System Disease

    Directory of Open Access Journals (Sweden)

    Ragesh Panikkath MD

    2016-07-01

    Full Text Available A 46-year-old African American woman presented with severe respiratory distress requiring intubation and was diagnosed with nonischemic cardiomyopathy. She had the typical phenotype of familial partial lipodystrophy 2 (FPLD2. Sequence analysis of LMNA gene showed a heterozygous missense mutation at exon 8 (c.1444C>T causing amino acid change, p.R482W. She later developed severe coronary artery disease requiring multiple percutaneous coronary interventions and coronary artery bypass surgery. She was later diagnosed with diabetes, primary hyperparathyroidism, and euthyroid multinodular goiter. She had sinus nodal and atrioventricular nodal disease and had an implantable cardioverter defibrillator implantation due to persistent left ventricular dysfunction. The device eroded through the skin few months after implantation and needed a re-implant on the contralateral side. She had atrial flutter requiring ablation. This patient with FPLD2 had most of the reported cardiac complications of FPLD2. This case is presented to improve the awareness of the presentation of this disease among cardiologists and internists.

  9. Takotsubo cardiomyopathy in a snake bite victim: a case report ...

    African Journals Online (AJOL)

    Takotsubo cardiomyopathy occurs in patients with severe emotional or physiologic stress. The prognosis is usually favorable, and the left ventricular wall motion dyskinesis normalizes within days to weeks. In this paper we report a case of snake bite complicated by takotsubo cardiomyopathy. We advise physicians to ...

  10. Multivessel myocardial bridging in a patient with spiral hypertrophic cardiomyopathy

    OpenAIRE

    Fritz, Timothy; Abdallah, Wissam; McNamara, Richard

    2016-01-01

    Myocardial bridging is commonly observed in hypertrophic cardiomyopathy, usually confined to the left anterior descending (LAD), and correlates to the hypertrophic septum. We present a patient with unique spiral hypertrophic cardiomyopathy (HCM) and compression of all three coronary arteries corresponding to this hypertrophy pattern.

  11. Septal alcoholization in hypertrophic cardiomyopathy: about 11 cases

    African Journals Online (AJOL)

    Outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy are not enough studied in all centers. The purpose of this study was to determine the outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy in our hospital. A retrospective and prospective descriptive study focused on all ...

  12. Dental Implant Surgery

    Science.gov (United States)

    ... here to find out more. Dental Implant Surgery Dental Implant Surgery Dental implant surgery is, of course, surgery, ... here to find out more. Dental Implant Surgery Dental Implant Surgery Dental implant surgery is, of course, surgery, ...

  13. Radiology and pathology correlation in common infiltrative cardiomyopathies

    International Nuclear Information System (INIS)

    Varzeshi, Neda; Hansen, Mark; Rezaee, Amir; Slaughter, Richard; Dixon, Natalie; Duhig, Edwina

    2012-01-01

    Infiltrative cardiomyopathies generally pose a diagnostic dilemma as current diagnostic tools are imprecise. Invasive endomyocardial biopsy is considered as the gold standard however it has some limitations. Recently cardiovascular magnetic resonance (CMR) is emerging as an excellent technique in diagnosing infiltrative cardiomyopathies and is increasingly being used. Characteristic pathologic and radiologic findings in most common infiltrative cardiomyopathies (amyloid, sarcoid and Fabry's) are discussed and correlated with relative CMR and histologic examples. There is fairly good correlation between the non-invasive radiologic and the invasive histologic findings in common infiltrative cardiomyopathies. Non-invasive CMR with its high sensitivity and specificity has an excellent role in establishing the diagnosis and improving the prognosis of common infiltrative cardiomyopathies.

  14. Basal wall hypercontraction of Takotsubo cardiomyopathy in a patient who had been diagnosed with dilated cardiomyopathy: a case report.

    Science.gov (United States)

    Ichihara, Noboru; Fujita, Shuichi; Kanzaki, Yumiko; Fujisaka, Tomohiro; Ozeki, Michishige; Ishizaka, Nobukazu

    2017-12-12

    Takotsubo cardiomyopathy is characterized by the basal hypercontractility and apical ballooning of the left ventriculum and T-wave inversion in the electrocardiogram. It has been suggested that Takotsubo cardiomyopathy might underlie the pathogenesis of persistent cardiac dysfunction; however, few reports are present demonstrating the advent of Takotsubo cardiomyopathy in patients with idiopathic cardiomyopathy. A 64-year-old women was admitted due to dyspnea on effort and lower extremity edema. She had been diagnosed with idiopathic dilated cardiomyopathy 2.5 years before owing to the reduced left ventricular ejection fraction (24%), normal coronary artery, and interstitial fibrosis of the myocardial samples. On admission, her electrocardiogram showed giant negative T wave in II, III, aVF, and precordial leads. Echocardiography showed dyskinesis of the left ventricular apex and hypercontraction of the basal wall, which had not been observed in the previous examinations. Coronary angiography showed normal coronary arteries, and apical ballooning and basal hypercontractility was confirmed by left ventriculography. On day 15 of admission, contraction of apical wall was recovered, and basal hypercontraction was disappeared. The present case is the first report demonstrating appearance the transient basal wall hypercontraction along with the advent of Takotsubo cardiomyopathy in a patient diagnosed with dilated cardiomyopathy. Whether such findings are indicative of fair prognosis and have the utility of understanding the pathogenesis of dilated cardiomyopathy needs further investigation.

  15. Cardiomyopathy Associated with Celiac Disease in Childhood

    Directory of Open Access Journals (Sweden)

    Aleksandra Boskovic

    2012-01-01

    Full Text Available Celiac disease is predominantly a disease of the small intestine characterized by chronic malabsorption in genetically susceptible individuals who ingest grains containing gluten, such as wheat, barley, and rye. Although previously believed to be uncommon, celiac disease may be present in up to 1% of the adult and children population. Celiac disease is associated frequently with iron-deficiency anemia, dermatitis herpetiformis, selective IgA deficiency, thyroid disorders, diabetes mellitus, and various connective tissue disorders but is rarely associated with cardiomyopathy.

  16. The Role of Echocardiography in Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Jing Ping Sun

    2017-02-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is a common genetic cardiovascular disease and appears in all ethnic groups. HCM is diagnosed on the basis of left ventricular hypertrophy. Echocardiography is a key technique in the diagnosis of HCM, the prognosis of patients with HCM, the management strategy for HCM, and the follow-up of patients with HCM. This review briefly describes and discusses the practical use of established echocardiography techniques and the current and emerging echocardiographic methods that can help physicians in the correct diagnostic and pathophysiological assessment of patients with HCM.

  17. Dilated cardiomyopathy following use of xenadrine EFX

    Science.gov (United States)

    Riccioni, Graziano; Speziale, Giuseppe; Scotti, Luca; Bucciarelli, Valentina; Cappetti, Silvia; Nasso, Giuseppe; Gallina, Sabina; Bucciarelli, Tonino

    2015-01-01

    We describe a case of a 35-year-old man presented at the emergency room of our institution with acute onset of dyspnea and dizziness. He was a body builder and had been using Xenadrine EFX for weight loss reduction. The laboratory analyses were normal. A chest radiograph showed an enlarged cardiac silhouette with clear lung fields. Transtoracic two-dimensional color Doppler echocardiography revealed a diffuse hypokinesia with a marked decreased in systolic function and a high teledyastolic diameter. This case document the possible relation to use of Xenadrine EFX for weight loss and the recurrence of dilated cardiomyopathy. PMID:26680256

  18. [Spanish implantable cardioverter-defibrillator registry. Seventh official report of the spanish society of cardiology working group on implantable cardioverter-defibrillators (2010)].

    Science.gov (United States)

    Alzueta, Javier; Fernández, José Maria

    2011-11-01

    The authors summarize the findings of the Spanish Implantable Cardioverter-Defibrillator Registry for 2010 compiled by the Spanish Society of Cardiology Working Group on Implantable Cardioverter-Defibrillators. Members of the Spanish Society of Cardiology were prospectively surveyed; data were recorded voluntarily by each implantation team on one-page questionnaires. In total, 4627 device implantations were reported, comprising 85.6% of the overall estimated number of implantations. The reported implantation rate was 100.61 per million population and the estimated total implantation rate was 117.50 per million. The proportion of first implantations was 73.87%. We collected data from 143 hospitals (9 more than in 2009). The majority of the implantable cardioverter-defibrillator implantations were performed in men (81%). The mean age was 62.5 ± 13 years. Most of the patients had severe or moderate-to-severe ventricular dysfunction and were in New York Heart Association functional class II. Ischemic heart disease was the most frequent underlying cardiac condition, followed by dilated cardiomyopathy. The number of implantable cardioverter-defibrillator implantations indicated for primary prevention increased over the previous year and now accounts for 65.6% of first implantations. In all, 76.1% of the implantable cardioverter-defibrillator implantations were performed by cardiac electrophysiologists. The 2010 Spanish Implantable Cardioverter-Defibrillator Registry includes data on almost 86% of all the implantable cardioverter-defibrillator implantations performed in Spain. Although the number has continued to increase, it still remains far lower than the European average. There has been a significant increase in the number of implantations indicated for primary prevention. Copyright © 2011 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

  19. Right ventricular bifocal stimulation in the treatment of dilated cardiomyopathy with heart failure

    Directory of Open Access Journals (Sweden)

    José Carlos Pachón Mateos

    1999-12-01

    Full Text Available OBJECTIVE: To describe a new more efficient method of endocardial cardiac stimulation, which produces a narrower QRS without using the coronary sinus or cardiac veins. METHODS: We studied 5 patients with severe dilated cardiomyopathy, chronic atrial fibrillation and AV block, who underwent definitive endocardial pacemaker implantation, with 2 leads, in the RV, one in the apex and the other in the interventricular septum (sub pulmonary, connected, respectively, to ventricular and atrial bicameral pacemaker outputs. Using Doppler echocardiography, we compared, in the same patient, conventional (VVI, high septal ("AAI" and bifocal ("DDT" with AV interval ~ 0 stimulation. RESULTS: The RV bifocal stimulation had the best results with an increase in ejection fraction and cardiac output and reduction in QRS duration, mitral regurgitation and in the left atrium area (p <= 0.01. The conventional method of stimulation showed the worst result. CONCLUSION: These results suggest that, when left ventricular stimulation is not possible, right ventricular bifocal stimulation should be used in patients with severe cardiomyopathy where a pacemaker is indicated.

  20. Percutaneous Septal Ablation in Hypertrophic Obstructive Cardiomyopathy: From Experiment to Standard of Care

    Science.gov (United States)

    2014-01-01

    Hypertrophic cardiomyopathy (HCM) is one of the more common hereditary cardiac conditions. According to presence or absence of outflow obstruction at rest or with provocation, a more common (about 60–70%) obstructive type of the disease (HOCM) has to be distinguished from the less common (30–40%) nonobstructive phenotype (HNCM). Symptoms include exercise limitation due to dyspnea, angina pectoris, palpitations, or dizziness; occasionally syncope or sudden cardiac death occurs. Correct diagnosis and risk stratification with respect to prophylactic ICD implantation are essential in HCM patient management. Drug therapy in symptomatic patients can be characterized as treatment of heart failure with preserved ejection fraction (HFpEF) in HNCM, while symptoms and the obstructive gradient in HOCM can be addressed with beta-blockers, disopyramide, or verapamil. After a short overview on etiology, natural history, and diagnostics in hypertrophic cardiomyopathy, this paper reviews the current treatment options for HOCM with a special focus on percutaneous septal ablation. Literature data and the own series of about 600 cases are discussed, suggesting a largely comparable outcome with respect to procedural mortality, clinical efficacy, and long-term outcome. PMID:26556411

  1. Percutaneous Septal Ablation in Hypertrophic Obstructive Cardiomyopathy: From Experiment to Standard of Care

    Directory of Open Access Journals (Sweden)

    Lothar Faber

    2014-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is one of the more common hereditary cardiac conditions. According to presence or absence of outflow obstruction at rest or with provocation, a more common (about 60–70% obstructive type of the disease (HOCM has to be distinguished from the less common (30–40% nonobstructive phenotype (HNCM. Symptoms include exercise limitation due to dyspnea, angina pectoris, palpitations, or dizziness; occasionally syncope or sudden cardiac death occurs. Correct diagnosis and risk stratification with respect to prophylactic ICD implantation are essential in HCM patient management. Drug therapy in symptomatic patients can be characterized as treatment of heart failure with preserved ejection fraction (HFpEF in HNCM, while symptoms and the obstructive gradient in HOCM can be addressed with beta-blockers, disopyramide, or verapamil. After a short overview on etiology, natural history, and diagnostics in hypertrophic cardiomyopathy, this paper reviews the current treatment options for HOCM with a special focus on percutaneous septal ablation. Literature data and the own series of about 600 cases are discussed, suggesting a largely comparable outcome with respect to procedural mortality, clinical efficacy, and long-term outcome.

  2. Lamin A/C truncation in dilated cardiomyopathy with conduction disease

    Directory of Open Access Journals (Sweden)

    Huber Jill M

    2003-07-01

    Full Text Available Abstract Background Mutations in the gene encoding the nuclear membrane protein lamin A/C have been associated with at least 7 distinct diseases including autosomal dominant dilated cardiomyopathy with conduction system disease, autosomal dominant and recessive Emery Dreifuss Muscular Dystrophy, limb girdle muscular dystrophy type 1B, autosomal recessive type 2 Charcot Marie Tooth, mandibuloacral dysplasia, familial partial lipodystrophy and Hutchinson-Gilford progeria. Methods We used mutation detection to evaluate the lamin A/C gene in a 45 year-old woman with familial dilated cardiomyopathy and conduction system disease whose family has been well characterized for this phenotype 1. Results DNA from the proband was analyzed, and a novel 2 base-pair deletion c.908_909delCT in LMNA was identified. Conclusions Mutations in the gene encoding lamin A/C can lead to significant cardiac conduction system disease that can be successfully treated with pacemakers and/or defibrillators. Genetic screening can help assess risk for arrhythmia and need for device implantation.

  3. The role of interpolation in PVC-induced cardiomyopathy.

    Science.gov (United States)

    Olgun, Hilal; Yokokawa, Miki; Baman, Timir; Kim, Hyungjin Myra; Armstrong, William; Good, Eric; Chugh, Aman; Pelosi, Frank; Crawford, Thomas; Oral, Hakan; Morady, Fred; Bogun, Frank

    2011-07-01

    Frequent premature ventricular complexes (PVCs) can cause cardiomyopathy. The mechanism is not known and may be multifactorial. This study assessed the role of PVC interpolation in PVC-induced cardiomyopathy. In 51 consecutive patients (14 women, age 49 ± 15 years, ejection fraction (EF) 0.49 ± 0.14) with frequent PVCs, 24-hour Holter recordings were performed. The amount of interpolation was determined and correlated with the presence of PVC-induced cardiomyopathy. In addition, parameters measured during an electrophysiology study were correlated with the Holter findings. Fourteen of the 21 patients (67%) with cardiomyopathy had interpolated PVCs, compared with only 6 of 30 patients (20%) without PVC-induced cardiomyopathy (P PVC burden than patients without interpolation (28% ± 12% vs. 15% ± 15%; P = .002). The burden of interpolated PVCs correlated with the presence of PVC cardiomyopathy (21% ± 30% vs. 4% ± 13%; P = .008). Both PVC burden and interpolation independently predicted PVC-induced cardiomyopathy (odds ratio 1.07, 95% confidence interval 1.01 to 1.13, P = .02; and odds ratio 4.43, 95% confidence interval 1.06 to 18.48, P = .04, respectively). The presence of ventriculoatrial block at a ventricular pacing cycle length of 600 ms correlated with the presence of interpolation (P = .004). Patients with interpolation had a longer mean ventriculoatrial block cycle length than patients without interpolated PVCs (520 ± 110 ms vs. 394 ± 92 ms; P = .01). The presence of interpolated PVCs was predictive of the presence of PVC cardiomyopathy. Interpolation may play an important role in the generation of PVC-induced cardiomyopathy. Copyright © 2011 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  4. Homozygous Desmocollin-2 Mutations and Arrhythmogenic Cardiomyopathy.

    Science.gov (United States)

    Lorenzon, Alessandra; Pilichou, Kalliopi; Rigato, Ilaria; Vazza, Giovanni; De Bortoli, Marzia; Calore, Martina; Occhi, Gianluca; Carturan, Elisa; Lazzarini, Elisabetta; Cason, Marco; Mazzotti, Elisa; Poloni, Giulia; Mostacciuolo, Maria Luisa; Daliento, Luciano; Thiene, Gaetano; Corrado, Domenico; Basso, Cristina; Bauce, Barbara; Rampazzo, Alessandra

    2015-10-15

    Dominant mutations in desmocollin-2 (DSC2) gene cause arrhythmogenic cardiomyopathy (ACM), a progressive heart muscle disease characterized by ventricular tachyarrhythmias, heart failure, and risk of juvenile sudden death. Recessive mutations are rare and are associated with a cardiac or cardiocutaneous phenotype. Here, we evaluated the impact of a homozygous founder DSC2 mutation on clinical expression of ACM. An exon-by-exon analysis of the DSC2 coding region was performed in 94 ACM index patients. The c.536A>G (p.D179G) mutation was identified in 5 patients (5.3%), 4 of which resulted to be homozygous carriers. The 5 subjects shared a conserved haplotype, strongly indicating a common founder. Genetic and clinical investigation of probands' families revealed that p.D179G homozygous carriers displayed severe forms of biventricular cardiomyopathy without hair or skin abnormalities. The only heterozygous proband, who carried an additional variant of unknown significance in αT-catenin gene, showed a mild form of ACM without left ventricular involvement. All heterozygous family members were clinically asymptomatic. In conclusion, this is the first homozygous founder mutation in DSC2 gene identified among Italian ACM probands. Our findings provide further evidence of the occurrence of recessive DSC2 mutations in patients with ACM predominantly presenting with biventricular forms of the disease. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. Takotsubo Cardiomyopathy in Intensive Care Unit: Prevention, Diagnosis and Management

    Directory of Open Access Journals (Sweden)

    Hannah Masoud

    2016-01-01

    Full Text Available Accurate diagnosis of Takotsubo Cardiomyopathy has substantial prognostic implications in an intensive care unit, given its increased mortality risk and association with life-threatening complications. This report seeks to discuss diagnostic modalities that can be useful in accurately differentiating Takotsubo Cardiomyopathy from Acute Coronary Syndrome, and also briefly discuss prevention and management of this cardiomyopathy in an intensive care unit. For critically ill Takotsubo patients, intensive clinicians can consider establishment of diagnosis by specific electrocardiograph changes, distinctive marked release of cardiac enzymes, characteristic echocardiograph findings, as well as invasive coronary angiography or noninvasive cardiac magnetic imaging.

  6. Life threatening causes of syncope: channelopathies and cardiomyopathies.

    Science.gov (United States)

    Herman, Adam; Bennett, Matthew T; Chakrabarti, Santabhanu; Chakrabarti, Santabahnu; Krahn, Andrew D

    2014-09-01

    Syncope is common, has a high recurrence rate and carries a risk of morbidity and, dependent on the cause, mortality. Although the majority of patients with syncope have a benign prognosis, syncope as a result of cardiomyopathy or channelopathy carries a poor prognosis. In addition, the identification of these disorders allows for the institution of treatments, which are effective at reducing the risk of both syncope and mortality. It is for these reasons that the identification of a cardiomyopathy or channelopathy in patients with syncope is crucial. This review article will describe the characteristics of common cardiomyopathies and channelopathies and their investigation. Copyright © 2014 Elsevier B.V. All rights reserved.

  7. Reversible catecholamine-induced cardiomyopathy due to pheochromocytoma: case report.

    Science.gov (United States)

    Satendra, Milan; de Jesus, Cláudia; Bordalo e Sá, Armando L; Rosário, Luís; Rocha, José; Bicha Castelo, Henrique; Correia, Maria José; Nunes Diogo, António

    2014-03-01

    Pheochromocytoma is a tumor originating from chromaffin tissue. It commonly presents with symptoms and signs of catecholamine excess, such as hypertension, tachycardia, headache and sweating. Cardiovascular manifestations include catecholamine-induced cardiomyopathy, which may present as severe left ventricular dysfunction and congestive heart failure. We report a case of pheochromocytoma which was diagnosed following investigation of dilated cardiomyopathy. We highlight the dramatic symptomatic improvement and reversal of cardiomyopathy, with recovery of left ventricular function after treatment. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  8. Stem Cell-Based Therapies in Chagasic Cardiomyopathy.

    Science.gov (United States)

    de Carvalho, Antonio Carlos Campos; Carvalho, Adriana Bastos

    2015-01-01

    Chagas disease is caused by Trypanosoma cruzi and can lead to a dilated cardiomyopathy decades after the prime infection by the parasite. As with other dilated cardiomyopathies, conventional pharmacologic therapies are not always effective and as heart failure progresses patients need heart transplantation. Therefore alternative therapies are highly desirable and cell-based therapies have been investigated in preclinical and clinical studies. In this paper we review the main findings of such studies and discuss future directions for stem cell-based therapies in chronic chagasic cardiomyopathy.

  9. Acute peritonitis as the first presentation of valvular cardiomyopathy.

    LENUS (Irish Health Repository)

    Higgins, Nikki

    2012-02-01

    Valvular cardiomyopathy can present a diagnostic challenge in the absence of overt cardiac symptoms. This report describes the case of a 46-year-old woman who presented with acute peritonitis associated with vomiting and abdominal distension. Subsequent abdominal computed tomography and ultrasound revealed bibasal pleural effusions, ascites, and normal ovaries. An echocardiogram revealed that all cardiac chambers were dilated with a global decrease in contractility and severe mitral, tricuspid, and aortic regurgitation. A diagnosis of cardiomyopathy with acute heart failure, secondary to valvular heart disease, was secured. Acute peritonitis as the presenting feature of valvular cardiomyopathy is a rare clinical entity.

  10. Acute peritonitis as the first presentation of valvular cardiomyopathy.

    Science.gov (United States)

    Higgins, Nikki; Burke, John P; McCreery, Charles J

    2012-01-01

    Valvular cardiomyopathy can present a diagnostic challenge in the absence of overt cardiac symptoms. This report describes the case of a 46-year-old woman who presented with acute peritonitis associated with vomiting and abdominal distension. Subsequent abdominal computed tomography and ultrasound revealed bibasal pleural effusions, ascites, and normal ovaries. An echocardiogram revealed that all cardiac chambers were dilated with a global decrease in contractility and severe mitral, tricuspid, and aortic regurgitation. A diagnosis of cardiomyopathy with acute heart failure, secondary to valvular heart disease, was secured. Acute peritonitis as the presenting feature of valvular cardiomyopathy is a rare clinical entity.

  11. Current perspectives in hypertrophic cardiomyopathy with the focus on patients in the Finnish population: a review.

    Science.gov (United States)

    Kuusisto, Johanna; Sipola, Petri; Jääskeläinen, Pertti; Naukkarinen, Anita

    2016-11-01

    Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease, with the prevalence of about 1/500. During the last two decades, the knowledge of the etiology, pathogenesis, risk stratification and prevention of sudden death in HCM has substantially advanced. Most often, HCM is familial and caused by mutations in sarcomere genes, inherited in an autosomal dominant manner. In Finland, genetic background of HCM is unique, with a few founder mutations in cardiac sarcomere genes accounting for a considerable proportion of the disease. Pathogenic mechanisms induced by disease-causing mutations are still poorly understood, although alterations in intracellular calcium handling and inefficient generation of contractile force in myocytes are considered key features in triggering the hypertrophic response. Clinical features of the disease are highly variable from no symptoms to the spectrum of exertional dyspnea, angina, palpitations, syncope and sudden death. In the current patient care, implantable cardioverter defibrillators (ICDs) are successfully used to prevent sudden cardiac death in high risk subjects. Targeted genetic testing is recommended to confirm the diagnosis in patients with HCM and to identify family members with the disease. Future research is needed to elucidate key cellular mechanisms leading to HCM, which may allow specific prevention and treatment of the disease. Key messages Hypertrophic cardiomyopathy, most often caused by defects in sarcomere genes, is the most common inherited heart disease, and a common cause of sudden cardiac death (SCD) in athletes and young subjects. Cardiac imaging, ECG and genetic testing are pivotal in the diagnosis of the disease in patients and first-degree relatives. Implantable cardioverter defibrillators in patients with high risk for SCD and tailored pharmacotherapy are efficient tools in patient care, but so far, exact mechanisms leading to cardiac hypertrophy in HCM are only partially understood, and

  12. [Sudden cardiac death in familial hypertrophic cardiomyopathy. Identification of high-risk patients].

    Science.gov (United States)

    Pellnitz, C; Geier, C; Perrot, A; Dietz, R; Osterziel, K J; Haverkamp, W

    2005-05-06

    Hypertrophic cardiomyopathy (HCM) is a relatively frequent, genetically determined primary cardiomyopathy, characterized by most often asymmetric hypertrophy of the ventricular septum with or without systolic obstruction of the left ventricular outflow tract. HCM is a genetically heterogeneous disease, with 12 different disease-causing genes beeing indentified to date. Histologically the disease is characterized by hypertophy and disarray of myofibrils as well as by an increase in myocardial fibrosis. Clinically, these changes may lead to palpitations, dyspnoe on exertion, and/or angina pectoris. However, they also lead to an increased propensity to the development of severe ventricular tachyarrhythmias and sudden cardiac death. The incidence of sudden death is significantly increased in HCM, particularly in affected young subjects. Risk stratification in HCM should include a complete clinical-cardiological evaluation that should also consider new diagnostic features, e. g. MR imaging. Major risk factors for sudden cardiac death include a survived cardiac arrest (ventricular fibrillation), non-sustained and sustained ventricular tachycardia, a history of premature familial sudden death, unexplained syncope, an abnormal blood pressure response on exercise, and left ventricular thickness greater than or equal to 3 cm. Ideally, risk stratification should also include genetic testing, since some gene mutations seem to be associated with a higher risk for sudden cardiac death than others. However, genetic testing in HCM in not yet available on a routine basis. The implantation of a cardioverter/defibrillator is first-line therapy in patients with documented ventricular tachycardia/fibrillation or patients who have survived sudden cardiac death. These devices also play an important role in the primary prevention of sudden cardiac death in HCM. Algorithms and scores are available to estimate the risk of sudden death, however, the decision to implant a cardioverter

  13. RARE CASES OF HYPERTROPHIC CARDIOMYOPATHY: VARIANTS AND CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    V. Yu. Zimina

    2015-09-01

    Full Text Available Hypertrophic cardiomyopathy belongs to a group of hereditary diseases due to sarcomere gene mutation. This abnormality is characterized by the development of symmetric or asymmetric hypertrophy of left ventricular myocardium with its normal contractile function or hypercontractility. Authors provide a brief overview of variants of hypertrophic cardiomyopathy and phenocopies of this disease, when structural changes in the heart are not the result of classic sarcomere gene mutation. In patients with some phenocopies concentric left ventricular hypertrophy can transform into its dilatation with reduced contractility. Such variant of hypertrophic cardiomyopathy is presented in the first clinical observation. The second case shows that hypertrophic cardiomyopathy can be one of the symptoms of the disease with other reasons for poor outcome.

  14. RARE CASES OF HYPERTROPHIC CARDIOMYOPATHY: VARIANTS AND CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    V. Yu. Zimina

    2014-01-01

    Full Text Available Hypertrophic cardiomyopathy belongs to a group of hereditary diseases due to sarcomere gene mutation. This abnormality is characterized by the development of symmetric or asymmetric hypertrophy of left ventricular myocardium with its normal contractile function or hypercontractility. Authors provide a brief overview of variants of hypertrophic cardiomyopathy and phenocopies of this disease, when structural changes in the heart are not the result of classic sarcomere gene mutation. In patients with some phenocopies concentric left ventricular hypertrophy can transform into its dilatation with reduced contractility. Such variant of hypertrophic cardiomyopathy is presented in the first clinical observation. The second case shows that hypertrophic cardiomyopathy can be one of the symptoms of the disease with other reasons for poor outcome.

  15. Genetics Home Reference: dilated cardiomyopathy with ataxia syndrome

    Science.gov (United States)

    ... usual to recharge between beats . The irregular heartbeats (arrhythmia) can lead to fainting (syncope) or cardiac arrest ... Topic: Cardiomyopathy Health Topic: Movement Disorders Health Topic: Newborn Screening Genetic and Rare Diseases Information Center (1 ...

  16. Advanced quantitative echocardiography in arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Hastrup Svendsen, Jesper; Sogaard, Peter

    2007-01-01

    BACKGROUND: Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is a regional disease of the RV myocardium with variable degrees of left ventricular involvement. Three-dimensional echocardiography and Doppler tissue imaging (DTI) are new echocardiographic modalities for the evaluation of ...

  17. Noncompaction Cardiomyopathy with Charcot-Marie-Tooth Disease

    Directory of Open Access Journals (Sweden)

    Sherif Ali Eltawansy

    2015-01-01

    Full Text Available We report a case of a 53-year-old female presenting with a new-onset heart failure that was contributed secondary to noncompaction cardiomyopathy. The diagnosis was made by echocardiogram and confirmed by cardiac MRI. Noncompaction cardiomyopathy (also known as ventricular hypertrabeculation is a newly discovered disease. It is considered to be congenital (genetic cardiomyopathy. It is usually associated with genetic disorders and that could explain the genetic pathogenesis of the non-compaction cardiomyopathy. Our case had a history of Charcot-Marie-Tooth disease. There is a high incidence of arrhythmia and embolic complications. The treatment usually consists of the medical management, defibrillator placement, and lifelong anticoagulation. Heart transplantation will be the last resort.

  18. Dynamic electrocardiographic changes in patients with arrhythmogenic right ventricular cardiomyopathy.

    LENUS (Irish Health Repository)

    Quarta, Giovanni

    2010-04-01

    Electrocardiographic (ECG) abnormalities of depolarisation and repolarisation contribute to the diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC). The development of diagnostic ECG features were investigated in a genotyped cohort with ARVC to provide more sensitive markers of early disease.

  19. Psychological distress and personality factors in takotsubo cardiomyopathy

    NARCIS (Netherlands)

    Smeijers, L; Szabó, B M; Kop, W J

    2016-01-01

    Background Takotsubo cardiomyopathy (TCC) is a transient condition characterised by severe left ventricular dysfunction combined with symptoms and signs mimicking myocardial infarction. Emotional triggers are common, but little is known about the psychological background characteristics of TCC. This

  20. Atypical aortic coarctation as a cause of a cardiomyopathy

    OpenAIRE

    Alsemgeest, F.; Kamp, O.; Marcu, C.B.

    2015-01-01

    Atypical locations for aortic coarctation have been previously described. However, to our knowledge, no case has been described of a rapidly progressive dilated cardiomyopathy caused by an atypical coarctation, with a rapid normalisation of ventricular function after treatment.

  1. Trace elements in diabetic cardiomyopathy: An electrophysiological overview

    OpenAIRE

    Ozturk, Nihal; Olgar, Yusuf; Ozdemir, Semir

    2013-01-01

    There is a growing body of evidence that Diabetes Mellitus leads to a specific cardiomyopathy apart from vascular disease and bring about high morbidity and mortality throughout the world. Recent clinical and experimental studies have extensively demonstrated that this cardiomyopathy causes impaired cardiac performance manifested by early diastolic and late systolic dysfunction. This impaired cardiac performance most probably have emerged upon the expression and activity of regulatory protein...

  2. Hypertrophic cardiomyopathy with mid-ventricular obstruction and apical aneurysm

    Directory of Open Access Journals (Sweden)

    N.D. Oryshchyn

    2016-11-01

    Full Text Available A case report of apical left ventricular aneurysm in patient with hypertrophic cardiomyopathy with mid-ventricular obstruction (diagnosis and surgical treatment is presented. We revealed apical aneurysm and mid-ventricular obstruction during echocardiography and specified anatomical characteristics of aneurysm during computer tomography. There was no evidence of obstructive coronary artery disease during coronary angiography. Taking into consideration multiple cerebral infarcts, aneurysm resection and left ventricular plastics was performed. Electronic microscopy of myocardium confirmed the diagnosis of hypertrophic cardiomyopathy.

  3. Quality of life metrics in arrhythmogenic right ventricular cardiomyopathy patients: The impact of age, shock and sex.

    Science.gov (United States)

    Rhodes, Ashley C; Murray, Brittney; Tichnell, Crystal; James, Cynthia A; Calkins, Hugh; Sears, Samuel F

    2017-12-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) patients face many medical and psychosocial challenges. However, the quality of life (QOL) outcomes of these patients remains largely unexplored. 159 ARVC patients completed a psychosocial survey including the Short Form Health Survey, Florida Shock Anxiety Scale, and Florida Patient Acceptance Survey. These cross-sectional data were used to examine the general and device-specific QOL of these patients compared to normative samples, and to determine the impact of age, shock and sex on these outcome measures. ARVC patients reported lower physical and mental QOL compared to a normative U.S sample. Compared to an implantable cardioverter defibrillator (ICD) sample, they reported higher mental and physical QOL. Compared to a hypertrophic cardiomyopathy sample, they reported lower physical but higher mental QOL. ARVC patients aged 18-35 reported significantly lower mental QOL than older patients. Male patients with no implantable defibrillator shock history reported significantly higher mental QOL scores than those with shock history. Shock anxiety scores were significantly higher among individuals who had experienced at least one shock. Female ARVC patients reported significantly higher shock anxiety and lower mental QOL compared to male patients. ARVC patients report lower mental and physical QOL than a U.S. normative sample, but report mostly superior QOL compared to relevant cardiac samples. Younger ARVC patients, female patients, and those who have experienced at least one device shock are at risk for psychosocial difficulties, including poorer QOL outcomes. Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.

  4. Left ventricular assist for pediatric patients with dilated cardiomyopathy using the Medos VAD cannula and a centrifugal pump.

    Science.gov (United States)

    Huang, Shu-Chien; Chi, Nai-Hsin; Chen, Chun-An; Chen, Yih-Sharng; Chou, Nai-Kuan; Ko, Wen-Je; Wang, Shoei-Shen

    2009-11-01

    Ventricular assist devices for small pediatric patients are expensive and commercially unavailable in Taiwan. We used the Medos ventricular assist device cannula (Medos, Aachen, Germany) and a centrifugal pump to support pediatric patients with dilated cardiomyopathy and decompensated heart failure. From January 2007 to December 2008, three pediatric patients with dilated cardiomyopathy were supported using a centrifugal pump as the left ventricular assist device. The Medos arterial cannula was sutured to the ascending aorta, and the Apex cannula was fixed into the left ventricular apex. When the patient was weaned off of cardiopulmonary bypass, the left ventricular assist device pump was started. The pump flow was gradually titrated according to the filling status of the left ventricle. All the left ventricular assist devices were successfully implanted and functioned well. Two patients on extracorporeal membrane oxygenation had severe lung edema before left ventricular assist device implantation. Both patients required extracorporeal membrane oxygenation for the postoperative period until the pulmonary edema was resolved. Among the three patients, two successfully bridged to heart transplantation after support for 6 and 11 days, respectively. The first patient (10 kg) expired due to systemic emboli 30 days after left ventricular assist device support. In summary, these results suggest that the Medos ventricular assist device cannula and a centrifugal pump is an option for temporary left ventricular assist device support in patients with intractable heart failure and as a bridge to heart transplantation.

  5. MT-CYB mutations in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Hagen, Christian M; Aidt, Frederik H; Havndrup, Ole

    2013-01-01

    Mitochondrial dysfunction is a characteristic of heart failure. Mutations in mitochondrial DNA, particularly in MT-CYB coding for cytochrome B in complex III (CIII), have been associated with isolated hypertrophic cardiomyopathy (HCM). We hypothesized that MT-CYB mutations might play an important...... and m.15482T>C; p.S246P were identified. Modeling showed that the p.C93Y mutation leads to disruption of the tertiary structure of Cytb by helix displacement, interfering with protein-heme interaction. The p.S246P mutation induces a diproline structure, which alters local secondary structure and induces...... of HCM patients. We propose that further patients with HCM should be examined for mutations in MT-CYB in order to clarify the role of these variants....

  6. Dilated cardiomyopathy in cats - A case report

    Directory of Open Access Journals (Sweden)

    K. Jeyaraja

    2013-08-01

    Full Text Available Two cats were brought to Madras Veterinary College Teaching Hospital with the history and clinical signs suggestive of congestive heart failure ie, coughing, exercise intolerance, dyspnea, abdominal distension etc. There was history of feeding the cat with home made diet in one case and in other with commercial dog food. Based on electrocardiographic, radiographic and echocardiographic findings, the diagnosis of dilated cardiomyopathy was done in both the cases. The cases were managed with enalapril maleate, furosemide, dietary taurine supplementation and other supportive therapy. Among these two cases, one cat died on 2nd day of treatment and the other showed recovery after 8 days of treatment. [Vet World 2013; 6(4.000: 226-227

  7. Takotsubo cardiomyopathy in amiodarone-induced hyperthyroidism

    Directory of Open Access Journals (Sweden)

    Ismael Capel

    2017-02-01

    Full Text Available Takotsubo cardiomyopathy (TC is an atypical, severe but reversible form of acute heart insufficiency. It typically presents with left ventricular failure, transient apical and mid-segments hypokinesis, absence of significant coronary stenosis and new electrographic abnormalities and/or elevation in serum cardiac enzymes. Although TC (‘broken heart syndrome’ has classically been associated with emotional trauma, evidence suggests that other precipitants might exist, including iatrogenic and thyroid-mediated forms. Thyroid disease is a relatively common comorbidity in TC patients. We report a case of TC in a postmenopausal female with no history of emotional trauma or other potential precipitant factors who was diagnosed with amiodarone-induced hyperthyroidism during her hospital stay. Though some case reports of thyroidrelated TC exist, we are not aware of any other reported case of TC precipitated by amiodarone-induced hyperthyroidism.

  8. A Case Report of Reversible Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Abhishek Singhai

    2014-01-01

    Full Text Available Dilated cardiomyopathy (DCM is mostly an idiopathic disease with a progressive and irreversible course. It carries poor prognosis and outcome. Rarely, a reversible metabolic etiology that is amenable to specific therapy is identified. Alteration in thyroid status can lead to changes in systolic and diastolic function of left ventricle. Heart is sensitive to thyroid hormone changes, and cardiac disorders are commonly associated with both hyper and hypothyroidism. Diastolic dysfunction is the most common abnormality reported in hypothyroidism. In systolic function, prolonged systolic time interval or normal cardiac function has been reported by most workers. DCM is a rare presentation of hypothyroidism. Here, we report a case of 40-year-old female diagnosed with DCM due to hypothyroidism

  9. Sepsis-Induced Cardiomyopathy: Mechanisms and Treatments

    Directory of Open Access Journals (Sweden)

    Yan-Cun Liu

    2017-08-01

    Full Text Available Sepsis is a lethal syndrome with a high incidence and a weighty economy burden. The pathophysiology of sepsis includes inflammation, immune dysfunction, and dysfunction of coagulation, while sepsis-induced cardiomyopathy (SIC, defined as a global but reversible dysfunction of both sides of the heart induced by sepsis, plays a significant role in all of the aspects above in the pathogenesis of sepsis. The complex pathogenesis of SIC involves a combination of dysregulation of inflammatory mediators, mitochondrial dysfunction, oxidative stress, disorder of calcium regulation, autonomic nervous system dysregulation, and endothelial dysfunction. The treatments for SIC include the signal pathway intervention, Chinese traditional medicine, and other specific therapy. Here, we reviewed the latest literatures on the mechanisms and treatments of SIC and hope to provide further insights to researchers and create a new road for the therapy of sepsis.

  10. Implantation metallurgy

    International Nuclear Information System (INIS)

    Picraux, S.T.

    1975-01-01

    Important changes in the near-surface physical properties of metals were obtained by high-fluence ion implantation. Recently there have been an increasing number of studies of the behavior of implanted species with the aim of understanding the detailed physical processes that occur in an implanted metal layer. A key aspect of these implantation metallurgy studies has been the ability to form uniquely controlled systems in the near-surface regions of metals that can be studied with accurate depth resolution. Metallurgical parameters that may be difficult or impossible to obtain by other means can be measured. Also, parameters that depend on the implantation process, due to the athermal introduction of atoms and defects can be determined. Thus the dual objective of implantation metallurgy is to obtain information to improve understanding of the microscopic aspects of metallurgy and to understand how to form controlled new metallurgical systems. Examples of parameters studied include implanted impurity location, diffusion, enhanced diffusion, solubility, precipitation, and dissolution. (auth)

  11. Pediatric cardiomyopathy: importance of genetic and metabolic evaluation

    Science.gov (United States)

    Kindel, Steven J.; Miller, Erin M.; Gupta, Resmi; Cripe, Linda H.; Hinton, Robert B.; Spicer, Robert L.; Towbin, Jeffrey A.; Ware, Stephanie M.

    2012-01-01

    Background Cardiomyopathy is a heterogeneous disease with a strong genetic component. A research-based pediatric cardiomyopathy registry (PCMR) identified familial, syndromic, or metabolic causes in 30% of children. However, these results pre-dated clinical genetic testing. Methods and Results We determined the prevalence of familial, syndromic, or metabolic causes in eighty-three consecutive unrelated patients referred for genetic evaluation of cardiomyopathy from 2006–2009. Seventy-six percent of probands (n=63) were categorized as familial, syndromic, or metabolic. Forty-three percent (n=18) of hypertrophic cardiomyopathy (HCM) patients had mutations in sarcomeric genes, with MYH7 and MYBPC3 mutations predominating. Syndromic (17%, n=7) and metabolic (26%, n=11) causes were frequently identified in HCM patients. The metabolic subgroup was differentiated by decreased endocardial shortening fraction on echocardiography. Dilated cardiomyopathy (DCM) patients had similar rates of syndromic (20%, n=5) and metabolic (16%, n=4) causes, but fewer familial cases (24%, n=6) than HCM patients. Conclusions The cause of cardiomyopathy is identifiable in a majority of affected children. An underlying metabolic or syndromic cause is identified in greater than 35% of children with HCM or DCM. Identification of etiology is important for management, family based risk assessment, and screening. PMID:22555271

  12. Metabolic remodeling associated with subchronic doxorubicin cardiomyopathy

    International Nuclear Information System (INIS)

    Carvalho, Rui A.; Sousa, Rui P.B.; Cadete, Virgilio J.J.; Lopaschuk, Gary D.; Palmeira, Carlos M.M.; Bjork, James A.; Wallace, Kendall B.

    2010-01-01

    Doxorubicin (Adriamycin ® ) is a potent and broad-spectrum antineoplastic agent, the clinical utility of which is restricted by a cumulative and progressive cardiomyopathy that develops with repeated dosing. Fundamental to the cardiac failure is an interference with mitochondrial respiration and inhibition of oxidative phosphorylation. Global gene expression arrays in cardiac tissue indicate that inhibition of mitochondrial oxidative phosphorylation by doxorubicin (DOX) is accompanied by a decreased expression of genes related to aerobic fatty acid oxidation and a corresponding increase in expression of genes involved in anaerobic glycolysis, possibly as an alternate source for ATP production. The aim of this investigation was to determine whether this is also manifest at the metabonomic level as a switch in metabolic flux in cardiac tissue, and whether this can be averted by co-administering the cardioprotective drug, dexrazoxane (DZR). 13 C-isotopomer analysis of isolated perfused hearts from male Sprague-Dawley rats receiving 6 weekly s.c. injections of 2 mg/kg DOX demonstrated a shift from the preferential oxidation of fatty acids to enhanced oxidation of glucose and lactate plus pyruvate, indicative of a compensatory shift towards increased pyruvate dehydrogenase activity. Substrate-selective isotopomer analysis combined with western blots indicate an inhibition of long-chain fatty acid oxidation and not MCAD activity or fatty acyl-carnitine transport. Co-administering DZR averted many treatment-related changes in cardiac substrate metabolism, consistent with DZR being an effective cardioprotective agent against DOX-induced cardiomyopathy. This switch in substrate metabolism resembles that described for other models of cardiac failure; accordingly, this change in metabolic flux may represent a general compensatory response of cardiac tissue to imbalances in bioenergetic demand and supply, and not a characteristic unique to DOX-induced cardiac failure itself.

  13. Adderall induced inverted-Takotsubo cardiomyopathy.

    Science.gov (United States)

    Alsidawi, Said; Muth, James; Wilkin, James

    2011-11-15

    Takotsubo Cardiomyopathy (TTC), also known as stress-induced cardiomyopathy, was initially described in Japan in 1990. Both illicit and prescription drugs have added to the growing list of insulting stressors. We describe an interesting case of atypical TTC triggered by adderall overdose. A 19-year-old female was brought to the Emergency Department after ingesting 30 Adderall tablets. She was complaining of pressure like chest pain and shortness of breath. Her cardiac enzymes were elevated but the electrocardiogram was unremarkable. Echocardiography identified an ejection fraction (EF) of 25-30% with severe hypokinesis of the base and a preserved apex. Cardiac angiography demonstrated normal coronary arteries with an EF of 35%, hyperkinetic apex and akinetic base consistent with the diagnosis of inverted-TTC. Her symptoms resolved in 24 hrs. Repeat echocardiogram performed 3 days later showed an EF of 60% with no regional wall motion abnormalities. TTC can be identified as a rapid development of severe and reversible left ventricular dysfunction extending beyond the territory of a single epicardial coronary artery in the absence of coronary artery disease or pheochromocytoma. Clinical presentation can be challenging and very hard to distinguish from acute myocardial infarction. Medication induced-TTC has been reported. In our case, the patient overdosed on Adderall which is a sympathomimetic medication. Cardiac imaging identified wall motion abnormalities consistent with inverted type TTC. Restoration of left ventricular function within days confirms the diagnosis of TTC. In conclusion, this case offers an interesting insight into the pathophysiology of TTC. Copyright © 2011 Wiley Periodicals, Inc.

  14. Epidemiology of cardiomyopathy – A Clinical and Genetic Study of Restrictive Cardiomyopathy: The EPOCH-R Study

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    Mitali Kapoor

    2017-01-01

    Full Text Available Introduction: Restrictive cardiomyopathy (RCM is characterized by diastolic dysfunction, biatrial enlargement, and normal or near-normal systolic function. RCM is the rarest kind among cardiomyopathies with a severe outcome. Methods: Here, we present the clinical outcomes of thirty RCM patients recruited from a tertiary care unit of India, All India Institute of Medical Sciences, New Delhi. For clinical assessment, patients underwent electrocardiogram, echocardiography, and cardiac catheterization, and endomyocardial biopsy whenever required. Results: Out of 190 patients with cardiomyopathy, 100 had dilated cardiomyopathy, 60 had hypertrophic cardiomyopathy, and 30 had idiopathic RCM and were recruited for the study. Out of these thirty patients, 63.3% were males. A maximum number of patients were diagnosed in their second to third decade of life. Atrial fibrillation (73.3% and ST-T abnormalities (76.6% were common. Most of the patients showed the early age of onset with symptoms emerging in the first and second decades of life. Shortness of breath and fatigue were found to be common symptoms. No familial cases were found. Conclusion: RCM in India is a sporadic disease, rare, and occurs in the young. Prognosis of RCM is still worse than any other cardiomyopathy.

  15. Cochlear implant

    Science.gov (United States)

    ... made up of a microphone/receiver, a speech processor, and an antenna. This part of the implant ... ear. This sound is sent to a speech processor, which is most often connected to the microphone ...

  16. Towards Early Detection and Risk Stratification of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

    NARCIS (Netherlands)

    Riele, A.S.J.M. te

    2016-01-01

    Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by frequent ventricular arrhythmias and usually slowly progressive ventricular dysfunction. Since its initial description in 1982, sudden cardiac death (SCD) occurring in young and usually

  17. Subtle abnormalities in contractile function are an early manifestation of sarcomere mutations in dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Lakdawala, Neal K; Thune, Jens J; Colan, Steven D

    2012-01-01

    Sarcomere mutations cause both dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM); however, the steps leading from mutation to disease are not well described. By studying mutation carriers before a clinical diagnosis develops, we characterize the early manifestations of sarcomere...

  18. THE ROLE OF PARVOVIRUS B19 IN THE DEVELOPMENT OF INFLAMMATORY CARDIOMYOPATHY

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    A. Yu. Shchedrina

    2013-01-01

    Full Text Available The problem of inflammatory cardiomyopathy is discussed. The etiology, pathogenesis, diagnosis and treatment of inflammatory cardiomyopathy are considered with focus on the role of parvovirus B19.

  19. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY AS A SIDE-EFFECT OF DEXAMETHASONE TREATMENT FOR BRONCHOPULMONARY DYSPLASIA

    NARCIS (Netherlands)

    BRAND, PLP; VANLINGEN, RA; BRUS, F; TALSMA, MD; ELZENGA, NJ

    1993-01-01

    We report three infants who developed hypertrophic obstructive cardiomyopathy during dexamethasone treatment for bronchopulmonary dysplasia. In all three infants, echocardiography had ruled out cardiac abnormalities prior to the dexamethasone course. The hypertrophic obstructive cardiomyopathy

  20. Magnetic resonance imaging in familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion and cardiac enzymes

    International Nuclear Information System (INIS)

    Nishimura, Tsunehiko; Nagata, Seiki; Sakakibara, Hiroshi

    1988-01-01

    Gated magnetic resonance imaging (MRI) was performed in 6 patients with familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion, and 12 patients with ordinary hypertrophic cardiomyopathy. The patients with ordinary hypertrophic cardiomyopathy and abnormal thickening of the septal wall and normal left ventricular dimensions, while the patients with familial hypertrophic cardiomyopathy had focal wall thinning (usually involving the apical-septal wall) and dilated left ventricle in addition to hypertrophied heart. The quantitative measurement for cardiac dimensions using MRI was similar to that found on echocardiography in all cases. In addition, inhomogeneous signal intensities at left ventricular wall were observed in 3 cases of familial hypertrophic cardiomyopathy, which may suggest the existence of myocardial fibrosis. Gated MRI should be performed for early detection and follow-up of hypertrophic cardiomyopathy, since some patients will progress from hypertrophic cardiomyopathy to dilated cardiomyopathy. (author)

  1. Spanish implantable cardioverter-defibrillator registry. Eighth official report of the Spanish Society of Cardiology Working Group on Implantable Cardioverter-Defibrillators (2011).

    Science.gov (United States)

    Alzueta, Javier; Fernández, José María

    2012-11-01

    To summarize the findings of the Spanish Implantable Cardioverter-Defibrillator Registry for 2011 compiled by the Electrophysiology and Arrhythmia Section of the Spanish Society of Cardiology. Each implantation team voluntarily and prospectively recorded data on a data collection form, which was then sent to the Spanish Society of Cardiology. Overall, 4481 device implantations were notified, representing 83.6% of the estimated total number of implantations. The notified implantation rate was 97 per million population and the estimated total implantation rate was 116.2 per million. First implantations accounted for 70.2% of the total notified. Data were collected from 167 hospitals (22 more than in 2010). Most implantable cardioverter-defibrillator implantations took place in men (82.1%). The mean age was 62.4 (14.1) years. Most patients had severe or moderate-to-severe ventricular dysfunction and were in New York Heart Association functional class II. The most frequent underlying cardiac condition was ischemic heart disease, followed by dilated cardiomyopathy. The number of indications for primary prevention increased over the previous year and accounted for 70.6% of first implantations. Overall, 78.4% of implantable cardioverter-defibrillators were implanted by cardiac electrophysiologists. The 2011 Spanish Implantable Cardioverter-Defibrillator Registry includes data on almost 84% of all implantations of these devices performed in Spain. This was the first year in which the number of implants decreased slightly from the previous year, as also occurred in the rest of Europe. The percentage of implants for primary prevention continued to increase. Full English text available from:www.revespcardiol.org. Copyright © 2012 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

  2. Dobutamine Stress Echocardiography in Patients with Dilated Cardiomyopathy

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    Miloradovic Vladimir

    2016-06-01

    Full Text Available A clear distinction between two of the most common forms of dilated cardiomyopathy is very important due to their different prediction and therapeutic approaches. Dobutamine stress echocardiography appears to be a noninvasive selection method due to its clear differentiation potential. Major factors influence test interpretation, resulting in a wide interval of diagnostic accuracy for this test. Fraction flow reserve (FFR is a novel invasive method for estimating coronary artery stenosis responsible for myocardium ischaemia. Decisions about lesion significance in coronary blood vessels have thus far been based on angiographic estimations, but this approach is being replaced by FFR measurements, which serve as a new gold standard and involve a noninvasive test. The goal of this study was to clearly differentiate two forms of dilated cardiomyopathies through analysis of the segmented mobility of the left ventricular wall. Fifty patients were analysed: 20 with ischaemic dilated cardiomyopathy, which was confirmed not only through coronary angiography but also functionally through FFR measurement, and 30 patients with nonischaemic dilated cardiomyopathy, which was confirmed by coronary angiography. A standard dobutamine stress echocardiography protocol was implemented. A positive dobutamine stress echocardiography test was defined as the presence of emerging incidents in segment contractility or worsening of existing incidents in at least one segment. Statistically relevant diff erences in the movement dynamics of a number of differently characterised segments during the observed time intervals (ANOVA p=0.000 was noted in both groups of patients, as was variation in the index value of the summarized mobility of the left chamber wall. In patients with ischaemic cardiomyopathies, regional contractility worsened at the maximum dose of dobutamine; in contrast, this feature slightly improved in nonischaemic cardiomyopathy patients. The results indicate

  3. Characterization and Long-Term Prognosis of Postmyocarditic Dilated Cardiomyopathy Compared With Idiopathic Dilated Cardiomyopathy.

    Science.gov (United States)

    Merlo, Marco; Anzini, Marco; Bussani, Rossana; Artico, Jessica; Barbati, Giulia; Stolfo, Davide; Gigli, Marta; Muça, Matilda; Naso, Paola; Ramani, Federica; Di Lenarda, Andrea; Pinamonti, Bruno; Sinagra, Gianfranco

    2016-09-15

    Dilated cardiomyopathy (DC) is the final common pathway of different pathogenetic processes and presents a significant prognostic heterogeneity, possibly related to its etiologic variety. The characterization and long-term prognosis of postmyocarditic dilated cardiomyopathy (PM-DC) remain unknown. This study assesses the clinical-instrumental evolution and long-term prognosis of a large cohort of patients with PM-DC. We analyzed 175 patients affected with DC consecutively enrolled from 1993 to 2008 with endomyocardial biopsy (EMB) data available. PM-DC was defined in the presence of borderline myocarditis at EMB or persistent left ventricular dysfunction 1 year after diagnosis of active myocarditis at EMB. Other patients were defined as affected by idiopathic dilated cardiomyopathy (IDC). Analysis of follow-up evaluations was performed at 24, 60, and 120 months. We found 72 PM-DC of 175 enrolled patients (41%). Compared with IDC, patients with PM-DC were more frequently females and less frequently presented a familial history of DC. No other baseline significant differences were found. During the long-term follow-up (median 154, first to third interquartile range 78 to 220 months), patients with PM-DC showed a trend toward slower disease progression. Globally, 18 patients with PM-DC (25%) versus 49 with IDC (48%) experienced death/heart transplantation (p = 0.045). The prognostic advantage for patients with PM-DC became significant beyond 40 months of follow-up. At multivariable time-dependent Cox analysis, PM-DC was confirmed to have a global independent protective role (hazard ratio 0.53, 95% confidence interval 0.28 to 0.97, p = 0.04). In conclusion, PM-DC is characterized by better long-term prognosis compared with IDC. An exhaustive etiologic characterization appears relevant in the prognostic assessment of DC. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Delayed AICD therapy and cardiac arrest resulting from undersensing of ventricular fibrillation in a subject with hypertrophic cardiomyopathy-A case report.

    Science.gov (United States)

    Chin, Ashley; Healey, Jeffrey S; Ribas, Carlos S; Nair, Girish M

    2015-01-01

    Defibrillation testing is no longer routinely performed after automatic implantable cardioverter-defibrillator (AICD) implantation. However, certain subjects undergoing AICD implantation may be at higher risk of undersensing of ventricular arrhythmias resulting in potentially fatal outcomes. We present the case of a 30-year-old woman with hypertrophic cardiomyopathy (HCM; 'asymmetric septal hypertophy' morphologic variant) and prophylactic AICD who experienced an out of hospital cardiac arrest. AICD interrogation revealed undersensing as a result of intermittent high amplitude electrograms during an episode of ventricular fibrillation (VF). The subject underwent replacement and repositioning of the AICD lead along with pulse generator replacement (that utilized a different VF sensing algorithm) with appropriate sensing of VF and successful defibrillation testing. The presence of intermittent high amplitude electrograms during episodes of VF in AICDs using the AGC function should be recognized as a situation that may necessitate interventions to prevent undersensing and consequent delay in therapy.

  5. An unusual ST-segment elevation: apical hypertrophic cardiomyopathy shows the ace up its sleeve.

    Science.gov (United States)

    de Santis, Francesco; Pergolini, Amedeo; Zampi, Giordano; Pero, Gaetano; Pino, Paolo Giuseppe; Minardi, Giovanni

    2013-01-01

    Apical hypertrophic cardiomyopathy is part of the broad clinical and morphologic spectrum of hypertrophic cardiomyopathy. We report a patient with electrocardiographic abnormalities in whom acute coronary syndrome was excluded and apical hypertrophic cardiomyopathy was demonstrated by careful differential diagnosis. Copyright © 2012 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  6. Autosomal Dominant Polycystic Kidney Patients May Be Predisposed to Various Cardiomyopathies

    Directory of Open Access Journals (Sweden)

    Fouad T. Chebib

    2017-09-01

    Discussion: Coexistence of ADPKD and cardiomyopathy in our tertiary referral center cohort appears to be higher than expected by chance. We suggest that PKD1 and PKD2 mutations may predispose to primary cardiomyopathies and that genetic interactions may account for the observed coexistence of ADPKD and cardiomyopathies.

  7. Differentiation of infiltrative cardiomyopathy from hypertrophic cardiomyopathy using high-sensitivity cardiac troponin T: a case-control study.

    Science.gov (United States)

    Kubo, Toru; Baba, Yuichi; Hirota, Takayoshi; Tanioka, Katsutoshi; Yamasaki, Naohito; Yamanaka, Shigeo; Iiyama, Tatsuo; Kumagai, Naoko; Furuno, Takashi; Sugiura, Tetsuro; Kitaoka, Hiroaki

    2015-06-16

    Because infiltrative cardiomyopathy and hypertrophic cardiomyopathy (HCM) share clinical and hemodynamic features of left ventricular (LV) hypertrophy and abnormal diastolic function, it is often difficult to distinguish these entities. We investigated the potential role of high-sensitivity cardiac troponin T (hs-cTnT) for differentiation of infiltrative cardiomyopathy from HCM. The study group consisted of 46 consecutive patients with infiltrative cardiomyopathies or HCM in whom sarcomere protein gene mutations were identified at Kochi Medical School Hospital; of these, there were 11 patients with infiltrative cardiomyopathy (cardiac amyloidosis in 8 patients and Fabry disease in 3 patients) and 35 HCM patients. Serum hs-cTnT level was significantly higher in patients who had infiltrative cardiomyopathy than in those who had HCM (0.083 ± 0.057 ng/ml versus 0.027 ± 0.034 ng/ml, p  40 years at age), hs-cTnT level, maximum LV wall thickness, posterior wall thickness, peak early (E) transmitral filling velocity, peak early diastolic (Ea) velocity of tissue Doppler imaging at the lateral corner and E/Ea ratios at both the septal and lateral corners were significantly different between the two groups. As for diagnostic accuracy to differentiate the two groups by using receiver operating characteristic analysis, hs-cTnT was the highest value of area under the curve (0.939) and E/Ea (lateral) was second highest value (0.914). Serum hs-cTnT is a helpful diagnostic indicator for accurate differentiation between infiltrative cardiomyopathy and HCM.

  8. Ventricular evoked response in patients with hypertrophic obstructive cardiomyopathy treated with DDD pacing

    Directory of Open Access Journals (Sweden)

    João Ricardo M. Sant'Anna

    1999-08-01

    Full Text Available OBJECTIVE: To assess the changes in ventricular evoked responses (VER produced by the decrease in left ventricular outflow tract gradient (LVOTG in patients with hypertrophic obstructive cardiomyopathy (HOCM treated with dual-chamber (DDD pacing. METHODS: A pulse generator Physios CTM (Biotronik, Germany was implanted in 9 patients with severe drug-refractory HOCM. After implantation, the following conditions were assessed: 1 Baseline evaluation: different AV delay (ranging from 150ms to 50 ms were sequentially programmed during 5 to 10 minutes, and the LVOTG (as determined by Doppler echocardiography and VER recorded; 2 standard evaluation, when the best AV delay (resulting in the lowest LVOTG programmed at the initial evaluation was maintained so that its effect on VER and LVOTG could be assessed during each chronic pacing evaluation. RESULTS: LVOTG decreased after DDD pacing, with a mean value of 59 ± 24 mmHg after dual chamber pacemaker, which was significantly less than the gradient before pacing (98 + 22mmHg. An AV delay >100ms produced a significantly lower decrease in VER depolarization duration (VER DD when compared to an AV delay <=100ms. Linear regression analyses showed a significant correlation between the LVOTG values and the magnitude of VER (r=0.69; p<0.05 in the 9 studied patients. CONCLUSION: The telemetry obtained intramyocardial electrogram is a sensitive means to assess left ventricular dynamics in patients with HOCM treated with DDD pacing.

  9. Cardiomyopathy induced by artificial cardiac pacing: myth or reality sustained by evidence?

    Science.gov (United States)

    Ferrari, Andrés Di Leoni; Borges, Anibal Pires; Albuquerque, Luciano Cabral; Sussenbach, Carolina Pelzer; da Rosa, Priscila Raupp; Piantá, Ricardo Medeiros; Wiehe, Mario; Goldani, Marco Antônio

    2014-01-01

    Implantable cardiac pacing systems are a safe and effective treatment for symptomatic irreversible bradycardia. Under the proper indications, cardiac pacing might bring significant clinical benefit. Evidences from literature state that the action of the artificial pacing system, mainly when the ventricular lead is located at the apex of the right ventricle, produces negative effects to cardiac structure (remodeling, dilatation) and function (dissinchrony). Patients with previously compromised left ventricular function would benefit the least with conventional right ventricle apical pacing, and are exposed to the risk of developing higher incidence of morbidity and mortality for heart failure. However, after almost 6 decades of cardiac pacing, just a reduced portion of patients in general would develop these alterations. In this context, there are not completely clear some issues related to cardiac pacing and the development of this cardiomyopathy. Causality relationships among QRS widening with a left bundle branch block morphology, contractility alterations within the left ventricle, and certain substrates or clinical (previous systolic dysfunction, structural heart disease, time from implant) or electrical conditions (QRS duration, percentage of ventricular stimulation) are still subjecte of debate. This review analyses contemporary data regarding this new entity, and discusses alternatives of how to use cardiac pacing in this context, emphasizing cardiac resynchronization therapy. PMID:25372916

  10. Occurrence and Natural History of Clinically Silent Episodes of Atrial Fibrillation in Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Rowin, Ethan J; Orfanos, Alexander; Estes, N A Mark; Wang, Wendy; Link, Mark S; Maron, Martin S; Maron, Barry J

    2017-06-01

    Overt symptomatic atrial fibrillation (AF) occurs in over 20% of patients with hypertrophic cardiomyopathy (HC) leading to impaired quality of life, loss of productivity, and the risk for embolic stroke. However, the overall burden presented by AF in the HC population is unresolved due to the unknown frequency of silent asymptomatic episodes that do not necessarily achieve clinical recognition but nevertheless may have important disease-related implications. Therefore, stored electrograms were analyzed retrospectively for AF in 75 consecutive patients with HC (without AF history) implanted with dual-chamber cardioverter-defibrillators. Patients were followed for 5.0 ± 4.1 years at the Tufts Medical Center HCM Institute; ages were 50 ± 15 years, and 55% were male. Implantable cardioverter-defibrillator interrogation in the 75 patients showed AF to be absent in 54 (72%), 18 (24%) had clinically silent AF episodes, and the remaining 3 (4%) without previous asymptomatic episodes developed symptomatic and clinically overt paroxysmal AF. Of the 18 patients with clinically silent AF, 8 developed symptomatic AF, 4.1 ± 1.5 years later. Nonfatal embolic stroke occurred in 1 patient associated with asymptomatic AF and without other risk factors. In conclusion, clinically silent AF appears to be common in HC, occurring in almost 25% of patients. Such asymptomatic episodes of AF have important future implications, including potential thromboembolic risk, and development of symptomatic and clinically overt AF requiring prophylactic anticoagulation. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Management of patients with Arrhythmogenic Right Ventricular Cardiomyopathy in the Nordic countries.

    Science.gov (United States)

    Haugaa, Kristina H; Bundgaard, Henning; Edvardsen, Thor; Eschen, Ole; Gilljam, Thomas; Hansen, Jim; Jensen, Henrik Kjærulf; Platonov, Pyotr G; Svensson, Anneli; Svendsen, Jesper H

    2015-01-01

    Diagnostics of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are complex, and based on the 2010 Task Force document including different diagnostic modalities. However, recommendations for clinical management and follow-up of patients with ARVC and their relatives are sparse. This paper aims to give a practical overview of management strategies, risk stratification, and selection of appropriate therapies for patients with ARVC and their family members. This paper summarizes follow-up and treatment strategies in ARVC patients in the Nordic countries. The author group represents cardiologists who are actively involved in the Nordic ARVC Registry which was established in 2009, and contains prospectively collected clinical data from more than 590 ARVC patients from Denmark, Norway, Sweden, and Finland. Different approaches of management and follow-up are required in patients with definite ARVC and in genetic-mutation-positive family members. Furthermore, ARVC patients with and without implantable cardioverter defibrillators (ICDs) require different follow-up strategies. Careful follow-up is required in patients with ARVC diagnosis to evaluate the need of anti-arrhythmic therapy and ICD implantation. Mutation-positive family members should be followed regularly for detection of early disease and risk stratification of ventricular arrhythmias.

  12. Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report

    Directory of Open Access Journals (Sweden)

    Paraskevaidis Stylianos

    2009-06-01

    Full Text Available Abstract Background Hypertrophic cardiomyopathy (HCM is an extremely heterogeneous disease. An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular (LV apical aneurysms in the absence of coronary artery disease. Case presentation We describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family. This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test. The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2.5-y follow-up period. Conclusion The detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.

  13. Acute myocardial infarction and stress cardiomyopathy following the Christchurch earthquakes.

    Science.gov (United States)

    Chan, Christina; Elliott, John; Troughton, Richard; Frampton, Christopher; Smyth, David; Crozier, Ian; Bridgman, Paul

    2013-01-01

    Christchurch, New Zealand, was struck by 2 major earthquakes at 4:36 am on 4 September 2010, magnitude 7.1 and at 12:51 pm on 22 February 2011, magnitude 6.3. Both events caused widespread destruction. Christchurch Hospital was the region's only acute care hospital. It remained functional following both earthquakes. We were able to examine the effects of the 2 earthquakes on acute cardiac presentations. Patients admitted under Cardiology in Christchurch Hospital 3 week prior to and 5 weeks following both earthquakes were analysed, with corresponding control periods in September 2009 and February 2010. Patients were categorised based on diagnosis: ST elevation myocardial infarction, Non ST elevation myocardial infarction, stress cardiomyopathy, unstable angina, stable angina, non cardiac chest pain, arrhythmia and others. There was a significant increase in overall admissions (pearthquake. This pattern was not seen after the early afternoon February earthquake. Instead, there was a very large number of stress cardiomyopathy admissions with 21 cases (95% CI 2.6-6.4) in 4 days. There had been 6 stress cardiomyopathy cases after the first earthquake (95% CI 0.44-2.62). Statistical analysis showed this to be a significant difference between the earthquakes (pearthquake triggered a large increase in ST elevation myocardial infarction and a few stress cardiomyopathy cases. The early afternoon February earthquake caused significantly more stress cardiomyopathy. Two major earthquakes occurring at different times of day differed in their effect on acute cardiac events.

  14. Oxidative Stress in Dilated Cardiomyopathy Caused by MYBPC3 Mutation

    Directory of Open Access Journals (Sweden)

    Thomas L. Lynch

    2015-01-01

    Full Text Available Cardiomyopathies can result from mutations in genes encoding sarcomere proteins including MYBPC3, which encodes cardiac myosin binding protein-C (cMyBP-C. However, whether oxidative stress is augmented due to contractile dysfunction and cardiomyocyte damage in MYBPC3-mutated cardiomyopathies has not been elucidated. To determine whether oxidative stress markers were elevated in MYBPC3-mutated cardiomyopathies, a previously characterized 3-month-old mouse model of dilated cardiomyopathy (DCM expressing a homozygous MYBPC3 mutation (cMyBP-C(t/t was used, compared to wild-type (WT mice. Echocardiography confirmed decreased percentage of fractional shortening in DCM versus WT hearts. Histopathological analysis indicated a significant increase in myocardial disarray and fibrosis while the second harmonic generation imaging revealed disorganized sarcomeric structure and myocyte damage in DCM hearts when compared to WT hearts. Intriguingly, DCM mouse heart homogenates had decreased glutathione (GSH/GSSG ratio and increased protein carbonyl and lipid malondialdehyde content compared to WT heart homogenates, consistent with elevated oxidative stress. Importantly, a similar result was observed in human cardiomyopathy heart homogenate samples. These results were further supported by reduced signals for mitochondrial semiquinone radicals and Fe-S clusters in DCM mouse hearts measured using electron paramagnetic resonance spectroscopy. In conclusion, we demonstrate elevated oxidative stress in MYPBC3-mutated DCM mice, which may exacerbate the development of heart failure.

  15. Genetic advances in sarcomeric cardiomyopathies: state of the art.

    Science.gov (United States)

    Ho, Carolyn Y; Charron, Philippe; Richard, Pascale; Girolami, Francesca; Van Spaendonck-Zwarts, Karin Y; Pinto, Yigal

    2015-04-01

    Genetic studies in the 1980s and 1990s led to landmark discoveries that sarcomere mutations cause both hypertrophic and dilated cardiomyopathies. Sarcomere mutations also likely play a role in more complex phenotypes and overlap cardiomyopathies with features of hypertrophy, dilation, diastolic abnormalities, and non-compaction. Identification of the genetic cause of these important conditions provides unique opportunities to interrogate and characterize disease pathogenesis and pathophysiology, starting from the molecular level and expanding from there. With such insights, there is potential for clinical translation that may transform management of patients and families with inherited cardiomyopathies. If key pathways for disease development can be identified, they could potentially serve as targets for novel disease-modifying or disease-preventing therapies. By utilizing gene-based diagnostic testing, we can identify at-risk individuals prior to the onset of clinical disease, allowing for disease-modifying therapy to be initiated early in life, at a time that such treatment may be most successful. In this section, we review the current application of genetics in clinical management, focusing on hypertrophic cardiomyopathy as a paradigm; discuss state-of-the-art genetic testing technology; review emerging knowledge of gene expression in sarcomeric cardiomyopathies; and discuss both the prospects, as well as the challenges, of bringing genetics to medicine. © The Author 2015. Published by Oxford University Press on behalf of the European Society of Cardiology.

  16. Takotsubo cardiomyopathy after a dancing session: a case report.

    Science.gov (United States)

    Kaballo, Mohammed A; Yousif, Abdelazim; Abdelrazig, Awadalla M; Ibrahim, Ammar A; Hennessy, Terence G

    2011-10-31

    Stress-induced (Takotsubo) cardiomyopathy is a rare form of cardiomyopathy which presents in a manner similar to that of acute coronary syndrome. This sometimes leads to unnecessary thrombolysis therapy. The pathogenesis of this disease is still poorly understood. We believe that reporting all cases of Takotsubo cardiomyopathy will contribute to a better understanding of this disease. Here, we report a patient who, in the absence of any recent stressful events in her life, developed the disease after a session of dancing. A 69-year-old Caucasian woman presented with features suggestive of acute coronary syndrome shortly after a session of dancing. Echocardiography and a coronary angiogram showed typical features of Takotsubo cardiomyopathy and our patient was treated accordingly. Eight weeks later, her condition resolved completely and the results of echocardiography were totally normal. Takotsubo cardiomyopathy, though transient, is a rare and serious condition. Although it is commonly precipitated by stressful life events, these are not necessarily present. Our patient was enjoying one of her hobbies (that is, dancing) when she developed the disease. This case has particular interest in medicine, especially for the specialties of cardiology and emergency medicine. We hope that it will add more information to the literature about this rare condition.

  17. Implantable Cardioverter Defibrillator

    Science.gov (United States)

    ... To Health Topics / Implantable Cardioverter Defibrillators Implantable Cardioverter Defibrillators Also known as What Is an Implantable Cardioverter ... pacemakers and defibrillators. Comparison of an Implantable Cardioverter Defibrillator and a Pacemaker The image compares an ICD ...

  18. Breast reconstruction - implants

    Science.gov (United States)

    Breast implants surgery; Mastectomy - breast reconstruction with implants; Breast cancer - breast reconstruction with implants ... to close the skin flaps. Breast reconstruction with implants is usually done in two stages, or surgeries. ...

  19. Genetic bases of arrhythmogenic right ventricular cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Alessandra Rampazzo

    2010-05-01

    Full Text Available Arrhythmogenic right ventricular cardiomyopathy (ARVC is a heart muscle disease in which the pathological substrate is a fibro-fatty replacement of the right ventricular myocardium. The major clinical features are different types of arrhythmias with a left branch block pattern. ARVC shows autosomal dominant inheritance with incomplete penetrance. Recessive forms were also described, although in association with skin disorders. Ten genetic loci have been discovered so far and mutations were reported in five different genes. ARVD1 was associated with regulatory mutations of transforming growth factor beta-3 (TGFβ3, whereas ARVD2, characterized by effort-induced polymorphic arrhythmias, was associated with mutations in cardiac ryanodine receptor-2 (RYR2. All other mutations identified to date have been detected in genes encoding desmosomal proteins: plakoglobin (JUP which causes Naxos disease (a recessive form of ARVC associated with palmoplantar keratosis and woolly hair; desmoplakin (DSP which causes the autosomal dominant ARVD8 and plakophilin-2 (PKP2 involved in ARVD9. Desmosomes are important cell-to-cell adhesion junctions predominantly found in epidermis and heart; they are believed to couple cytoskeletal elements to plasma membrane in cell-to-cell or cell-to-substrate adhesions.

  20. A One Health Approach to Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Ueda, Yu; Stern, Joshua A

    2017-09-01

    Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease in humans and results in significant morbidity and mortality. Research over the past 25 years has contributed enormous insight into this inherited disease particularly in the areas of genetics, molecular mechanisms, and pathophysiology. Our understanding continues to be limited by the heterogeneity of clinical presentations with various genetic mutations associated with HCM. Transgenic mouse models have been utilized especially studying the genotypic and phenotypic interactions. However, mice possess intrinsic cardiac and hemodynamic differences compared to humans and have limitations preventing their direct translation. Other animal models of HCM have been studied or generated in part to overcome these limitations. HCM in cats shows strikingly similar molecular, histopathological, and genetic similarities to human HCM, and offers an important translational opportunity for the study of this disease. Recently, inherited left ventricular hypertrophy in rhesus macaques was identified and collaborative investigations have been conducted to begin to develop a non-human primate HCM model. These naturally-occurring large-animal models may aid in advancing our understanding of HCM and developing novel therapeutic approaches to this disease. This review will highlight the features of HCM in humans and the relevant available and developing animal models of this condition.

  1. [Recurrent postpartum pyoderma gangrenosum and fatal cardiomyopathy].

    Science.gov (United States)

    Naciri, I; Meziane, M; Benzekri, L; Ghaouti, M; Senouci, K; Hassam, B

    2017-12-06

    We report a case of recurrent post-partum pyoderma gangrenosum (PG) complicated by post-partum cardiomyopathy (PPCM). A 23-year-old woman presented with a previous medical history of aseptic abscess of the left breast in her fourth pregnancy, which developed after surgical drainage of an inflammatory ulceration treated by atraumatic topical care. During her fifth pregnancy, the patient presented a large and painful ulceration in relation to the scar of the Caesarean section, despite the introduction of broad-spectrum antibiotic therapy. Bacteriological samples were negative. Histological examination militated in favor of PG. One week after initiation of corticosteroid therapy, the patient suddenly showed signs of heart failure. Based on trans-thoracic echocardiography PPCM was diagnosed, and the outcome was fatal. This observation raises the question of the relationship between PG and pregnancy and describes the association of PG and PPCM. PG occurs rarely during pregnancy and it may be induced by the rise in G-CSF levels found in pregnant women. The association with PPCM seen in our patient could have been due to the development of an anti-angiogenic climate at the end of pregnancy, together with inflammatory myocardial aggression linked to the PG. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  2. Significance of myocardial scintigraphy in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Emoto, Tsugumichi; Shimizu, Yoshimi; Yuo, Hiroyuki (Kanazawa Univ. (Japan). School of Medicine) (and others)

    1994-02-01

    The study investigated the relationship between the occurrence incidence of abnormal findings on exercice Tl-201 myocardial scintigraphy and pathophysiology in hypertrophic cardiomyopathy (HCM). Fifty HCM patients underwent exercise Tl-201 scintigraphy. Simultaneously, 12-lead ECG was recorded during exercise. In addition, 17 patients, who were randomly selected from the 50 patients, underwent I-123 BMIPP myocardial scintigraphy. Abnormal findings were observed in 23 of the 50 patients (46%) on Tl images. Among these patients, 19 patients (38%) had redistribution on 3 hr images (the group of reversible defects (R)), and 4 (8%) had no redistribution (the group of fixed defects (F)). Regarding the occurrence incidence of ischemic ST changes and exercise tolerance ability, there was no difference between the group of normal findings and the group of R. In the group of F, however, ST changes were seen in all patients, although there was no difference in exercise tolerance ability. Of 17 patients who underwent I-123 BMIPP scintigraphy, 7 had normal findings on Tl images. Of these 7, 4 had abnormal findings on BMIPP images and decreased exercise tolerance ability as well. These findings suggest the necessity for careful follow-up even in HCM patients who had no abnormal findings on Tl images. (N.K.).

  3. Cardiomyopathy in Patients With Hereditary Bullous Epidermolysis.

    Science.gov (United States)

    Batalla, A; Vicente, A; Bartrons, J; Prada, F; Fortuny, C; González-Enseñat, M A

    In recent decades, an association has been reported between epidermolysis bullosa (EB) and dilated cardiomyopathy (DC). DC is typically in an advanced phase when detected, leading to a poorer prognosis. Our objective was to determine the prevalence of DC in patients with EB seen in Hospital San Joan de Déu in Barcelona, Spain, between May 1986 and April 2015. This was a descriptive, cross-sectional chart-review study in which we recorded the type and main subtypes of EB and the presence or absence of DC. Fifty-seven patients with EB were found, 19 with EB simplex, 10 with junctional EB, 27 with dystrophic EB (14 dominant dystrophic and 13 recessive dystrophic), and just 1 with Kindler syndrome. DC was detected in only 2 patients with recessive dystrophic EB. Twenty-three patients had presented factors that could have had a causal relationship with the potential onset of DC. DC is a possible complication of EB, particularly in recessive dystrophic EB. Periodic follow-up should be performed to make an early diagnosis and start treatment. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. Prooxidant Mechanisms in Iron Overload Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ching-Feng Cheng

    2013-01-01

    Full Text Available Iron overload cardiomyopathy (IOC, defined as the presence of systolic or diastolic cardiac dysfunction secondary to increased deposition of iron, is emerging as an important cause of heart failure due to the increased incidence of this disorder seen in thalassemic patients and in patients of primary hemochromatosis. At present, although palliative treatment by regular iron chelation was recommended; whereas IOC is still the major cause for mortality in patient with chronic heart failure induced by iron-overloading. Because iron is a prooxidant and the associated mechanism seen in iron-overload heart is still unclear; therefore, we intend to delineate the multiple signaling pathways involved in IOC. These pathways may include organelles such as calcium channels, mitochondria; paracrine effects from both macrophages and fibroblast, and novel mediators such as thromboxane A2 and adiponectin; with increased oxidative stress and inflammation found commonly in these signaling pathways. With further understanding on these complex and inter-related molecular mechanisms, we can propose potential therapeutic strategies to ameliorate the cardiac toxicity induced by iron-overloading.

  5. EBCT in diagnosis on primary cardiomyopathy

    International Nuclear Information System (INIS)

    Li Xiangmin; Zhou Xuhui; Meng Quanfei; Li Weiduo; Peng Qian; Tan Zhiyu

    2000-01-01

    Objective: To evaluate the diagnostic value of electron beam CT (EBCT) in primary cardiomyopathy (PCM). Methods: EBCT including coronary scanning and heart movie study was performed in 15 patients. The diagnosis of PCM was established by clinical and imaging findings or/and pathologic examination. Results: BECT findings related to the classification of PCM drawn up by WHO/ISFC were as follows: (1) dilated PCM (8 cases): dilated left ventricle (LV) was found in 7 of 8 cases and dilated right ventricle (RV) in the remaining one. Hypokinetic contraction of the LV wall with (250 ± 101) ml LVEDV and (18.9 ±6.6)% LVEF presented in the patients with dilated LV. (2) Hypertrophic PCM (5 cases): Hypertrophic ventricular septum (16.5 ± 2.3) mm was demonstrated in all cases, hypertrophy of the LV wall or the apex in 3 of 5 cases, and stenosis of the LV outflow tract in 2 cases. (3) Restrictive PCM (2 cases): Thickening of endocardium with lower density than cardiac muscle, reduced RV, obliteration of apex, hypokinetic contraction of the RV wall, and dilatation of right atrium (RA) with parietal thrombus formation of RA was identified in one patient. Both the LV and the RV were involved, with enlargement of atrium. Conclusion: The findings of PCM on EBCT seem to reflect pathologic and functional changes of PCM, and may be useful for diagnosis and classification of PCM

  6. Takotsubo Cardiomyopathy and Catatonia in the Setting of Benzodiazepine Withdrawal

    Directory of Open Access Journals (Sweden)

    Teng J. Peng

    2016-01-01

    Full Text Available We report two serious and unusual complications of benzodiazepine withdrawal in a single patient: takotsubo cardiomyopathy and catatonia. This 61-year-old female patient was brought to the emergency department with lethargy and within hours had declined into a state of catatonia. Although there was never a complaint of chest pain, ECG showed deep anterior T-wave inversions and cardiac enzymes were elevated. An echocardiogram was consistent with takotsubo cardiomyopathy. She later received 1 mg of midazolam and within minutes had resolution of catatonic symptoms. Careful history revealed that she had omitted her daily dose of lorazepam for 3 days prior to admission. To our knowledge, the case presented herein is the first report of simultaneous catatonia and takotsubo cardiomyopathy in the setting of benzodiazepine withdrawal. The pathogenesis of both conditions is poorly understood but may be indirectly related to the sudden decrease in γ-aminobutyric acid (GABA signaling during benzodiazepine withdrawal.

  7. Twenty Years of Alcohol Septal Ablation in Hypertrophic Obstructive Cardiomyopathy

    Science.gov (United States)

    Rigopoulos, Angelos G.; Seggewiss, Hubert

    2016-01-01

    Hypertrophic obstructive cardiomyopathy is the most common genetic cardiac disease and is generally characterised by asymmetric septal hypertrophy and intraventricular obstruction. Patients with severe obstruction and significant symptoms that persist despite optimal medical treatment are candidates for an invasive septal reduction therapy. Twenty years after its introduction, percutaneous transluminal alcohol septal ablation has been increasingly preferred for septal reduction in patients with drug refractory hypertrophic obstructive cardiomyopathy. Myocardial contrast echocardiography and injection of reduced alcohol volumes have increased safety, while efficacy is comparable to the surgical alternative, septal myectomy, which has for decades been regarded as the ‘gold standard’ treatment. Data on medium- and long-term survival show improved prognosis with survival being similar to the general population. Current guidelines have supported its use by experienced operators in centres specialised in the treatment of patients with hypertrophic obstructive cardiomyopathy. PMID:25563291

  8. Arrhythmia-Induced Cardiomyopathy: Prevalent, Under-recognized, Reversible.

    Science.gov (United States)

    Dhawan, Rahul; Gopinathannair, Rakesh

    2017-01-01

    Arrhythmia-induced cardiomyopathy (AIC) is a clinical condition in which a persistent tachyarrhythmia or frequent ectopy contribute to ventricular dysfunction leading to systolic heart failure. AIC can be partially or completely corrected with adequate treatment of the culprit arrhythmia. Several molecular and cellular alterations by which tachyarrhythmias lead to cardiomyopathy have been identified. AIC can affect children and adults, can be clinically silent in the form of asymptomatic tachycardia with cardiomyopathy, or can present with manifest heart failure. A high index of suspicion for AIC and aggressive treatment of the culprit arrhythmia can result in resolution of heart failure symptoms and improvement in cardiac function. Recurrent arrhythmia, following recovery from the index episode, can hasten the left ventricular dysfunction and result in HF, suggesting persistent adverse remodeling despite recovery of left ventricular function. Several aspects of AIC, such as predisposing factors, early diagnosis, preventive measures to avoid adverse remodeling, and long-term prognosis, remain unclear, and need further research.

  9. Spontaneous coronary artery dissection associated with apical hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Tuncer, M.; Gumrukcuoglu, H.A.; Ekim, H.; Gunes, Y.; Simsek, H.

    2010-01-01

    Apical hypertrophic cardiomyopathy (HCM) is a relatively uncommon inherited disease. Spontaneous coronary artery dissection (SCAD) is also uncommonly observed, which often occurs in pregnant or post partum women but is rare in men. This report describes a 38 years old man with apical hypertrophic cardiomyopathy who developed SCAD leading to acute inferior myocardial infarction. After emergent appendectomy operation at another hospital, he was immediately transferred to the Cardiology Department of our hospital due to acute myocardial infarction. He emergently underwent coronary angiography which showed a long dissection involving the right coronary. He underwent an emergent CABG with cardiopulmonary bypass. Postoperative recovery was uneventful and he was discharged. According to our knowledge, no case of spontaneous coronary artery dissection associated with apical hypertrophic cardiomyopathy unrelated to postpartum period or oral contraceptive use has been reported so far. (author)

  10. DKA-Induced Takotsubo Cardiomyopathy in Patient with Known HOCM

    Directory of Open Access Journals (Sweden)

    Ayla Gordon

    2017-01-01

    Full Text Available The first published case of Diabetic Ketoacidosis-induced Takotsubo cardiomyopathy was in 2009. Our patient is the 1st reported case of Diabetic Ketoacidosis- (DKA- induced Takotsubo cardiomyopathy (TC in a patient with known hypertrophic cardiomyopathy (HOCM in the United States. In the literature, there are only two examples linking DKA to TC; however, this report focuses on the biochemical and physiological causes of TC in a patient with known HOCM and new-onset DKA. TC in previously diagnosed HOCM poses particular complications. With the above patient’s baseline outflow tract obstruction due to septal hypertrophy, the acute reduction in EF due to TC resulted in transient drop in brain perfusion and, therefore, syncope.

  11. Takotsubo cardiomyopathy in myasthaenia gravis crisis confirmed by cardiac MRI.

    Science.gov (United States)

    Harries, I B; Levoir, H; Bucciarelli-Ducci, C; Ramcharitar, S

    2015-09-28

    Myasthaenia gravis crisis and Takotsubo cardiomyopathy are rare conditions that can be precipitated by emotional or physical stress. Myasthaenia gravis has a variety of cardiac manifestations but Takotsubo cardiomyopathy, particularly in male patients, has rarely been reported. We describe a unique case of a 70-year-old man who developed Takotsubo cardiomyopathy during his first presentation with a myasthaenia gravis crisis. He had not received plasmapharesis or immunoglobulin therapy. Striking ECG traces and cardiac MRI helped to confirm the diagnosis. Cardiac manifestations of myasthaenia gravis and myasthaenia gravis itself have overlapping symptoms; the importance of cardiac monitoring and clinical vigilance in such cases is discussed. The utility of cardiac MRI in assessing cardiac manifestations of myasthaenia gravis is also highlighted. 2015 BMJ Publishing Group Ltd.

  12. Reversible Stress Cardiomyopathy Presenting as Acute Coronary Syndrome with Elevated Troponin in the Absence of Regional Wall Motion Abnormalities: A Forme Fruste of Stress Cardiomyopathy?

    Directory of Open Access Journals (Sweden)

    Mahesh Anantha Narayanan

    2014-01-01

    Full Text Available We present a case of reversible stress cardiomyopathy in a surgical patient, described here as a forme fruste due to its atypical features. It is important to recognize such unusual presentation of stress cardiomyopathy that mimics acute coronary syndrome. Stress cardiomyopathy commonly presents as acute coronary syndrome and is characterized by typical or atypical variants of regional wall motion abnormalities. We report a 60-year-old Caucasian male with reversible stress cardiomyopathy following a sternal fracture fixation. Although the patient had several typical features of stress cardiomyopathy including physical stress, ST-segment elevation, elevated cardiac biomarkers and normal epicardial coronaries, there were few features that were atypical, including unusual age, gender, absence of regional wall motion abnormalities, high lateral ST elevation, and high troponin-ejection fraction product. In conclusion, this could represent a forme fruste of stress cardiomyopathy.

  13. Examining the Psychosocial Impact of Genetic Testing for Cardiomyopathies.

    Science.gov (United States)

    Wynn, Julia; Holland, David T; Duong, Jimmy; Ahimaz, Priyanka; Chung, Wendy K

    2017-12-15

    Inherited cardiomyopathies, including hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM), are the most common monogenic cause of cardiac disease and can rarely lead to sudden cardiac death (SCD). They are characterized by incomplete and age-dependent penetrance and are usually initially symptomatic in adulthood yet can present in childhood as well. Over 20 genes have been identified to cause HCM, and more than 40 genes are known to cause DCM. Genetic testing for these genes has been integrated into medical care; however, the psychological impact of genetic testing and the impact of the uncertainty that comes with receiving these results have not been well studied. This study surveyed 90 adult probands and relatives with a personal or family history of cardiomyopathy from a single hospital-based cardiac genetic program to determine the psychosocial impact of genetic testing for cardiomyopathies. Standardized psychological instruments including an adapted Multidimensional Impact of Cancer Risk Assessment (aMICRA), Impact of Event Scale (IES), and Satisfaction with Decision (SWD) scales were utilized. Patients with positive genetic test results had higher scores for intrusive thoughts, avoidance, and distress when compared to those with negative genetic test results and were also more likely to make or plan to make life changes because of the results of their genetic testing. Satisfaction with the decision to undergo genetic testing was similar regardless of genetic test results. The results of this study provide insight into the patient experience of genetic testing for cardiomyopathies and how these experiences are associated with genetic test results and cardiac history.

  14. Echocardiography and cardiac MRI in mutation-negative hypertrophic cardiomyopathy in an older patient: a case defining the need for ICD.

    Science.gov (United States)

    Rodriguez, Fatima; Degnan, Kathleen O; Seidman, Christine E; Mangion, Judy R

    2014-08-01

    We report the case of a 67-year-old man with hypertrophic cardiomyopathy who presented for a second opinion about implantable cardio-defibrillator (ICD) placement after a witnessed syncopal episode. Despite his older age, being mutation-negative, and having a maximal septal thickness of 2.2 cm on echocardiography, he demonstrated rapid progression of myocardial fibrosis on cardiac MRI, correlating to ventricular tachyarrhythmias and syncope. We review the role of echocardiography and cardiac MRI in optimizing medical care for such patients who may not otherwise meet criteria for an ICD placement or further interventions. © 2014, Wiley Periodicals, Inc.

  15. Cochlear Implants

    Science.gov (United States)

    ... For young children who are deaf or severely hard-of-hearing, using a cochlear implant while they are young exposes them to sounds during an optimal period to develop speech and language skills. Research has shown that when these children receive ...

  16. Inferior ST-Elevation Myocardial Infarction Associated with Takotsubo Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Oliver Koeth

    2010-01-01

    Full Text Available Takotsubo cardiomyopathy (TCM is usually characterized by transient left ventricular apical ballooning. Due to the clinical symptoms which include chest pain, electrocardiographic changes, and elevated myocardial markers, Takotsubo cardiomyopathy is frequently mimicking ST-elevation myocardial infarction in the absence of a significant coronary artery disease. Otherwise an acute occlusion of the left anterior descending coronary artery can produce a typical Takotsubo contraction pattern. ST-elevation myocardial infarction (STEMI is frequently associated with emotional stress, but to date no cases of STEMI triggering TCM have been reported. We describe a case of a female patient with inferior ST-elevation myocardial infarction complicated by TCM.

  17. [Congestive cardiomyopathy in addiction to clobenzorex, an anorexigenic drug].

    Science.gov (United States)

    Cornaert, P; Camblin, J; Graux, P; Anaye, B; Dutoit, A; Croccel, L

    1986-04-01

    Cardiac failure caused by high doses of amphetamine-like drugs is rare. We report a case of decompensated congestive cardiomyopathy occurring in a 29 year old woman addicted to clobenzorex (Dinintel). This patient had been taking 5 to 7 capsules per day for 5 years. No other cause of cardiac failure was detected. A rapid improvement was obtained by digitalis and diuretic therapy; no further episodes of cardiac failure were observed after one year. However, the drug could not be completely withdrawn and echocardiography has shown increasing left ventricular dilatation. The possible mechanisms of amphetamine induced myocardial toxicity are discussed and the analogy with the group of adrenergic cardiomyopathies is underlined.

  18. A Rare Occurance with Epidermolysis Bullosa Disease: Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Derya Cimen

    2014-02-01

    Full Text Available Epidermolysis bullosa is a congenital and herediter vesiculobullous disease. Dystrophic form of this disease is characterized by severe malnutrition, failure to thrive, adhesions at fingers, joint contractures related with the formation of scar tissues, carcinoma of the skin, anemia, hipoalbuminemia, wound enfections and sepsis. Rarely, mortal dilated cardiomyopathy may occur in patients. In this report we present a 13 year-old pediatric patient with dilated cardiomyopathy, clinically diagnosed with Epidermolysis bullosa as well as a review of recent related literature.

  19. Prognostic Implications of Defibrillation Threshold Testing in Patients With Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Francia, Pietro; Adduci, Carmen; Semprini, Lorenzo; Palano, Francesca; Santini, Daria; Musumeci, Beatrice; Santolamazza, Caterina; Volpe, Massimo; Autore, Camillo

    2017-01-01

    In hypertrophic cardiomyopathy (HCM) patients the need for defibrillation threshold (DFT) testing at the time of ICD implantation is debated. Moreover, its prognostic implications have never been explored. In a cohort of HCM patients we sought to (a) investigate factors prompting DFT testing, (b) evaluate ICD efficacy by testing DFT, (c) compare DFT in patients with and without massive LVH, and (d) assess whether DFT testing predicts shock efficacy for spontaneous VT/VF. We retrospectively analyzed a cohort of HCM patients implanted with an ICD. DFT was tested at the discretion of the implanting physician with a 10 J safety margin. During follow-up, ICD interventions were evaluated. The study population included 66 patients. DFT was determined in 25 (38%) patients. Age (HR: 0.95; 95%CI: 0.92-0.98; P = 0.004) and massive LVH (HR: 6.0; 95%CI: 2.03-18.8; P = 0.001) affected the decision to test DFT. DFT was at least 10 J less than maximal ICD output in 25/25. Safety margin was similar among patients with and without massive LVH (15 ± 3 J vs. 14 ± 2 J; P = 0.42). During follow-up (median 53 months) 15 shocks were delivered for 12 VT/VF in 7 patients. One VF ended spontaneously after a failed shock. Of 4 unsuccessful shocks, 2 occurred in 1 patient with DFT testing and 2 were delivered in 2 patients without. All unsuccessful shocks were ≤35 J. Young age and massive LVH prompt DFT testing. Contemporary ICDs are safe and effective in HCM patients independently from the magnitude of LVH. DFT testing does not predict shock efficacy for spontaneous VT/VF. © 2016 Wiley Periodicals, Inc.

  20. Intraventricular vortex properties in nonischemic dilated cardiomyopathy

    Science.gov (United States)

    Benito, Yolanda; Alhama, Marta; Yotti, Raquel; Martínez-Legazpi, Pablo; del Villar, Candelas Pérez; Pérez-David, Esther; González-Mansilla, Ana; Santa-Marta, Cristina; Barrio, Alicia; Fernández-Avilés, Francisco; del Álamo, Juan C.

    2014-01-01

    Vortices may have a role in optimizing the mechanical efficiency and blood mixing of the left ventricle (LV). We aimed to characterize the size, position, circulation, and kinetic energy (KE) of LV main vortex cores in patients with nonischemic dilated cardiomyopathy (NIDCM) and analyze their physiological correlates. We used digital processing of color-Doppler images to study flow evolution in 61 patients with NIDCM and 61 age-matched control subjects. Vortex features showed a characteristic biphasic temporal course during diastole. Because late filling contributed significantly to flow entrainment, vortex KE reached its maximum at the time of the peak A wave, storing 26 ± 20% of total KE delivered by inflow (range: 1–74%). Patients with NIDCM showed larger and stronger vortices than control subjects (circulation: 0.008 ± 0.007 vs. 0.006 ± 0.005 m2/s, respectively, P = 0.02; KE: 7 ± 8 vs. 5 ± 5 mJ/m, P = 0.04), even when corrected for LV size. This helped confining the filling jet in the dilated ventricle. The vortex Reynolds number was also higher in the NIDCM group. By multivariate analysis, vortex KE was related to the KE generated by inflow and to chamber short-axis diameter. In 21 patients studied head to head, Doppler measurements of circulation and KE closely correlated with phase-contract magnetic resonance values (intraclass correlation coefficient = 0.82 and 0.76, respectively). Thus, the biphasic nature of filling determines normal vortex physiology. Vortex formation is exaggerated in patients with NIDCM due to chamber remodeling, and enlarged vortices are helpful for ameliorating convective pressure losses and facilitating transport. These findings can be accurately studied using ultrasound. PMID:24414062

  1. Emotional stress triggers symptoms in hypertrophic cardiomyopathy: a survey of the Hypertrophic Cardiomyopathy Association.

    Science.gov (United States)

    Lampert, Rachel; Salberg, Lisa; Burg, Matthew

    2010-09-01

    Symptoms are among the most important factors impacting quality of life (QOL) in hypertrophic cardiomyopathy (HCM) patients, and reflect a poor prognosis. Whether emotional stress can trigger symptoms of chest pain, dyspnea, palpitations, and lightheadedness has not been described. Members of the Hypertrophic Cardiomyopathy Association (HCMA) received an electronic link via e-mail to an ongoing online survey, also accessed via links on the HCMA message-board and homepage. Between May 2007 and November 2008, there were 1,297 respondents. The survey queried demographic and self-reported clinical information, and types and triggers of symptoms. Respondents reported physical and emotional QOL on a 1-10 Likert scale. Symptoms reported included chest pain (49%), dyspnea (70%), palpitations (61%), and syncope/lightheadedness (59%). The most common symptom trigger was exertion, 64% describing symptoms while climbing stairs or hills. Forty-nine percent described experiencing symptoms during emotional stress. Those reporting chest pain were more likely to report emotion triggering (60%) than those reporting palpitations, syncope/lightheadedness, or dyspnea (50-54% each). Both physical and emotional QOL were significantly decreased in those describing emotion-triggered symptoms. Women were more likely than men to report symptoms overall, as well as emotion-triggered symptoms (50% vs 35%, P symptoms (79% vs 58%, P symptoms, both emotion- and exertion-triggered symptoms remained significantly more common in women. Triggering of symptoms by emotion is common in individuals with HCM. Further studies will determine pathways linking emotional stressors with chest pain, dyspnea, palpitations, and lightheadedness in these patients. ©2010, The Authors. Journal compilation ©2010 Wiley Periodicals, Inc.

  2. Application of radionuclide techniques in evaluation of dilated cardiomyopathy and ischemic cardiomyopathy

    International Nuclear Information System (INIS)

    Tian Yueqin; Liu Xiujie; Shi Rongfang

    2000-01-01

    Objective: To assess the clinical significance of radionuclide techniques in differentiating dilated cardiomyopathy (DCM) from ischemic cardiomyopathy (CAD-CM). Methods: 28 patients (pts) with DCM and 55 pts with CAD-CM were studied. All pts underwent 99 Tc m -MIBI myocardial perfusion SPECT and 18 F-FDG myocardial metabolic PET. 73 pts had 99 Tc m -RBC radionuclide ventriculography and 68 pts had coronary angiography. Results: 23 pts (82%) with DCM showed perfusion abnormalities with mild and not segmental distribution. 52 pts (95%) with CAD-CM showed perfusion abnormalities that distributed along the coronary vessel territories. Perfusion defects were found in 4 pts (14%) with DCM and 45 pts (82%) with CAD-CM (P<0.01). The average perfusion score was 4.5 +- 2.6 in DCM and 9.5 +- 2.9 in CAD-CM, the area of perfusion diminished uptake was significantly smaller in DCM than in CAD-CM (P < 0.001). 2 pts with DCM and 18 pts with CAD-CM had metabolic defect. The patterns of perfusion/metabolic imaging showed mismatch in most pts with CAD-CM but match in pts with DCM. The LVEF in pts with DCM and CAD-CM was decreased but no significant difference between DCM and CAD-CM was observed. The RVEF in pts with DCM was significantly lower than that in pts with CAD-CM (32.4% +- 13.9% vs 40.9% +- 15.4%, P < 0.05). Conclusions: The radionuclide techniques showed to be helpful for distinguishing DCM from CAD-CM. The discriminate analysis revealed that segmental perfusion abnormality and RVEF were the most important factors for differentiation of DCM from CAD-CM

  3. Short Implants: New Horizon in Implant Dentistry

    Science.gov (United States)

    Gulati, Manisha; Garg, Meenu; Pathak, Chetan

    2016-01-01

    The choice of implant length is an essential factor in deciding the survival rates of these implants and the overall success of the prosthesis. Placing an implant in the posterior part of the maxilla and mandible has always been very critical due to poor bone quality and quantity. Long implants can be placed in association with complex surgical procedures such as sinus lift and bone augmentation. These techniques are associated with higher cost, increased treatment time and greater morbidity. Hence, there is need for a less invasive treatment option in areas of poor bone quantity and quality. Data related to survival rates of short implants, their design and prosthetic considerations has been compiled and structured in this manuscript with emphasis on the indications, advantages of short implants and critical biomechanical factors to be taken into consideration when choosing to place them. Studies have shown that comparable success rates can be achieved with short implants as those with long implants by decreasing the lateral forces to the prosthesis, eliminating cantilevers, increasing implant surface area and improving implant to abutment connection. Short implants can be considered as an effective treatment alternative in resorbed ridges. Short implants can be considered as a viable treatment option in atrophic ridge cases in order to avoid complex surgical procedures required to place long implants. With improvement in the implant surface geometry and surface texture, there is an increase in the bone implant contact area which provides a good primary stability during osseo-integration. PMID:27790598

  4. Short Implants: New Horizon in Implant Dentistry.

    Science.gov (United States)

    Jain, Neha; Gulati, Manisha; Garg, Meenu; Pathak, Chetan

    2016-09-01

    The choice of implant length is an essential factor in deciding the survival rates of these implants and the overall success of the prosthesis. Placing an implant in the posterior part of the maxilla and mandible has always been very critical due to poor bone quality and quantity. Long implants can be placed in association with complex surgical procedures such as sinus lift and bone augmentation. These techniques are associated with higher cost, increased treatment time and greater morbidity. Hence, there is need for a less invasive treatment option in areas of poor bone quantity and quality. Data related to survival rates of short implants, their design and prosthetic considerations has been compiled and structured in this manuscript with emphasis on the indications, advantages of short implants and critical biomechanical factors to be taken into consideration when choosing to place them. Studies have shown that comparable success rates can be achieved with short implants as those with long implants by decreasing the lateral forces to the prosthesis, eliminating cantilevers, increasing implant surface area and improving implant to abutment connection. Short implants can be considered as an effective treatment alternative in resorbed ridges. Short implants can be considered as a viable treatment option in atrophic ridge cases in order to avoid complex surgical procedures required to place long implants. With improvement in the implant surface geometry and surface texture, there is an increase in the bone implant contact area which provides a good primary stability during osseo-integration.

  5. Penetrance of Hypertrophic Cardiomyopathy in Children Who Are Mutation Positive

    NARCIS (Netherlands)

    Vermeer, Alexa M. C.; Clur, Sally-Ann B.; Blom, Nico A.; Wilde, Arthur A. M.; Christiaans, Imke

    2017-01-01

    Objectives To investigate the presence of hypertrophic cardiomyopathy (HCM) at first cardiac evaluation and during follow-up and cardiac events in predictively tested children who are mutation positive. Study design The study included 119 predictively tested children who were mutation positive, with

  6. The KCNE genes in hypertrophic cardiomyopathy: a candidate gene study

    DEFF Research Database (Denmark)

    Hedley, Paula L; Haundrup, Ole; Andersen, Paal S

    2011-01-01

    as well as the T-tubules of the sarcolemma. It has been suggested that minK forms part of an "electro-mechanical feed-back" which links cardiomyocyte stretching to changes in ion channel function. We examined whether mutations in KCNE genes were associated with hypertrophic cardiomyopathy (HCM), a genetic...

  7. Cirrhotic cardiomyopathy: is there any correlation between the stage ...

    African Journals Online (AJOL)

    Rania Hammami

    2017-02-28

    Feb 28, 2017 ... To cite this article: Rania Hammami, Mouna Boudabbous, Jihen Jdidi, Fatma Trabelsi, Fakher. Mroua, Rahma Kallel, Ali Amouri, Dorra Abid, Nabil Tahri, Leila Abid & Samir Kammoun. (2017) Cirrhotic cardiomyopathy: is there any correlation between the stage of cardiac impairment and the severity of liver ...

  8. Hypertrophic Cardiomyopathy in a Middle Aged Man - A Case Report

    African Journals Online (AJOL)

    Background: Hypertrophic cardiomyopathy (HCM) is a disease of the myocardium with autosomal dominant pattern of inheritance. It is characterized by inappropriate hypertrophy involving either the interventricular septum, apex or left ventricular free wall in isolation or combined. The disease is often asymptomatic and may ...

  9. Hypertrophic cardiomyopathy in South African Blacks | Lewis | South ...

    African Journals Online (AJOL)

    Hypertrophic cardiomyopathy (HCM) has been considered rare among the Black population of southern Africa. We report 7 patients with the disease who presented during a 14-month period. Current concepts in the approach to the diagnosis and treatment of HCM are discussed. It is possible that with greater awareness of ...

  10. Prognosis in hypertrophic cardiomyopathy observed in a large clinic population

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel); D.J. Waldstein; J. Vos (Jeroen); F.J. ten Cate (Folkert)

    1993-01-01

    textabstractOverall annual cardiac mortality in hypertrophic cardiomyopathy (HC) has been reported to be between 2 and 4%, although these numbers are primarily from retrospective studies of patients referred to large research institutions. A clinic population of 113 patients with HC was

  11. Peripheral vascular structure and function in hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Rowley, N.J.; Green, D.J.; George, K.; Thijssen, D.H.J.; Oxborough, D.; Sharma, S.; Somauroo, J.D.; Jones, J.; Sheikh, N.; Whyte, G.

    2012-01-01

    BACKGROUND: Hypertrophic cardiomyopathy (HCM) is characterised by idiopathic cardiac enlargement and represents the most frequent cause of sudden cardiac death in athletes under the age of 35 years. Differentiation between physiological (ie, exercise-related) and pathological (ie, HCM-related)

  12. Cardiogenic shock in Takotsubo cardiomyopathy: a focus on management.

    Science.gov (United States)

    Doucet, Katie M; Labinaz, Marino X; Beauchesne, Luc M; Burwash, Ian G

    2015-01-01

    Takotsubo cardiomyopathy has become a well recognized mimicker of acute coronary syndrome. Patients generally do well, although a minority can develop life-threatening complications. We present a case of 1 such patient in a branched self-assessment format designed to challenge the reader's clinical management skills. Copyright © 2015 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  13. CARDIOMYOPATHIE DU POSTPARTUM: A propos de cinq cas au ...

    African Journals Online (AJOL)

    The main presenting symptom was congestive heart failure with acute pulmonary edema. In all cases, the chest x-ray showed ... The treatment administered to these patients included bed rest, salt restriction, digitalics, ... The main issues concerning the management of postpartum cardiomyopathy are raised in this article.

  14. Atrioventricular conduction after alcohol septal ablation for obstructive hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Axelsson, Anna; Weibring, Kristina; Havndrup, Ole

    2014-01-01

    AIMS: Lesion of the atrioventricular conduction system is a well known adverse effect of alcohol septal ablation (ASA) in patients with obstructive hypertrophic cardiomyopathy (HCM). We assessed the atrioventricular conduction at long-term follow-up after ASA. METHODS: In patients with a pacemaker...

  15. Symmetric Dimethylarginine in Cats with Hypertrophic Cardiomyopathy and Diabetes Mellitus

    DEFF Research Database (Denmark)

    Langhorn, R.; Kieler, I. N.; Koch, J.

    2018-01-01

    Background: Symmetric dimethylarginine (SDMA) has been increasingly used as a marker of early chronic kidney disease (CKD) in cats, but little is known about the influence of comorbidities on SDMA in this species. Hypothesis: Hypertrophic cardiomyopathy (HCM) and diabetes mellitus (DM), independe...... controls, a finding that needs further investigation and should be kept in mind when evaluating renal function of cats with this endocrinopathy....

  16. Thallium scintigraphy for the prognosis of idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Yabe, Toshikazu; Furuno, Takashi; Kitaoka, Hiroaki; Matsumura, Yoshihisa; Yamasaki, Naohito; Doi, Yoshinori

    2002-01-01

    This study evaluated the significance of perfusion defects demonstrated by thallium-201 and age in the prognosis of patients with idiopathic dilated cardiomyopathy. Seventy-four dilated cardiomyopathy patients underwent thallium scintigraphy as well as clinical and hemodynamic examination. Abnormal perfusion defects were present in 23 of 38 patients aged <60 years (61%) and in 26 of 36 elderly patients aged ≥60 years (72%; NS). Univariate analysis showed that such perfusion defects were a significant predictor of cardiac death only in patients aged <60 years (p=0.015). Stepwise discriminant analysis also revealed that perfusion defects were a significant predictor in patients aged <60 years (Wilks' lambda 0.499, chi-square test 20.2, p=0.003). Perfusion defects were not more important than the history of syncope or stroke in elderly dilated cardiomyopathy patients. Twenty-one patients died of disease-related causes during 58±43 months. The five-year survival rate was better in patients aged <60 years without than in those with perfusion defects (100% vs 58.4%, respectively), but not affected in patients aged ≥60 years (66.7% vs 62.2%). Thallium scintigraphy is valuable for the prognosis of patients with dilated cardiomyopathy aged <60 years who are usually candidates for heart transplantation. Absence of thallium perfusion defects may indicate good long-term prognosis. (author)

  17. Private mitochondrial DNA variants in danish patients with hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Hagen, Christian M; Aidt, Frederik H; Havndrup, Ole

    2015-01-01

    Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disease primarily caused by mutations in genes coding for sarcomeric proteins. A molecular-genetic etiology can be established in ~60% of cases. Evolutionarily conserved mitochondrial DNA (mtDNA) haplogroups are susceptibility factors for HCM...

  18. Penetrance of Hypertrophic Cardiomyopathy in Children and Adolescents

    DEFF Research Database (Denmark)

    Jensen, Morten K; Havndrup, Ole; Christiansen, Michael

    2013-01-01

    The penetrance of hypertrophic cardiomyopathy (HCM) during childhood and adolescence has been only sparsely described. We studied the penetrance of HCM and the short- and long-term outcomes of clinical screening and predictive genetic testing of child relatives of patients with HCM....

  19. Cardiac symptoms before sudden cardiac death caused by hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Lynge, Thomas Hadberg; Risgaard, Bjarke; Jabbari, Reza

    2016-01-01

    AIMS: Hypertrophic cardiomyopathy (HCM) is a frequent cause of sudden cardiac death (SCD) among the young (SCDY). The aim of this study was to characterize symptoms before SCDY due to HCM. METHODS AND RESULTS: Through review of all death certificates, we identified all SCDs in Danes aged 1-35 years...

  20. Stress Cardiomyopathy in the Setting of COPD Exacerbation

    Directory of Open Access Journals (Sweden)

    Kevin Landefeld

    2015-10-01

    Full Text Available Introduction. Stress cardiomyopathy, or takotsubo cardiomyopathy, is an acute, reversible left ventricular dysfunction usually initiated by a psychological or physical stress. We report this case of stress cardiomyopathy following a chronic obstructive pulmonary disease exacerbation and the subsequent treatment. Case Description. A 49-year-old white female with a history of chronic obstructive pulmonary disease presented to the emergency room via emergency medical services with worsening severe shortness of breath and productive cough for 2 weeks but denied any chest pain on arrival. On presentation, she was noted to be tachypneic, using her accessory muscles and with bilateral coarse expiratory wheezing on lung auscultation. Initial electrocardiogram demonstrated sinus tachycardia. She was treated with multiple albuterol treatments. Soon afterwards, the course was complicated by hypoxic respiratory failure eventually requiring intubation. Her repeat electrocardiogram showed acute changes consistent with myocardial infarction, and an echocardiograph demonstrated apical akinesia with an ejection fraction of 25% to 30%. The patient was urgently taken for cardiac catheterization, which showed no angiographic evidence of coronary artery disease. Three days after initial presentation, a repeat transthoracic echocardiogram showed overall left ventricular systolic function improvement. Discussion. This case provided a unique look at the difficulty of balancing catecholamines in a patient with bronchospasm and stress cardiomyopathy.

  1. Atrioventricular conduction after alcohol septal ablation for obstructive hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Axelsson, Anna; Weibring, Kristina; Havndrup, Ole

    2014-01-01

    AIMS: Lesion of the atrioventricular conduction system is a well known adverse effect of alcohol septal ablation (ASA) in patients with obstructive hypertrophic cardiomyopathy (HCM). We assessed the atrioventricular conduction at long-term follow-up after ASA. METHODS: In patients with a pacemake...

  2. ALCOHOLIC CARDIOMYOPATHY: BASIC ASPECTS OF EPIDEMIOLOGY, PATHOGENESIS AND PHARMACOTHERAPY

    Directory of Open Access Journals (Sweden)

    A. O. Yusupova

    2014-01-01

    Full Text Available Data on the epidemiology and pathogenesis of alcoholic cardiomyopathy (ACMP are presented. Special aspects of clinical manifestations as well as management of these patients are considered. Psychotropic therapy aimed at reduction in motivation to alcohol consumption in patients with ACMP is discussed separately.

  3. Early molecular events in the development of the diabetic cardiomyopathy.

    NARCIS (Netherlands)

    Monkemann, H.; Vriese, A.S. de; Blom, H.J.; Kluijtmans, L.A.J.; Heil, S.G.; Schild, H.H.; Golubnitschaja, O.

    2002-01-01

    Oxidative damage to DNA has been well documented in cardiac cells isolated from diabetic patients and rats with streptozotocin-induced diabetes mellitus (DM). This study evaluates possible molecular mechanisms for early events in the development of DM-induced cardiomyopathy. Methods: To analyze the

  4. Takotsubo cardiomyopathy: A known unknown foe of asthma.

    Science.gov (United States)

    Kotsiou, Ourania S; Douras, Alexandros; Makris, Demosthenes; Mpaka, Nikoleta; Gourgoulianis, Konstantinos I

    2017-10-01

    Patients with uncontrolled asthma are at a greater risk of asthma attacks requiring emergency room visits or hospital admissions. Takotsubo cardiomyopathy is potentially a significant complication in a course of status asthmaticus. We describe a 43-year-old female patient who presented with status asthmaticus that was further complicated with takotsubo cardiomyopathy. Recognizing apical ballooning syndrome is challenging in patients with a history of respiratory disease because the symptoms of the last entity may complicate the diagnostic approach. It is difficult to distinguish clinically apical ballooning syndrome from the acute airway exacerbation itself. Both asthma and takotsubo cardiomyopathy share the same clinical presentation with dyspnea and chest tightness. In our patient, the electrocardiographic abnormalities, the rapidly reversible distinctive characteristics of echocardiography, and the modest elevation of serum cardiac biomarkers levels, in combination with the presence of a stress trigger (severe asthma attack), strongly supported the diagnosis of broken heart syndrome. Clinicians should re-evaluate asthma management and be aware of the complications associated with asthma attacks such as stress-induced cardiomyopathy.

  5. [Plasma selenium and peripartum cardiomyopathy in Bamako, Mali].

    Science.gov (United States)

    Cénac, A; Touré, K; Diarra, M B; Sergeant, C; Jobic, Y; Sanogo, K; Dembele, M; Fayol, V; Simonoff, M

    2004-01-01

    Peripartum heart failure due to unexplained dilated cardiomyopathy is a common disorder as Savannak-Sahelian Africa. One of the many suspected risk factors identified is selenium deficiency. The purpose of this study was to measure plasma selenium levels in patients with peripartum heart failure due to cardiomyopathy in Bamako, Republic of Mali and compare data with healthy Sahalian women with the same obstetrical status. Plasma selenium was measured in a patient group consisting of 28 Malian women presenting peripartum heart failure and in a control group of 28 healthy breast-feeding Nigerien women of comparable age. The criteria for matching the two groups was parity (similar number of deliveries) since multiparity is a risk factor for peripartum cardiomyopathy. The Wilcoxon test (nonparametric) was used to compare the 2 groups considering up value < 0.05 as significant. Plasma selenium was significantly lower in patients from Mali than in controls from Niger (65 +/- 17 ng/ml vs. 78 +/- 17 ng/ml, p = 0.01). The results of this study showing lower plasma selenium in Bamako patients with peripartum cardiomyopathy than in a matching healthy control population confirms the previous data from the Niamey study.

  6. Potential role of vitamin D deficiency on Fabry cardiomyopathy

    NARCIS (Netherlands)

    Drechsler, C.; Schmiedeke, B.; Niemann, M.; Schmiedeke, D.; Kramer, J.; Turkin, I.; Blouin, K.; Emmert, A.; Pilz, S.; Obermayer-Pietsch, B.; Weidemann, F.; Breunig, F.; Wanner, C.

    2014-01-01

    Patients with Fabry disease frequently develop left ventricular (LV) hypertrophy and renal fibrosis. Due to heat intolerance and an inability to sweat, patients tend to avoid exposure to sunlight. We hypothesized that subsequent vitamin D deficiency may contribute to Fabry cardiomyopathy. This study

  7. Arrhythmogenic right ventricular cardiomyopathy in a patient with schizophrenia

    OpenAIRE

    Kawasaki, Kenta; Miyaji, Kotaro; Kodera, Satoshi; Suzuki, Yoshio; Kanda, Junji; Ikeda, Masayuki

    2015-01-01

    Key Clinical Message People with schizophrenia are at greater risk of cardiovascular morbidity and mortality than the general population. Arrhythmogenic right ventricular cardiomyopathy is a recognized cause of sudden cardiac death in young people. This report discusses the necessity for close cardiac evaluation to reduce incidence of sudden death in people with schizophrenia.

  8. Psychological Features of Takotsubo Cardiomyopathy: Report of Four Cases.

    Science.gov (United States)

    Jenab, Yaser; Hashemi, Seyedeh Roghaieh; Ghaffari-Marandi, Neda; Zafarghandi, Hoda; Shahmansouri, Nazila

    2017-04-01

    Takotsubo or stress-induced cardiomyopathy is a cardiomyopathy in which the patient has a sudden onset, reversible left ventricular systolic dysfunction without any significant coronary artery disease. Four women, who were at a mean age of 64 years and suffered from chest pain exacerbated by emotional stress, were admitted as cases of acute coronary syndrome and were completely evaluated through precise history taking, physical examination, and ECG. Coronary angiography or coronary multidetector computed tomography was used to exclude significant coronary artery disease. In these patients with confirmed Takotsubo cardiomyopathy, in addition to the Diagnostic and Statistical Manual of the American Psychiatric Association (DSM-IV) criteria, a 71-item form of the Minnesota Multiphasic Personality Inventory (MMPI)-Mini-Mult-was employed for psychological assessment. The main common elevated scale was hypochondriasis. Individuals with high scores on this scale are obsessed with themselves, especially in regard to their body, and often use their disease symptoms in order to manipulate others. They are mainly passive aggressive, critical, and demanding, which stems from their lack of effective verbal abilities as a means of communication, specifically when it comes to anger or hostility expression. To the best of our knowledge, there is no available study evaluating patients with Takotsubo cardiomyopathy using the Mini-Mult questionnaire for psychological assessment.

  9. Dystrophic Cardiomyopathy: Complex Pathobiological Processes to Generate Clinical Phenotype

    Directory of Open Access Journals (Sweden)

    Takeshi Tsuda

    2017-09-01

    Full Text Available Duchenne muscular dystrophy (DMD, Becker muscular dystrophy (BMD, and X-linked dilated cardiomyopathy (XL-DCM consist of a unique clinical entity, the dystrophinopathies, which are due to variable mutations in the dystrophin gene. Dilated cardiomyopathy (DCM is a common complication of dystrophinopathies, but the onset, progression, and severity of heart disease differ among these subgroups. Extensive molecular genetic studies have been conducted to assess genotype-phenotype correlation in DMD, BMD, and XL-DCM to understand the underlying mechanisms of these diseases, but the results are not always conclusive, suggesting the involvement of complex multi-layers of pathological processes that generate the final clinical phenotype. Dystrophin protein is a part of dystrophin-glycoprotein complex (DGC that is localized in skeletal muscles, myocardium, smooth muscles, and neuronal tissues. Diversity of cardiac phenotype in dystrophinopathies suggests multiple layers of pathogenetic mechanisms in forming dystrophic cardiomyopathy. In this review article, we review the complex molecular interactions involving the pathogenesis of dystrophic cardiomyopathy, including primary gene mutations and loss of structural integrity, secondary cellular responses, and certain epigenetic and other factors that modulate gene expressions. Involvement of epigenetic gene regulation appears to lead to specific cardiac phenotypes in dystrophic hearts.

  10. Molecular genetics of dilated cardiomyopathy in the Dobermann dog

    NARCIS (Netherlands)

    Stabej, Polona

    2005-01-01

    Canine dilated cardiomyopathy (DCM) is a disease of the myocardium associated with dilatation and impaired contraction of the ventricles. It primarily affects large and giant breed dogs with Dobermanns being one of the most frequently affected. The high prevalence of DCM in specific breeds suggests

  11. Stress-induced cardiomyopathy (Takotsubo – broken heart and mind?

    Directory of Open Access Journals (Sweden)

    Redfors B

    2013-04-01

    Full Text Available Björn Redfors, Yangzhen Shao, Elmir Omerovic Department of Molecular and Clinical Medicine, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden Abstract: Stress-induced cardiomyopathy (SIC, also known as Takotsubo cardiomyopathy, is characterized by severe but potentially reversible regional left ventricular wall motion abnormalities, ie, akinesia, in the absence of explanatory angiographic evidence of a coronary occlusion. The typical pattern is that of an akinetic apex with preserved contractions in the base, but other variants are also common, including basal or midmyocardial akinesia with preserved apical function. The pathophysiology of SIC remains largely unknown but catecholamines are believed to play a pivotal role. The diverse array of triggering events that have been linked to SIC are arbitrarily categorized as either emotional or somatic stressors. These categories can be considered as different elements of a continuous spectrum, linked through the interface of neurology and psychiatry. This paper reviews our current knowledge of SIC, with focus on the intimate relationship between the brain and the heart. Keywords: stress-induced cardiomyopathy, takotsubo cardiomyopathy, catecholamine, cerebral injury, emotional stress, somatic stress

  12. Hypertrophic cardiomyopathy: from mutation to functional analysis of defective protein

    Czech Academy of Sciences Publication Activity Database

    Čapek, P.; Vondrášek, Jiří; Škvor, J.; Brdička, R.

    2011-01-01

    Roč. 52, č. 3 (2011), s. 384-391 ISSN 0353-9504 Grant - others:GA MŠk(CZ) LN00B107 Program:LN Institutional research plan: CEZ:AV0Z40550506 Keywords : myosin heavy chain * homology modeling * molecular simulation * inherited cardiomyopathies Subject RIV: CE - Biochemistry Impact factor: 1.796, year: 2011

  13. Thallium scintigraphy for the prognosis of idiopathic dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Yabe, Toshikazu; Furuno, Takashi; Kitaoka, Hiroaki; Matsumura, Yoshihisa; Yamasaki, Naohito; Doi, Yoshinori [Kochi Medical School, Nankoku (Japan)

    2002-11-01

    This study evaluated the significance of perfusion defects demonstrated by thallium-201 and age in the prognosis of patients with idiopathic dilated cardiomyopathy. Seventy-four dilated cardiomyopathy patients underwent thallium scintigraphy as well as clinical and hemodynamic examination. Abnormal perfusion defects were present in 23 of 38 patients aged <60 years (61%) and in 26 of 36 elderly patients aged {>=}60 years (72%; NS). Univariate analysis showed that such perfusion defects were a significant predictor of cardiac death only in patients aged <60 years (p=0.015). Stepwise discriminant analysis also revealed that perfusion defects were a significant predictor in patients aged <60 years (Wilks' lambda 0.499, chi-square test 20.2, p=0.003). Perfusion defects were not more important than the history of syncope or stroke in elderly dilated cardiomyopathy patients. Twenty-one patients died of disease-related causes during 58{+-}43 months. The five-year survival rate was better in patients aged <60 years without than in those with perfusion defects (100% vs 58.4%, respectively), but not affected in patients aged {>=}60 years (66.7% vs 62.2%). Thallium scintigraphy is valuable for the prognosis of patients with dilated cardiomyopathy aged <60 years who are usually candidates for heart transplantation. Absence of thallium perfusion defects may indicate good long-term prognosis. (author)

  14. Oxidative Stress in Dilated Cardiomyopathy Caused by MYBPC3 Mutation

    NARCIS (Netherlands)

    T.L. Lynch (Thomas L.); M. Sivaguru (Mayandi); M. Velayutham (Murugesan); A.J. Cardounel (Arturo J.); M. Michels (Michelle); D. Barefield (David); S. Govindan (Suresh); C.D. Remedios (Cristobal Dos); J. van der Velden (Jolanda); S. Sadayappan (Sakthivel)

    2015-01-01

    textabstractCardiomyopathies can result from mutations in genes encoding sarcomere proteins including MYBPC3, which encodes cardiac myosin binding protein-C (cMyBP-C). However, whether oxidative stress is augmented due to contractile dysfunction and cardiomyocyte damage in MYBPC3-mutated

  15. Pheochromocytoma presenting as takotsubo-like cardiomyopathy following delivery.

    Science.gov (United States)

    Jóźwik-Plebanek, Katarzyna; Pęczkowska, Mariola; Klisiewicz, Anna; Wrzesiński, Kazimierz; Prejbisz, Aleksander; Niewada, Maciej; Kabat, Marek; Szperl, Małgorzata; Eisenhofer, Graeme; Lenders, Jacques W; Januszewicz, Andrzej

    2014-12-01

    Diagnosis of pheochromocytoma during pregnancy can be difficult, and the tumor carries an unfavorable prognosis if not diagnosed and treated in a timely manner. To present a case of Takotsubo-like cardiomyopathy characterized by transient left ventricular apical ballooning due to pheochromocytoma following delivery. A few hours after Caesarean section, a 32-year-old Caucasian female presented with pulmonary edema followed by cardiac arrest with echocardiographic and ventriculographic evidence of reversible acute myocardial failure characteristic of Takotsubo-like cardiomyopathy. A previously unrecognized adrenal pheochromocytoma was found during her clinical work-up. Left ventricle (LV) function normalized after surgical removal of the tumor, which was carried out after implementing an alpha-adrenoreceptor blockade. Hemorrhagic necrosis of the pheochromocytoma was seen on histopathologic analysis; this may have triggered the sequence of events leading to the development of Takotsubo-like cardiomyopathy and hemodynamic collapse. To the best of our knowledge, this is the first reported case of Takotsubo-like cardiomyopathy related to pheochromocytoma following delivery. This emphasizes the increased cardiovascular risk if pheochromocytoma is not diagnosed and treated in a timely manner, especially during pregnancy.

  16. Recurrence of PVCs in patients with PVC-induced cardiomyopathy.

    Science.gov (United States)

    Baser, Kazim; Bas, Hatice Duygu; LaBounty, Troy; Yokokawa, Miki; Good, Eric; Latchamsetty, Rakesh; Morady, Fred; Bogun, Frank

    2015-07-01

    The natural history of premature ventricular complex (PVC)-induced cardiomyopathy is incompletely understood. The purpose of this study was to assess long term follow-up data in patients who underwent successful PVC ablation for PVC-induced cardiomyopathy. The subjects of this study were 60 patients (17 women; mean age 52.5 ± 16.8 years; ejection fraction [EF] 37.3 ± 8.5%, median 40%, interquartile range [IQR] 15) with PVC-induced cardiomyopathy who underwent successful ablation of their predominant PVCs between 2005 and 2012. Patients were followed up for a mean of 23.6 ± 17.2 months. EF improved to 57.2 ± 4.7% (median 55%, IQR 5; P = .0001) within 9.6 ± 8.4 months of the ablation procedure. During follow-up, 10 of 60 patients (16.7%) had recurrent frequent PVCs and 50 patients (83.3%) did not. Patients underwent repeat assessment of EF and PVC burden. During follow-up of 23.6 ± 17.2 months, 10 patients had recurrent frequent PVCs, with an increase of their PVC burden from 1.4 ± 0.9% (median 1.05%, IQR 1.59) after the initial ablation to 27.2 ± 8.8% (median 26.0%, IQR 18.2; P = .018). Their EF decreased from 55.7 ± 3.4% (median 55%, IQR 5.8) after the initial ablation to 40.2 ± 5.1% (median 40%, IQR 15; P = .005). In the remaining patients with PVC-induced cardiomyopathy, EF and PVC burden remained unchanged during follow-up. Patients with PVC recurrence had a higher number of pleomorphic PVC morphologies during initial presentation (4.7 ± 2.2 vs 2.5 ± 2.8, P = .002). Recurrence of frequent PVCs in patients with a history of PVC cardiomyopathy can result in recurrence of cardiomyopathy. Follow-up in patients with PVC-induced cardiomyopathy is important, especially if patients were asymptomatic from the PVCs and have pleomorphic PVCs. Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  17. Five-week use of a monopivot centrifugal blood pump as a right ventricular assist device in severe dilated cardiomyopathy.

    Science.gov (United States)

    Inoue, Takamichi; Kitamura, Tadashi; Torii, Shinzo; Hanayama, Naoji; Oka, Norihiko; Itatani, Keiichi; Tomoyasu, Takahiro; Irisawa, Yusuke; Shibata, Miyuki; Hayashi, Hidenori; Ono, Minoru; Miyaji, Kagami

    2014-03-01

    Right heart failure is a critical complication in patients requiring mechanical ventricular support. However, it is often difficult to provide adequate right ventricular support in the acute phase. A 41-year-old woman diagnosed with dilated cardiomyopathy with severe right heart failure underwent implantation of a paracorporeal pulsatile left ventricular assist device (LVAD, Nipro Corporation, Tokyo, Japan) and a MERA monopivot centrifugal pump (Senko Medical Instrument Manufacturing Co., Ltd., Tokyo, Japan) as a right ventricular assist device (RVAD). The patient developed ischemic enteritis 3 weeks after surgery, necessitating fasting and reversal of anticoagulation therapy. A target international normalized ratio of 1.5 was selected, and aspirin administration was discontinued. Following recovery without thromboembolic events, the patient failed the RVAD discontinuation test. Five weeks after surgery, the monopivot centrifugal pump was exchanged for a pulsatile pump. No thrombus was evident on the centrifugal pump. The patient was undergoing cardiac rehabilitation at the time of this writing and awaiting heart transplantation.

  18. Clinical Features and Echocardiographic Findings in Children with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Cristina Blesneac

    2013-12-01

    Full Text Available Background: Hypertrophic cardiomyopathy, one of the most common inherited cardiomyopathies, is a heterogeneous disease resulting from sarcomeric protein mutations, with an incidence in the adult population of 1:500. Current information on the epidemiology and outcomes of this disease in children is limited. Methods: Thirty-four children diagnosed with hypertrophic cardiomyopathy in the Pediatric Cardiology Department from Tîrgu Mureș were evaluated concerning familial and personal history, clinical, paraclinical and therapeutic aspects. Hypertrophic cardiomyopathy was defined by the presence of a hypertrophied, non-dilated ventricle, in the absence of a cardiac or systemic disease that could produce ventricular hypertrophy. Results: The youngest diagnosed child was a neonate, a total of 10 patients being diagnosed until 1 year of age. In 6 cases a positive familial history was found. Noonan syndrome was found in 2 cases. Only 21 patients were symptomatic, the predominant symptoms being shortness of breath on exertion with exercise limitations. Left ventricular outflow tract obstruction was present in 21 cases (61.7%. Twenty-four patients were on β-blocking therapy, while 4 patients underwent septal myectomy. Conclusions: Hypertrophic cardiomyopathy is a heterogeneous disorder in terms of evolution, age of onset, type and extent of hypertrophy, and the risk of sudden death. It can affect children of any age. There is a need for a complex evaluation, including familial and personal anamnesis, clinical examination, electrocardiogram and echocardiography of all patients. It is highly important to develop screening strategies, including genetic testing, for an early diagnosis, especially in asymptomatic patients with a positive familial background

  19. Left ventricular function in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Takahashi, Hiromi; Yamaguchi, Ryutaro; Ifuku, Masayasu

    1985-01-01

    The present study was to investigate of left ventricular (LV) function during exercise in 26 patients with hypertrophic cardiomyopathy(HCM) usingTc-99m equilibrium angiocardiography, and to elucidate the mechanism of impaired functional reserve during exercise. In patients with HCM, LV ejection fraction decreased from 65 ± 8 (mean ± SD) % at rest to 59 ± 18 % at peak exercise, in contrast to an increase among controls (from 56 ± 9 % to 64 ± 9 %). As compared with resting values, cardiac output increased to 168 ± 24 % at peak exercise in HCM, but the increase was significantly less than that in controls (215 ± 47 %). Stroke volume decreased gradually to 83 ± 16 % during exercise in HCM, while it increased to 114 ± 10 % at an exercise level of half intensity, and it decreased slightly to 106 ± 16 % at peak exercise. LV end-systolic volume decreased among controls to 78 ± 27 % at peak exercise, but remained unchanged in HCM (118 ± 58 %). An increase in peak ejection rate at peak exercise was less in HCM than in controls (143 ± 26 % vs 170 ± 42 %). No significant differences were observed between the two groups concerning changes in indices of LV diastolic function including LV end-diastolic volume, peak filling rate or 1/3 filling rate during exercise. In the analysis of LV function curves, pulmonary arterial diastolic pressure increased to a greater extent in HCM than in controls (19 ± 6 mmHg vs 11 ± 6 mmHg); whereas, an increase in the stroke work index was less in HCM (80 ± 26 g.m/m 2 /beat vs 121 ± 21 g.m/m 2 /beat) at peak exercise. Thus, the LV function curve shifted downward and to the right in patients with HCM. The above findings indicate that LV functional reserve during exercise is impaired, especially as to systolic function in patients with HCM, while deterioration of diastolic function may be partly compromised by elevated filling pressure. (J.P.N.)

  20. Takotsubo cardiomyopathy in amiodarone-induced hyperthyroidism.

    Science.gov (United States)

    Capel, Ismael; Tasa-Vinyals, Elisabet; Cano-Palomares, Albert; Bergés-Raso, Irene; Albert, Lara; Rigla, Mercedes; Caixàs, Assumpta

    2017-01-01

    Takotsubo cardiomyopathy (TC) is an atypical, severe but reversible form of acute heart insufficiency. It typically presents with left ventricular failure, transient apical and mid-segments hypokinesis, absence of significant coronary stenosis and new electrographic abnormalities and/or elevation in serum cardiac enzymes. Although TC ('broken heart syndrome') has classically been associated with emotional trauma, evidence suggests that other precipitants might exist, including iatrogenic and thyroid-mediated forms. Thyroid disease is a relatively common comorbidity in TC patients. We report a case of TC in a postmenopausal female with no history of emotional trauma or other potential precipitant factors who was diagnosed with amiodarone-induced hyperthyroidism during her hospital stay. Though some case reports of thyroid-related TC exist, we are not aware of any other reported case of TC precipitated by amiodarone-induced hyperthyroidism. TC is a relatively new, rare, transient, severe, but reversible cardiovascular condition that is characterized by an acute left ventricular cardiac failure, which can clinically, analytically and electrocardiographically mimic an acute myocardial infarction.Many precipitant factors have been described in TC, being the most classical and emotional trauma. However, thyroid dysfunction is also a significant condition frequently found in patients with TC.A hypercatecholaminergic state leading to cardiomyocyte damage has been established as the main fact of TC physiopathology. Hyperthyroidism induces an upregulation of β-adrenergic receptors.Both hyperthyroidism and hypothyroidism have been related with TC development. Most reported cases of TC involving thyroid dysfunction correspond to hyperthyroidism due to Graves-Basedow disease, but there are also descriptions with severe hypothyroidism, radioiodine treatment or thyroid surgery.Amiodarone is a class III antiarrhythmic agent widely used, and it is a well-known cause of thyroid

  1. Comparison of Benefits from Cardiac Resynchronization Therapy between Patients with Ischemic Cardiomyopathy and Patients with Idiopathic Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Talia Alenabi

    2009-06-01

    Full Text Available Background: Cardiac resynchronization therapy (CRT is an effective treatment for patients with moderate to severe heart failure. However, 20-30% of patients remain non-responders to CRT. We sought to identify which patients benefit the most from CRT in regard to the etiology of heart failure. Methods: Eighty-three consecutive patients (62 men who had a biventricular pacemaker inserted at Tehran Heart Center between May 2004 and March 2007 were evaluated retrospectively. The inclusion criteria were comprised of New York Heart Association (NYHA class III or IV, left ventricular ejection fraction120ms. After 6 months, response was defined as being alive, no hospitalization for cardiac decompensation, and an improvement in NYHA class>1 grade. Results: After 6 months, 60 patients out of the 83 patients were responders. Amongst the 83 patients, 48 had ischemic cardiomyopathy and 35 had non-ischemic cardiomyopathy. A cross-tabulation of response versus etiology showed no significant difference between ischemic versus non-ischemic cardiomyopathy with regard to response to CRT (P=0.322. Conclusion: According to our study, there was no difference in response to CRT between ischemic versus non-ischemic cardiomyopathy at six months’ follow-up.

  2. Implantation, recoil implantation, and sputtering

    International Nuclear Information System (INIS)

    Kelly, R.

    1984-01-01

    Underlying ion-beam modification of surfaces is the more basic subject of particle-surface interaction. The ideas can be grouped into forward and backward features, i.e. those affecting the interior of the target and those leading to particle expulsion. Forward effects include the stopping of the incident particles and the deposition of energy, both governed by integral equations which are easily set up but difficult to solve. Closely related is recoil implantation where emphasis is placed not on the stopping of the incident particles but on their interaction with target atoms with resulting implantation of these atoms. Backward effects, all of which are denoted as sputtering, are in general either of collisional, thermal, electronic, or exfoliational origin. (Auth.)

  3. Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies

    Energy Technology Data Exchange (ETDEWEB)

    Grothoff, Matthias; Lehmkuhl, Lukas; Gutberlet, Matthias [University of Leipzig - Heart Center, Department of Diagnostic and Interventional Radiology, Leipzig (Germany); Pachowsky, Milena [Klinik fuer Strahlenheilkunde, Charite, Campus Virchow-Klinikum, Berlin (Germany); Hoffmann, Janine [University of Leipzig, Department of Obstetrics, Leipzig (Germany); Posch, Maximilian [Department of Cardiothoracic Surgery, Deutsches Herzzentrum Berlin, Berlin (Germany); Klaassen, Sabine [Experimental and Clinical Research Center, Charite Medical Faculty and Max Delbrueck Center for Molecular Medicine, Berlin (Germany)

    2012-12-15

    To analyse the value of cardiovascular magnetic resonance (CMR)-derived myocardial parameters to differentiate left ventricular non-compaction cardiomyopathy (LVNC) from other cardiomyopathies and controls. We retrospectively analysed 12 patients with LVNC, 11 with dilated and 10 with hypertrophic cardiomyopathy and compared them to 24 controls. LVNC patients had to fulfil standard echocardiographic criteria as well as additional clinical and imaging criteria. Cine steady-state free precession and late gadolinium enhancement (LGE) imaging was performed. The total LV myocardial mass index (LV-MMI), compacted (LV-MMI{sub compacted}), non-compacted (LV-MMI{sub non-compacted}), percentage LV-MM{sub non-compacted}, ventricular volumes and function were calculated. Data were compared using analysis of variance and Dunnett's test. Additionally, semi-quantitative segmental analyses of the occurrence of increased trabeculation were performed. Total LV-MMI{sub non-compacted} and percentage LV-MM{sub non-compacted} were discriminators between patients with LVCN, healthy controls and those with other cardiomyopathies with cut-offs of 15 g/m{sup 2} and 25 %, respectively. Furthermore, trabeculation in basal segments and a ratio of non-compacted/compacted myocardium of {>=}3:1 were criteria for LVNC. A combination of these criteria provided sensitivities and specificities of up to 100 %. None of the LVNC patients demonstrated LGE. Absolute CMR quantification of the LV-MMI{sub non-compacted} or the percentage LV-MM{sub non-compacted} and increased trabeculation in basal segments allows one to reliably diagnose LVNC and to differentiate it from other cardiomyopathies. (orig.)

  4. The Subcutaneous Implantable Cardioverter-Defibrillator: A Practical Review and Real-World Use and Application

    Directory of Open Access Journals (Sweden)

    Mark E. Panna Jr, MD, FACC, FHRS

    2016-02-01

    Full Text Available The subcutaneous implantable cardioverter-defibrillator (ICD is a novel technology using a subcutaneous (extrathoracic system for treatment of potential lethal ventricular arrhythmias. It avoids many of the risks of transvenous ICD implantation. It may be considered in patients having an ICD indication who do not have a pacing and/or cardiac resynchronization therapy indication, and who are unlikely to benefit from antitachycardia pacing therapy. We review patient selection, system components, the implantation technique, and screening considerations for subcutaneous ICD implantation. Its uses in specific patient populations, including children, patients with congenital heart disease, hypertrophic cardiomyopathy, or end-stage renal disease, and patients with preexisting pacemakers, are highlighted. Areas of future investigation are reviewed, including potential use with leadless pacing and magnetic resonance imaging.

  5. Cochlear Implant

    Directory of Open Access Journals (Sweden)

    Mehrnaz Karimi

    1993-03-01

    Full Text Available People with profound hearing loss are not able to use some kinds of conventional amplifiers due to the nature of their loss. In these people, hearing sense is stimulated only when the auditory nerve is activated via electrical stimulation. This stimulation is possible through cochlear implant. In fact, for the deaf people who have good mental health and can not use surgical and medical treatment and also can not benefit from air and bone conduction hearing aids, this device is used if they have normal central auditory system. The basic parts of the device included: Microphone, speech processor, transmitter, stimulator and receiver, and electrode array.

  6. Cochlear Implant

    Directory of Open Access Journals (Sweden)

    Mehrnaz Karimi

    1992-04-01

    Full Text Available People with profound hearing loss are not able to use some kinds of conventional amplifiers due to the nature of their loss . In these people, hearing sense is stimulated only when the auditory nerve is activated via electrical stimulation. This stimulation is possible through cochlear implant. In fact, for the deaf people who have good mental health and can not use surgical and medical treatment and also can not benefit from air and bone conduction hearing aids, this device is used if they have normal central auditory system. The basic parts of the device included: Microphone, speech processor, transmitter, stimulator and receiver, and electrode array.

  7. Spanish Implantable Cardioverter-Defibrillator Registry. Sixth official report of the Spanish Society Of Cardiology Working Group on Implantable Cardioverter-defibrillators (2009).

    Science.gov (United States)

    Alzueta, Javier; Linde, Antonio; Barrera, Alberto; Peña, Jose; Peinado, Rafael

    2010-12-01

    This article describes the findings of the 2009 Spanish Implantable Cardioverter-Defibrillator (ICD) Registry compiled by the Working Group on Implantable Cardioverter-Defibrillators of the Spanish Society of Cardiology's Electrophysiology and Arrhythmias Section. Each implantation team voluntarily sent prospective data recorded on a single-page document to the Spanish Society of Cardiology. In total, 4108 device implantations were reported, which comprised 88.6% of the estimated total number of implantations carried out. The number of implants reported corresponded to 89 per million population and the estimated total number was 100.2 per million. The proportion of first implantations among those reported was 71.3%. Data were received from 134 centers, 17 more than in 2008. There continued to be significant regional variations between the various Spanish autonomous regions. The highest implantation rate (81%) was in men (mean age 62 years) who had severe or moderate-to-severe ventricular dysfunction and were in New York Heart Association functional class II. The most common heart condition was ischemic heart disease, followed by dilated cardiomyopathy. Indications for primary prevention accounted for 55.9% of first implantations; this figure was lower than the previous year's for the first time since 2003. The most significant increase observed was in patients with ischemic heart disease. The 2009 Spanish ICD registry included data on almost 89% of all ICD implantations performed in the country. Although the number of implantations has continued to increase, it still remains far from the European average. The percentage of implantations performed for primary prevention was observed to have stabilized.

  8. [Experience with the use of an implantable automatic cardioverter defibrillator].

    Science.gov (United States)

    Dórticos, F; Zayas, R; Dorantes, M; Tain, J; Bueno, J; Carballido, J; Sainz, H

    1990-01-01

    An automatic implantable cardioverter-defibrillator with pacemaker was implanted in Cuba, in ten patients with malignant ventricular arrhythmias, sudden cardiac collapse, and ventricular tachycardia with syncope, after a previous electrophysiological study for analysis of the arrhythmia and pharmacological evaluation. The patients were 9 males, ranging in age from 23 a 70 years, with a mean of 48 years, and an ejection fraction of 32% (18-62%). The etiologies were: an old myocardial infarction (7 cases) and dilated cardiomyopathy (3 cases). During the follow-up, mean from 2 to 25 months, four patients received effective shocks for rapid palpitations and presyncope. Two patients died, one due to incessant ventricular tachycardia and one of a cause unrelated to device. We concluded that the GUARDIAN 4201 and 4202 device are useful to prevent sudden cardiac death in high risk patients who experienced a life threatening arrhythmia.

  9. Concomitant Thoracic Aortobifemoral Bypass With Left Ventricular Assist Device Implantation.

    Science.gov (United States)

    Bishawi, Muath; Shah, Asad A; McCann, Richard L; Milano, Carmelo A

    2016-11-01

    Improved quality of life for patients after left ventricular assist device (LVAD) implantation can be greatly limited by peripheral vascular disease even if heart failure symptoms are resolved by LVAD support. We present a case of concomitant thoracic aortobifemoral bypass and LVAD implantation in a patient with ischemic cardiomyopathy, severe peripheral vascular disease, and multiple previous failed revascularization attempts. In this patient, we used the LVAD outflow to provide the inflow to the femoral artery bypass graft. This graft has remained patent at a 2-year follow-up, without claudication symptoms. Performing concomitant major vascular operations safely and successfully is feasible in patients with LVADs. Quality of life after ventricular assist device placement can be limited by vascular disease, but it can be markedly improved after vascular surgical intervention. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  10. Cardiomiopatía periparto Peripartum cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Jorge E Velásquez V

    2008-02-01

    Full Text Available La cardiomiopatía periparto es una entidad clínica con una frecuencia variable de acuerdo con la zona en estudio. Se caracteriza por disfunción sistólica del ventrículo izquierdo y posterior aparición de síntomas de falla cardiaca, los cuales ocurren durante el último mes de gestación y los primeros meses post- parto. Su etiología aún no es clara, pero se plantean diferentes teorías, las cuales se basan en fenómenos inflamatorios, infecciosos y auto-inmunes. Recientemente, se describieron alteraciones relacionadas con el estrés oxidativo, que podrían explicar en gran medida esta patología. Su presentación clínica guarda gran similitud con las demás causas de falla cardíaca, aunque se han descrito presentaciones atípicas. Su diagnóstico requiere alto nivel de sospecha y debe considerarse en toda mujer con síntomas de falla cardíaca durante el periparto. El tratamiento convencional de la falla cardiaca crónica que incluye beta-bloqueadores, inhibidores de la enzima convertidora de angiotensina y diuréticos, además de los adelantos en el diagnóstico y manejo de la falla cardiaca aguda, permitió cambiar la historia de la enfermedad al disminuir la mortalidad y recuperar la función sistólica del ventrículo izquierdo. Las gestaciones posteriores al desarrollo de esta entidad, dependerán de la recuperación completa de la función cardíaca, sin disminuir el riesgo de recurrencia. Todavía existen múltiples preguntas por responder en áreas como etiología, factores de riesgo, tratamiento y marcadores pronósticos que permitan prevenir y manejar en forma oportuna y segura tanto a la madre como a su hijo.Peripartum cardiomyopathy is a clinical entity with a variable frequency according to the zone of the study. It is characterized by a systolic dysfunction of the left ventricle and posterior appearance of heart failure symptoms that occur during the last month of pregnancy and the first post-partum months. Its etiology

  11. Complications after cardiac implantable electronic device implantations

    DEFF Research Database (Denmark)

    Kirkfeldt, Rikke Esberg; Johansen, Jens Brock; Nohr, Ellen Aagaard

    2014-01-01

    Complications after cardiac implantable electronic device (CIED) treatment, including permanent pacemakers (PMs), cardiac resynchronization therapy devices with defibrillators (CRT-Ds) or without (CRT-Ps), and implantable cardioverter defibrillators (ICDs), are associated with increased patient...

  12. Age-dependent heterogeneity of familiar hypertrophic cardiomyopathy phenotype: a role of cardiovascular magnetic resonance.

    Science.gov (United States)

    Glaveckaitė, Sigita; Rudys, Alfredas; Mikštienė, Violeta; Valevičienė, Nomeda; Palionis, Darius; Laucevičius, Aleksandras

    2013-01-01

    In this case report, we present familiar hypertrophic cardiomyopathy with age-dependent heterogeneity of the disease phenotype among the members of one family who carry the same mutation of the myosin-binding protein C gene. Phenotypic heterogeneity is common in patients with familial forms of hypertrophic cardiomyopathy, both in clinical expression and outcome. Compared with other noninvasive cardiac imaging modalities, cardiovascular magnetic resonance provides an opportunity to more accurately characterize the varying phenotypic presentations of hypertrophic cardiomyopathy.

  13. Reversal of cardiomyopathy in propionic acidemia after liver transplantation: a 10-year follow-up.

    Science.gov (United States)

    Arrizza, Chiara; De Gottardi, Andrea; Foglia, Ezio; Baumgartner, Matthias; Gautschi, Matthias; Nuoffer, Jean-Marc

    2015-12-01

    Cardiomyopathy is a frequent complication in propionic acidemia. It is mostly rapidly fatal and independent of the metabolic control or medical intervention. Here, we present the reversal of a severe cardiomyopathy after liver transplantation in a patient with propionic acidemia and the long-term stability after ten years. Liver transplantation in patients with propionic acidemia may be considered a valid and long-lasting treatment when cardiomyopathy is progressive and unresponsive to medical therapy. © 2015 Steunstichting ESOT.

  14. RESTRICTIVE CARDIOMYOPATHY AND SECONDARY CONGESTIVE HEART FAILURE IN A MCDOWELL'S CARPET PYTHON (MORELIA SPILOTA MCDOWELLI).

    Science.gov (United States)

    Schilliger, Lionel; Chetboul, Valérie; Damoiseaux, Cécile; Nicolier, Alexandra

    2016-12-01

    Echocardiography is an established and noninvasive diagnostic tool used in herpetologic cardiology. Various cardiac lesions have been previously described in reptiles with the exception of restrictive cardiomyopathy. In this case report, restrictive cardiomyopathy and congestive heart failure associated with left atrial and sinus venosus dilation were diagnosed in a 2-yr-old captive lethargic McDowell's carpet python ( Morelia spilota mcdowelli), based on echocardiographic, Doppler, and histopathologic examinations. This cardiomyopathy was also associated with thrombosis within the sinus venosus.

  15. Muscle Stem Cell Therapy for the Treatment of DMD Associated Cardiomyopathy

    Science.gov (United States)

    2015-12-01

    Translating Stem Cells and Regenerative Therapies to the Treatment of Liver Diseases”, Cleveland, OH, April 12-13, 2015. 36. Invited Speaker, Gene, Cell ...Award Number: W81XWH-11-1-0803 TITLE: Muscle Stem Cell Therapy for the Treatment of DMD Associated Cardiomyopathy PRINCIPAL INVESTIGATOR... Stem Cell Therapy for the Treatment of DMD Associated Cardiomyopathy 5a. CONTRACT NUMBER Cardiomyopathy 5b. GRANT NUMBER W81XWH-11-1-0803

  16. [From arrhythmogenic right ventricular dysplasia/cardiomyopathy(ARVD/ARVC) to a broader concept of ABCDE syndrome].

    Science.gov (United States)

    Sekiguchi, M; Kinoshita, O

    2000-01-01

    The concept on the presence of a peculiar disease, arrhythmogenic right ventricular cardiomyopathy, has been widely recognized to be involved in the category of cardiomyopathy. The authors believe this disease entity should be further expanded, and propose the new term "ABCDE Syndrome", abbreviated from Arrhythmogenicity, Brugada syndrome, Competitive sports, Death(especially sudden death) and Electric disturbance type of cardiomyopathy(ECM), be incorporated to encompass all facets of this category.

  17. Midventricular Hypertrophic Cardiomyopathy with Apical Aneurysm: Potential for Underdiagnosis and Value of Multimodality Imaging

    Directory of Open Access Journals (Sweden)

    Archana Sivanandam

    2016-01-01

    Full Text Available We illustrate a case of midventricle obstructive HCM and apical aneurysm diagnosed with appropriate use of multimodality imaging. A 75-year-old African American woman presented with a 3-day history of chest pain and dyspnea with elevated troponins. Her electrocardiogram showed sinus rhythm, left atrial enlargement, left ventricular hypertrophy, prolonged QT, and occasional ectopy. After medical therapy optimization, she underwent coronary angiography for an initial diagnosis of non-ST segment elevation myocardial infarction. Her coronaries were unremarkable for significant disease but her left ventriculogram showed hyperdynamic contractility of the midportion of the ventricle along with a large dyskinetic aneurysmal apical sac. A subsequent transthoracic echocardiogram provided poor visualization of the apical region of the ventricle but contrast enhancement identified an aneurysmal pouch distal to the midventricular obstruction. To further clarify the diagnosis, cardiac magnetic resonance imaging with contrast was performed confirming the diagnosis of midventricular hypertrophic cardiomyopathy with apical aneurysm and fibrosis consistent with apical scar on delayed enhancement. The patient was medically treated and subsequently underwent elective implantable defibrillator placement in the ensuing months for recurrent nonsustained ventricular tachycardia and was initiated on prophylactic oral anticoagulation with warfarin for thromboembolic risk reduction.

  18. [Arrhythmogenic right ventricular cardiomyopathy. Case report and a brief literature review].

    Science.gov (United States)

    Izurieta, Carlos; Curotto-Grasiosi, Jorge; Rocchinotti, Mónica; Torres, María J; Moranchel, Manuel; Cañas, Sebastián; Cardús, Marta E; Alasia, Diego; Cordero, Diego J; Angel, Adriana

    2013-01-01

    A 51-year-old man was admitted to this hospital because of palpitations and a feeling of dizziness for a period of 2h. The electrocardiogram revealed a regular wide-QRS complex tachycardia at a rate of 250 beats per minute, with superior axis and left bundle branch block morphology without hemodynamically decompensation, the patient was cardioverted to sinus rhythm after the administration of a loading and maintenance dose of amiodarone. The elechtrophysiological study showed the ventricular origin of the arrhythmia. In order to diagnose the etiology of the ventricular tachycardia we performed a coronary arteriography that showed normal epicardial vessels, thus ruling out coronary disease. Doppler echocardiography revealed systolic and diastolic functions of both left and right ventricles within normal parameters, and normal diameters as well. A cardiac magnetic resonance with late enhancement was done, showing structural abnormalities of the right ventricle wall with moderate impairment of the ejection fraction, and a mild dysfunction of the left ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was performed as 2 major Task Force criteria were met. We implanted an automatic cardioverter defibrillator as a prophylactic measure. The patient was discharged without complications. Copyright © 2012 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  19. An overlap of Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy/dysplasia

    Directory of Open Access Journals (Sweden)

    Shohei Kataoka, MD

    2016-02-01

    Full Text Available Overlapping characteristics of Brugada syndrome (BrS and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D have been reported in recent studies, but little is known about the overlapping disease state of BrS and ARVC/D. A 36-year-old man, hospitalized at our institution for syncope, presented with this overlapping disease state. The electrocardiogram showed spontaneous coved-type ST-segment elevation, and ventricular fibrillation was induced by right ventricular outflow tract stimulation in an electrophysiological study. BrS was subsequently diagnosed; additionally, the presence of epsilon-like waves and right ventricular structural abnormalities met with the 2010 revised task force criteria for ARVC/D. After careful investigation for both BrS and ARVC/D, an implantable cardioverter defibrillator was inserted in the patient. This case revealed 2 important clinical findings: (1 BrS and ARVC/D clinical features can coexist in a single patient, and EPS might be useful for determining the phenotype of overlapping disease (e.g., BrS-like or ARVC/D-like. (2 An overlapping disease state of BrS and ARVC/D can change phenotypically during its clinical course. Therefore, careful examination and attentive follow-up are required for patients with BrS or ARVC/D.

  20. National experience with long-term use of the wearable cardioverter defibrillator in patients with cardiomyopathy.

    Science.gov (United States)

    Lamichhane, Madhab; Gardiner, Joseph C; Bianco, Nicole R; Szymkiewicz, Steven J; Thakur, Ranjan K

    2017-01-01

    The wearable cardioverter defibrillator (WCD) is generally used for short periods of sudden cardiac death (SCD) risk; circumstances may occasionally result in prolonged use (over 1 year). The aim of this study was to determine the benefits and risks of prolonged use in patients with systolic heart failure (HF). ZOLL's post-market US database included adult patients (≥18 years) with ischemic and/or non-ischemic cardiomyopathy (ICM, NICM) and at least 1 year of use. Cox-regression was used to identify factors associated with survival with WCD use, and reasons for stopping use were entered as time-dependent factors. Among 220 patients, age (mean ± SD) 55.4 ± 14.8 years, WCD use 451.4 ± 289.9 days, and 67.3 % were male and their left ventricle ejection fraction (EF) averaged 20.9 ± 7.2 %. Eighty-nine (40.5 %) were continuing WCD use at the last follow-up. Thirty-six (16.4 %) and 56 (25.5 %) patients discontinued WCD use because of EF recovery and implantable cardioverter (ICD) implantation, respectively. Nine patients (4.1 %) received appropriate shock therapy for 13 episodes of sustained ventricular tachyarrhythmia with 12 (92.3 %) successful shocks. One patient died of refractory ventricular fibrillation. One patient died from sinus bradycardia transitioning to asystole. Eight patients (3.6 %) had nine episodes of non-fatal inappropriate shocks. Long-term use of the WCD is safe and effective. Recovery of EF was seen in significant number of patients even after 1 year of WCD use.

  1. Clinical significance of left ventricular apical aneurysms in hypertrophic cardiomyopathy patients: the role of diagnostic electrocardiography.

    Science.gov (United States)

    Ichida, Masaru; Nishimura, Yoshioki; Kario, Kazuomi

    2014-10-01

    Some patients with hypertrophic cardiomyopathy (HCM) develop left ventricular apical aneurysm, leading to serious cardiovascular complications. The aims of this study were to identify the incidence and clinical course of HCM patients with apical aneurysms in Japan, and to evaluate the role of electrocardiography (ECG) as a screening test to detect apical aneurysms in HCM patients. In a retrospective, single center analysis of a population of 247 HCM patients, 21 patients (8.5%) had left ventricular apical aneurysms. Their mean age was 60 ± 14 years (range: 23-77 years) at study entry. Over 4.7 ± 3.3 years of follow-up, 10 patients (47.6%) experienced an adverse clinical event (annual event rate: 10.1%/y), including five implantable cardioverter-defibrillator (ICD) implantations for ventricular tachycardia/ventricular fibrillation (VT/VF), an appropriate discharge of ICD for VT/VF, and four nonfatal thromboembolic strokes. Two patients developed systolic dysfunction (ejection fraction <50%). No sudden cardiac death or progressive heart failure was detected. Fourteen patients showed ST-segment elevation (≥ 1 mm) in V3 through V5 of ECG. In four patients, progression of the ST-segment elevation was recognized. When the ST-segment elevation was used to identify apical aneurysms in HCM patients, the sensitivity was 66.7%, and the specificity was 98.7%. Apical aneurysms in HCM patients in Japan are not rare, and are associated with serious cardiovascular complications. The early diagnosis of apical aneurysms can be achieved by serial ECG. Copyright © 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  2. Orthostatic blood pressure test for risk stratification in patients with hypertrophic cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Julia Münch

    Full Text Available Hypertrophic cardiomyopathy (HCM is the most common cause of sudden cardiac death (SCD in young adults, mainly ascribed to ventricular tachycardia (VT. Assuming that VT is the major cause of (pre- syncope in HCM patients, its occurrence is essential for SCD risk stratification and primarily preventive ICD-implantation. However, evidence of VT during syncope is often missing. As the differentiation of potential lethal causes for syncope such as VT from more harmless reasons is crucial, HCM patients were screened for orthostatic dysregulation by using a simple orthostatic blood pressure test.Over 15 months (IQR [9;20] 100 HCM patients (55.8±16.2 yrs, 61% male were evaluated for (pre-syncope and VT (24h-ECGs, device-memories within the last five years. Eighty patients underwent an orthostatic blood pressure test. Logistic regression models were used for statistical analysis.In older patients (>40 yrs a positive orthostatic test result increased the chance of (pre- syncope by a factor of 63 (95%-CI [8.8; 447.9], p<0.001; 93% sensitivity, 95%-CI [76; 99]; 74% specificity, 95%-CI [58; 86]. No correlation with VT was shown. A prolonged QTc interval also increased the chance of (pre- syncope by a factor of 6.6 (95%-CI [2.0; 21.7]; p=0.002.The orthostatic blood pressure test is highly valuable for evaluation of syncope and presyncope especially in older HCM patients, suggesting that orthostatic syncope might be more relevant than previously assumed. Considering the high complication rates due to ICD therapies, this test may provide useful information for the evaluation of syncope in individual risk stratification and may help to prevent unnecessary device implantations, especially in older HCM patients.

  3. Spanish Implantable Cardioverter-defibrillator Registry. Ninth official report of the Spanish Society of Cardiology Electrophysiology and Arrhythmias Section (2012).

    Science.gov (United States)

    Alzueta, Javier; Fernández, José María

    2013-11-01

    To summarize the findings of the Spanish Implantable Cardioverter-defibrillator Registry for 2012 compiled by the Electrophysiology and Arrhythmias Section of the Spanish Society of Cardiology. Prospective data recorded voluntarily on single-page questionnaires were sent to the Spanish Society of Cardiology by each implantation team. Overall, 4216 device implantations were reported, representing 80.8% of the estimated total number of implantations. The reported implantation rate was 91.2 per million population and the estimated total implantation rate was 113 per million. The proportion of first implantations was 69.4%. We collected data from 161 hospitals (6 fewer than in 2011). The majority of implantable cardioverter-defibrillator recipients were men (83.4%). Mean age was 61.8 (13.4) years. Most patients had severe or moderate-to-severe ventricular dysfunction and were in New York Heart Association functional class II. Ischemic heart disease was the most frequent underlying cardiac condition, followed by dilated cardiomyopathy. The number of indications for primary prevention decreased over the previous year and now account for 58.1% of first implantations. Overall, 81% of the implantable cardioverter-defibrillator were implanted by cardiac electrophysiologists. The 2012 Spanish Implantable Cardioverter-defibrillator Registry includes data on 80.8% of all implantable cardioverter-defibrillators implantations performed in Spain. This is the second consecutive year in which the number of implantations has slightly decreased compared to the previous year. This year, the percentage of implantations for primary prevention indications also decreased. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

  4. Evaluation of left cardiac function by exercise in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Konishi, Tokuji; Horayama, Norihisa; Hamada, Masayuki; Nakano, Takeshi; Takezawa, Hideo

    1981-01-01

    Left ventricular systolic and diastolic features at rest and exercise in hypertrophic cardiomyopathy were evaluated by Fourier analysis of blood pool scintigraphy (intracorporeal labelling with sup(99m)Tc-RBC). In the normal group (17 subjects), the left ventricular ejection fraction showed a linear increase, but no abnormality of regional ventricular wall motion, by multistage exercises. The hypertrophic cardiomyopathy group showed higher left ventricular ejection fractions at rest than those of the normal group, and in the HCM group (non-obstructive, from morphological features; 7 cases) the left ventricular ejection fraction did not increase any more when it reached a certain plateau in accordance with increased stress. In the HOCM (obstructive; 5 cases), the left ventricular ejection fraction showed a decreasing tendency as the stress was increased and also showed contractile abnormalities from the left ventricular center to the apex. Fourier analysis was effective for the evaluation of these changes. (Chiba, N.)

  5. Takotsubo Cardiomyopathy in a Patient with Undiscovered Sigmoid Colon Cancer

    Directory of Open Access Journals (Sweden)

    Huang Po-Yen

    2017-01-01

    Full Text Available Takotsubo cardiomyopathy (TTC is a stress-related cardiomyopathy that is characterized by reversible left systolic dysfunction, which appears to be precipitated by sudden emotional or physical stress in the absence of myocardial infarction. Here we present a rare case that clinically presented with intermittent abdominal pain, initially impressed as non-ST elevation myocardial infarction and congestive heart failure but with a normal coronary angiogram. Her symptoms relieved spontaneously without returning. Sigmoid colon cancer was diagnosed via colonoscopy later due to persistent abdominal discomfort. In the absence of detectable emotional or physical stress factors, the newly diagnosed sigmoid colon cancer was the only possible trigger factor of TTC. We offer this case as a reminder that cancer should be considered in the differential diagnosis of patients presenting with the etiology of TTC.

  6. Atlas of the clinical genetics of human dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Haas, Jan; Frese, Karen S; Peil, Barbara

    2015-01-01

    AIM: Numerous genes are known to cause dilated cardiomyopathy (DCM). However, until now technological limitations have hindered elucidation of the contribution of all clinically relevant disease genes to DCM phenotypes in larger cohorts. We now utilized next-generation sequencing to overcome......, leading to 99.1% coverage of the target region with at least 50-fold and a mean read depth of 2415. In this well characterized cohort, we find the highest number of known cardiomyopathy mutations in plakophilin-2, myosin-binding protein C-3, and desmoplakin. When we include yet unknown but predicted......: This is to our knowledge, the first study that comprehensively investigated the genetics of DCM in a large-scale cohort and across a broad gene panel of the known DCM genes. Our results underline the high analytical quality and feasibility of Next-Generation Sequencing in clinical genetic diagnostics and provide...

  7. Genomic Insights into Cardiomyopathies: A Comparative Cross-Species Review.

    Science.gov (United States)

    Simpson, Siobhan; Rutland, Paul; Rutland, Catrin Sian

    2017-03-21

    In the global human population, the leading cause of non-communicable death is cardiovascular disease. It is predicted that by 2030, deaths attributable to cardiovascular disease will have risen to over 20 million per year. This review compares the cardiomyopathies in both human and non-human animals and identifies the genetic associations for each disorder in each species/taxonomic group. Despite differences between species, advances in human medicine can be gained by utilising animal models of cardiac disease; likewise, gains can be made in animal medicine from human genomic insights. Advances could include undertaking regular clinical checks in individuals susceptible to cardiomyopathy, genetic testing prior to breeding, and careful administration of breeding programmes (in non-human animals), further development of treatment regimes, and drugs and diagnostic techniques.

  8. Myocardial ischemia in hypertrophic cardiomyopathy; Isquemia miocardica na cardiomiopatia hipertrofica

    Energy Technology Data Exchange (ETDEWEB)

    Lima Filho, Moyses de Oliveira; Figueiredo, Geraldo L.; Simoes, Marcus V.; Pyntia, Antonio O.; Marin Neto, Jose Antonio [Sao Paulo Univ., Ribeirao Preto, SP (Brazil). Faculdade de Medicina. Div. de Cardiologia

    2000-08-01

    Myocardial ischemia in hypertrophic cardiomyopathy is multifactorial and explains the occurrence of angina, in about 50% of patients. The pathophysiology of myocardial ischemia may be explained by the increase of the ventricular mass and relative paucity of the coronary microcirculation; the elevated ventricular filling pressures and myocardial stiffness causing a compression of the coronary microvessels; the impaired coronary vasodilator flow reserve caused by anatomic and functional abnormalities; and the systolic compression of epicardial vessel (myocardial bridges). Myocardial ischemia must be investigated by perfusion scintigraphic methods since its presence influences the prognosis and has relevant clinical implications for management of patients. Patients with hypertrophic cardiomyopathy and documented myocardial ischemia usually need to undergo invasive coronary angiography to exclude the presence of concomitant atherosclerotic coronary disease. (author)

  9. Baking soda pica associated with rhabdomyolysis and cardiomyopathy in pregnancy.

    Science.gov (United States)

    Scolari Childress, Katherine M; Myles, Thomas

    2013-08-01

    Pica is a commonly underappreciated disorder in pregnancy that can lead to several complications, including severe metabolic derangements and other adverse outcomes. We report a case of baking soda pica in pregnancy associated with both rhabdomyolysis and cardiomyopathy. A multigravid woman at 37 weeks of gestation presented with weakness and severe hypokalemia. She subsequently had development of rhabdomyolysis and presumed peripartum cardiomyopathy. After delivery, it was discovered that the patient had a long history of consumption of large quantities of baking soda. Her condition improved with cessation of the pica. Clinicians must have a high index of suspicion for pica in pregnancy because it can lead to complex diagnostic challenges and pregnancy complications. The diagnosis should be considered in a patient with unexplained metabolic abnormalities.

  10. The Safety of Exercise in Individuals With Cardiomyopathy.

    Science.gov (United States)

    Finocchiaro, Gherardo; Sharma, Sanjay

    2016-04-01

    The cardiomyopathies are a heterogeneous group of primary myocardial diseases characterized by a propensity to fatal arrhythmias and are the leading cause of sudden cardiac death in young athletes. Apart from the underlying pathologic substrate, a combination of neurohormonal, mechanical, and oxidative stressors; dehydration; electrolyte abnormalities; and acid-base disturbances may trigger fatal arrhythmias during intensive exercise. Current consensus-based documents recommend that affected athletes abstain from most competitive sports, with the exception of those involving minimal dynamic or static components, to minimize the risk of sudden cardiac death. This article aims to describe the rationale underlying current recommendations and provides guidance for recreational exercise in many asymptomatic individuals. The article concludes with pragmatic recommendations for symptomatic patients with cardiomyopathy in whom physical activity is associated with beneficial effects on the quality and, possibly, the quantity of life. Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  11. Primary amyloid heart disease presenting as hypertrophic obstructive cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Weston, L.T.; Raybuck, B.D.; Robinowitz, M.; Brinker, J.A.; Oetgen, W.J.

    1986-01-01

    This report describes the unusual presentation of a patient with primary cardiac amyloidosis. Initial clinical symptoms and hemodynamic studies, including Technetium-99m-pyrophosphate scintigraphy, suggested hypertrophic obstructive cardiomyopathy, but endomyocardial biopsy revealed diffuse amyloid infiltration. Only two other cases of left ventricular outflow tract obstruction due to cardiac amyloidosis have been reported. The false-negative technetium-99m-pyrophosphate scintigram in this patient argues for the use of endomyocardial biopsy to aid in the diagnosis of left ventricular hypertrophy.

  12. Microvascular dysfunction in nonfailing arrhythmogenic right ventricular cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Paul, Matthias [University Hospital Muenster, Department of Cardiology and Angiology, Muenster (Germany); University Hospital Muenster, Institute for Genetics of Heart Diseases, Muenster (Germany); Rahbar, Kambiz; Kies, Peter; Schober, Otmar [University Hospital Muenster, Department of Nuclear Medicine, Muenster (Germany); Gerss, Joachim [University of Muenster, Institute of Biostatistics and Clinical Research, Muenster (Germany); Schaefers, Klaus; Schaefers, Michael [University of Muenster, European Institute for Molecular Imaging - EIMI, Muenster (Germany); Breithardt, Guenter [University Hospital Muenster, Department of Cardiology and Angiology, Muenster (Germany); Schulze-Bahr, Eric [University Hospital Muenster, Institute for Genetics of Heart Diseases, Muenster (Germany); Wichter, Thomas [Marienhospital Osnabrueck, Department of Cardiology, Niels-Stensen-Kliniken, Osnabrueck (Germany)

    2012-03-15

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a nonischaemic cardiomyopathy and leading cause of sudden death in the young. It has been shown that microvascular dysfunction reflected by an impaired myocardial blood flow (MBF) response to stress is present in patients with other forms of nonischaemic cardiomyopathy, e.g. dilated cardiomyopathy, and that the reduced MBF may be related to a poor prognosis. Therefore, we quantified MBF, coronary flow reserve and coronary vascular resistance in patients with nonfailing ARVC using H{sub 2}{sup 15} O and PET. In ten male patients with ARVC (mean age 49 {+-} 14 years), MBF was quantified at rest and during adenosine-induced hyperaemia using H{sub 2}{sup 15} O PET. Results were compared with those obtained in 20 age-matched healthy male control subjects (mean age 46 {+-} 14 years). Resting MBF was not significantly different between patients with ARVC and controls (MBF{sub rest} 1.19 {+-} 0.29 vs. 1.12 {+-} 0.20 ml/min/ml). However, hyperaemic MBF was significantly lower in patients with ARVC than in controls (2.60 {+-} 0.96 vs. 3.68 {+-} 0.84 ml/min/ml; p = 0.005). Consequently, patients with ARVC had a significantly lower coronary flow reserve than control subjects (2.41 {+-} 1.34 vs. 3.39 {+-} 0.93; p = 0.030). In addition, hyperaemic coronary vascular resistance was increased in patients with ARVC (36.79 {+-} 12.91 vs. 26.31 {+-} 6.49 mmHg x ml{sup -1} x min x ml; p = 0.007), but was found to be unchanged at rest. In this small well-characterized cohort of patients with nonfailing ARVC, we found a significantly reduced hyperaemic MBF and increased coronary vascular resistance. Further studies are necessary to corroborate this potential new functional aspect of the pathophysiological mechanisms underlying ARVC. (orig.)

  13. Hypertrophic Cardiomyopathy Complicated by Pulmonary Edema in the Postpartum Period

    Directory of Open Access Journals (Sweden)

    Kate Hanneman

    2013-01-01

    Full Text Available We report the case of a 42-year-old patient with hypertrophic cardiomyopathy (HCM who presented to the emergency department with severe shortness of breath one week following uneventful cesarean delivery. Thoracic CT ruled out pulmonary embolus and confirmed pulmonary edema. Asymmetric interventricular septal thickening was clearly identified, demonstrating that the heart may be evaluated even on a non-ECG gated study. Acute pulmonary edema in the postpartum period is an unusual clinical presentation of HCM.

  14. Comparison among patients with hypertrophic cardiomyopathy, hypertrophic cardiomyopathy with hypertension and hypertensive heart disease by {sup 123}I-BMIPP myocardial scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Yoneyama, Satoshi; Sugihara, Hiroki; Ito, Kazuki [Kyoto Prefectural Univ. of Medicine (Japan)] [and others

    1997-12-01

    The usefulness of {sup 123}I-BMIPP myocardial SPECT in discriminating hypertrophic cardiomyopathy (46 patients), hypertrophic cardiomyopathy with hypertension (23 patients), and hypertensive hypertrophic heart (20 patients) was studied. SPECT image was divided into 17 domains, and dimension of decreased accumulation was decided visually at each domain as four classes called defect score (DS). Summation of DS (TDS) of each group was used to compare frequency and dimension of decreased accumulation, and characteristic of each site. Frequency of decreased accumulation and TDS in hypertrophic cardiomyopathy were similar in dimension with those in hypertrophic cardiomyopathy with hypertension, and those data in hypertensive hypertrophic heart were lower than those in above-mentioned 2 groups. In the cases of hypertrophic cardiomyopathy and hypertrophic cardiomyopathy with hypertension, decreased accumulation site was similar and was anterior wall-septum junction, septum-posterior wall junction and apex of heart. In the case of hypertensive hypertrophic heart, decreased accumulation site was only the posterior wall. Frequency, dimension and site of decreased accumulation in hypertrophic cardiomyopathy were different from those in hypertensive hypertrophic heart, and BMIPP was thought to be useful in discriminating these diseases. (K.H.)

  15. High Incidence of De Novo and Subclinical Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy and Cardiac Rhythm Management Device.

    Science.gov (United States)

    Wilke, Iris; Witzel, Katrin; Münch, Julia; Pecha, Simon; Blankenberg, Stephan; Reichenspurner, Hermann; Willems, Stephan; Patten, Monica; Aydin, Ali

    2016-07-01

    Atrial fibrillation (AF) is an important prognostic parameter in patients with hypertrophic cardiomyopathy (HCM). Though cardiac rhythm management (CRM) devices (e.g., ICD, pacemaker or implantable loop recorder) can detect subclinical AF, data describing the incidence of AF are rare. We therefore investigated the incidence and clinical impact of de novo and subclinical AF detected by CRM devices in patients with HCM. In our retrospective single-center study, we included patients with HCM and need for CRM devices. The primary endpoint of the study was the incidence of clinical and subclinical de novo AF. During follow-up, patients were screened for adverse events like stroke, ventricular arrhythmia, heart failure, or death. From 192 HCM patients, 44 patients received a CRM device (38 ICDs, 5 pacemakers, 1 implantable loop recorder). In 14 of these patients (32%), AF had been documented before device implantation. Thirty (68%) patients were free from AF at the time of implantation. During a median follow-up of 595 days (interquartile range, 367-890 days), de novo AF was recorded in 16 of these 30 patients (53%). Fourteen (88%) of the 16 patients with de novo AF were free from any clinical symptoms, so these patients were classified to have subclinical AF. In logistic regression analysis, age was the only significant predictor for an increased risk of AF. AF is common in patients with HCM who need a CRM device. More than 50% of these patients develop de novo AF that was predominantly subclinical in our cohort. © 2016 Wiley Periodicals, Inc.

  16. Personalized medicine: genetic diagnosis for inherited cardiomyopathies/channelopathies.

    Science.gov (United States)

    Ackerman, Michael J; Marcou, Cherisse A; Tester, David J

    2013-04-01

    Major advances in the field of molecular genetics have expanded our ability to identify genetic substrates underlying the pathogenesis of various disorders that follow Mendelian inheritance patterns. Included among these disorders are the potentially lethal and heritable channelopathies and cardiomyopathies for which the underlying genetic basis has been identified and is now better understood. Clinical and genetic heterogeneity are hallmark features of these disorders, with thousands of gene mutations being implicated within these divergent cardiovascular diseases. Genetic testing for several of these heritable channelopathies and cardiomyopathies has matured from discovery to research-based genetic testing to clinically/commercially available diagnostic tests. The purpose of this review is to provide the reader with a basic understanding of human medical genetics and genetic testing in the context of cardiovascular diseases of the heart. We review the state of clinical genetic testing for the more common channelopathies and cardiomyopathies, discuss some of the pertinent issues that arise from genetic testing, and discuss the future of personalized medicine in cardiovascular disease. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

  17. Update on Myocarditis and Inflammatory Cardiomyopathy: Reemergence of Endomyocardial Biopsy.

    Science.gov (United States)

    Dominguez, Fernando; Kühl, Uwe; Pieske, Burkert; Garcia-Pavia, Pablo; Tschöpe, Carsten

    2016-02-01

    Myocarditis is defined as an inflammatory disease of the heart muscle and is an important cause of acute heart failure, sudden death, and dilated cardiomyopathy. Viruses account for most cases of myocarditis or inflammatory cardiomyopathy, which could induce an immune response causing inflammation even when the pathogen has been cleared. Other etiologic agents responsible for myocarditis include drugs, toxic substances, or autoimmune conditions. In the last few years, advances in noninvasive techniques such as cardiac magnetic resonance have been very useful in supporting diagnosis of myocarditis, but toxic, infectious-inflammatory, infiltrative, or autoimmune processes occur at a cellular level and only endomyocardial biopsy can establish the nature of the etiological agent. Furthermore, after the generalization of immunohistochemical and viral genome detection techniques, endomyocardial biopsy provides a definitive etiological diagnosis that can lead to specific treatments such as antiviral or immunosuppressive therapy. Endomyocardial biopsy is not commonly performed for the diagnosis of myocarditis due to safety reasons, but both right- and left endomyocardial biopsies have very low complication rates when performed by experienced operators. This document provides a state-of-the-art review of myocarditis and inflammatory cardiomyopathy, with special focus on the role of endomyocardial biopsy to establish specific treatments. Copyright © 2015 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  18. Unusual Presentation of Propionic Acidemia as Isolated Cardiomyopathy

    Science.gov (United States)

    Lee, T. M.; Addonizio, L. J.; Barshop, B. A.; Chung, W. K.

    2013-01-01

    Summary Propionic acidemia (PA) is an autosomal recessive disease that results from deficiency of propionyl-CoA carboxylase (PCC). In virtually all reported cases of PA, the phenotype includes metabolic acidosis and/or neurological deficits. We report on a 14-year-old Asian male with PA who presented with isolated cardiomyopathy without any episodes of metabolic acidosis or evidence of any neurocognitive deficits. On routine metabolic screening, the patient was found to have urine organic acids suggestive of PA. Biochemical and genetic characterization confirmed a PCC deficiency with two novel mutations in PCCB: IVS7+2 T>G (c.763+2 T>G) and p.R410Q (c.1229 G>A). Residual enzyme activity likely explains our patient’s mild phenotype. Splicing mutations tend to result in a milder phenotype as these mutations may still produce small amounts of normal enzyme. In addition, the similar p.R410W mutation has been shown to have partial residual activity. Moreover, this case illustrates the important but under-recognized manifestation of isolated cardiomyopathy as the sole clinical presentation in PA. A thorough metabolic evaluation should be performed in all pediatric patients with cardiomyopathy. Such an evaluation has important implications for clinical management and genetic counseling. PMID:19238581

  19. Takotsubo Cardiomyopathy in the Setting of Tension Pneumothorax

    Directory of Open Access Journals (Sweden)

    Michael Gale

    2015-01-01

    Full Text Available Background. Takotsubo cardiomyopathy is defined as a transient left ventricular dysfunction, usually accompanied by electrocardiographic changes. The literature documents only two other cases of Takotsubo cardiomyopathy in the latter setting. Methods. A 78-year-old female presented to the ED with severe shortness of breath, hypertension, and tachycardia. On physical exam, heart sounds (S1 and S2 were regular and wheezing was noticed bilaterally. We found laboratory results with a WBC of 20.0 (103/μL, troponin of 16.52 ng/mL, CK-mb of 70.6%, and BNP of 177 pg/mL. The patient was intubated for acute hypoxemic respiratory failure. A chest X-ray revealed a large left-sided tension pneumothorax. Initial echocardiogram showed apical ballooning with a LVEF of 10–15%. A cardiac angiography revealed normal coronary arteries with no coronary disease. After supportive treatment, the patient’s condition improved with a subsequent echocardiogram showing a LVEF of 60%. Conclusion. The patient was found to have Takotsubo cardiomyopathy in the setting of a tension pneumothorax. The exact mechanisms of ventricular dysfunction have not been clarified. However, multivessel coronary spasm or catecholamine cardiotoxicity has been suggested to have a causative role. We suggest that, in our patient, left ventricular dysfunction was induced by the latter mechanism related to the stress associated with acute pneumothorax.

  20. Hypertrophic cardiomyopathy: an autopsy analysis of 14 cases.

    Directory of Open Access Journals (Sweden)

    Phadke R

    2001-07-01

    Full Text Available BACKGROUND: Hypertrophic cardiomyopathy (HCM is one of the less common forms of primary cardiomyopathies. There is little data available on HCM in Indian literature. AIMS: To assess the incidence and analyse the clinicopathological features of HCM. SETTINGS: Analysis of data of 15 years from a tertiary care centre. METHODS AND MATERIAL: The clinical and pathological data in fourteen cases of HCM with respect to their gross and microscopic features and clinical presentation were reviewed. RESULTS: Incidence of HCM amongst the autopsied primary cardiomyopathies (N = 101 was 13.9% (n=14. Males were affected more. Common presenting symptoms were exertional dyspnoea, angina and palpitations. Concentric and asymmetric hypertrophy was equally seen. Obliterative small vessel disease was noted in 50% of the cases. Although significant myofibre disarray (>5% was seen in all fourteen cases, it could be demonstrated in only 40- 50% of an average of twenty sections studied. Type IA myofibre disarray was the commonest. Six of the fourteen patients died suddenly. Cardiac failure was the commonest cause of death. CONCLUSIONS: Myofibre disarray is a highly sensitive and specific marker for HCM only when considered in a quantitative rather than a qualitative fashion. In this context, the rationale for performing endomyocardial biopsy is to rule out mimics of HCM.

  1. Trace elements in diabetic cardiomyopathy: An electrophysiological overview.

    Science.gov (United States)

    Ozturk, Nihal; Olgar, Yusuf; Ozdemir, Semir

    2013-08-15

    There is a growing body of evidence that Diabetes Mellitus leads to a specific cardiomyopathy apart from vascular disease and bring about high morbidity and mortality throughout the world. Recent clinical and experimental studies have extensively demonstrated that this cardiomyopathy causes impaired cardiac performance manifested by early diastolic and late systolic dysfunction. This impaired cardiac performance most probably have emerged upon the expression and activity of regulatory proteins such as Na(+)/Ca(2+) exchanger, sarcoplasmic reticulum Ca(2+)-ATPase, ryanodine receptor and phospholamban. Over years many therapeutic strategies have been recommended for treatment of diabetic cardiomyopathy. Lately, inorganic elements have been suggested to have anti-diabetic effects due to their suggested ability to regulate glucose homeostasis, reduce oxidative stress or suppress phosphatases. Recent findings have shown that trace elements exert many biological effects including insulin-mimetic or antioxidant activity and in this manner they have been recommended as potential candidates for treatment of diabetes-induced cardiac complications, an effect based on their modes of action. Some of these trace elements are known to play an essential role as component of enzymes and thus modulate the organ function in physiological and pathological conditions. Besides, they can also manipulate redox state of the channels via antioxidant properties and thus contribute to the regulation of [Ca(2+)]i homeostasis and cardiac ion channels. On account of little information about some trace elements, we discussed the effect of vanadium, selenium, zinc and tungstate on diabetic heart complications.

  2. Arrhythmogenic right ventricular cardiomyopathy in a dog : case report

    Directory of Open Access Journals (Sweden)

    A.J. Möhr

    2000-07-01

    Full Text Available An 8-month-old Labrador retriever bitch was evaluated for sudden-onset, progressive abdominal distension. Physical examination revealed an exaggerated inspiratory effort, severe ascites, bilateral jugular vein distension, and hypokinetic femoral arterial pulses. Thoracic auscultation detected tachycardia with muffled heart sounds, without audible cardiac murmurs. Thoracic radiographs identified severe right ventricular enlargement and pleural effusion. The electrocardiogram was consistent with incomplete right bundle branch block or right ventricular enlargement. Echocardiography demonstrated severe right ventricular and atrial dilation, secondary tricuspid regurgitation, and thinning and hypocontractility of the right ventricular myocardium. Left heart chamber sizes were slightly decreased, with normal left ventricular contractility. Adiagnosis of arrhythmogenic right ventricular cardiomyopathy was reached, based on the characteristic clinical, electrocardiographic, radiographic and echocardiographic findings, and the exclusion of other causes of isolated right ventricular failure. Treatment effected good control of clinical signs, until acutely decompensated congestive right heart failure led to euthanasia after 4 months. Arrhythmogenic right ventricular cardiomyopathy is a well-described clinical entity in humans, and has previously been documented in 3 male dogs. The condition is characterised by progressive fibro-adipose replacement of right ventricular myocardium, while the left ventricle usually remains unaffected. It should be considered a differential diagnosis in any young dog presented with isolated right heart failure, syncope, or unexplained ventricular tachyarrhythmias. This article reports the 1st case of arrhythmogenic right ventricular cardiomyopathy in a female dog, and highlights its echocardiographic features.

  3. Human herpesvirus 6-induced inflammatory cardiomyopathy in immunocompetent children

    Directory of Open Access Journals (Sweden)

    Surabhi Reddy

    2017-01-01

    Full Text Available Over the last decade, human herpesvirus 6 (HHV-6 has been implicated in the etiology of pediatric myocarditis and subsequent dilated cardiomyopathy (DCM. This review provides an overview of recent literature investigating the pathophysiological relevance of HHV-6 in inflammatory cardiomyopathy. We examined 11 cases of previously published pediatric myocarditis and/or DCM associated with HHV-6 and also our experience of detection of virus particles in vascular endothelium of HHV-6 positive endomyocardial biopsy tissue by electron microscopy. The exact role of the presence of HHV-6 and its load remains controversial as the virus is also found in the heart of healthy controls. Therefore, the question remains open whether and how cardiac HHV-6 may be of pathogenetic importance. Quantitative polymerase chain reaction or mRNA testing allows differentiation between low-level latent virus found in asymptomatic myocardium and active HHV-6 infection. Although only a small number of pediatric cases have been reported in literature, HHV-6 should be considered as a causative agent of inflammatory cardiomyopathy, especially in children under three who might be experiencing a primary infection. Future studies are needed to establish a threshold for determining active infection in biopsy samples and the role of coinfections other cardiotropic viruses.

  4. Chagas cardiomyopathy in the context of the chronic disease transition.

    Directory of Open Access Journals (Sweden)

    Alicia I Hidron

    2010-05-01

    Full Text Available Patients with Chagas disease have migrated to cities, where obesity, hypertension and other cardiac risk factors are common.The study included adult patients evaluated by the cardiology service in a public hospital in Santa Cruz, Bolivia. Data included risk factors for T. cruzi infection, medical history, physical examination, electrocardiogram, echocardiogram, and contact 9 months after initial data collection to ascertain mortality. Serology and PCR for Trypanosoma cruzi were performed. Of 394 participants, 251 (64% had confirmed T. cruzi infection by serology. Among seropositive participants, 109 (43% had positive results by conventional PCR; of these, 89 (82% also had positive results by real time PCR. There was a high prevalence of hypertension (64% and overweight (body mass index [BMI] >25; 67%, with no difference by T. cruzi infection status. Nearly 60% of symptomatic congestive heart failure was attributed to Chagas cardiomyopathy; mortality was also higher for seropositive than seronegative patients (p = 0.05. In multivariable models, longer residence in an endemic province, residence in a rural area and poor housing conditions were associated with T. cruzi infection. Male sex, increasing age and poor housing were independent predictors of Chagas cardiomyopathy severity. Males and participants with BMI cardiomyopathy remains an important cause of congestive heart failure in this hospital population, and should be evaluated in the context of the epidemiological transition that has increased risk of obesity, hypertension and chronic cardiovascular disease.

  5. Dominant de novo DSP mutations cause erythrokeratodermia-cardiomyopathy syndrome.

    Science.gov (United States)

    Boyden, Lynn M; Kam, Chen Y; Hernández-Martín, Angela; Zhou, Jing; Craiglow, Brittany G; Sidbury, Robert; Mathes, Erin F; Maguiness, Sheilagh M; Crumrine, Debra A; Williams, Mary L; Hu, Ronghua; Lifton, Richard P; Elias, Peter M; Green, Kathleen J; Choate, Keith A

    2016-01-15

    Disorders of keratinization (DOK) show marked genotypic and phenotypic heterogeneity. In most cases, disease is primarily cutaneous, and further clinical evaluation is therefore rarely pursued. We have identified subjects with a novel DOK featuring erythrokeratodermia and initially-asymptomatic, progressive, potentially fatal cardiomyopathy, a finding not previously associated with erythrokeratodermia. We show that de novo missense mutations clustered tightly within a single spectrin repeat of DSP cause this novel cardio-cutaneous disorder, which we term erythrokeratodermia-cardiomyopathy (EKC) syndrome. We demonstrate that DSP mutations in our EKC syndrome subjects affect localization of desmosomal proteins and connexin 43 in the skin, and result in desmosome aggregation, widening of intercellular spaces, and lipid secretory defects. DSP encodes desmoplakin, a primary component of desmosomes, intercellular adhesion junctions most abundant in the epidermis and heart. Though mutations in DSP are known to cause other disorders, our cohort features the unique clinical finding of severe whole-body erythrokeratodermia, with distinct effects on localization of desmosomal proteins and connexin 43. These findings add a severe, previously undescribed syndrome featuring erythrokeratodermia and cardiomyopathy to the spectrum of disease caused by mutation in DSP, and identify a specific region of the protein critical to the pathobiology of EKC syndrome and to DSP function in the heart and skin. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  6. Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice

    DEFF Research Database (Denmark)

    Pinto, Yigal M; Elliott, Perry M; Arbustini, Eloisa

    2016-01-01

    In this paper the Working Group on Myocardial and Pericardial Disease proposes a revised definition of dilated cardiomyopathy (DCM) in an attempt to bridge the gap between our recent understanding of the disease spectrum and its clinical presentation in relatives, which is key for early diagnosis...... and the institution of potential preventative measures. We also provide practical hints to identify subsets of the DCM syndrome where aetiology directed management has great clinical relevance....

  7. Identification of established arrhythmogenic right ventricular cardiomyopathy mutation in a patient with the contrasting phenotype of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Bainbridge, Matthew Neil; Li, Lili; Tan, Yanli; Cheong, Benjamin Y; Marian, Ali J

    2017-03-03

    Advances in the nucleic acid sequencing technologies have ushered in the era of genetic-based "precision medicine". Applications of the genetic discoveries to practice of medicine, however, are hindered by phenotypic variability of the genetic variants. The report illustrates extreme pleiotropic phenotypes associated with an established causal mutation for hereditary cardiomyopathy. We report a 61-year old white female who presented with syncope and echocardiographic and cardiac magnetic resonance (CMR) imaging findings consistent with the diagnosis of hypertrophic cardiomyopathy (HCM). The electrocardiogram, however, showed a QRS pattern resembling an Epsilon wave, a feature of arrhythmogenic right ventricular cardiomyopathy (ARVC). Whole exome sequencing (mean depth of coverage of exons 178X) analysis did not identify a pathogenic variant in the known HCM genes but identified an established causal mutation for ARVC. The mutation involves a canonical splice accepter site (c.2146-1G > C) in the PKP2 gene, which encodes plakophillin 2. Sanger sequencing confirmed the mutation. PKP2 is the most common causal gene for ARVC but has not been implicated in HCM. Findings on echocardiography and CMR during the course of 4-year follow up showed septal hypertrophy and a hyperdynamic left ventricle, consistent with the diagnosis of HCM. However, neither baseline nor follow up echocardiography and CMR studies showed evidence of ARVC. The right ventricle was normal in size, thickness, and function and there was no evidence of fibro-fatty infiltration in the myocardium. The patient carries an established pathogenic mutation for ARVC and a subtle finding of ARVC but exhibits the classic phenotype of HCM, a contrasting phenotype to ARVC. The case illustrates the need for detailed phenotypic characterization for patients with hereditary cardiomyopathies as well as the challenges physicians face in applying the genetic discoveries in practicing genetic-based "precision medicine".

  8. Individual titanium zygomatic implant

    Science.gov (United States)

    Nekhoroshev, M. V.; Ryabov, K. N.; Avdeev, E. V.

    2018-03-01

    Custom individual implants for the reconstruction of craniofacial defects have gained importance due to better qualitative characteristics over their generic counterparts – plates, which should be bent according to patient needs. The Additive Manufacturing of individual implants allows reducing cost and improving quality of implants. In this paper, the authors describe design of zygomatic implant models based on computed tomography (CT) data. The fabrication of the implants will be carried out with 3D printing by selective laser melting machine SLM 280HL.

  9. The Role of Serum Adiponectin for Outcome Prediction in Patients with Dilated Cardiomyopathy and Advanced Heart Failure

    Directory of Open Access Journals (Sweden)

    Vaida Baltrūnienė

    2017-01-01

    Full Text Available Clinical interpretation of patients’ plasma adiponectin (APN remains challenging; its value as biomarker in dilated cardiomyopathy (DCM is equivocal. We evaluated whether circulating APN level is an independent predictor of composite outcome: death, left ventricle assist device (LVAD implantation, and heart transplantation (HT in patients with nonischemic DCM. 57 patients with nonischemic DCM (average LV diastolic diameter 6.85 cm, LV ejection fraction 26.63%, and pulmonary capillary wedge pressure 22.06  mmHg were enrolled. Patients underwent echocardiography, right heart catheterization, and endomyocardial biopsy. During a mean follow-up of 33.42 months, 15 (26% patients died, 12 (21% patients underwent HT, and 8 (14% patients were implanted with LVAD. APN level was significantly higher in patients who experienced study endpoints (23.4 versus 10.9 ug/ml, p=0.01. APN was associated with worse outcome in univariate Cox proportional hazards model (HR 1.04, CI 1.02–1.07, p=0.001 but lost significance adjusting for other covariates. Average global strain (AGS is an independent outcome predictor (HR 1.42, CI 1.081–1.866, p=0.012. Increased circulating APN level was associated with higher mortality and may be an additive prognostic marker in DCM with advanced HF. Combination of serum (APN, BNP, TNF-α and echocardiographic (AGS markers may increase the HF predicting power for the nonischemic DCM patients.

  10. Dizzy Spells in a Patient with Hypertrophic Obstructive Cardiomyopathy and a Pacemaker

    OpenAIRE

    Wilschut, J. M.; de Voogt, W. G.

    2010-01-01

    Pacemaker syndrome represents the clinical consequences of the haemodynamic adverse effects of atrioventricular asynchrony during pacing. Patients suffering from hypertrophic cardiomyopathy may be particularly sensitive to these effects because of the importance of atrial systolic contribution to left ventricular diastolic filling. In this case report, we describe the symptoms and cause of pacemaker syndrome in a patient with hypertrophic obstructive cardiomyopathy.

  11. Cardiomyopathies with particular reference to the diagnostic relevance of endomyocardial biopsies

    NARCIS (Netherlands)

    Becker, A. E.

    1988-01-01

    A distinction is made between heart muscle diseases with a known cause, or occurring as part of a systemic disease, and those of unknown cause. The first category is termed "specific heart muscle disease". The term "cardiomyopathy" is then limited to the latter category. Cardiomyopathies are

  12. Beneficial effects of biventricular pacing in a patient with hypertrophic cardiomyopathy and intraventricular conduction delay

    OpenAIRE

    Rinaldi, C A; Bucknall, C A; Gill, J S

    2002-01-01

    The beneficial use of biventricular pacing is reported in a patient with hypertrophic cardiomyopathy and intraventricular conduction delay. This resulted in improvements in symptomatic status and exercise tolerance that may be related to cardiac resynchronisation. The improvement in symptoms by biventricular pacing in a patient with hypertrophic cardiomyopathy and intraventricular conduction delay is previously undocumented and requires further investigation.

  13. High prevalence of myocardial monoclonal antimyosin antibody uptake in patients with chronic idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Obrador, D.; Ballester, M.; Carrio, I.; Berna, L.; Pons-Llado, G.

    1989-01-01

    Monoclonal antimyosin antibody studies were undertaken to assess the presence of myocardial uptake in patients with chronic idiopathic dilated cardiomyopathy. Three groups were studied: 17 patients with chronic (greater than 12 months) idiopathic dilated cardiomyopathy, 12 patients with a large, poorly contracting left ventricle not due to dilated cardiomyopathy (control patients) and 8 normal individuals. The patients in the cardiomyopathy and control groups showed a similar degree of clinical and functional impairment. Imaging was undertaken 48 h after antimyosin injection. The heart/lung ratio of antimyosin uptake was used to assess the results. The mean ratio in the cardiomyopathy group was 1.83 +/- 0.36 (range 1.40 to 2.80), a value significantly higher than that obtained in the control patients without cardiomyopathy (mean 1.46 +/- 0.04, range 1.38 to 1.50) or normal subjects (mean 1.46 +/- 0.13, range 1.31 to 1.6) (p less than 0.01). No difference in the ratio was noted between the normal subjects and control patients. Abnormal antimyosin uptake was seen in 12 (70%) of the 17 patients with cardiomyopathy and in only 1 (8%) of the 12 control patients. Positive monoclonal antimyosin antibody studies are highly prevalent in chronic idiopathic dilated cardiomyopathy

  14. Stress-related cardiomyopathy in a 31-year-old woman.

    Science.gov (United States)

    Pfister, Shirley; Wagar, Phyllis; Casserly, Ivan P

    2010-10-01

    Stress-related cardiomyopathy (SRC), initially referred to as Tako-Tsubo cardiomyopathy and later as apical ballooning syndrome, has been largely observed in postmenopausal women. It is frequently precipitated by a stressful event. This is a case report of a 31-year-old woman who experienced SRC immediately after a bilateral mastectomy.

  15. Influence of Septal Thickness on the Clinical Outcome After Alcohol Septal Alation in Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Jacobsson, Linda; Almaas, Vibeke Marie

    2016-01-01

    BACKGROUND: We assessed the influence of interventricular septal thickness (IVSd) on the clinical outcome and survival after alcohol septal ablation (ASA) in patient with hypertrophic cardiomyopathy. METHODS AND RESULTS: We analyzed 531 patients with hypertrophic cardiomyopathy (age: 56±14 years...

  16. HYPERTROPHIC CARDIOMYOPATHY AS A PART OF INHERITED MALFORMATION SYNDROMES IN INFANTS

    Directory of Open Access Journals (Sweden)

    M.V. Tural'chuk

    2011-01-01

    Full Text Available The data of clinical and instrumental examination of two infantile patients with obstructive hypertrophic cardiomyopathy in association with marked multisystem involvement as a picture of inherited malformation syndromes are given.Key words: infants, hypertrophic cardiomyopathy, LEOPARD syndrome, Noonan syndrome.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3: 166–169

  17. Genetic inhibition of PKA phosphorylation of RyR2 prevents dystrophic cardiomyopathy

    NARCIS (Netherlands)

    Sarma, Satyam; Li, Na; van Oort, Ralph J.; Reynolds, Corey; Skapura, Darlene G.; Wehrens, Xander H. T.

    2010-01-01

    Aberrant intracellular Ca(2+) regulation is believed to contribute to the development of cardiomyopathy in Duchenne muscular dystrophy. Here, we tested whether inhibition of protein kinase A (PKA) phosphorylation of ryanodine receptor type 2 (RyR2) prevents dystrophic cardiomyopathy by reducing SR

  18. Current and future role of echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy

    NARCIS (Netherlands)

    Mast, Thomas P.; Teske, Arco J.; Doevendans, Pieter A.; Cramer, Maarten J.

    Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited progressive cardiomyopathy, clinically characterized by ventricular arrhythmias and increased risk of sudden cardiac death. Echocardiography has a role in the diagnosis and prognosis of ARVD/C. However, in the current

  19. Arrhythmogenic cardiomyopathy in a patient with a rare loss-of-function KCNQ1 mutation.

    Science.gov (United States)

    Xiong, Qinmei; Cao, Qing; Zhou, Qiongqiong; Xie, Jinyan; Shen, Yang; Wan, Rong; Yu, Jianhua; Yan, Sujuan; Marian, Ali J; Hong, Kui

    2015-01-23

    Ventricular tachycardia (VT) is a common manifestation of advanced cardiomyopathies. In a subset of patients with dilated cardiomyopathy, VT is the initial and the cardinal manifestation of the disease. The molecular genetic basis of this subset of dilated cardiomyopathy is largely unknown. We identified 10 patients with dilated cardiomyopathy who presented with VT and sequenced 14 common causal genes for cardiomyopathies and arrhythmias. Functional studies included cellular patch clamp, confocal microscopy, and immunoblotting. We identified nonsynonymous variants in 4 patients, including a rare missense p.R397Q mutation in the KCNQ1 gene in a 60-year-old man who presented with incessant VT and had mild cardiac dysfunction. The p.R397Q mutation was absent in an ethnically matched control group, affected a conserved amino acid, and was predicted by multiple algorithms to be pathogenic. Co-expression of the mutant KCNQ1 with its partner unit KCNE1 was associated with reduced tail current density of slowly activating delayed rectifier K(+) current (IKs). The mutation reduced membrane localization of the protein. Dilated cardiomyopathy with an initial presentation of VT may be a forme fruste of arrhythmogenic cardiomyopathy caused by mutations in genes encoding the ion channels. The findings implicate KCNQ1 as a possible causal gene for arrhythmogenic cardiomyopathy. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  20. Contemporary trends in the epidemiology and management of cardiomyopathy and pericarditis in sub‐Saharan Africa

    Science.gov (United States)

    Mayosi, Bongani M

    2007-01-01

    Heart failure in sub‐Saharan Africans is mainly due to non‐ischaemic causes, such as hypertension, rheumatic heart disease, cardiomyopathy and pericarditis. The two endemic diseases that are major contributors to the clinical syndrome of heart failure in Africa are cardiomyopathy and pericarditis. The major forms of endemic cardiomyopathy are idiopathic dilated cardiomyopathy, peripartum cardiomyopathy and endomyocardial fibrosis. Endomyocardial fibrosis, which affects children, has the worst prognosis. Other cardiomyopathies have similar epidemiological characteristics to those of other populations in the world. HIV infection is associated with occurrence of HIV‐associated cardiomyopathy in patients with advanced immunosuppression, and the rise in the incidence of tuberculous pericarditis. HIV‐associated tuberculous pericarditis is characterised by larger pericardial effusion, a greater frequency of myopericarditis, and a higher mortality than in people without AIDS. Population‐based studies on the epidemiology of heart failure, cardiomyopathy and pericarditis in Africans, and studies of new interventions to reduce mortality, particularly in endomyocardial fibrosis and tuberculous pericarditis, are needed. PMID:17890693

  1. Survival and sudden cardiac death after septal ablation for hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Kvistholm; Havndrup, Ole; Hassager, Christian

    2011-01-01

    Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse.......Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse....

  2. Cardiac resynchronization therapy in patients with end-stage hypertrophic cardiomyopathy.

    Science.gov (United States)

    Killu, Ammar M; Park, Jae-Yoon; Sara, Jaskanwal D; Hodge, David O; Gersh, Bernard J; Nishimura, Rick A; Asirvatham, Samuel J; McLeod, Christopher J

    2018-01-01

    A dilated/end-stage phase of hypertrophic cardiomyopathy (HCM) is rare but well-recognized. The role for cardiac resynchronization therapy (CRT) in this subset of patients remains unexplored. We aimed to clarify the impact of bi-ventricular pacing CRT in dilated/end-stage HCM. The Mayo Clinic HCM database was interrogated to identify patients with ejection fraction (EF) average age at CRT-D implant was 44.8 ± 14.8 years, an average of 17.3 ± 10.3 years after HCM diagnosis. The indication for CRT was based on New York Heart Association class ≥II symptoms (mean 2.7 ± 0.4) and EF  0.05 for all). There was no difference in the combined end-point of left ventricular assist device (LVAD), cardiac transplant, or death between groups (P = 0.90). At last follow-up [mean duration 12.9 ± 8.3 (median 10.7) years], 8 (89%) in the CRT group were alive. Three and 2 patients underwent LVAD implantation and cardiac heart transplantation, respectively, 15.0 ± 10.1 years from HCM diagnosis and 2.6 ± 0.9 years from CRT implant. In the control group, 4 (44.4%) patients were alive at last follow-up [mean duration 12.0 ± 7.1 (median 12.7) years]. One patient each had LVAD and cardiac transplant. CRT in patients with dilated/end-stage HCM does not appear to confer a salutary effect on ventricular function. In medium-term follow-up, however, left ventricular function did not appear to deteriorate further, yet advanced heart failure therapy was common in this group. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For Permissions, please email: journals.permissions@oup.com.

  3. Hypertrophic Obstructive Cardiomyopathy Masked by Tako-Tsubo Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Y. Daralammori

    2012-01-01

    Full Text Available Introduction. Left ventricular outflow obstruction might be part of the pathophysiological mechanism of Tako-tsubo cardiomyopathy. This obstruction can be masked by Tako-tsubo cardiomyopathy and diagnosed only by followup. Case Presentation. A 70-year-old female presented with Tako-tsubo cardiomyopathy and masked obstructive hypertrophic cardiomyopathy at presentation. Conclusion. Tako-tsubo cardiomyopathy typically presents like an acute MI and is characterized by severe, but transient, regional left ventricular systolic dysfunction. Prompt evaluation of the coronary status is, therefore, mandatory. The prognosis under medical treatment of heart failure symptoms and watchful waiting is favourable. Previous studies showed that LVOT obstruction might be part of the pathophysiological mechanism of TCM. This paper supports this theory. However, TCM may also mask any preexisting LVOT obstruction.

  4. Frequency and echocardiographic study of dilated cardiomyopathy in children presenting with cardiac failure

    International Nuclear Information System (INIS)

    Khan, M.A.; Mohammad, J.; Hussain, M.

    2004-01-01

    Objective: To evaluate the role of echocardiography in diagnosis of dilated cardiomyopathy as a cause of cardiac failure in children. Design: This was descriptive study. Children presenting with cardiac failure from indoor patients were selected and echocardiography along with chest X- ray, ECG, cardiac enzymes and ASO titre was performed in all patients. Subject: Fifty hospitalized patients with congestive heart failure were selected consecutively from hospitalized patients. Main Outcome: Role of echocardiography in the diagnosis of dilated cardiomyopathy in children presenting with cardiac failure. Results: Out of fifty patients admitted with cardiac failure 27 (54%) cases were found to be dilated cardiomyopathy while congenital heart disease, myocarditis and rheumatic heart disease were found in 12 (24%), 8 (16%) and 3 (6%) cases respectively. Conclusion: Dilated cardiomyopathy is an important cause of cardiac failure in children and echocardiography is an important tool to diagnose and differentiate dilated cardiomyopathy from other causes of cardiac failure. (author)

  5. Takotsubo Cardiomyopathy Triggered by Venous Air Embolism During Craniotomy in the Sitting Position.

    Science.gov (United States)

    Raimann, Florian; Senft, Christian; Honold, Jörg; Zacharowski, Kai; Seifert, Volker; Mersmann, Jan

    2017-11-01

    We present a case of stress-induced cardiomyopathy (Takotsubo cardiomyopathy) caused by a venous air embolism during a craniotomy performed in the sitting position. A 69-year-old woman was admitted to the neurosurgical department and scheduled for elective resection of a cerebellar metastasis in the sitting position. After craniotomy and opening of the posterior fossa, a venous air embolism was detected via transesophageal echocardiography. The patient immediately presented with cardiac decompensation with signs of takotsubo or stress-induced cardiomyopathy. Intensivists and anesthesiologists in the operating room and in intensive care units need to be aware of stress-induced cardiomyopathy as a probably underdiagnosed disease entity, especially as management differs significantly from other forms of cardiogenic shock. Diagnosis can be accomplished quickly by bedside echocardiography, emphasizing the need for availability of this tool and the integration of stress-induced cardiomyopathy in diagnostic algorithms in the intensive care unit. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. Association Between Hypertensive Disorders of Pregnancy and Later Risk of Cardiomyopathy

    DEFF Research Database (Denmark)

    Behrens, Ida; Basit, Saima; Lykke, Jacob Alexander

    2016-01-01

    whether hypertensive disorders of pregnancy are associated with cardiomyopathy beyond the peripartum period. DESIGN, SETTING, AND PARTICIPANTS: Nationwide register-based cohort study using Cox regression to compare rates of cardiomyopathy in women with and without a history of hypertensive disorders......-years [95% CI, 7.3-8.2]), women with a history of hypertensive disorders of pregnancy had significantly increased rates of cardiomyopathy (in 173,062 person-years of follow-up among women with severe preeclampsia, n = 27 cardiomyopathy events; 15.6/100,000 person-years [95% CI, 10.7-22.7]; adjusted hazard...... postpregnancy chronic hypertension. In this cohort, 11% of all cardiomyopathy events occurred in women with a history of hypertensive disorders of pregnancy. CONCLUSIONS AND RELEVANCE: Women with a history of hypertensive disorders of pregnancy, compared with women without such a history, had a small...

  7. An Upgrade on the Rabbit Model of Anthracycline-Induced Cardiomyopathy: Shorter Protocol, Reduced Mortality, and Higher Incidence of Overt Dilated Cardiomyopathy

    Science.gov (United States)

    Talavera, Jesús; Fernández-Del-Palacio, María Josefa; García-Nicolás, Obdulio; Seva, Juan; Brooks, Gavin; Moraleda, Jose M.

    2015-01-01

    Current protocols of anthracycline-induced cardiomyopathy in rabbits present with high premature mortality and nephrotoxicity, thus rendering them unsuitable for studies requiring long-term functional evaluation of myocardial function (e.g., stem cell therapy). We compared two previously described protocols to an in-house developed protocol in three groups: Group DOX2 received doxorubicin 2 mg/kg/week (8 weeks); Group DAU3 received daunorubicin 3 mg/kg/week (10 weeks); and Group DAU4 received daunorubicin 4 mg/kg/week (6 weeks). A cohort of rabbits received saline (control). Results of blood tests, cardiac troponin I, echocardiography, and histopathology were analysed. Whilst DOX2 and DAU3 rabbits showed high premature mortality (50% and 33%, resp.), DAU4 rabbits showed 7.6% premature mortality. None of DOX2 rabbits developed overt dilated cardiomyopathy; 66% of DAU3 rabbits developed overt dilated cardiomyopathy and quickly progressed to severe congestive heart failure. Interestingly, 92% of DAU4 rabbits showed overt dilated cardiomyopathy and 67% developed congestive heart failure exhibiting stable disease. DOX2 and DAU3 rabbits showed alterations of renal function, with DAU3 also exhibiting hepatic function compromise. Thus, a shortened protocol of anthracycline-induced cardiomyopathy as in DAU4 group results in high incidence of overt dilated cardiomyopathy, which insidiously progressed to congestive heart failure, associated to reduced systemic compromise and very low premature mortality. PMID:26788502

  8. A Case of Acute ST-Segment Elevation Myocardial Infarction Mimicking Stress Induced Cardiomyopathy; Demonstration of Typical Echocardiographic Finding Correlated with Unusual Distribution of Left Anterior Descending Coronary Artery.

    Science.gov (United States)

    Shin, Sung Kyun; Jin, Seon-Ah; Park, Yong Kyu; Park, Jae-Hyeong

    2010-09-01

    Stress-induced cardiomyopathy (SCMP) is diagnosed in 1-2% of patients presenting with symptoms suggestive of acute coronary syndrome. Because of sharing many common clinical features with SCMP, acute ST-segment elevation myocardial infarction (STEMI) can be misdiagnosed as SCMP. However, it can be associated with fatal outcome of the patient. Also, diagnosis of SCMP seems to be always challenging to clinicians, especially in the decision of taking coronary angiography which is still invasive and even risky. Here, we present a case with acute STEMI mimicking SCMP as a result of anatomical variation of coronary circulation. In this patient, prompt and early coronary angiography and stent implantation was very helpful.

  9. Breast Implants: Saline vs. Silicone

    Science.gov (United States)

    ... differ in material and consistency, however. Saline breast implants Saline implants are filled with sterile salt water. ... of any age for breast reconstruction. Silicone breast implants Silicone implants are pre-filled with silicone gel — ...

  10. [Biomaterials in cochlear implants].

    Science.gov (United States)

    Stöver, T; Lenarz, T

    2009-05-01

    Cochlear implants (CI) represent the "gold standard" for the treatment of congenitally deaf children and postlingually deafened adults. Thus, cochlear implantation is a success story of new bionic prosthesis development. Owing to routine application of cochlear implants in adults but also in very young children (below the age of one), high demands are placed on the implants. This is especially true for biocompatibility aspects of surface materials of implant parts which are in contact with the human body. In addition, there are various mechanical requirements which certain components of the implants must fulfil, such as flexibility of the electrode array and mechanical resistance of the implant housing. Due to the close contact of the implant to the middle ear mucosa and because the electrode array is positioned in the perilymphatic space via cochleostomy, there is a potential risk of bacterial transferral along the electrode array into the cochlea. Various requirements that have to be fulfilled by cochlear implants, such as biocompatibility, electrode micromechanics, and although a very high level of technical standards has been carried out there is still demand for the improvement of implants as well as of the materials used for manufacturing, ultimately leading to increased implant performance. General considerations of material aspects related to cochlear implants as well as potential future perspectives of implant development will be discussed.

  11. Riboflavin alleviates cardiac failure in Type I diabetic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Xue Zhao

    2011-09-01

    Full Text Available Heart failure (HF is a common and serious comorbidity of diabetes. Oxidative stress has been associated with the pathogenesis of chronic diabetic complications including cardiomyopathy. The ability of antioxidants to inhibit injury has raised the possibility of new therapeutic treatment for diabetic heart diseases. Riboflavin constitutes an essential nutrient for humans and animals and it is an important food additive. Riboflavin, a precursor of flavin mononucleotide (FMN and flavin adenine dinucleotide (FAD, enhances the oxidative folding and subsequent secretion of proteins. The objective of this study was to investigate the cardioprotective effect of riboflavin in diabetic rats. Diabetes was induced in 30 rats by a single injection of streptozotocin (STZ (70 mg /kg. Riboflavin (20 mg/kg was orally administered to animals immediately after induction of diabetes and was continued for eight weeks. Rats were examined for diabetic cardiomyopathy by left ventricular (LV remadynamic function. Myocardial oxidative stress was assessed by measuring the activity of superoxide dismutase (SOD, the level of malondialdehyde (MDA as well as heme oxygenase-1 (HO-1 protein level. Myocardial connective tissue growth factor (CTGF level was measured by Western blot in all rats at the end of the study. In the untreated diabetic rats, left ventricular systolic pressure (LVSP rate of pressure rose (+dp/dt, and rate of pressure decay (−dp/dt were depressed while left ventricular enddiastolic pressure (LVEDP was increased, which indicated the reduced left ventricular contractility and slowing of left ventricular relaxation. The level of SOD decreased, CTGF and HO-1 protein expression and MDA content rose. Riboflavin treatment significantly improved left ventricular systolic and diastolic function in diabetic rats, there were persistent increases in significant activation of SOD and the level of HO-1 protein, and a decrease in the level of CTGF. These results suggest

  12. Efficacy of Gd-DTPA-enhanced MRI in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Okamoto, Shinya; Aoki, Toshikazu; Konishi, Tokuji; Nakano, Takeshi; Yamakado, Kyoichiro; Sakuma, Hajime; Takeda, Kann; Nakagawa, Takashi

    1991-01-01

    The cabability of magnetic resonance (MR) imaging to detect tissue characterization or myocardial degeneration process of the hypertrophied myocardium was evaluated in 15 patients with hypertrophic cardiomyopathy. T1-weighted MR images were obtained with a 1.5 T MR unit by using ECG-gated spin-echo techniques. MR images were visually reviewed before and after enhancement of Gd-DTPA. Four patients had an increase in signal intensity mainly in the endocardium of the left ventricular septum on non-enhanced MR images, 3 of whom had widespread high intensity in addition to two-thirds of the wall. Gd-DTPA enhanced-MR images showed high intensity over the whole septum in 5 patients and also in the antero-lateral endocardium in 4 patients. Decreased intensity on non-enhanced MR images, as shown in 4 patients, became clear on enhanced-MR images. According to findings on enhanced-MR images, signal intensity was defined as normal (N), septum (S), and diffuse (D). Patients in Group D tended to be younger and have more frequently family history. Regarding both interventricular septum thickness and left ventricular posterior wall thickness, there was no significant difference among the three groups. Both left ventricular diastolic diameter and left ventricular systolic diameter were significantly larger in Group D than the other two groups. Left ventricular ejection fraction was significantly lower in both Group S and Group D. Widespread abnormal intensity on Gd-DTPA enhanced MR images was associated with findings similar to dilated cardiomyopathy, such as dilated left ventricular lumen and decreased ejection fraction. Gd-DTPA enhanced MR imaging seemed to be useful for visualizing myocardial degeneration in hypertrophic cardiomyopathy.(N.K.)

  13. Simultaneous interstitial pneumonitis and cardiomyopathy induced by venlafaxine

    Directory of Open Access Journals (Sweden)

    Pedro Gonçalo Ferreira

    2014-06-01

    Full Text Available Venlafaxine is a serotonin-norepinephrine reuptake inhibitor used as an antidepressant. Interindividual variability and herb-drug interactions can lead to drug-induced toxicity. We report the case of a 35-year-old female patient diagnosed with synchronous pneumonitis and acute cardiomyopathy attributed to venlafaxine. The patient sought medical attention due to dyspnea and dry cough that started three months after initiating treatment with venlafaxine for depression. The patient was concomitantly taking Centella asiatica and Fucus vesiculosus as phytotherapeutic agents. Chest CT angiography and chest X-ray revealed parenchymal lung disease (diffuse micronodules and focal ground-glass opacities and simultaneous dilated cardiomyopathy. Ecocardiography revealed a left ventricular ejection fraction (LVEF of 21%. A thorough investigation was carried out, including BAL, imaging studies, autoimmune testing, right heart catheterization, and myocardial biopsy. After excluding other etiologies and applying the Naranjo Adverse Drug Reaction Probability Scale, a diagnosis of synchronous pneumonitis/cardiomyopathy associated with venlafaxine was assumed. The herbal supplements taken by the patient have a known potential to inhibit cytochrome P450 enzyme complex, which is responsible for the metabolization of venlafaxine. After venlafaxine discontinuation, there was rapid improvement, with regression of the radiological abnormalities and normalization of the LVEF. This was an important case of drug-induced cardiopulmonary toxicity. The circumstantial intake of inhibitors of the CYP2D6 isoenzyme and the presence of a CYP2D6 slow metabolism phenotype might have resulted in the toxic accumulation of venlafaxine and the subsequent clinical manifestations. Here, we also discuss why macrophage-dominant phospholipidosis was the most likely mechanism of toxicity in this case.

  14. Arrhythmogenic Right Ventricular Cardiomyopathy in the Boxer Dog: An Update.

    Science.gov (United States)

    Meurs, Kathryn M

    2017-09-01

    Arrhythmogenic right ventricular cardiomyopathy is an inheritable form of myocardial disease characterized most commonly by ventricular tachycardias, syncope, and sometimes systolic dysfunction and heart failure. A genetic mutation in the striatin gene has been identified in many affected dogs. Dogs with only one copy of the mutation (heterozygous) have a variable prognosis, with many dogs remaining asymptomatic or being successfully managed on antiarrhythmic drugs for years. Dogs that are homozygous for the mutation seem to have a worse prognosis. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. An unusual cardiomyopathy after physical stress in a child.

    Science.gov (United States)

    Fabi, Marianna; Testa, Gabriella; Gesuete, Valentina; Balducci, Anna; Ragni, Luca

    2013-01-01

    Takotsubo cardiomyopathy, or broken heart syndrome, is characterized by transient left ventricular dysfunction associated to chest pain, elevation of cardiac enzymes, and electrocardiographic changes, mimicking an acute coronary syndrome, especially in older women after a physical or emotional stress. It is extremely infrequent in children as well as after infective stress. We described a celiac 4-year-old girl, following a gluten-free diet, who developed features of cardiac failure few days after episodes of acute diarrhea with fever. The patient was treated with oral anticongestive therapy and intravenous immunoglobulins, and she had a dramatic and rapid improvement; echocardiographic features normalized in 48 hours. © 2012 Wiley Periodicals, Inc.

  16. Collecting, Analyzing, and Publishing Massive Data about the Hypertrophic Cardiomyopathy

    Science.gov (United States)

    Montserrat, Lorenzo; Cotelo-Lema, Jose Antonio; Luaces, Miguel R.; Seco, Diego

    We present in this paper the architecture and some implementation details of a Document Management System and Workflow to help in the diagnosis of the hypertrophic cardiomyopathy, one of the most frequent genetic cardiovascular diseases. The system allows a gradual and collaborative creation of a knowledge base about the mutations associated with this disease. The system manages both the original documents of the scientific papers and the data extracted from these papers by the experts. Furthermore, a semiautomatic report generation module exploits this knowledge base to create high quality reports about the studied mutations.

  17. Genotype-specific pathogenic effects in human dilated cardiomyopathy.

    Science.gov (United States)

    Bollen, Ilse A E; Schuldt, Maike; Harakalova, Magdalena; Vink, Aryan; Asselbergs, Folkert W; Pinto, Jose R; Krüger, Martina; Kuster, Diederik W D; van der Velden, Jolanda

    2017-07-15

    Mutations in genes encoding cardiac troponin I (TNNI3) and cardiac troponin T (TNNT2) caused altered troponin protein stoichiometry in patients with dilated cardiomyopathy. TNNI3 p.98trunc resulted in haploinsufficiency, increased Ca 2+ -sensitivity and reduced length-dependent activation. TNNT2 p.K217del caused increased passive tension. A mutation in the gene encoding Lamin A/C (LMNA p.R331Q ) led to reduced maximal force development through secondary disease remodelling in patients suffering from dilated cardiomyopathy. Our study shows that different gene mutations induce dilated cardiomyopathy via diverse cellular pathways. Dilated cardiomyopathy (DCM) can be caused by mutations in sarcomeric and non-sarcomeric genes. In this study we defined the pathogenic effects of three DCM-causing mutations: the sarcomeric mutations in genes encoding cardiac troponin I (TNNI3 p.98truncation ) and cardiac troponin T (TNNT2 p.K217deletion ; also known as the p.K210del) and the non-sarcomeric gene mutation encoding lamin A/C (LMNA p.R331Q ). We assessed sarcomeric protein expression and phosphorylation and contractile behaviour in single membrane-permeabilized cardiomyocytes in human left ventricular heart tissue. Exchange with recombinant troponin complex was used to establish the direct pathogenic effects of the mutations in TNNI3 and TNNT2. The TNNI3 p.98trunc and TNNT2 p.K217del mutation showed reduced expression of troponin I to 39% and 51%, troponin T to 64% and 53%, and troponin C to 73% and 97% of controls, respectively, and altered stoichiometry between the three cardiac troponin subunits. The TNNI3 p.98trunc showed pure haploinsufficiency, increased Ca 2+ -sensitivity and impaired length-dependent activation. The TNNT2 p.K217del mutation showed a significant increase in passive tension that was not due to changes in titin isoform composition or phosphorylation. Exchange with wild-type troponin complex corrected troponin protein levels to 83% of controls in the TNNI3

  18. Genotype‐specific pathogenic effects in human dilated cardiomyopathy

    Science.gov (United States)

    Schuldt, Maike; Harakalova, Magdalena; Vink, Aryan; Asselbergs, Folkert W.; Pinto, Jose R.; Krüger, Martina; Kuster, Diederik W. D.; van der Velden, Jolanda

    2017-01-01

    Key points Mutations in genes encoding cardiac troponin I (TNNI3) and cardiac troponin T (TNNT2) caused altered troponin protein stoichiometry in patients with dilated cardiomyopathy. TNNI3p.98trunc resulted in haploinsufficiency, increased Ca2+‐sensitivity and reduced length‐dependent activation. TNNT2p.K217del caused increased passive tension.A mutation in the gene encoding Lamin A/C (LMNA p.R331Q) led to reduced maximal force development through secondary disease remodelling in patients suffering from dilated cardiomyopathy.Our study shows that different gene mutations induce dilated cardiomyopathy via diverse cellular pathways. Abstract Dilated cardiomyopathy (DCM) can be caused by mutations in sarcomeric and non‐sarcomeric genes. In this study we defined the pathogenic effects of three DCM‐causing mutations: the sarcomeric mutations in genes encoding cardiac troponin I (TNNI3p.98truncation) and cardiac troponin T (TNNT2p.K217deletion; also known as the p.K210del) and the non‐sarcomeric gene mutation encoding lamin A/C (LMNAp.R331Q). We assessed sarcomeric protein expression and phosphorylation and contractile behaviour in single membrane‐permeabilized cardiomyocytes in human left ventricular heart tissue. Exchange with recombinant troponin complex was used to establish the direct pathogenic effects of the mutations in TNNI3 and TNNT2. The TNNI3p.98trunc and TNNT2p.K217del mutation showed reduced expression of troponin I to 39% and 51%, troponin T to 64% and 53%, and troponin C to 73% and 97% of controls, respectively, and altered stoichiometry between the three cardiac troponin subunits. The TNNI3p.98trunc showed pure haploinsufficiency, increased Ca2+‐sensitivity and impaired length‐dependent activation. The TNNT2p.K217del mutation showed a significant increase in passive tension that was not due to changes in titin isoform composition or phosphorylation. Exchange with wild‐type troponin complex corrected troponin protein levels to 83% of

  19. Tetralogy of Fallot and hypertrophic cardiomyopathy: a rare association

    Directory of Open Access Journals (Sweden)

    Carvalho Angela Maria Férrer

    2003-01-01

    Full Text Available Tetralogy of Fallot is known as the most common cyanotic congenital heart disease and has a prevalence of 10% of all congenital heart diseases. Although many other heart anomalies may coexist, the association of tetralogy of Fallot and hypertrophic cardiomyopathy is extremely rare. We report this association in a 15-month-old female, cyanotic since birth, in her first hospital admission for diagnosis and treatment of recurring cyanotic crises. In addition, a review of the literature and of the problems related to the treatment is provided.

  20. Telomere shortening and telomerase activity in ischaemic cardiomyopathy patients

    DEFF Research Database (Denmark)

    Sawhney, V; Campbell, N G; Brouilette, S W

    2016-01-01

    associated with incidence of ICD therapy (p=0.02, p=0.02). ROC analyses demonstrated that the sensitivity and specificity of these telomere dynamics in predicting potentially-fatal VA was higher than the current gold-standard - left ventricular ejection fraction (AUC 0.82 versus 0.47). CONCLUSION: The load......-of-short telomeres and telomerase activity had a significant association with ICD therapy (for VA) in ischaemic cardiomyopathy patients. These biomarkers should be tested in prospective studies to assess their clinical utility in predicting VA after myocardial infarction and guiding primary prevention ICD...

  1. First Reported Case of Arrhythmogenic Right Ventricular Cardiomyopathy in Oman

    Directory of Open Access Journals (Sweden)

    Hatim Al Lawati

    2017-07-01

    Full Text Available Arrhythmogenic right ventricular cardiomyopathy (ARVC is a rare genetic disorder characterized by fatty degeneration of the right ventricular myocardium with variable involvement of the left ventricle. The condition is associated with exercise-mediated ventricular tachycardia and is one of the recognized causes of sudden cardiac death in the young and in athletes. Here, we report the first confirmed case of ARVC in Oman and present its electrocardiographic, echocardiographic features, and radiological findings on gated, contrast-enhanced cardiac computed tomography. Our patient was a 22-year-old male who had presented to our hospital for evaluation and investigation of syncope and symptomatic palpitations.

  2. FOLFOX Induced Takotsubo Cardiomyopathy Treated with Impella Assist Device

    Directory of Open Access Journals (Sweden)

    Swethika Sundaravel

    2017-01-01

    Full Text Available Chemotherapy induced cardiotoxicity is becoming increasingly prevalent with several new agents being used recently. The incidence of Takotsubo cardiomyopathy due to 5-fluorouracil based chemotherapeutic regimens like FOLFOX is not uncommon. It is also seen with platinum based chemotherapy. Most of these patients have reversible cardiotoxicity and the cardiac function recovers within a short period with supportive treatment. Here we have a patient who presented with cardiogenic shock after 5 days of receiving FOLFOX regimen for colorectal adenocarcinoma. She was treated with a percutaneous left ventricular assist device, Impella CP, for hemodynamic support with excellent outcome.

  3. Hypertrophic Cardiomyopathy in Children, Adolescents, and Young Adults Associated With Low Cardiovascular Mortality With Contemporary Management Strategies.

    Science.gov (United States)

    Maron, Barry J; Rowin, Ethan J; Casey, Susan A; Lesser, John R; Garberich, Ross F; McGriff, Deepa M; Maron, Martin S

    2016-01-05

    Youthful age has been considered the time of greatest risk for patients with hypertrophic cardiomyopathy (HCM), largely because of the possibility of sudden death. The last 2 decades have witnessed more reliable identification of at-risk patients and utilization of implantable cardioverter-defibrillators for prevention of sudden death, and other contemporary treatment options. Whether such management advances have significantly altered the considerable mortality rate for young HCM patients remains unresolved. We studied long-term outcome in 474 consecutive HCM patients between 7 and 29 years of age presenting at 2 referral institutions. Over 7.1±5.1 years of follow-up (6.0 [3.0, 10.0]), 452 patients (95%) survived, with 95% experiencing no or mild symptoms. HCM-related death occurred in 18 patients (3%; 0.54%/y): arrhythmic sudden death (n=12), progressive heart failure and heart transplant complications (n=5), or postoperatively (n=1). In contrast, aborted life-threatening events occurred in 63 other high-risk patients (13%) with implantable cardioverter-defibrillator interventions for ventricular tachyarrhythmias (n=31), resuscitated out-of-hospital cardiac arrest (n=20), or heart transplant for advanced heart failure (n=12), 1.8%/y, 3-fold higher than HCM mortality. Five- and 10-year survival (considering only HCM deaths) was high (97% and 94%, respectively), virtually identical to that reported in middle-aged adult HCM patients (98% and 94%, P=0.23). In a large hospital-based cohort of young HCM patients, representing an age group considered at greatest risk, low mortality rates can be achieved with the application of contemporary cardiovascular treatment strategies, largely because of reliable identification of high-risk patients who benefited from implantable cardioverter-defibrillators for sudden death prevention, thereby creating the opportunity for extended longevity and good quality of life. © 2015 American Heart Association, Inc.

  4. Implantable cardioverter-defibrillator

    Science.gov (United States)

    ... medlineplus.gov/ency/article/007370.htm Implantable cardioverter-defibrillator To use the sharing features on this page, please enable JavaScript. An implantable cardioverter-defibrillator (ICD) is a device that detects any life- ...

  5. Implantable cardioverter defibrillator - discharge

    Science.gov (United States)

    ... medlineplus.gov/ency/patientinstructions/000108.htm Implantable cardioverter defibrillator - discharge To use the sharing features on this page, please enable JavaScript. An implantable cardioverter-defibrillator (ICD) is a device that detects a life- ...

  6. About Implantable Contraception

    Science.gov (United States)

    ... a tube was inserted, and get a new contraceptive implant on schedule or switch to another method ... STDs. Possible Side Effects Young women who get contraceptive implants might notice such side effects as: irregular ...

  7. Urinary incontinence - collagen implants

    Science.gov (United States)

    ... gov/ency/article/007373.htm Urinary incontinence - injectable implant To use the sharing features on this page, please enable JavaScript. Injectable implants are injections of material into the urethra to ...

  8. Moessbauer spectroscopy of implanted sources

    International Nuclear Information System (INIS)

    Niesen, L.

    1983-01-01

    A review is given of the field of Moessbauer spectroscopy of ion-implanted sources. After an introduction to the various aspects of the ion-implantation method, the following topics are treated: final site selection of implanted impurities; trapping of defects at implanted ions; on-line implantation; implantation in metals, semiconductors and insulators. (Auth.)

  9. Implantable electronic medical devices

    CERN Document Server

    Fitzpatrick, Dennis

    2014-01-01

    Implantable Electronic Medical Devices provides a thorough review of the application of implantable devices, illustrating the techniques currently being used together with overviews of the latest commercially available medical devices. This book provides an overview of the design of medical devices and is a reference on existing medical devices. The book groups devices with similar functionality into distinct chapters, looking at the latest design ideas and techniques in each area, including retinal implants, glucose biosensors, cochlear implants, pacemakers, electrical stimulation t

  10. COCHLEAR IMPLANTATION: MY EXPERIENCE

    OpenAIRE

    Shankar

    2015-01-01

    Cochlear implant is a small, surgically implanted complex electronic device that can help to provide a sense of sound to a person with severe to profound sensorineural hearing loss. This type of hearing loss, typically involves damage to hair cells in the cochlea, as a result sound cannot reach the auditory nerve which usually receives information from hair cells. A cochlear implant skips the damaged hair cells and to stimulate the auditory nerve directly. An implant does not rest...

  11. Diagnosis of cardiomyopathies: tips and tricks for internists and general practitioners

    Directory of Open Access Journals (Sweden)

    Giuseppe Palmiero

    2017-04-01

    Full Text Available Cardiomyopathies are little known to internists and general practitioners (GPs, and not always able to arouse the interest of cardiologists. Probably, this happens because cardiomyopathies are perceived as rare and complex disorders, a prerogative of a few dedicated centers. This may partly explain why the diagnosis of cardiomyopathy is often missed and, consequently, why cardiomyopathies are largely underdiagnosed. Internists and general practitioners should have an interest in these conditions, because cardiomyopathies are not as rare as generally perceived, and because their complexity can be unravelled with knowledge and methodology. Cardiomyopathies are defined as myocardial disorders in which the heart is structurally and functionally abnormal in the absence of coronary artery disease or abnormal loading conditions. Irrespective of the cardiac imaging technique used, a limited number of phenotypes are defined based on ventricular morphology and function. These basic phenotypes include hypertrophic, dilated, restrictive and right ventricular arrhythmogenic cardiomyopathies. Aim of this review is to describe a simplified approach to the detection of the underlying causes of specific phenotypes. We will focus our attention on the basic phenotypes, presenting a diagnostic work-up and a suggestive clinical case for each phenotype.

  12. Cell therapy in dilated cardiomyopathy: from animal models to clinical trials

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    C. del Corsso

    2011-05-01

    Full Text Available Dilated cardiomyopathy can be the end-stage form and common denominator of several cardiac disorders of known cause, such as hypertensive, ischemic, diabetic and Chagasic diseases. However, some individuals have clinical findings, such as an increase in ventricular chamber size and impaired contractility (classical manifestations of dilated cardiomyopathy even in the absence of a diagnosed primary disease. In these patients, dilated cardiomyopathy is classified as idiopathic since its etiology is obscure. Nevertheless, regardless of all of the advances in medical, pharmacological and surgical procedures, the fate of patients with dilated cardiomyopathy (of idiopathic or of any other known cause is linked to arrhythmic episodes, severe congestive heart failure and an increased risk of sudden cardiac death. In this review, we will summarize present data on the use of cell therapies in animal models of dilated cardiomyopathies and will discuss the few clinical trials that have been published so far involving patients affected by this disease. The animal models discussed here include those in which the cardiomyopathy is produced by genetic manipulation and those in which disease is induced by chemical or infectious agents. The specific model used clearly creates restrictions to translation of the proposed cell therapy to clinical practice, insofar as most of the clinical trials performed to date with cell therapy have used autologous cells. Thus, translation of genetic models of dilated cardiomyopathy may have to wait until the use of allogeneic cells becomes more widespread in clinical trials of cell therapies for cardiac diseases.

  13. Identification of high risk patients with hypertrophic cardiomyopathy in a northern Greek population

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    Karvounis Charalambos

    2009-07-01

    Full Text Available Abstract Background The percentage of hypertrophic cardiomyopathy (HCM patients who are in high risk for Sudden Death (SD constitutes only a minority of all HCM population but the incidence of SD in this subset is high (at least 5% annually. The identification of this small but important proportion of high risk HCM patients has been the clue in the clinical evaluation of these patients. Methods Our study cohort consisted from 123 patients with HCM who are currently followed up in our Institution. Five clinical risk factors were assessed: a family history of premature SD, unexplained syncope, Non Sustained Ventricular Tachycardia (NSVT on 24-h ECG monitoring, Abnormal Blood Pressure Response (ABPR during upright exercise testing and Maximum left ventricular Wall Thickness (MWT ≥30 mm. The purpose of our study was the identification of high risk HCM patients coming from Northern Greece. Results Fifteen patients (12.2% of the whole cohort had MWT ≥ 30 mm, 30 patients (24.4% had an ABPR to exercise, 17 patients (13.8% had episodes of NSVT in 24-h Holter monitoring, 17 patients (13.8% suffered from syncope, and 8 patients (6.5% had a positive family history of premature SD. Data analysis revealed that 74 patients (60.1% had none risk factor. Twenty four patients (19.5% had 1 risk factor, 17 patients (13.8% had 2 risk factors, 4 patients (3.25% had 3 risk factors, and 4 patients (3.25% had 4 risk factors, while none patient had 5 risk factors. Twenty five patients (20.3% had 2 or more risk factors. Conclusion This study for the first time confirms that, although a 60% of patients with HCM coming from a regional Greek population are in low risk for SD, a substantial proportion (almost 20% carries a high risk for SD justifying prophylactic therapy with amiodaron or ICD implantation.

  14. The value of myocardial perfusion imaging in differentiating between idiopathic dilated cardiomyopathy from the ischemic form

    International Nuclear Information System (INIS)

    Fad, A.; Emami, F.; Eftekhari, M.; Saghari, M.; Fallahi, B.; Beiki, D.; Tkavar, A.

    2004-01-01

    Introduction: differentiating between ischemic cardiomyopathy and idiopathic dilated cardiomyopathy is important as coronary revascularization can improve prognosis in the ischemic subgroup. Due to inherent problems of coronary angiography in patients with depressed ejection fraction introducing a noninvasive tool to diagnose those who will benefit from angiography seems to be rewarding. We examined usefulness of myocardial perfusion scan in this group of patients. Materials and methods: study was performed on 64 patients (62 male and 2 female) aged 57.1 ± 6.7 y (mean ± SD) all with dilation of the left ventricular cavity and ejection fraction less than 40 % by echocardiography. Myocardial perfusion scan was performed in stress and rest phases. All the patients had coronary angiography which was used as the gold standard test. On each set of images, heart was arbitrary divided into 17 segments and perfusion abnormality in each segment was scored by a 5 grade scoring system (0-4). Summed stress Score was used as the scan criteria to differentiate dilated ischemic from idiopathic cardiomyopathy. Scores more than 17 were considered ischemic, and less than that, idiopathic. Results were compared with angiography. Results: from total 40 cases of ischemic cardiomyopathy (proved by angiography) 39 were correctly diagnosed by scan and only one case was mis categorized as idiopathic dilated cardiomyopathy . All 24 cases of idiopathic dilated cardiomyopathy were correctly diagnosed by scintigraphy. Sensitivity, specificity, positive predictive value, and negative predictive value of myocardial perfusion imaging for discrimination between ischemic and idiopathic dilated cardiomyopathy were 97.5 %, 100 %, 100 %, and 96 % respectively. Conclusion: Considering excellent accuracy of myocardial perfusion scan with scoring system in discrimination of ischemic dilated cardiomyopathy from idiopathic cardiomyopathy, this noninvasive test could be considered the main diagnostic test

  15. Women's experiences of Takotsubo cardiomyopathy in a short-term perspective--a qualitative content analysis.

    Science.gov (United States)

    Dahlviken, Rønnaug M; Fridlund, Bengt; Mathisen, Lars

    2015-06-01

    Takotsubo cardiomyopathy is a reversible condition mimicking acute myocardial infarction. The phenomenon is associated with emotional and physical stressful trigger events. Evidence-based patient counselling should be based on disease-specific knowledge of patient experiences. The aim of the study was to describe women's experiences of Takotsubo cardiomyopathy in a short-term perspective. The study design was explorative and descriptive. Semi-structured interviews were conducted with 14 women diagnosed with Takotsubo cardiomyopathy, 1 day to 9 months after hospitalisation. The transcriptions underwent qualitative content analysis. The main theme that emerged was Takotsubo cardiomyopathy as a continuous process of making sense and adapting. To begin with, understanding and coping with signs and symptoms were described as having a diversity of signs and symptoms, taking actions towards signs and symptoms, receiving treatment for suspected ST/non ST-elevation myocardial infarction diagnosis and finally being diagnosed with Takotsubo cardiomyopathy. Understanding the context of illness was expressed as getting treated for Takotsubo cardiomyopathy diagnosis and having previous stressful conditions of life. The changing perspective that emanated was a combination of having prospects and expectations and experiencing limitations. Finally, managing to live with Takotsubo cardiomyopathy was manifested as returning home with the illness and receiving follow-up health care. Information on regaining prior health status and capacity within a short-term perspective may not be accurate. These women struggle and require education and counselling from healthcare professionals to comprehend and manage having a Takotsubo cardiomyopathy diagnosis. Women experiencing Takotsubo cardiomyopathy may be a target group for holistic and individual health care with a longer duration of follow-up. © 2014 Nordic College of Caring Science.

  16. Left Atrial Function in Patients with Chronic Chagasic Cardiomyopathy.

    Science.gov (United States)

    Fragata, Claudia da Silva; Matsumoto, Afonso Y; Ramires, Felix J A; Fernandes, Fabio; Buck, Paula de Cássia; Salemi, Vera Maria C; Nastari, Luciano; Mady, Charles; Ianni, Barbara Maria

    2015-07-01

    Chagas disease is a cause of dilated cardiomyopathy, and information about left atrial (LA) function in this disease still lacks. To assess the different LA functions (reservoir, conduit and pump functions) and their correlation with the echocardiographic parameters of left ventricular (LV) systolic and diastolic functions. 10 control subjects (CG), and patients with Chagas disease as follows: 26 with the indeterminate form (GI); 30 with ECG alterations (GII); and 19 with LV dysfunction (GIII). All patients underwent M-mode and two-dimensional echocardiography, pulsed-wave Doppler and tissue Doppler imaging. Reservoir function (Total Emptying Fraction: TEF): (p <0.0001), lower in GIII as compared to CG (p = 0.003), GI (p <0.001) and GII (p <0.001). Conduit function (Passive Emptying Fraction: PEF): (p = 0.004), lower in GIII (GIII and CG, p = 0.06; GI and GII, p = 0.06; and GII and GIII, p = 0.07). Pump function (Active Emptying Fraction: AEF): (p = 0.0001), lower in GIII as compared to CG (p = 0.05), GI (p<0.0001) and GII (p = 0.002). There was a negative correlation of E/e' (average) with the reservoir and pump functions (TEF and AEF), and a positive correlation of e' (average) with s' wave (both septal and lateral walls) and the reservoir, conduit and pump LA functions. An impairment of LA functions in Chagas cardiomyopathy was observed.

  17. The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results.

    Science.gov (United States)

    Cardim, Nuno; Brito, Dulce; Rocha Lopes, Luís; Freitas, António; Araújo, Carla; Belo, Adriana; Gonçalves, Lino; Mimoso, Jorge; Olivotto, Iacopo; Elliott, Perry; Madeira, Hugo

    2018-01-01

    We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease. Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. An Unusual Presentation of a Myocardial Crypt in Hypertrophic Cardiomyopathy

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    Danny A. J. P. van de Sande

    2014-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is a common inherited cardiovascular disease with prevalence of 0.2% in the population. More than 1000 mutations in more than 10 genes encoding for proteins of the cardiac sarcomere have been identified. Cardiac magnetic resonance imaging (CMR is used to characterize left ventricular morphology with great precision in patients with HCM and it identifies unique structural abnormalities in patients with HCM. We present a case of a 56-year-old man who had positive family history of HCM who was a carrier of the genetic MYH-7 2770 G > C, exon 23 mutation. Transthoracic echocardiography showed thickening of the interventricular septum (16 mm and in particular the basal septum. CMR confirmed the diagnosis of HCM in the anteroseptal myocardium with a thickness of 23 mm and also revealed large and deep myocardial crypts in the anterior wall. These myocardial crypts are rarely found in the so-called genotype positive and phenotype positive patients, as in our case. Also the crypts in this case are deeper and wider than those reported in other cases. So in conclusion, this case reveals an uncommon finding of a myocardial crypt at an unusual myocardial site with the unusual morphology in a patient with genotypic and phenotypic expression of hypertrophic cardiomyopathy.

  19. Climatic variables in Takotsubo cardiomyopathy: role of temperature.

    Science.gov (United States)

    Novo, Giuseppina; Mariano, Enrica; Giambanco, Salvatore; Bonomo, Vito; Manno, Girolamo; Viele, Annalisa; Evola, Salvatore; Giambanco, Francesco; Assennato, Pasquale; Novo, Salvatore; Romeo, Francesco

    2017-03-01

    Recent studies documented a seasonal (summer) and circadian (morning) temporal distribution of takotsubo cardiomyopathy (TTC). The aim of our study was to investigate whether there is a relationship among season, temperature and the occurrence of TTC. A second aim of our study was the comparison of climatic variables in Takotsubo cardiomyopathy versus acute myocardial infarction (AMI). We enrolled consecutive patients with TTC in three Italian centres and, for comparison consecutive patients with AMI. The frequency of TTC and AMI patients according to month, season and quartiles of temperature (I quartile: 9.8-15°, II quartile: 15-19°, III quartile: 19-25° and IV quartile: 25-38°C) was reported. Climatic variables of TTC and AMI patients were compared. We included in the study 85 patients with TTC and 900 patients with AMI. It was not observed a significant peak in the occurrence of TTC during summer time; however, when compared with AMI, TTC was more frequent in summer. We found an absolute higher frequency of TTC cases with warmer temperatures. TTC cases occurred during warmer temperatures than AMI. Our study does not confirm a summer preference for TTC occurrence, as reported by previous studies, even if, compared with AMI, TTC is more frequent in summer. During warmest days, it was recorded the highest incidence of TTC.

  20. Left Atrial Function in Patients with Chronic Chagasic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Claudia da Silva Fragata

    2015-01-01

    Full Text Available Background: Chagas disease is a cause of dilated cardiomyopathy, and information about left atrial (LA function in this disease still lacks. Objective: To assess the different LA functions (reservoir, conduit and pump functions and their correlation with the echocardiographic parameters of left ventricular (LV systolic and diastolic functions. Methods: 10 control subjects (CG, and patients with Chagas disease as follows: 26 with the indeterminate form (GI; 30 with ECG alterations (GII; and 19 with LV dysfunction (GIII. All patients underwent M-mode and two-dimensional echocardiography, pulsed-wave Doppler and tissue Doppler imaging. Results: Reservoir function (Total Emptying Fraction: TEF: (p <0.0001, lower in GIII as compared to CG (p = 0.003, GI (p <0.001 and GII (p <0.001. Conduit function (Passive Emptying Fraction: PEF: (p = 0.004, lower in GIII (GIII and CG, p = 0.06; GI and GII, p = 0.06; and GII and GIII, p = 0.07. Pump function (Active Emptying Fraction: AEF: (p = 0.0001, lower in GIII as compared to CG (p = 0.05, GI (p<0.0001 and GII (p = 0.002. There was a negative correlation of E/e’ average with the reservoir and pump functions (TEF and AEF, and a positive correlation of e’ average with s’ wave (both septal and lateral walls and the reservoir, conduit and pump LA functions. Conclusion: An impairment of LA functions in Chagas cardiomyopathy was observed.

  1. Characterisation of peripartum cardiomyopathy by cardiac magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Mouquet, Frederic; Groote, Pascal de; Bouabdallaoui, Nadia; Dagorn, Joel; Lamblin, Nicolas; Bauters, Christophe [Pole de Cardiologie et Maladies Vasculaires, CHRU Lille et Universite Lille 2, Lille Cedex (France); Lions, Christophe; Willoteaux, Serge; Beregi, Jean Paul [Radiologie et Imagerie Cardiovasculaire, CHRU Lille et Universite Lille 2, Lille Cedex (France); Deruelle, Philippe [Gynecologie-Maternite, CHRU Lille et Universite Lille 2, Lille Cedex (France)

    2008-12-15

    Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure. Only half of the patients recover normal cardiac function. We assessed the usefulness of magnetic resonance imaging (MRI) and late enhancement imaging to detect myocardial fibrosis in order to predict cardiac function recovery in patients with peripartum cardiomyopathy. Among a consecutive series of 1,037 patients referred for heart failure treatment or prognostic evaluation between 1999 and 2006, eight women had confirmed PPCM. They all underwent echocardiography and cardiac MRI for assessment of left ventricular anatomy, systolic function and detection of myocardial fibrosis through late enhancement imaging. Mean ({+-} SD) baseline left ventricular ejection fraction (LVEF) was 28 {+-} 4%. After a follow-up of 50 {+-} 9 months, half the patients recovered normal cardiac function (LVEF = 58 {+-} 4%) and four did not (LVEF = 35 {+-} 6%). None of the eight patients exhibited abnormal myocardial late enhancement. No difference in MRI characteristics was observed between the two groups. Patients with PPCM do not exhibit a specific cardiac MRI pattern and particularly no myocardial late enhancement. It suggests that myocardial fibrosis does not play a major role in the limitation of cardiac function recovery after PPCM. (orig.)

  2. Brain natriuretic peptide and left ventricular dysfunction in chagasic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Andre Talvani

    2004-10-01

    Full Text Available Global left ventricular (LV systolic dysfunction is the strongest predictor of morbidity and mortality in Chagas disease. Echocardiography is considered the gold standard for the detection of LV dysfunction, but not always available in endemic areas where chagasic cardiomyopathy is most common. Brain natriuretic peptide (BNP is a neurohormone that has been recently described as a simple and inexpensive diagnostic and prognostic marker for patients with congestive heart failure. Chagasic patients (n = 63 and non-infected healthy individuals (n = 18 were recruited prospectively and underwent complete clinical examination, echocardiography and 24-h Holter monitoring. BNP was measured from thawed plasma samples using the Triage BNP test. We observed high levels of BNP in association with depression of LV ejection fraction, with increase of LV end-diastolic diameter and with LV premature complexes. An elevated concentration of BNP, defined as a concentration of 60 pg/ml or more, had a sensitivity of 91.7%, specificity of 82.8%, positive predictive value of 52.4%, and negative predictive value of 98% for detecting LV dysfunction (LV ejection fraction < 40%.BNP measurement using a simple, relatively inexpensive and rapid test has a promising role in identifying LV dysfunction associated with chagasic cardiomyopathy. Equally important, patients with Trypanosoma cruzi infection who have low levels of BNP level in plasma have a very low likelihood of severe cardiac involvement, and echocardiography is probably not necessary.

  3. Mid-Ventricular Variant of Dobutamine-Induced Stress Cardiomyopathy.

    Science.gov (United States)

    Chandraprakasam, Satish; Kanuri, Swapna; Hunter, Claire

    2015-05-01

    Dobutamine stress testing is a commonly used modality in detecting and estimating the prognosis in coronary artery disease (CAD). Although it is well tolerated by most patients, adverse events have been reported. Rarely, transient wall motion abnormalities can occur in the absence of obstructive CAD to suggest stress cardiomyopathy. We report a 48-year-old female with intermittent chest pain. Her physical exam, cardiac enzymes and transthoracic echocardiogram were unremarkable. She underwent dobutamine stress echocardiogram to rule out obstructive CAD. After 40 micrograms (mcg)/kg/minute and 0.5 mg atropine, she complained of intense chest pain and became hypertensive. Stress echocardiogram demonstrated mid-anterior and mid-septal hypokinesis. Emergent coronary angiogram demonstrated normal coronaries. Left ventricular angiogram in the right anterior oblique projection revealed mid-ventricular ballooning during systole with apical and basal hypercontractility. Patient demonstrated excellent recovery with expectant management. The mechanism of mid-variant of Dobutamine-induced stress cardiomyopathy remains unclear. We think that multiple mechanisms are involved and this risk should be considered in patients with comorbid psychiatric conditions and with use of centrally acting stimulants.

  4. Radiologic evaluation of adriamycin induced toxic cardiomyopathy in childhood leukemia

    International Nuclear Information System (INIS)

    Kim, Young Joo; Moon, Young Hee; Kang, Kyung Jin; Kim, Ok Hwa; Kim, Choon Yul; Bahk, Yong Whee

    1992-01-01

    The cardiomyopathy associated with Adriamycin is frequently fatal and full clinical recovery is uncommon. To evaluate the radiological manifestation and the outcome of Adriamycin induced cardiac toxicity, we retrospectively reviewed the serial chest X-ray films of children treated with Adriamycin. Among 154 children with leukemia, fourteen patients developed clinical and radiologic evidence of congestive heart failure (CHF). Six out of 14 (43%) died of CHF within 2 weeks after attack and eight children survived after their acute episodes of CHF, were controlled following digoxin and diuretic therapy. Despite the improving clinical evidence of heart failure, the follow-up chest roentgenograms of these 8 children showed definite cardiomegaly as compared with the pre-treatment chest X-ray. Three children among 8 had minimal cardiomegaly and the remaining five children showed persistent, marked cardiomegaly during the period of 9-25 months of follow up. In summary, when CHF develops during chemotherapy in leukemic children, the possibility of Adriamycin induced cardiac toxicity should be suspected. Our findings showed that persistence of cardiomegaly represented significant cardiomyopathy despite clinical improvement of CHF

  5. Family communication in a population at risk for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Batte, Brittany; Sheldon, Jane P; Arscott, Patricia; Huismann, Darcy J; Salberg, Lisa; Day, Sharlene M; Yashar, Beverly M

    2015-04-01

    Encouraging family communication is an integral component of genetic counseling; therefore, we sought to identify factors impacting communication to family members at risk for Hypertrophic Cardiomyopathy (HCM). Participants (N = 383) completed an online survey assessing: 1) demographics (gender, genetic test results, HCM family history, and disease severity); 2) illness representations; 3) family functioning and cohesiveness; 4) coping styles; 5) comprehension of HCM autosomal dominant inheritance; and 6) communication of HCM risk information to at-risk relatives. Participants were a national sample of individuals with HCM, recruited through the Hypertrophic Cardiomyopathy Association. Data from 183 participants were analyzed using a logistic regression analysis, with family communication as a dichotomous dependent variable. We found that female gender and higher comprehension of autosomal dominant inheritance were significant predictors of participants' communication of HCM risk information to all their siblings and children. Our results suggest that utilizing interventions that promote patient comprehension (e.g., a teaching-focused model of genetic counseling) are important and may positively impact family communication within families with HCM.

  6. Takotsubo cardiomyopathy: an overlooked cause of chest pain

    Directory of Open Access Journals (Sweden)

    Leonardo Hackbart Bermudes

    2014-06-01

    Full Text Available Takotsubo cardiomyopathy (TTC, also known as apical ballooning syndrome, broken heart syndrome, or stress-induced cardiomyopathy, is defined as a transient disturbance of the left ventricle, which is quite often associated with electrocardiographic abnormalities that may mimic acute myocardial infarction. The syndrome is also characterized by a mild alteration of cardiac biomarkers in absence of coronary blood flow obstruction on the coronariography. Clinical presentation is often manifested by angina, dyspnea, syncope, and arrhythmias. Peculiarly, the left ventricle takes the form of “tako-tsubo” (a Japanese word for “octopus trap” on the imaging workup. The authors report the case of a post-menopausal, hypertensive, dyslipidemic and type-II diabetic woman admitted at the emergency service with acute chest pain post physical exertion. Electrocardiogram showed signs of ischemia and myocardial necrosis markers were mildly increased. Echocardiography and ventriculography showed apical and mid-ventricular akinesia, with mild atherosclerotic coronary lesions. Thus diagnostic workup and the outcome followed the diagnostic criteria for TTC. The authors called attention to the potential of overlooking this diagnosis, since this syndrome is still not widely recognized.

  7. Multiple Genetic Associations with Irish Wolfhound Dilated Cardiomyopathy

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    Siobhan Simpson

    2016-01-01

    Full Text Available Cardiac disease is a leading cause of morbidity and mortality in dogs and humans, with dilated cardiomyopathy being a large contributor to this. The Irish Wolfhound (IWH is one of the most commonly affected breeds and one of the few breeds with genetic loci associated with the disease. Mutations in more than 50 genes are associated with human dilated cardiomyopathy (DCM, yet very few are also associated with canine DCM. Furthermore, none of the identified canine loci explain many cases of the disease and previous work has indicated that genotypes at multiple loci may act together to influence disease development. In this study, loci previously associated with DCM in IWH were tested for associations in a new cohort both individually and in combination. We have identified loci significantly associated with the disease individually, but no genotypes individually or in pairs conferred a significantly greater risk of developing DCM than the population risk. However combining three loci together did result in the identification of a genotype which conferred a greater risk of disease than the overall population risk. This study suggests multiple rather than individual genetic factors, cooperating to influence DCM risk in IWH.

  8. Diagnostic Imaging for Implants

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    Sanjay M Mallya

    2004-01-01

    Full Text Available Dental implants are a popular modality for permanent tooth replacement. The key to successful implant placement, its subsequent osseointegration and the final prosthetic rehabilitation is proper preoperative assessment. Diagnostic imaging plays an important role in the pre- and post-surgical evaluation process. Imaging is used to evaluate suitability of implant sites, aid in selection of appropriate implants, and finally evaluate implant placement and osseointegration. This article reviews the role of diagnostic imaging in the various phases and the advantages and limitations of the numerous imaging modalities.

  9. Cardiomiopatia hipertrófica apical associada a doença arterial coronária Apical hypertrophic cardiomyopathy associated with coronary artery disease

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    Francisco Manes Albanesi Fº

    1998-08-01

    Full Text Available Homem de 59 anos, portador de cardiomiopatia hipertrófica apical há 12 anos, apresentou agudização de quadro anginoso. Foi detectada doença arterial coronária com lesão trivascular, e indicada cirurgia de revascularização, com anastomose de artéria mamária e a colocação de duas pontes de veia safena, tendo evoluído com melhora do quadro anginoso e redução da dimensão do átrio esquerdo.A fifty-nine year old man, known to have hypertrophic cardiomyopathy, presented worsening of angina. Multivessel coronary artery disease was diagnosed, and he underwent myocardial revascularization (mammary and two safenous grafts were implanted with good evolution and reduction of left atrium dimension.

  10. Determination of multidirectional myocardial deformations in cats with hypertrophic cardiomyopathy by using two-dimensional speckle-tracking echocardiography.

    Science.gov (United States)

    Suzuki, Ryohei; Mochizuki, Yohei; Yoshimatsu, Hiroki; Teshima, Takahiro; Matsumoto, Hirotaka; Koyama, Hidekazu

    2017-12-01

    Objectives Hypertrophic cardiomyopathy, a primary disorder of the myocardium, is the most common cardiac disease in cats. However, determination of myocardial deformation with two-dimensional speckle-tracking echocardiography in cats with various stages of hypertrophic cardiomyopathy has not yet been reported. This study was designed to measure quantitatively multidirectional myocardial deformations of cats with hypertrophic cardiomyopathy. Methods Thirty-two client-owned cats with hypertrophic cardiomyopathy and 14 healthy cats serving as controls were enrolled and underwent assessment of myocardial deformation (peak systolic strain and strain rate) in the longitudinal, radial and circumferential directions. Results Longitudinal and radial deformations were reduced in cats with hypertrophic cardiomyopathy, despite normal systolic function determined by conventional echocardiography. Cats with severely symptomatic hypertrophic cardiomyopathy also had lower peak systolic circumferential strain, in addition to longitudinal and radial strain. Conclusions and relevance Longitudinal and radial deformation may be helpful in the diagnosis of hypertrophic cardiomyopathy. Additionally, the lower circumferential deformation in cats with severe hypertrophic cardiomyopathy may contribute to clinical findings of decompensation, and seems to be related to severe cardiac clinical signs. Indices of multidirectional myocardial deformations by two-dimensional speckle-tracking echocardiography may be useful markers and help to distinguish between cats with hypertrophic cardiomyopathy and healthy cats. Additionally, they may provide more detailed assessment of contractile function in cats with hypertrophic cardiomyopathy.

  11. Myofilament Remodeling and Function Is More Impaired in Peripartum Cardiomyopathy Compared with Dilated Cardiomyopathy and Ischemic Heart Disease.

    Science.gov (United States)

    Bollen, Ilse A E; Ehler, Elisabeth; Fleischanderl, Karin; Bouwman, Floor; Kempers, Lanette; Ricke-Hoch, Melanie; Hilfiker-Kleiner, Denise; Dos Remedios, Cristobal G; Krüger, Martina; Vink, Aryan; Asselbergs, Folkert W; van Spaendonck-Zwarts, Karin Y; Pinto, Yigal M; Kuster, Diederik W D; van der Velden, Jolanda

    2017-12-01

    Peripartum cardiomyopathy (PPCM) and dilated cardiomyopathy (DCM) show similarities in clinical presentation. However, although DCM patients do not recover and slowly deteriorate further, PPCM patients show either a fast cardiac deterioration or complete recovery. The aim of this study was to assess if underlying cellular changes can explain the clinical similarities and differences in the two diseases. We, therefore, assessed sarcomeric protein expression, modification, titin isoform shift, and contractile behavior of cardiomyocytes in heart tissue of PPCM and DCM patients and compared these with nonfailing controls. Heart samples from ischemic heart disease (ISHD) patients served as heart failure control samples. Passive force was only increased in PPCM samples compared with controls, whereas PPCM, DCM, and ISHD samples all showed increased myofilament Ca 2+ sensitivity. Length-dependent activation was significantly impaired in PPCM compared with controls, no impairment was observed in ISHD samples, and DCM samples showed an intermediate response. Contractile impairments were caused by impaired protein kinase A (PKA)-mediated phosphorylation because exogenous PKA restored all parameters to control levels. Although DCM samples showed reexpression of EH-myomesin, an isoform usually only expressed in the heart before birth, PPCM and ISHD did not. The lack of EH-myomesin, combined with low PKA-mediated phosphorylation of myofilament proteins and increased compliant titin isoform, may explain the increase in passive force and blunted length-dependent activation of myofilaments in PPCM samples. Copyright © 2017 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  12. Misclassification of hypertrophic cardiomyopathy: validation of diagnostic codes

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    Magnusson P

    2017-08-01

    Full Text Available Peter Magnusson,1,2 Andreas Palm,2,3 Eva Branden,2,4 Stellan Mörner5 1Cardiology Research Unit, Department of Medicine, Karolinska Institutet, Stockholm, 2Centre for Research and Development, Uppsala University, Region Gävleborg, Gävle, 3Department of Medical Sciences, Respiratory, Allergy and Sleep Research, Uppsala University, Uppsala, 4Department of Medicine, Karolinska Institutet, Stockholm, 5Heart Center and Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden Purpose: To validate diagnostic codes for hypertrophic cardiomyopathy (HCM, analyze misclassfications, and estimate the prevalence of HCM in an unselected Swedish regional cohort.Patients and methods: Using the hospitals’ electronic medical records (used for the Swedish National Patient Register, we identified 136 patients from 2006 to 2016 with the HCM-related codes 142.1 and 142.2 (International Classification of Diseases.Results: Of a total of 129 residents in the catchment area, 88 patients were correctly classified as HCM (positive predictive value 68.2% and 41 patients (31.8% were misclassified as HCM. Among the 88 HCM patients (52.2% males, 74 were alive and 14 were dead (15.9%. This yields an HCM prevalence of 74/183,337, that is, 4.0 diagnosed cases per 10,000 in the adult population aged ≥18 years. The underlying diagnoses of misclassified cases were mainly hypertension (31.7% and aortic stenosis (22.0%. Other types of cardiomyopathies accounted for several cases of misclassification: dilated (nonischemic or ischemic, left ventricular noncompaction, and Takotsubo. Miscellaneous diagnoses were amyloidosis, pulmonary stenosis combined with ventricular septal defect, aortic insufficiency, athelete’s heart, and atrioventricular conduction abnormality. The mean age was not significantly different between HCM and misclassified patients (65.8±15.8 vs 70.1±13.4 years; P=0.177. There were 47.8% females among HCM and 60.8% females among

  13. PVCs, PVC-Induced Cardiomyopathy, and the Role of Catheter Ablation.

    Science.gov (United States)

    Sharma, Esseim; Arunachalam, Karuppiah; Di, Mengyang; Chu, Antony; Maan, Abhishek

    2017-06-01

    Premature ventricular contractions (PVCs) are common arrhythmias noticed in the clinical setting because of premature depolarization of the ventricular myocytes. Although often thought to be reflective of underlying disease rather than intrinsically harmful, PVCs have recently been linked with worse outcomes in patients without significant cardiac disease. Long-term exposure to a high PVC burden can lead to the development of PVC-induced cardiomyopathy. The pathogenesis of this condition is poorly understood at the current time. Many studies have suggested that catheter ablation of these PVCs may result in reversal of the PVC-induced cardiomyopathy. This article will go over the natural history of PVCs and PVC-induced cardiomyopathy, as well as review the current literature on the role of catheter ablation in treating PVC-induced cardiomyopathy.

  14. Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly

    Directory of Open Access Journals (Sweden)

    Ouarda Taghli-Lamallem

    2016-02-01

    Full Text Available Cardiovascular diseases and, among them, channelopathies and cardiomyopathies are a major cause of death worldwide. The molecular and genetic defects underlying these cardiac disorders are complex, leading to a large range of structural and functional heart phenotypes. Identification of molecular and functional mechanisms disrupted by mutations causing channelopathies and cardiomyopathies is essential to understanding the link between an altered gene and clinical phenotype. The development of animal models has been proven to be efficient for functional studies in channelopathies and cardiomyopathies. In particular, the Drosophila model has been largely applied for deciphering the molecular and cellular pathways affected in these inherited cardiac disorders and for identifying their genetic modifiers. Here we review the utility and the main contributions of the fruitfly models for the better understanding of channelopathies and cardiomyopathies. We also discuss the investigated pathological mechanisms and the discoveries of evolutionarily conserved pathways which reinforce the value of Drosophila in modeling human cardiac diseases.

  15. Recurrent Stress Cardiomyopathy During COPD Exacerbation: Are Beta-adrenergic Agonists Only to Blame?

    Science.gov (United States)

    Katsa, Ioanna; Christia, Panagiota; Massera, Daniele; Faillace, Robert

    2017-04-14

    Takotsubo cardiomyopathy (TCM) is a variant of stress-induced cardiomyopathy, characterized by transient left ventricular dysfunction that may be associated with emotional or physical triggers. We present the case of a 51-year-old Caucasian female with severe chronic obstructive pulmonary disease (COPD) who presented with syncope and was found to have her second lifetime episode of stress-induced cardiomyopathy. Eight months prior, she had been admitted with a COPD exacerbation and was found to have left ventricular (LV) dysfunction with ejection fraction (EF) of 22% attributed to TCM with subsequent normalization of her left ventricular function. Recurrence of stress-induced cardiomyopathy associated with COPD is a rare phenomenon and its presentation raises the possibility of a common underlying mechanism.

  16. Nutritional deficiency of selenium secondary to weight loss (bariatric) surgery associated with life-threatening cardiomyopathy.

    Science.gov (United States)

    Boldery, Rachel; Fielding, George; Rafter, Tony; Pascoe, Andrew L; Scalia, Gregory M

    2007-04-01

    Nutritional deficiencies of vitamins and minerals have been associated with reversible and irreversible cardiomyopathic processes. Selenium deficient dilated cardiomyopathy, first described in 1935 in the Keshan Province of China, was sometimes reversed with selenium supplementation. In the past three decades, selenium deficient cardiomyopathy has re-emerged in western medicine secondary to gastrointestinal disorders, long-term total parenteral nutrition (TPN) and gastrointestinal surgery. This report describes a case of selenium deficient cardiomyopathy secondary to the bariatric (weight loss) surgical operation bilio-pancreatic diversion (Scopinaro procedure). This patient presented with life-threatening heart failure nine months after this surgery, having lost 100 kg of body weight. Multiple nutritional deficiencies were detected and corrected including severely depleted selenium levels. Cardiac function and the clinical scenario improved dramatically over three weeks. Screening patients at risk of malnourishment for selenium deficiency as a potential cause of cardiomyopathy is indicated.

  17. Large Genomic Rearrangements of Desmosomal Genes in Italian Arrhythmogenic Cardiomyopathy Patients

    NARCIS (Netherlands)

    Pilichou, Kalliopi; Lazzarini, Elisabetta; Rigato, Ilaria; Celeghin, Rudy; De Bortoli, Marzia; Marra, Marina Perazzolo; Cason, Marco; Jongbloed, Jan; Calore, Martina; Rizzo, Stefania; Regazzo, Daniela; Poloni, Giulia; Iliceto, Sabino; Daliento, Luciano; Delise, Pietro; Corrado, Domenico; Van Tintelen, J. Peter; Thiene, Gaetano; Rampazzo, Alessandra; Basso, Cristina; Bauce, Barbara; Lorenzon, Alessandra; Occhi, Gianluca

    2017-01-01

    BACKGROUND: Arrhythmogenic cardiomyopathy (AC) is an inherited heart muscle disease associated with point mutations in genes encoding for cardiac desmosome proteins. Conventional mutation screening is positive in approximate to 50% of probands. Copy number variations (CNVs) have recently been linked

  18. Cardiac resynchronization therapy in patients with chronic Chagas cardiomyopathy: long-term follow up.

    Science.gov (United States)

    Araújo, Edgard Ferreira de; Chamlian, Eduardo Gregório; Peroni, Alexey Pomares; Pereira, Wilson Lopes; Gandra, Sylvio Matheus de Aquino; Rivetti, Luiz Antonio

    2014-01-01

    Chagas disease is a major cause of cardiomyopathy and sudden death in our country. It has a high mortality when their patients develop New York Heart Association (NYHA) class IV. The objective of this study is to analyze the clinical outcome of patients with Chagas' cardiomyopathy with congestive heart failure with optimized pharmacological therapy, undergoing cardiac resynchronization therapy. Between January 2004 and February 2009, 72 patients with Chagas' cardiomyopathy in NYHA class III and IV underwent cardiac resynchronization therapy and were monitored to assess their clinical evolution. We used the t test or the Wilcoxon test to compare the same variable in two different times. A P value chronic Chagas cardiomyopathy undergoing cardiac resynchronization therapy, we found the following statistically significant changes: improvement in NYHA class and increase of left ventricle ejection fraction, a decrease of the systolic final diameter and systolic final left ventricle volume and improvement of patient survival.

  19. Echocardiographic strain imaging to assess early and late consequences of sarcomere mutations in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Ho, Carolyn Y; Carlsen, Christian; Thune, Jens Jakob

    2009-01-01

    BACKGROUND: Genetic testing identifies sarcomere mutation carriers (G+) before clinical diagnosis of hypertrophic cardiomyopathy (HCM), allowing characterization of initial disease manifestations. Previous studies demonstrated that impaired relaxation develops before left ventricular hypertrophy...

  20. Echocardiographic evaluation of pre-diagnostic development in young relatives genetically predisposed to hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Havndrup, Ole; Christiansen, Michael

    2015-01-01

    Identification of the first echocardiographic manifestations of hypertrophic cardiomyopathy may be important for clinical management and our understanding of the pathogenesis. We studied the development of pre-diagnostic echocardiographic changes in young relatives to HCM patients during long...

  1. Mitochondrial dysfunction and oxidative stress in Naturally-Occurring Feline Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Christiansen, Liselotte Bruun

    Background: Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease, characterized by unexplained hypertrophy of the left ventricle. HCM features similar clinical and pathological characteristics in human beings and cats and is a common cause of sudden death and heart failure...

  2. [Application of next-generation semiconductor sequencing technologies in genetic diagnosis of inherited cardiomyopathies].

    Science.gov (United States)

    Zhao, Yue; Zhang, Hong; Xia, Xue-shan

    2015-07-01

    Inherited cardiomyopathy is the most common hereditary cardiac disease. It also causes a significant proportion of sudden cardiac deaths in young adults and athletes. So far, approximately one hundred genes have been reported to be involved in cardiomyopathies through different mechanisms. Therefore, the identification of the genetic basis and disease mechanisms of cardiomyopathies are important for establishing a clinical diagnosis and genetic testing. Next-generation semiconductor sequencing (NGSS) technology platform is a high-throughput sequencer capable of analyzing clinically derived genomes with high productivity, sensitivity and specificity. It was launched in 2010 by Life Technologies of USA, and it is based on a high density semiconductor chip, which was covered with tens of thousands of wells. NGSS has been successfully used in candidate gene mutation screening to identify hereditary disease. In this review, we summarize these genetic variations, challenge and application of NGSS in inherited cardiomyopathy, and its value in disease diagnosis, prevention and treatment.

  3. Safety profile and utility of treadmill exercise in patients with high-gradient hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Sorensen, Lars Lindholm; Liang, Hsin-Yueh; Pinheiro, Aurelio

    2017-01-01

    BACKGROUND: Exercise echocardiography in the evaluation of hypertrophic cardiomyopathy (HCM) provides valuable information for risk stratification, selection of optimal treatment, and prognostication. However, HCM patients with left ventricular outflow tract gradients ≥30mm Hg are often excluded ...

  4. Cardiomyopathy and cardiac computed tomography: What the radiologist needs to know

    International Nuclear Information System (INIS)

    Troupis, J.M.; Singh Pasricha, S.; Gunaratnam, K.; Nasis, A.; Cameron, J.; Seneviratne, S.

    2013-01-01

    Coronary computed tomographic angiography (CCTA) is an established tool for the investigation of shortness of breath and chest pain. Although CCTA is performed to assess vessels that could well be diseased, one must review the study for evidence of cardiomyopathy, which can provoke similar symptoms. Cardiomyopathies can coexist with various causes of chest pain including obstructive coronary artery disease and may, therefore, be identifiable at CCTA. Furthermore, symptoms such as shortness of breath and chest pain that the clinician may suspect are secondary to coronary disease leading to investigation with CCTA, may be secondary to cardiomyopathy. We review several important causes of cardiomyopathy that may be detected by CCTA, which are important for radiologists to identify given the implications for further management and prognosis.

  5. Comparison of myocardial thallium and β-methyl iodophenyl pentadecanoic acid (BMIPP) distribution in cardiomyopathy hamster

    International Nuclear Information System (INIS)

    Nishimura, Tsunehiko; Sago, Masayoshi

    1989-01-01

    The usefulness of fatty acid imaging in the detection of cardiomyopathy was evaluated by comparing thallium and BMIPP myocardial distribution in Bio 14.6 Syrian Hamster (25 week ages). Autoradiography was performed in 3 using 3.7 MBq (100 μCi) of 125 I-BMIPP and 37 MBq (1 mCi) of 201 TlCl. In vivo pin-hole imaging was performed in 3 using 37 MBq (1 mCi) of 123 I-BMIPP and 37 MBq (1 mCi) of 201 TlCl. In all cardiomyopathy hamsters, decreased uptake of BMIPP compared to that of thallium was demonstrated. These findings suggest dilated cardiomyopathy is associated with severe focal alternation in the substrate used for the performance of myocardial work. In conclusion, myocardial imaging using BMIPP may be useful for early detection of myocardial degeneration compared to thallium in patients with dilated cardiomyopathy. (author)

  6. Usefulness of immunostaining for plakoglobin as a diagnostic marker of arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Munkholm, Julie; Christensen, Alex H; Svendsen, Jesper H

    2012-01-01

    The clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) is often challenging due to phenotypic variation, reduced/age-related penetrance, and lack of a diagnostic test. A single report has suggested quantitative myocardial immunoanalysis for the desmosomal protein...

  7. Ten-year incidence of Chagas cardiomyopathy among asymptomatic Trypanosoma cruzi-seropositive former blood donors.

    Science.gov (United States)

    Sabino, Ester C; Ribeiro, Antonio L; Salemi, Vera M C; Di Lorenzo Oliveira, Claudia; Antunes, Andre P; Menezes, Marcia M; Ianni, Barbara M; Nastari, Luciano; Fernandes, Fabio; Patavino, Giuseppina M; Sachdev, Vandana; Capuani, Ligia; de Almeida-Neto, Cesar; Carrick, Danielle M; Wright, David; Kavounis, Katherine; Goncalez, Thelma T; Carneiro-Proietti, Anna Barbara; Custer, Brian; Busch, Michael P; Murphy, Edward L

    2013-03-12

    Very few studies have measured disease penetrance and prognostic factors of Chagas cardiomyopathy among asymptomatic Trypanosoma cruzi-infected persons. We performed a retrospective cohort study among initially healthy blood donors with an index T cruzi-seropositive donation and age-, sex-, and period-matched seronegatives in 1996 to 2002 in the Brazilian cities of São Paulo and Montes Claros. In 2008 to 2010, all subjects underwent medical history, physical examination, ECGs, and echocardiograms. ECG and echocardiogram results were classified by blinded core laboratories, and records with abnormal results were reviewed by a blinded panel of 3 cardiologists who adjudicated the outcome of Chagas cardiomyopathy. Associations with Chagas cardiomyopathy were tested with multivariate logistic regression. Mean follow-up time between index donation and outcome assessment was 10.5 years for the seropositives and 11.1 years for the seronegatives. Among 499 T cruzi seropositives, 120 (24%) had definite Chagas cardiomyopathy, and among 488 T cruzi seronegatives, 24 (5%) had cardiomyopathy, for an incidence difference of 1.85 per 100 person-years attributable to T cruzi infection. Of the 120 seropositives classified as having Chagas cardiomyopathy, only 31 (26%) presented with ejection fraction <50%, and only 11 (9%) were classified as New York Heart Association class II or higher. Chagas cardiomyopathy was associated (P<0.01) with male sex, a history of abnormal ECG, and the presence of an S3 heart sound. There is a substantial annual incidence of Chagas cardiomyopathy among initially asymptomatic T cruzi-seropositive blood donors, although disease was mild at diagnosis.

  8. Ten-Year Incidence of Chagas Cardiomyopathy Among Asymptomatic Trypanosoma cruzi–Seropositive Former Blood Donors

    Science.gov (United States)

    Sabino, Ester C.; Ribeiro, Antonio L.; Salemi, Vera M.C.; Di Lorenzo Oliveira, Claudio; Antunes, Andre P.; Menezes, Marcia M.; Ianni, Barbara M.; Nastari, Luciano; Fernandes, Fabio; Patavino, Giuseppina M.; Sachdev, Vandana; Capuani, Ligia; de Almeida-Neto, Cesar; Carrick, Danielle M.; Wright, David; Kavounis, Katherine; Goncalez, Thelma T.; Carneiro-Proietti, Anna Barbara; Custer, Brian; Busch, Michael P.; Murphy, Edward L.

    2013-01-01

    Background Very few studies have measured disease penetrance and prognostic factors of Chagas cardiomyopathy among asymptomatic Trypanosoma cruzi–infected persons. Methods and Results We performed a retrospective cohort study among initially healthy blood donors with an index T cruzi–seropositive donation and age-, sex-, and period-matched seronegatives in 1996 to 2002 in the Brazilian cities of São Paulo and Montes Claros. In 2008 to 2010, all subjects underwent medical history, physical examination, ECGs, and echocardiograms. ECG and echocardiogram results were classified by blinded core laboratories, and records with abnormal results were reviewed by a blinded panel of 3 cardiologists who adjudicated the outcome of Chagas cardiomyopathy. Associations with Chagas cardiomyopathy were tested with multivariate logistic regression. Mean follow-up time between index donation and outcome assessment was 10.5 years for the seropositives and 11.1 years for the seronegatives. Among 499 T cruzi seropositives, 120 (24%) had definite Chagas cardiomyopathy, and among 488 T cruzi seronegatives, 24 (5%) had cardiomyopathy, for an incidence difference of 1.85 per 100 person-years attributable to T cruzi infection. Of the 120 seropositives classified as having Chagas cardiomyopathy, only 31 (26%) presented with ejection fraction <50%, and only 11 (9%) were classified as New York Heart Association class II or higher. Chagas cardiomyopathy was associated (P<0.01) with male sex, a history of abnormal ECG, and the presence of an S3 heart sound. Conclusions There is a substantial annual incidence of Chagas cardiomyopathy among initially asymptomatic T cruzi–seropositive blood donors, although disease was mild at diagnosis. PMID:23393012

  9. Ischemic Stroke in a Young Patient Heralding a Left Ventricular Noncompaction Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Fanny Lestienne

    2017-08-01

    Full Text Available Strokes in young patients may be the clinical expression of many complex and extremely rare diseases. Uncommon causes constitute less than 5% of all strokes, but are present in 30% of strokes in young patients. We report the case of a young woman whose ischemic stroke led to the diagnosis of a rare embolic cardiomyopathy, left ventricular noncompaction cardiomyopathy, requiring a heart transplant.

  10. The Comparision of Left Atrial Functions Between Hypertrophic Cardiomyopathy and Hypertension

    Directory of Open Access Journals (Sweden)

    Ahmet Akdi

    2016-12-01

    Full Text Available INTRODUCTION: The presence of left ventricule hypertrophy (LVH due to arterial hypertension may impair atrial function. Also, hypertrophic cardiomyopathy (HCM represents a generalized myopathic process affecting both ventricular and atrial myocardium. In this study we aimed to evaluate left atrial volumes, phasic functions in hypertrophic cardiomyopathy and hypertensive heart disease. METHODS: The study consisted of 42 patients (25 men, 17 women; mean age 51.2 years with hypertrophic cardiomyopathy (n = 20 or hypertensive heart disease (n = 22. Two-dimensional echocardiographic left atrial volumes (maximal, minimal and pre-contraction volumes were obtained and left atrial phasic functions (reservoir, conduit, and pumping functions were calculated. The findings were compared with those of age- and sex-matched 20 controls (9 men, 11 women; mean age 44.2 years without structural heart disease. RESULTS: Left atrial volumes were found to be significantly increased in patients with hypertrophic cardiomyopathy and hypertensive heart disease compared with control groups. Left atrial reservoir and conduit functions were significantly lower in patients with hypertrophic cardiomyopathy than in those with hypertensive heart disease and control groups (p < 0.01 and p < 0.01, respectively. There was no significant difference of left atrial pumping functions between study groups (p = 0.2. DISCUSSION AND CONCLUSION: This study showed that left atrial minimal, maximal and pre-contraction volume indexes are increased in hypertensive and hypertrophic cardiomyopathy groups compared with control group, but there was no significant difference between hypertrophic cardiomyopathy and hypertensive patients. In hypertrophic cardiomyopathy group left atrial phasic functions, including conduit, reservoir and pump, were decreased significantly more than hypertensive and control group. In hypertensive patients left atrial phasic functions were decreased but compared with

  11. Asymmetric left ventricular hypertrophy associated with morbid obesity mimicking familial hypertrophic cardiomyopathy.

    Science.gov (United States)

    Wong, Raymond Ching-Chiew; Tan, Kong Bing

    2014-12-01

    Asymmetric septal hypertrophy with systolic anterior motion of the mitral valve is frequently a phenotypic, but not pathognomonic, expression of genetic hypertrophic cardiomyopathy (HCM) with or without obstruction. It can, however, be associated nonspecifically with other forms of increased left ventricular (LV) afterload. We herein report the case of a young man with obesity cardiomyopathy and heart failure who presented with asymmetric septal hypertrophy and marked LV hypertrophy, and endomyocardial biopsy ruled out genetic HCM.

  12. Acute pulmonary edema caused by takotsubo cardiomyopathy in a pregnant woman undergoing transvaginal cervical cerclage

    OpenAIRE

    Lee, Jae-Young; Kwon, Hyun-Jung; Park, Sang-Wook; Lee, Yu-Mi

    2017-01-01

    Abstract Background: The physiological changes associated with pregnancy may predispose pregnant women to pulmonary edema. Other known causes of pulmonary edema during pregnancy include tocolytic drugs, preeclampsia, eclampsia, and peripartum cardiomyopathy. Methods: We describe a rare case of pulmonary edema caused by takotsubo cardiomyopathy in a pregnant woman at 14 weeks of gestation who was undergoing emergency transvaginal cervical cerclage. Results: Intraoperative chest radiography rev...

  13. Pregnancy in women with a cardiomyopathy: Outcomes and predictors from a retrospective cohort.

    Science.gov (United States)

    Billebeau, Gilles; Etienne, Martin; Cheikh-Khelifa, Riadh; Vauthier-Brouzes, Daniele; Gandjbakhch, Estelle; Isnard, Richard; Nizard, Jacky; Komajda, Michel; Dommergues, Marc; Charron, Philippe

    2017-10-25

    Pregnancies in women with pre-existing cardiomyopathies are considered at high risk for complications. However, few data are available to characterize their natural history and predict the outcome. Our aim was to evaluate the prevalence and predictors of acute cardiac and obstetric events in women with a cardiomyopathy during pregnancy, excluding peripartum cardiomyopathy. In this retrospective study in a referral centre for cardiomyopathies, we included 43 consecutive pregnancies in 36 women with dilated, hypertrophic, arrhythmogenic right ventricular or tachycardia-induced cardiomyopathy, or left ventricular non-compaction. We observed a major cardiovascular event during 15 pregnancies (35%), including three cardiac deaths, which occurred in patients who did not follow our usual early multidisciplinary protocol. The Carpreg score was predictive of maternal complication rate (67%, 36% and 31% in women with a Carpreg score of 2, 1 and 0, respectively). However, major cardiac complications occurred in four women with no risk factors. Left ventricular ejection fraction alone, gradient in hypertrophic cardiomyopathy, the Zahara score and the modified World Health Organization score appeared to be less discriminant than Carpreg for maternal outcome. There were two intrauterine fetal deaths, nine premature deliveries (23%), 17 low neonatal birth weights (40%) and 11 cases of hypoglycaemia (26%). Pregnancy in women with a cardiomyopathy is at high risk for both women and neonates. The highest risks are observed in women who do not benefit from early multidisciplinary team management, and in patients with dilated cardiomyopathy. Our findings suggest that the Carpreg score is the most appropriate predictor of maternal complications, although the stratification might be improved. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  14. High?Sensitivity Troponin: A Clinical Blood Biomarker for Staging Cardiomyopathy in Fabry Disease

    OpenAIRE

    Seydelmann, Nora; Liu, Dan; Kr?mer, Johannes; Drechsler, Christiane; Hu, Kai; Nordbeck, Peter; Schneider, Andreas; St?rk, Stefan; Bijnens, Bart; Ertl, Georg; Wanner, Christoph; Weidemann, Frank

    2016-01-01

    Background High?sensitivity troponin (hs?TNT), a biomarker of myocardial damage, might be useful for assessing fibrosis in Fabry cardiomyopathy. We performed a prospective analysis of hs?TNT as a biomarker for myocardial changes in Fabry patients and a retrospective longitudinal follow?up study to assess longitudinal hs?TNT changes relative to fibrosis and cardiomyopathy progression. Methods and Results For the prospective analysis, hs?TNT from 75 consecutive patients with genetically confirm...

  15. Comparison Between Clinical and Echocardiographic Findings in Infants and Children Diagnosed with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Cristina Blesneac

    2015-06-01

    Full Text Available Background: Hypertrophic cardiomyopathy is a rather common hereditary disease with an autozomal dominant character, caused by mutations of genes that code for proteins of the cardiac sarcomere. The observed prevalence of this disease is much lower in pediatric patients compared to adults, because it’s late gene expression. Hypertrophic cardiomyopathy presenting in infancy has been shown to have a very high mortality.

  16. Postoperative Takotsubo cardiomyopathy triggered by intraoperative fluid overload and acute hypertensive crisis.

    Science.gov (United States)

    Varutti, Rosanna; Setti, Tommaso; Ezri, Tiberiu; Nicolosi, Gianluigi; Rellini, Gianluigi; Cassin, Matteo; Leykin, Yigal

    2015-04-01

    The Takotsubo cardiomyopathy is a rare haemodynamic dysfunction, only recently reported perioperatively. While the diagnostic criteria have been established and the outcome is known as favorable, the pathophysiological mechanisms are not entirely understood. Here we present the case of a patient scheduled for laparoscopic hysterectomy and adnexectomy, who early postoperatively developed a Takotsubo cardiomyopathy supposedly triggered by an acute hypertensive crisis due to intraoperative fluid overload.

  17. Is hepatitis C virus a cause of idiopathic dilated cardiomyopathy? A systematic review of literature

    OpenAIRE

    Reis, Francisco José Farias Borges dos; Sousa, Tiago Almeida de; Oliveira, Manoela S.; Dantas, Ney; Silveira, Martha; Braghiroly, Maria Ignês Freitas Melro; Paraná, Raymundo

    2006-01-01

    p.199-202 Recent studies have suggested that some patients with idiopathic dilated cardiomyopathy (IDC) are also afflicted with insidious forms of viral myocarditis. Participation of hepatitis C virus (HCV) in this process has been postulated. The objective of this study was to evaluate a possible association between hepatitis C virus and idiopathic dilated cardiomyopathy. Systematic review of the literature using electronic databases (MEDLINE, EMBASES, LILACS and COCHRANE) for the period ...

  18. Newborn infant with maternal anti-SSA antibody-induced complete heart block accompanying cardiomyopathy.

    Science.gov (United States)

    Iida, Midori; Inamura, Noboru; Takeuchi, Makoto

    2006-01-01

    Newborn case of maternal anti-SSA antibody-induced congenital complete heart block (CCHB) accompanying cardiomyopathy is presented. Unexpectedly, she died of ventricular tachycardia, not bradycardia, 6 days after birth. Autopsy revealed left ventricular cardiomyopathy with endocardial fibroelastosis. Thus, when evaluating fetal cardiac performance in cases of maternal anti-SSA antibody-induced CCHB, it is necessary to pay attention to myocardial attributes such as endocardial hyperplasia.

  19. Lethal Cardiomyopathy in Mice Lacking Transferrin Receptor in the Heart.

    Science.gov (United States)

    Xu, Wenjing; Barrientos, Tomasa; Mao, Lan; Rockman, Howard A; Sauve, Anthony A; Andrews, Nancy C

    2015-10-20

    Both iron overload and iron deficiency have been associated with cardiomyopathy and heart failure, but cardiac iron utilization is incompletely understood. We hypothesized that the transferrin receptor (Tfr1) might play a role in cardiac iron uptake and used gene targeting to examine the role of Tfr1 in vivo. Surprisingly, we found that decreased iron, due to inactivation of Tfr1, was associated with severe cardiac consequences. Mice lacking Tfr1 in the heart died in the second week of life and had cardiomegaly, poor cardiac function, failure of mitochondrial respiration, and ineffective mitophagy. The phenotype could only be rescued by aggressive iron therapy, but it was ameliorated by administration of nicotinamide riboside, an NAD precursor. Our findings underscore the importance of both Tfr1 and iron in the heart, and may inform therapy for patients with heart failure. Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.

  20. Lethal Cardiomyopathy in Mice Lacking Transferrin Receptor in the Heart

    Directory of Open Access Journals (Sweden)

    Wenjing Xu

    2015-10-01

    Full Text Available Both iron overload and iron deficiency have been associated with cardiomyopathy and heart failure, but cardiac iron utilization is incompletely understood. We hypothesized that the transferrin receptor (Tfr1 might play a role in cardiac iron uptake and used gene targeting to examine the role of Tfr1 in vivo. Surprisingly, we found that decreased iron, due to inactivation of Tfr1, was associated with severe cardiac consequences. Mice lacking Tfr1 in the heart died in the second week of life and had cardiomegaly, poor cardiac function, failure of mitochondrial respiration, and ineffective mitophagy. The phenotype could only be rescued by aggressive iron therapy, but it was ameliorated by administration of nicotinamide riboside, an NAD precursor. Our findings underscore the importance of both Tfr1 and iron in the heart, and may inform therapy for patients with heart failure.

  1. Evidence for a possible calcium flux dependent cardiomyopathy in hyperthyroidism

    International Nuclear Information System (INIS)

    Barat, J.L.; Wicker, P.; Manley, W.

    1985-01-01

    This study was designed to test the hypothesis that the impaired functional cardiac reserve to exercise in hyperthyroidism is related to alterations in the regulation of calcium transport. In 2l hyperthyroid patients, the left ventricular ejection fraction (LVEF) was measured using equilibrium gated radionuclide angiocardiography at rest and during supine dynamic exercise. After a recovery period, the patients performed a second exercise study after random administration of Verapamil, a calcium entry blocker (11 pts), or propanolol, a beta adrenergic antagonist (10 pts) for comparison. The results showed i) normal resting LVEF with no significant change during exercise before any medication, ii) resting LVEF significantly decreased after Propanolol, and no significantly changed after Verapamil, iii) during exercise, significant increase of LVEF after Verapamil, and no significant change after Propanolol. These results are consistent with previous studies showing that abnormal change in LVEF during exercise in hyperthyroidism seems independent of beta adrenergic activation, and suggest a reversible functional cardiomyopathy dependent of calcium transporting systems

  2. Data of methylome and transcriptome derived from human dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Bong-Seok Jo

    2016-12-01

    Full Text Available Alterations in DNA methylation and gene expression have been implicated in the development of human dilated cardiomyopathy (DCM. Differentially methylated probes (DMPs and differentially expressed genes (DEGs were identified between the left ventricle (LV, a pathological locus for DCM and the right ventricle (RV, a proxy for normal hearts. The data in this DiB are for supporting our report entitled “Methylome analysis reveals alterations in DNA methylation in the regulatory regions of left ventricle development genes in human dilated cardiomyopathy” (Bong-Seok Jo, In-Uk Koh, Jae-Bum Bae, Ho-Yeong Yu, Eun-Seok Jeon, Hae-Young Lee, Jae-Joong Kim, Murim Choi, Sun Shim Choi, 2016 [1].

  3. Ventricular Arrhythmias in Hypertrophic Cardiomyopathy- Can We Ever Predict Them?

    Directory of Open Access Journals (Sweden)

    Narayanan Namboodiri

    2010-03-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is characterized by gross cardiac and myocyte hypertrophy, myocyte disarray, and interstitial fibrosis. This condition is relatively common, with a prevalence of about 1:500 in the general population. Most patients with HCM are either asymptomatic or have only minimal symptoms. In general, HCM is a relatively benign disease with an annual mortality rate of slightly less than 1% in unselected HCM populations [1,2]. However, sudden cardiac death (SCD may be the first manifestation of the disease. Approximately 60% to 70% of all patients with HCM die suddenly [3], and the fatal event is generally assumed, though not proven so far, due to ventricular arrhythmias.

  4. Cardiovascular genetics: technological advancements and applicability for dilated cardiomyopathy.

    Science.gov (United States)

    Kummeling, G J M; Baas, A F; Harakalova, M; van der Smagt, J J; Asselbergs, F W

    2015-07-01

    Genetics plays an important role in the pathophysiology of cardiovascular diseases, and is increasingly being integrated into clinical practice. Since 2008, both capacity and cost-efficiency of mutation screening of DNA have been increased magnificently due to the technological advancement obtained by next-generation sequencing. Hence, the discovery rate of genetic defects in cardiovascular genetics has grown rapidly and the financial threshold for gene diagnostics has been lowered, making large-scale DNA sequencing broadly accessible. In this review, the genetic variants, mutations and inheritance models are briefly introduced, after which an overview is provided of current clinical and technological applications in gene diagnostics and research for cardiovascular disease and in particular, dilated cardiomyopathy. Finally, a reflection on the future perspectives in cardiogenetics is given.

  5. Hepatorenal Syndrome with Cirrhotic Cardiomyopathy: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Luis Mocarzel

    2015-01-01

    Full Text Available The hepatorenal syndrome (HRS is defined as a potentially reversible kidney failure in patients with cirrhosis and ascites. An association of HRS and cirrhotic cardiomyopathy has been reported recently, but there are no result studies about the use of positive inotropes as part of the acute phase treatment. We report the case of a patient diagnosed with HRS, with high levels of NT pro-BNP, but with normal ejection fraction of the left ventricle, which showed abnormalities in systolic function through speckle tracking in echocardiography, reversible after the infusion of dobutamine. The patient showed clinical and laboratory improvement of his renal function after the infusion of dobutamine. Clinical studies are needed on HRS therapeutic approach taking into account the myocardial dysfunction as a major contributing factor to renal dysfunction.

  6. Role of ventricular tachycardia ablation in arrhythmogenic right ventricular cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Alberto Cipriani

    2017-11-01

    Full Text Available Arrhythmogenic right ventricular cardiomyopathy (ARVC is characterized by progressive fibro-fatty replacement of the myocardium that represents the substrate for recurrent sustained ventricular tachycardia (VT. These arrhythmias characterize the clinical course of a sizeable proportion of patients and have significant implications for their quality of life and long-term prognosis. Antiarrhythmic drugs are often poorly tolerated and usually provide incomplete control of arrhythmia relapses. Catheter ablation is a potentially effective strategy to treat frequent VT episodes and ICD shocks in ARVC patients. The aims of this review are to discuss the electrophysiological and electroanatomic substrates of ventricular tachycardia in patients with ARVC and to analyze the role of catheter ablation in their management with particular reference to selection of patients, technical issues, potential complications and outcomes.

  7. Takotsubo cardiomyopathy: The challenging diagnosis in clinical routine

    Directory of Open Access Journals (Sweden)

    Eglė Kazakauskaitė

    2014-01-01

    Full Text Available Takotsubo cardiomyopathy is rapidly reversible heart failure syndrome that usually mimics the symptoms of acute myocardial infarction with the characteristic regional wall-motion abnormalities (classically with a virtual apical ballooning caused by hypokinetic or akinetic apical or midventricular myocardium and hypercontraction of the basal segments and absence of obstructive coronary artery disease. TC is usually associated with identifiable emotional, psychological or physical stress event and most commonly appears in postmenopausal women. The certain pathophysiological mechanism remains unknown. However, the central hypothesis is supported by the excess of catecholamines and hyperactivity of nervous system. In the last decades the frequency of the TC diagnosis is increasing rapidly but at the initial presentation the diagnosis remains challenging due to the close similarities between TC and ST elevation myocardial infarction clinical presentations that consider TC as an important part of differential diagnosis in acute coronary syndrome.

  8. Hypertrophic Cardiomyopathy: How do Mutations Lead to Disease?

    Energy Technology Data Exchange (ETDEWEB)

    Marsiglia, Júlia Daher Carneiro, E-mail: julia.marsiglia@usp.br; Pereira, Alexandre Costa [Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil)

    2014-03-15

    Hypertrophic cardiomyopathy (HCM) is the most common monogenic genetic cardiac disease, with an estimated prevalence of 1:500 in the general population. Clinically, HCM is characterized by hypertrophy of the left ventricle (LV) walls, especially the septum, usually asymmetric, in the absence of any cardiac or systemic disease that leads to a secondary hypertrophy. The clinical course of the disease has a large inter- and intrafamilial heterogeneity, ranging from mild symptoms of heart failure late in life to the onset of sudden cardiac death at a young age and is caused by a mutation in one of the genes that encode a protein from the sarcomere, Z-disc or intracellular calcium modulators. Although many genes and mutations are already known to cause HCM, the molecular pathways that lead to the phenotype are still unclear. This review focus on the molecular mechanisms of HCM, the pathways from mutation to clinical phenotype and how the disease's genotype correlates with phenotype.

  9. Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis.

    Science.gov (United States)

    Cavalli, Giulio; Berti, Alvise; Fragasso, Gabriele; De Cobelli, Francesco

    2016-12-01

    : Almost invariably associated with chronic HCV infection, cryoglobulinemic vasculitis is a small-vessel vasculitis commonly affecting the skin, kidneys, and peripheral nervous system. Cardiac involvement, possibly due to cardiac microcirculation involvement, is an utterly rare and severe complication. We describe a case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis. Evaluation with transthoracic cardiac ultrasound and cardiac MRI evidenced severe left ventricular hypertrophy and diffuse hypokinesia, a marked decrease in left ventricular ejection fraction, and a subtle late enhancement of inferior and lateral left ventricular walls. Upon clinical stabilization, the patient received treatment with anti-CD20 monoclonal antibody rituximab. Clinical and radiological follow-up with cardiac ultrasound and cardiac MRI documented a dramatic and sustained clinical improvement, with marked reduction of left ventricular hypertrophy, resolution of late enhancement, recovery of left ventricular contractility and function.

  10. Immersion Pulmonary Edema in the Setting of Takotsubo Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Tara Reed

    2016-01-01

    Full Text Available Immersion Pulmonary Edema (IPE is a unique medical condition being increasingly described in the medical literature as sudden-onset pulmonary edema in the setting of scuba diving and/or swimming. We report on three patients with unique presentations of IPE with associated development of Takotsubo cardiomyopathy (TTC. All three cases occurred in Oahu, Hawaii and were seen by the same cardiologist within a span of seven years. Each patient was scuba diving with sudden onset dyspnea with pulmonary edema on chest x-ray. Cardiac catheterization revealed no significant epicardial stenosis or thrombosis. EKGs showed typical evolution of symmetric T wave inversion. Wall motion abnormalities resolved. IPE and TTC may occur together and may be more common than initially thought. Physical and emotional stressors are known to trigger TTC. TTC should be considered as a possible complication of IPE. Initial workup should include EKGs, cardiac enzymes, echocardiogram and, in the appropriate situation, cardiac catheterization.

  11. Pathomorphological Changes of the Myocardium in Canine Dilated Cardiomyopathy (DCM

    Directory of Open Access Journals (Sweden)

    Janus Izabela

    2015-04-01

    Full Text Available The study was conducted on ventricular and atrial wall preparations from 11 dogs with clinically diagnosed dilated cardiomyopathy. After fixation, the specimens were stained with haematoxylin and eosin and Masson-Goldner trichrome technique. Parenchymal changes (fibrosis and fatty infiltration, vascular changes (congestion and coronary vessel wall hypertrophy, degenerative changes (loss of striation, changes in cardiomycyte and nuclei structure, and presence of inflammatory infiltrates (mononuclear and polynuclear were estimated. Complex histological changes in both ventricular and atrial muscles were shown. It was not determined whether the processes occurring in the myocardium have a primary character, or are a consequence of developing heart failure. Such issues will be put under further and more detailed examination.

  12. Hypertrophic cardiomyopathy and ultra-endurance running - two incompatible entities?

    Directory of Open Access Journals (Sweden)

    Wilson Mathew G

    2011-11-01

    Full Text Available Abstract Regular and prolonged exercise is associated with increased left ventricular wall thickness that can overlap with hypertrophic cardiomyopathy (HCM. Differentiating physiological from pathological hypertrophy has important implications, since HCM is the commonest cause of exercise-related sudden cardiac death in young individuals. Most deaths have been reported in intermittent 'start-stop' sports such as football (soccer and basketball. The theory is that individuals with HCM are unable to augment stroke volume sufficiently to meet the demands of endurance sports and are accordingly 'selected-out' of participation in such events. We report the case of an ultra-endurance athlete with 25 years of > 50 km competitive running experience, with genetically confirmed HCM; thereby demonstrating that these can be two compatible entities.

  13. Hypertrophic cardiomyopathy and ultra-endurance running - two incompatible entities?

    Science.gov (United States)

    Wilson, Mathew G; Chandra, Navin; Papadakis, Michael; O'Hanlon, Rory; Prasad, Sanjay K; Sharma, Sanjay

    2011-11-29

    Regular and prolonged exercise is associated with increased left ventricular wall thickness that can overlap with hypertrophic cardiomyopathy (HCM). Differentiating physiological from pathological hypertrophy has important implications, since HCM is the commonest cause of exercise-related sudden cardiac death in young individuals. Most deaths have been reported in intermittent 'start-stop' sports such as football (soccer) and basketball. The theory is that individuals with HCM are unable to augment stroke volume sufficiently to meet the demands of endurance sports and are accordingly 'selected-out' of participation in such events. We report the case of an ultra-endurance athlete with 25 years of > 50 km competitive running experience, with genetically confirmed HCM; thereby demonstrating that these can be two compatible entities.

  14. Ultrastructural myocardial changes in seven cats with spontaneous hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Christiansen, Liselotte Bruun; Prats Gavalda, Clara; Hyttel, Poul

    2015-01-01

    OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is the most common heart disease in cats and shares clinical and pathological characteristics with human HCM. Little is known about the pathogenic mechanisms underlying development of spontaneous feline HCM. ANIMALS: The study population consisted...... of seven cats diagnosed with HCM and eight age-matched cats with no evidence of cardiac disease. METHODS: Fresh myocardial biopsies taken from the middle of the left ventricular posterior free wall were obtained and examined with transmission electron microscopy. RESULTS: Electron microscopic examination...... showed ultrastructural aberrations of the myocardial cytoarchitecture and of the interstitium in the seven cats with HCM. In the most severely affected cats the myofibrils were disorganized and subsarcolemmal mitochondria were depleted. In control cats, contraction band artifacts were commonly seen...

  15. Cardiomyopathies as a Cause of Sudden Cardiac Death (SCD in Egypt: Recognition and Preventive Strategies Needed

    Directory of Open Access Journals (Sweden)

    Nora Fnon

    2016-06-01

    Full Text Available This study aimed at evaluating the epidemiological characteristics and pathological features of different types of cardiomyopathies in Egypt, highlighting the role of the forensic pathologist in identifying cases of cardiomyopathies and initiating for their families a possible genetic study aiming at prevention of sudden death. All cases with sudden cardiac death (SCD due to cardiomyopathies during the period from the beginning of January 2010 until the end of December 2014 (5 years were included in this study. All hearts underwent detailed gross and histological examination. Circumstances of death, medical history, and post-mortem pathological findings were thoroughly  investigated. Out of 535 cases of sudden cardiac death, there were 22 cases (4.1% diagnosed as having cardiomyopathies; sudden death was their first presentation. Eighteen cases (81.8% were male, with the 4th decade (11 cases, 50% being the most affected age; severe physical activity and exertion were evident in death circumstances of 14 cases (63.6%; pathological evaluation revealed that hypertrophic cardiomyopathy was the most frequent type, being diagnosed in 10 cases (45%. Cardiomyopathies are an infrequent cause of sudden cardiac death. Most deaths are in children and adults, so cases are of high social impact that demands multidisciplinary research and resources. In all cases of SCD, forensic autopsy should be done. Forensic study is the key to identifying an affected family and the starting point regarding assessing them.

  16. Role of left ventricular twist mechanics in cardiomyopathies, dance of the helices.

    Science.gov (United States)

    Kauer, Floris; Geleijnse, Marcel Leonard; van Dalen, Bastiaan Martijn

    2015-08-26

    Left ventricular twist is an essential part of left ventricular function. Nevertheless, knowledge is limited in "the cardiology community" as it comes to twist mechanics. Fortunately the development of speckle tracking echocardiography, allowing accurate, reproducible and rapid bedside assessment of left ventricular twist, has boosted the interest in this important mechanical aspect of left ventricular deformation. Although the fundamental physiological role of left ventricular twist is undisputable, the clinical relevance of assessment of left ventricular twist in cardiomyopathies still needs to be established. The fact remains; analysis of left ventricular twist mechanics has already provided substantial pathophysiological understanding on a comprehensive variety of cardiomyopathies. It has become clear that increased left ventricular twist in for example hypertrophic cardiomyopathy may be an early sign of subendocardial (microvascular) dysfunction. Furthermore, decreased left ventricular twist may be caused by left ventricular dilatation or an extensive myocardial scar. Finally, the detection of left ventricular rigid body rotation in noncompaction cardiomyopathy may provide an indispensible method to objectively confirm this difficult diagnosis. All this endorses the value of left ventricular twist in the field of cardiomyopathies and may further encourage the implementation of left ventricular twist parameters in the "diagnostic toolbox" for cardiomyopathies.

  17. Hypertrophic cardiomyopathy: prognostic factors and survival analysis in 128 Egyptian patients.

    Science.gov (United States)

    El-Saiedi, Sonia A; Seliem, Zeinab S; Esmail, Reem I

    2014-08-01

    Hypertrophic cardiomyopathy is an important cause of disability and death in patients of all ages. Egyptian children may differ from Western and Asian patients in the pattern of hypertrophy distribution, clinical manifestations, and risk factors. The aim of our study was to report the clinical characteristics and outcomes of Egyptian children with hypertrophic cardiomyopathy studied over a 7-year duration and to determine whether the reported adult risk factors for sudden cardiac death are predictive of the outcome in these affected children. This retrospective study included 128 hypertrophic cardiomyopathy children. The data included personal history, family history, physical examination, baseline laboratory measurements, electrocardiogram, and Holter and echocardiographic results. Logistic regression analysis was used for the detection of risk factors of death. Fifty-one out of 128 patients died during the period of the study. Of the 51 deaths, 36 (70.5%) occurred in patients presenting before 1 year of age. Only eight patients had surgical intervention. Extreme left ventricular hypertrophy, that is, interventricular septal wall thickness or posterior wall thickness Z-score >6, sinus tachycardia, and supraventricular tachycardia were found to be independent risk factors for prediction of death in patients with hypertrophic cardiomyopathy. At our Egyptian tertiary care centre, hypertrophic cardiomyopathy has a relatively worse prognosis when compared with reports from Western and Asian series. Infants have a worse outcome than children presenting after the age of 1 year. A poorer prognosis in childhood hypertrophic cardiomyopathy is predicted by an extreme left ventricular hypertrophy, the presence of sinus tachycardia, and supraventricular tachycardia.

  18. [Gene mutation and clinical phenotype analysis of patients with Noonan syndrome and hypertrophic cardiomyopathy].

    Science.gov (United States)

    Liu, X H; Ding, W W; Han, L; Liu, X R; Xiao, Y Y; Yang, J; Mo, Y

    2017-10-02

    Objective: To analyze the gene mutations and clinical features of patients with Noonan syndrome and hypertrophic cardiomyopathy. Method: Determined the mutation domain in five cases diagnosed with Noonan syndrome and hypertrophic cardiomyopathy and identified the relationship between the mutant domain and hypertrophic cardiomyopathy by searching relevant articles in pubmed database. Result: Three mutant genes (PTPN11 gene in chromosome 12, RIT1 gene in chromosome 1 and RAF1 gene in chromosome 3) in five cases all had been reported to be related to hypertrophic cardiomyopathy. The reported hypertrophic cardiomyopathy relevant genes MYPN, MYH6 and MYBP3 had also been found in case 1 and 2. Patients with same gene mutation had different clinical manifestations. Both case 4 and 5 had RAF1 mutation (c.770C>T). However, case 4 had special face, low IQ, mild pulmonary artery stenosis, and only mild ventricular hypertrophy. Conclusion: Noonan syndrome is a genetic heterogeneity disease. Our study identified specific gene mutations that could result in Noonan syndrome with hypertrophic cardiomyopathy through molecular biology methods. The results emphasize the importance of gene detection in the management of Noonan syndrome.

  19. Prevalence and Risk Factors of Stress Cardiomyopathy After Convulsive Status Epilepticus in ICU Patients.

    Science.gov (United States)

    Belcour, Dominique; Jabot, Julien; Grard, Benjamin; Roussiaux, Arnaud; Ferdynus, Cyril; Vandroux, David; Vignon, Philippe

    2015-10-01

    Although stress cardiomyopathy has been described in association with epilepsy, its frequency in patients with convulsive status epilepticus remains unknown. Accordingly, we sought to determine the prevalence and risk factors of stress cardiomyopathy in patients admitted to the ICU for convulsive status epilepticus. Prospective, descriptive, single-center study. Medical-surgical ICU of a teaching hospital. Thirty-two consecutive ventilated patients (21 men; age, 50 ± 18 yr; Simplified Acute Physiology Score II, 53 ± 15; Sequential Organ Failure Assessment, 6 ± 2) hospitalized in the ICU for convulsive status epilepticus. None. Hemodynamic parameters, transthoracic echocardiography, biological data, and electrocardiogram were obtained serially on ICU admission (H0), and after 6, 12, 24, and 48 hours of hospitalization (H6, H12, H24, and H48). Stress cardiomyopathy was defined as a 20% decrease in left ventricular ejection fraction between H0 or H6 and H48. Stress cardiomyopathy was diagnosed in 18 patients (56%; 95% CI, 38-74%). Mean left ventricular ejection fraction, left ventricular stroke index and cardiac index were initially (at H0 or H6 according to lowest individual values) significantly reduced in stress cardiomyopathy patients (45 ± 14% vs 61 ± 6%, p ICU for convulsive status epilepticus. Accordingly, these patients should be screened for stress cardiomyopathy and monitored if they present with hemodynamic compromise.

  20. Association of caspase-1 polymorphisms with Chagas cardiomyopathy among individuals in Santa Cruz, Bolivia.

    Science.gov (United States)

    Fu, Katherine Yih-Jia; Zamudio, Roxana; Henderson-Frost, Jo; Almuedo, Alex; Steinberg, Hannah; Clipman, Steven Joseph; Duran, Gustavo; Marcus, Rachel; Crawford, Thomas; Alyesh, Daniel; Colanzi, Rony; Flores, Jorge; Gilman, Robert Hugh; Bern, Caryn

    2017-01-01

    Trypanosoma cruzi (Tc) infection is usually acquired in childhood in endemic areas, leading to Chagas disease, which progresses to Chagas cardiomyopathy in 20-30% of infected individuals over decades. The pathogenesis of Chagas cardiomyopathy involves the host inflammatory response to T. cruzi, in which upstream caspase-1 activation prompts the cascade of inflammatory chemokines/cytokines, cardiac remodeling, and myocardial dysfunction. The aim of the present study was to examine the association of two caspase-1 single nucleotide polymorphisms (SNPs) with cardiomyopathy. We recruited infected (Tc+, n = 149) and uninfected (Tc-, n = 87) participants in a hospital in Santa Cruz, Bolivia. Cardiac status was classified (I, II, III, IV) based on Chagas cardiomyopathy-associated electrocardiogram findings and ejection fractions on echocardiogram. Genotypes were determined using Taqman probes via reverse transcription-polymerase chain reaction of peripheral blood DNA. Genotype frequencies were analyzed according to three inheritance patterns (dominant, recessive, additive) using logistic regression adjusted for age and sex. The AA allele for the caspase-1 SNP rs501192 was more frequent in Tc+ cardiomyopathy (classes II, III, IV) patients compared to those with a normal cardiac status (class I) [odds ratio (OR) = -2.18, p = 0.117]. This trend approached statistical significant considering only Tc+ patients in class I and II (OR = -2.64, p = 0.064). Caspase-1 polymorphisms may play a role in Chagas cardiomyopathy development and could serve as markers to identify individuals at higher risk for priority treatment.

  1. Magnetic resonance imaging of dilated cardiomyopathy; MRT bei dilatativen Kardiomyopathien

    Energy Technology Data Exchange (ETDEWEB)

    D' Anastasi, M. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Greif, M. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Medizinische Klinik und Poliklinik I, Muenchen (Germany); Reiser, M.F.; Theisen, D. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Deutsches Zentrum fuer Herzkreislaufforschung (DZHK), Muenchen (Germany)

    2013-01-15

    Dilated cardiomyopathy (DCM) is the most common type of cardiomyopathy with a prevalence of 1 out of 2,500 in adults. Due to mild clinical symptoms in the early phase of the disease, the true prevalence is probably even much higher. Patients present with variable clinical symptoms ranging from mild systolic impairment of left ventricular function to congestive heart failure. Even sudden cardiac death may be the first clinical symptom of DCM. The severity of the disease is defined by the degree of impairment of global left ventricular function. Arrhythmias, such as ventricular or supraventricular tachycardia, atrioventricular (AV) block, ventricular extrasystole and atrial fibrillation are common cardiac manifestations of DCM. Magnetic resonance imaging (MRI) plays an important role in the exact quantification of functional impairment of both ventricles and in the evaluation of regional wall motion abnormalities. With its excellent ability for the assessment of myocardial structure, it is becoming increasingly more important for risk stratification and therapy guidance. (orig.) [German] Die dilatative Kardiomyopathie (DCM) ist die haeufigste Form der Kardiomyopathie mit einer Praevalenz von 1/2500 Erwachsenen. Aufgrund der zunaechst milden klinischen Symptomatik ist jedoch von einer relativ hohen Dunkelziffer auszugehen. Die klinische Praesentation ist variabel, die Schwere der Erkrankung wird vom Ausmass der systolischen Funktionseinschraenkung bestimmt. Herzrhythmusstoerungen, wie ventrikulaere oder supraventrikulaere Tachykardien, AV-Blockierungen, ventrikulaere Extrasystolen und Vorhofflimmern sind moegliche klinische Manifestationen. Bei manchen Patienten ist der ploetzliche Herztod die erste klinische Manifestation der Erkrankung. Die kardiale MRT spielt eine bedeutende Rolle fuer die Beurteilung des Ausmasses der ventrikulaeren Dilatation, Dysfunktion und fuer die Beurteilung regionaler Wandbewegungsstoerungen. Darueber hinaus kann sie zur Anwendung kommen

  2. Hypertensive cardiomyopathy in horses: 5 cases (1995-2011).

    Science.gov (United States)

    Navas de Solis, Cristobal; Slack, JoAnn; Boston, Raymond C; Reef, Virginia B

    2013-07-01

    To describe the prognosis and clinical, echocardiographic, and pathological features of hypertensive cardiomyopathy in horses. Retrospective case series. 5 horses with cardiac hypertrophy and systemic hypertension. Demographics, history, physical and cardiological examination findings, diagnosis, clinical progression, prognosis, and pathological findings were obtained from medical records. The primary diagnosis was chronic laminitis in 3 horses and chronic renal failure in 2. Persistent tachycardia, hypertension, chronic laminitis, or a combination of these prompted the cardiac evaluations. Blood pressure values (median [range]), measured noninvasively, were determined as 190 mm Hg (183 to 261 mm Hg) for systolic pressure, 126 mm Hg (100 to 190 mm Hg) for diastolic pressure, and 155 mm Hg (126 to 222 mm Hg) for mean pressure. No arrhythmias were reported. For the left ventricle, all horses had increased relative wall thickness, mean wall thickness, and ventricular mass. The interventricular septum was thickened at end diastole (n = 5) and in peak systole (4). The left ventricular internal diameter was small at end diastole (n = 4) and in peak systole (3). The left ventricular free wall was thickened at end diastole (n = 3) and in peak systole (4). No associations between blood pressure and variables consistent with hypertrophy were detected. All horses were euthanized because of the grave prognosis of the primary diseases. All 3 horses that underwent postmortem evaluation had cardiovascular abnormalities. Hypertensive cardiomyopathy should be considered as a comorbid diagnosis in horses with laminitis or chronic renal failure. Information about the development, progression, reversibility, importance of early detection, and long-term sequelae of this condition is needed.

  3. Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy.

    Science.gov (United States)

    Alexander, Peta M A; Daubeney, Piers E F; Nugent, Alan W; Lee, Katherine J; Turner, Christian; Colan, Steven D; Robertson, Terry; Davis, Andrew M; Ramsay, James; Justo, Robert; Sholler, Gary F; King, Ingrid; Weintraub, Robert G

    2013-10-29

    Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy. The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to time of diagnosis. Survival free from death or transplantation was 74% (95% confidence interval, 67-80) 1 year after diagnosis, 62% (95% confidence interval, 55-69) at 10 years, and 56% (95% confidence interval, 46-65) at 20 years. In multivariable analysis, age at diagnosis 5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy. Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication.

  4. Sudden death associated with group A streptococcal infection in an 8-year-old girl with undiagnosed hypertrophic cardiomyopathy.

    OpenAIRE

    Bragonier, R.; Oades, P.

    1998-01-01

    An 8-year-old girl died suddenly without prior symptoms. Post-mortem examination identified both systemic group A streptococcal infection and hypertrophic cardiomyopathy. She had no history of cardiac symptoms and was not in a high-risk group for sudden death due to hypertrophic cardiomyopathy. We believe the disseminated but asymptomatic group A streptococcal infection precipitated her early death from hypertrophic cardiomyopathy. Sudden unexpected death during systemic infection should be f...

  5. Animal models of arrhythmogenic right ventricular cardiomyopathy: what have we learned and where do we go? Insight for therapeutics.

    Science.gov (United States)

    Padrón-Barthe, Laura; Domínguez, Fernando; Garcia-Pavia, Pablo; Lara-Pezzi, Enrique

    2017-09-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically-determined cardiac heart muscle disorder characterized by fibro-fatty replacement of the myocardium that results in heart failure and sudden cardiac death (SCD), predominantly in young males. The disease is often caused by mutations in genes encoding proteins of the desmosomal complex, with a significant minority caused by mutations in non-desmosomal proteins. Existing treatment options are based on SCD prevention with the implantable cardioverter defibrillator, antiarrhythmic drugs, and anti-heart failure medication. Heart transplantation may also be required and there is currently no cure. Several genetically modified animal models have been developed to characterize the disease, assess its progression, and determine the influence of potential environmental factors. These models have also been very valuable for translational therapeutic approaches, to screen new treatment options that prevent and/or reverse the disease. Here, we review the available ARVC animal models reported to date, highlighting the most important pathophysiological findings and discussing the effect of treatments tested so far in this setting. We also describe gaps in our knowledge of the disease, with the goal of stimulating research and improving patient outcomes.

  6. Sudden cardiac arrest in a young patient with hypertrophic cardiomyopathy and zero canonical risk factors: the inherent limitations of risk stratification in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Kohorst, John J; Bos, J Martijn; Hagler, Donald J; Ackerman, Michael J

    2014-01-01

    Hypertrophic cardiomyopathy is the most common heritable cardiovascular disease and a common cause of sudden cardiac death (SCD) in young adolescents and athletes. Clinical risk stratification for SCD is predicated on the presence of established risk factors; however, this assessment is far from perfect. Herein, we present a 16-year-old male who was resuscitated successfully from his sentinel event of out-of-hospital cardiac arrest. Prior to this event, he was asymptomatic and lacked all traditional SCD-predisposing risk factors for hypertrophic cardiomyopathy. © 2013 Wiley Periodicals, Inc.

  7. HA-Coated Implant

    DEFF Research Database (Denmark)

    Daugaard, Henrik; Søballe, Kjeld; Bechtold, Joan E

    2014-01-01

    The goal of osseointegration of orthopedic and dental implants is the rapid achievement of a mechanically stable and long lasting fixation between living bone and the implant surface. In total joint replacements of cementless designs, coatings of calcium phosphates were introduced as a means...... of improving the fixation of implants. Of these, hydroxyapatite (HA) is the most widely used and most extensively investigated. HA is highly osseoconductive, and the positive effect is well documented in both basic and long-term clinical research [1–6]. This chapter describes experimental and clinical studies...... evaluating bone-implant fixation with HA coatings....

  8. Ion implantation technology

    CERN Document Server

    Downey, DF; Jones, KS; Ryding, G

    1993-01-01

    Ion implantation technology has made a major contribution to the dramatic advances in integrated circuit technology since the early 1970's. The ever-present need for accurate models in ion implanted species will become absolutely vital in the future due to shrinking feature sizes. Successful wide application of ion implantation, as well as exploitation of newly identified opportunities, will require the development of comprehensive implant models. The 141 papers (including 24 invited papers) in this volume address the most recent developments in this field. New structures and possible approach

  9. ENDEAVOUR: Phase 3 Multicenter Study of Revusiran (ALN-TTRSC) in Patients With Transthyretin (TTR) Mediated Familial Amyloidotic Cardiomyopathy (FAC)

    Science.gov (United States)

    2017-12-08

    Transthyretin (TTR) Mediated Familial Amyloidotic Cardiomyopathy (FAC); Amyloidosis, Hereditary; Amyloid Neuropathies, Familial; Amyloid Neuropathies; Amyloidosis, Hereditary, Transthyretin-Related; Familial Transthyretin Cardiac Amyloidosis

  10. Myocardial perfusion in patients with non-ischaemic systolic heart failure and type 2 diabetes

    DEFF Research Database (Denmark)

    Byrne, Christina; Hasbak, Philip; Kjaer, Andreas

    2018-01-01

    reserve (myocardial flow reserve (MFR) = stress flow/rest flow) as a measure of the myocardial vasomotor function; 28 patients with T2DM and 123 without T2DM were included. All patients received heart failure treatment according to guidelines. Multiple regression analysis was performed to assess...... the association between T2DM and MFR. Age [68 (60-75) years vs. 68 (62-72) years; P = 0.84] and female sex (21% vs. 33%; P = 0.26) were similar between patients with and without T2DM. Patients with T2DM had higher body mass index, (29.9 vs. 26.5 kg/m2; P = 0.02), higher blood glucose (6.2 vs. 5.7 mmol/L; P = 0.......03), more often hypertension (50 vs. 27%; P = 0.02) and received more cholesterol lowering medication (61 vs. 35%; P = 0.02). Apart from this, the groups were similar. In a multivariable analysis, MFR was 16% lower in patients with T2DM compared with patients without [estimate - 16%; 95% confidence interval...

  11. [Myocardial regional thickness in patients with and without cardiomyopathy assessed by cardiac magnetic resonance].

    Science.gov (United States)

    de Zan, Macarena; Carrascosa, Patricia; Deviggiano, Alejandro; Capuñay, Carlos; Rodríguez-Granillo, Gastón A

    To explore regional differences in myocardial wall thickness (WT) among the most prevalent cardiomyopathies and in individuals without structural heart disease using cardiac magnetic resonance. Patients older than 18 years referred to cardiac magnetic resonance during the period between January 2014 and September 2014, with a diagnosis of hypertrophic cardiomyopathy, idiopathic dilated cardiomyopathy, ischemic cardiomyopathy, and myocarditis were retrospectively selected from our database. One hundred twenty patients patients were included. The control group had an average WT of 5.9±1.1mm, with a WT index of 2.9±0.8. Significantly lower mean WT in the apical segments were identified in both the control group (basal 6.7±1.3 vs. mid 6.0±1.3 vs. apical 4.6±1.0mm, P<.0001) and in all evaluated cardiomyopathies (hypertrophic cardiomyopathy: basal 10.5±2.4 vs. mid 10.8±2.7 vs. apical 7.3±3.3mm, P<.0001; idiopathic dilated cardiomyopathy: basal 7.7±1.7 vs. mid 7.6±1.3 vs. apical 5.4±1.3mm, P<.0001; ischemic cardiomyopathy: basal 7.4±1.7 vs. mid 7.5±1.9 vs. apical 5.5±1.8mm, P<.0001; myocarditis: basal 7.1±1.5 vs. mid 6.4±1.1 vs. apical 5.1±0.8, P<.0001). Significant gender differences were also evident regarding the mean WT both in the control group (male 6.5±2.1 vs. female 5.2±1.7mm, P<.0001), as in hypertrophic cardiomyopathy (10.5±5.3 vs. 8.5±5.7mm, P<.0001) and myocarditis (6.6±2.0 vs. 5.2±1.6mm, P<.0001). We found a relatively high prevalence of segments commonly deemed thinned among patients without structural heart disease. We also observed a marked asymmetry and longitudinal gradient in wall thickness both in controls and in the various cardiomyopathies evaluated. Copyright © 2016 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

  12. Antimicrobial coatings for implant surfaces

    OpenAIRE

    Brunetto, Priscilla S.; Fromm, Katharina M.

    2008-01-01

    Body-foreign materials are used more and more frequently in our lives: joint implants (hips, knees, fingers, etc.), catheters, pacemakers, dental and aesthetic implants, etc. The increasing numbers of patients requiring such implants also raises the absolute numbers of implant-related infections. Thus, it is known that body-foreign materials are prone to bacterial adhesion and subsequent biofilm formation, either via bacterial debris on implant materials, infections during implantation or, la...

  13. Minimally Invasive Implantation of HeartWare Assist Device and Simultaneous Tricuspid Valve Reconstruction Through Partial Upper Sternotomy.

    Science.gov (United States)

    Hillebrand, Julia; Hoffmeier, Andreas; Djie Tiong Tjan, Tonny; Sindermann, Juergen R; Schmidt, Christoph; Martens, Sven; Scherer, Mirela

    2017-05-01

    Left ventricular assist device (LVAD) implantation is a well-established therapy to support patients with end-stage heart failure. However, the operative procedure is associated with severe trauma. Third generation LVADs like the HeartWare assist device (HeartWare, Inc., Framingham, MA, USA) are characterized by enhanced technology despite smaller size. These devices offer new minimally invasive surgical options. Tricuspid regurgitation requiring valve repair is frequent in patients with the need for mechanical circulatory support as it is strongly associated with ischemic and nonischemic cardiomyopathy. We report on HeartWare LVAD implantation and simultaneous tricuspid valve reconstruction through minimally invasive access by partial upper sternotomy to the fifth left intercostal space. Four male patients (mean age 51.72 ± 11.95 years) suffering from chronic heart failure due to dilative (three patients) and ischemic (one patient) cardiomyopathy and also exhibiting concomitant tricuspid valve insufficiency due to annular dilation underwent VAD implantation and tricuspid valve annuloplasty. Extracorporeal circulation was established via the ascending aorta, superior vena cava, and right atrium. In all four cases the LVAD implantation and tricuspid valve repair via partial median sternotomy was successful. During the operative procedure, no conversion to full sternotomy was necessary. One patient needed postoperative re-exploration because of pericardial effusion. No postoperative focal neurologic injury was observed. New generation VADs are advantageous because of the possibility of minimally invasive implantation procedure which can therefore minimize surgical trauma. Concomitant tricuspid valve reconstruction can also be performed simultaneously through partial upper sternotomy. Nevertheless, minimally invasive LVAD implantation is a challenging operative technique. © 2016 International Center for Artificial Organs and Transplantation and Wiley Periodicals

  14. Percutaneous and skeletal biocarbon implants

    Science.gov (United States)

    Mooney, V.

    1977-01-01

    Review of carbon implants developed by NASA discussed four different types of implants and subsequent improvements. Improvements could be of specific interest to rehabilitation centers and similar organizations.

  15. Peri-Implant Diseases

    Science.gov (United States)

    ... Dentists about How Often They Floss Their Teeth Oral Hygiene Habits and Hypertension Risk Alcohol Consumption and Gum ... peri-implant disease include previous periodontal disease diagnosis, poor ... tooth. With a proper oral health routine, your dental implant can last a ...

  16. Biocompatible implant surface treatments

    Directory of Open Access Journals (Sweden)

    Bikash Pattanaik

    2012-01-01

    Limitation of this study is that we tried to give a broader overview related to implant surface treatments. It does not give any conclusion regarding the best biocompatible implant surface treatment investigated till date. Unfortunately, the eventually selected studies were too heterogeneous for inference of data.

  17. Ion implantation into diamond

    International Nuclear Information System (INIS)

    Sato, Susumu

    1994-01-01

    The graphitization and the change to amorphous state of diamond surface layer by ion implantation and its characteristics are reported. In the diamond surface, into which more than 10 16 ions/cm 2 was implanted, the diamond crystals are broken, and the structure changes to other carbon structure such as amorphous state or graphite. Accompanying this change of structure, the electric conductivity of the implanted layer shows two discontinuous values due to high resistance and low resistance. This control of structure can be done by the temperature of the base during the ion implantation into diamond. Also it is referred to that by the base temperature during implantation, the mutual change of the structure between amorphous state and graphite can be controlled. The change of the electric resistance and the optical characteristics by the ion implantation into diamond surface, the structural analysis by Raman spectroscopy, and the control of the structure of the implanted layer by the base temperature during implantation are reported. (K.I.)

  18. HA-Coated Implant

    DEFF Research Database (Denmark)

    Daugaard, Henrik; Søballe, Kjeld; Bechtold, Joan E

    2014-01-01

    The goal of osseointegration of orthopedic and dental implants is the rapid achievement of a mechanically stable and long lasting fixation between living bone and the implant surface. In total joint replacements of cementless designs, coatings of calcium phosphates were introduced as a means...

  19. Dynamic ion implantation

    International Nuclear Information System (INIS)

    Oppenheim, I.F.C.

    1988-01-01

    The Dynamic Ion Implantation Technique consists of ion implantation of a film during the film-deposition process. This technique was investigated theoretically and experimentally with ions whose incident energy is of the order of a few times 100 keV. It was found to be a viable alternative low-temperature method for the preparation of thick zirconium nitride films (∼1 μm) with good mechanical properties. Theoretical modeling of the processes involved during dynamic ion implantation lead to analytical expressions for the ions' depth-profile distributions. Numerical evaluations of these equations indicated that the depth distributions of dynamically implanted ions are in general more uniform than those predicted by the model for ions implanted by more conventional techniques. Mechanical properties of stoichiometric RF sputter-deposited zirconium nitride films post implanted with krypton and rubidium ions were investigated. Scratch-adhesion critical load and Vickers microhardness of samples implanted with doses varying from 1 x 10 15 to 5 x 10 16 ions/cm 2 and energies ranging from 300 to 500 keV were studied. In general, best mechanical properties were observed for 300- keV krypton implantations

  20. Reversal of glycogen storage disease type IIIa-related cardiomyopathy with modification of diet

    Science.gov (United States)

    Zori, R. T.; McCune, H.; Maisenbacher, M. K.; Ivsic, T.; Weinstein, D. A.

    2013-01-01

    Summary Glycogen storage disease type III (GSD III) is caused by a deficiency in debranching enzyme, which leads to an accumulation of abnormal glycogen called limit dextrin in affected tissues. Muscle and liver involvement is present in GSD type IIIa, while the defect is limited to the liver only in GSD type IIIb. Besides skeletal muscle involvement, a cardiomyopathy resembling idiopathic hypertrophic cardiomyopathy is seen. Management consists of maintaining normoglycaemia by supplementation with cornstarch therapy and/or protein. While studies are lacking regarding the best treatment for skeletal muscle disease, a high-protein diet was previously reported to be beneficial. No cases of improvement in cardiomyopathy have been reported. Our patient presented in infancy with hypoglycaemia and hepatomegaly. His prescribed management consisted of cornstarch supplementation and a high-protein diet providing 20% of his total energy needs. At 16 years of age, he developed a severe cardiomyopathy with a left ventricular mass index of 209 g/m2. The cardiomyopathy remained stable on a protein intake of 20–25% of total energy. At age 22 years, the diet was changed to increase his protein intake to 30% of total energy and minimize his cornstarch therapy to only what was required to maintain normoglycaemia. Dramatic improvement in the cardiomyopathy occurred. Over one year, his left ventricular mass index decreased from 159.7 g/m2 to 78 g/m2 (normal 50–86 g/m2) and the creatine kinase levels decreased from 455 U/L to 282 U/L. Avoidance of overtreatment with carbohydrate and a high-protein diet can reverse and may prevent cardiomyopathy. PMID:19322675