Secondary Cardiomyopathy Research Articles

Role of echocardiography for takotsubo cardiomyopathy: clinical and prognostic implications.

Takotsubo cardiomyopathy (TTC) is newly-described secondary cardiomyopathy characterized by transient left ventricular (LV) dysfunction, which is increasingly recognized in the field of cardiology. TTC occurs in approximately 2% of the patients with acute coronary syndrome. Its onset is rare; however, its specific features play a crucial role in diagnosing the chest pain in clinical practice. TTC has generally favorable outcome with rapid recovery of LV function; however, an increasing evidence suggests that it should be regarded as a more serious acute cardiac disorder with a variety of complications. Owing to its widespread availability, even in emergency settings, transthoracic echocardiography plays a key role in the diagnostic assessment of TTC and contributes to an increased number of disease detection and incidence reports in contemporary clinical practice. This review focuses on the role of echocardiography in understanding the clinical prognostic implications in patients with TTC.

Predictive factors of left ventricular reverse remodeling in patients with idiopathic or secondary dilated cardiomyopathy

To investigate the occurrence of left ventricular reverse remodeling (LVRR) and its predictive factors in patients with idiopathic or secondary dilated cardiomyopathy (DCM). A cross-sectional survey was conducted in a consecutive cohort of patients with DCM admitted in our department between January, 2012 and June, 2016. Based on dynamic echocardiographic findings, LVRR was defined as an absolute increase in left ventricular ejection fraction (LVEF) by ≥100% or an absolute value of LVEF ≥45% with simultaneously an absolute decrease in end-diastolic diameter (LVEDD) ≥10 mm or an absolute value of LVEDD ≤55 mm (in men) or ≤50 mm (in women). The patients with LVRR and those without LVRR were compared for clinical data at admission to identify the potential factors for predicting LVRR. A total of 462 patients, who were followed up for 24.13±15.60 months, were included in this survey. In patients with idiopathic DCM who had LVRR, LVEDD was reduced (P < 0.01), LVEF was improved (P < 0.01) and the mean exercise tolerance was increased significantly (P < 0.01) compared with those in patients without LVRR. Multiple logistic regression analysis showed that a shorter course of heart failure (OR=0.913, P < 0.01), a high systolic blood pressure (OR=1.062, P < 0.01), absence of electrolyte imbalance (OR=0.347, P < 0.01), a low red cell distribution width (OR=0.205, P < 0.01), a smaller LVEDD (OR=0.799, P < 0.01) and a greater LVEF (OR= 1.142, P < 0.01) were independent predictors of LVRR in the idiopathic patients. In patients with secondary DCM, LVEDD was reduced (P < 0.01), LVEF was improved (P < 0.01), and the mean exercise tolerance was increased significantly (P < 0.01) compared with those in patients without LVRR. Multiple logistic regression analysis showed that a shorter course of heart failure (OR= 0.954, P < 0.01), a low red cell distribution width (OR=1.011, P < 0.01), and implementation of etiological treatment (OR=1.073, P < 0.01) were independent predictors of LVRR in patients with secondary DCM. The exercise tolerance, cardiac structure and function can be reversed in some of the patients with idiopathic or secondary DCM by administration of standard therapy for heart failure and etiological treatment.

Сравнительная оценка влияния треосульфан- и бусульфан- содержащих миелоаблативных режимов кондиционирования на поздние осложнения аллогенных ТГСК при злокачественных заболеваниях у детей

Treosulfan-based regimens used in conditioning for allogeneic HSCT (allo-HSCT) in children allow to reduce incidence of transplant-related mortality and early toxicity, but there are no data about long-term effects. We propose a retrospective comparative study of late complications after allo-HSCT in 135 children with hematologic malignancies, who got allo-HSCT between January 1994 and July 2011 and survived at least 1 year after HSCT. Five-year event free survival was 75.6% in the group of children who got busulfan and 66.5% in patients with treosulfan-based conditioning; 5-year nonrelapse mortality was 6% and 10.2%, respectively. Late effects analysis demonstrated that treosulfan-containing conditioning allowed to reduce the incidence of some late complications: hypergonadotropic hypogonadism cumulative incidence was 0 compared to 32.8% in busulfan-conditioning group (р = 0.012); the decrease in subclinical hypothyroidism, secondary cardiomyopathy and organic central nervous system pathology incidence was revealed. Thus treosulfan-based conditioning regimen is an effective approach in reducing of serious late complications after allogeneic HSCT when compared with data of busulfan-based conditioning long-term effects.

Detection of cardiomyopathy in children with Marfan Syndrome with 2D strain echocardiography

Studies have found cardiomyopathy in adult patients with marfan syndrome (MS) with systolic dysfunction. Sophisticated techniques such as 2D strain echocardiography or “speckle tracking imaging” (STI) have been reported to detect early cardiac dysfunction and to be more accurately. The aim of our study was to evaluate the validity of STI for the study of myocardial function in children with MS, and to assess the interest for cardiomyopathy detection in this population. Echocardiographic parameters of left ventricular systolic function were compared between two populations in standard 2D, STI and cardiac MRI. We included 39 MS (clinically confirmed from the 2010 modified criteria of Ghent or genetically with mutations on the FBN1 gene) and 41 healthy children, aged 4 to 18 years old (respectively mean ± age SD 12.07 ± 4.81 and 11.82 ± 3.64). They were paired on the body surface. The values of global strain longitudinal peak (SLG) of MS were compared with values of ejection fraction of the left ventricle (LVEF) assessed by cardiac MRI (gold standard). Relations between the two types of mutations (PTC and inframe) in SM and altered strain were studied. Impaired SLG was significant for 7 segments: basal inferoseptal ( P = 0.0001), mid anterolateral ( P = 0.0083), basal anterolateral ( P < 0.0001), basal inferior ( P = 0.0057), apical lateral ( P = 0.0084), basal anterior ( P < 0.0001) and basal inferolateral ( P = 0.0103). This segmental impairment predominated the basal level with apex-to-base gradient. Patients with more altered SLG appeared to have a greater dilatation of the ascending aorta ( P = 0.03). This study is the largest pediatric cohort who compare 2D strain echocardiography and MRI in children with SM. The interest for primary or secondary cardiomyopathy detection remains to be validated and consolidated with larger studies and could justify not only to focus on the aorta.

Bloqueo auriculoventricular fetal completo: enfoque diagnóstico y terapéutico; reporte de caso en Bogotá, Colombia y revisión de la literatura

Objetivo: reportar un caso de bloqueo auriculoventricular completo congénito y realizar una revisión de la literatura del diagnóstico y tratamiento.Materiales y métodos: se reporta el caso de una gestante de 27 años quien consulta a un hospital general de alto nivel de complejidad, con un embarazo de 33 semanas, con feto único, con diagnóstico de bloqueo auriculoventricular completo y cardiomiopatía dilatada secundaria. Se inició manejo prenatal con betamimético con pobre respuesta, por lo cual fue necesario finalizar el embarazo. El recién nacido requiere implantación de marcapasos ventricular en el primer día de vida con excelentes resultados en el seguimiento a un año. Se realiza revisión de la literatura publicada en las bases de datos: Medline vía PubMed, Lilacs y SciELO mediante los términos: “fetal complete atrioventricular block”, “congenital complete heart block”, con límites de año de 2000 a 2016, en español e inglés.Resultados: se obtuvieron 21 publicaciones; siete reportes de caso, diez revisiones de literatura, cuatro estudios de cohorte. El diagnóstico se basa en la ecocardiografía fetal para determinar el intervalo PR, la relación auriculoventricular y la detección de anomalías intracardiacas, entre las que se encuentran: regurgitación valvular, hiperecogenicidad miocárdica/valvular-fibroelastosis endocárdica, contracciones atriales prematuras y efusión pericárdica. Respecto al tratamiento prenatal, los medicamentos más utilizados son los corticoesteroides y los betamiméticos. El tratamiento de la bradiarritimia severa refractaria neonatal puede requerir la implantación de un marcapaso como manejo definitivo.Conclusión: el bloqueo AV congénito de tercer grado requiere diagnóstico temprano y tratamiento oportuno, ya que se acompaña de alta morbimortalidad perinatal. Se requieren estudios con mayor calidad metodológica que permitan avalar otras opciones y esquemas terapéuticos prometedores.

Dilated Cardiomyopathy in Children at Saiful Anwar General Hospital

Background: Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by ventricular dilatation and decreased systolic function. The cause of DCM is multifactorial. Although most cases are idiopathic, nowadays secondary dilated cardiomyopathy due to systemic disease such as collagen disease, severe infection or after anthracycline administration.

Cardiovascular Benefits of Moderate Exercise Training in Marfan Syndrome: Insights From an Animal Model.

BackgroundMarfan syndrome (MF) leads to aortic root dilatation and a predisposition to aortic dissection, mitral valve prolapse, and primary and secondary cardiomyopathy. Overall, regular physical exercise is recommended for a healthy lifestyle, but dynamic sports are strongly discouraged in MF patients. Nonetheless, evidence supporting this recommendation is lacking. Therefore, we studied the role of long‐term dynamic exercise of moderate intensity on the MF cardiovascular phenotype.Methods and ResultsIn a transgenic mouse model of MF (Fbn1 C1039G/+), 4‐month‐old wild‐type and MF mice were subjected to training on a treadmill for 5 months; sedentary littermates served as controls for each group. Aortic and cardiac remodeling was assessed by echocardiography and histology. The 4‐month‐old MF mice showed aortic root dilatation, elastic lamina rupture, and tunica media fibrosis, as well as cardiac hypertrophy, left ventricular fibrosis, and intramyocardial vessel remodeling. Over the 5‐month experimental period, aortic root dilation rate was significantly greater in the sedentary MF group, compared with the wild‐type group (∆mm, 0.27±0.07 versus 0.13±0.02, respectively). Exercise significantly blunted the aortic root dilation rate in MF mice compared with sedentary MF littermates (∆mm, 0.10±0.04 versus 0.27±0.07, respectively). However, these 2 groups were indistinguishable by aortic root stiffness, tunica media fibrosis, and elastic lamina ruptures. In MF mice, exercise also produced cardiac hypertrophy regression without changes in left ventricular fibrosis.ConclusionsOur results in a transgenic mouse model of MF indicate that moderate dynamic exercise mitigates the progression of the MF cardiovascular phenotype.

SY15-1 - Diagnosis of Secondary Cardiomyopathy According to Endomyocardial Cardiac Biopsy

SY15-1 - Diagnosis of Secondary Cardiomyopathy According to Endomyocardial Cardiac Biopsy

SY15-2 - Approach to Diagnosis of Secondary Cardiomyopathies With Left Ventricular Hypertrophy

SY15-2 - Approach to Diagnosis of Secondary Cardiomyopathies With Left Ventricular Hypertrophy

WITHDRAWN Ventricular Arrhythmias in Non-ischemic Cardiomyopathy

Abstract Non-ischemic cardiomyopathies (NICMs) are composed of variable disease entities, including primary and secondary cardiomyopathies. Determining the etiology of NICM provides pivotal roles of not only the understanding of the individual pathogenesis, but also the clinical management, such as risk stratification, pharmacological treatment, and intervention therapies. Despite the diverse causes of NICM, these cases mostly require clinical attention owing to progressive myocardial injury, resulting in ventricular dysfunction and heart failure. The interaction between the diseased ventricular substrates and systemic/neurophysiological factors contributes to the cornerstones responsible for ventricular arrhythmogenesis and sudden cardiac death (SCD). Prevention of SCD and diminishing ventricular tachyarrhythmias are the important mainstays for the management of NICM patients. Given the understanding of the abnormal ventricular substrates and advancement of navigation systems, radiofrequency catheter ablation (RFCA) has become an adjunctive or alternative strategy for NICM patients who experience drug-refractory ventricular tachycardias (VTs). Successful ablation can frequently be achieved at the expense of an epicardial intervention. A recent study has proven the survival benefits for NICM patients who are free from recurrent VTs after a successful RFCA, regardless of the New York Heart Association (NYHA) functional class status or left ventricular ejection fraction. Additionally, recent evidence has highlighted the better delineation of a diseased myocardium through the incorporation of cardiovascular magnetic resonance imaging (CMRI) and 3-D mapping systems, which can facilitate the identification of critical ventricular arrhythmogenic substrates in NICM patients.

Intravenous immunoglobulins in children with new onset dilated cardiomyopathy.

Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy. Methods and results In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy - for example, genetic, auto-immune or structural defects - had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis "probable or definite viral myocarditis". Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank=0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0-4.6; p=0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle. In our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulins.

Imaging of Left Ventricular Hypertrophy: a Practical Utility for Differential Diagnosis and Assessment of Disease Severity.

Left ventricular hypertrophy (LVH) is often encountered in clinical practice, and it is a risk factor for cardiac mortality and morbidity. Determination of the etiology and disease severity is important for the management of patients with LVH. The aim of this review is to show the remarkable progress in cardiac imaging and its importance in clinical practice. This review focuses on clinical features and characteristic cardiac imaging in LVH caused by various diseases including hypertension, aortic valve stenosis, hypertrophic cardiomyopathy, and secondary cardiomyopathies. The usefulness of echocardiography as a tool of general versatility including hemodynamic evaluation and the usefulness of cardiac magnetic resonance imaging for assessment of cardiac morphology and myocardial tissue characteristics of relevance for LVH are described. Imaging modalities now have central roles in the differentiation and prognostic assessment of LVH.

Invited Commentary on "Radiologic-Pathologic Correlation of Primary and Secondary Cardiomyopathies".

Invited Commentary on "Radiologic-Pathologic Correlation of Primary and Secondary Cardiomyopathies".

Radiologic-Pathologic Correlation of Primary and Secondary Cardiomyopathies: MR Imaging and Histopathologic Findings in Hearts from Autopsy and Transplantation.

Cardiac magnetic resonance (MR) imaging with late gadolinium enhancement (LGE) is used to detect and assess the myocardial damage seen with a variety of cardiomyopathies. Gadolinium-based contrast material accumulates in the expanded interstitial space of the myocardium. Areas with LGE correspond to replacement fibrosis, fibrofatty change, epithelioid granuloma, inflammatory cell infiltration, cardiomyocyte necrosis, and amyloid deposition-conditions that represent a focal increase in interstitial space. Areas without LGE correspond to interstitial or plexiform fibrosis, mildly degenerated cardiomyocytes, inflammatory cell infiltration, and diffuse amyloid deposition-conditions that represent diffuse increases in interstitial space. LGE MR imaging cannot depict these diffuse changes and does not enable quantitative evaluation of this increased interstitial space because on inversion-recovery MR images, the inversion time is adjusted to null the signal from normal-appearing or the least enhancing regions of the myocardium. Thus, the absence of LGE does not always indicate normal myocardial tissue. The use of current T1 mapping techniques enables one to overcome these drawbacks of LGE imaging, detect diffuse myocardial abnormalities, and perform quantitative analysis of the interstitial space. The authors describe the histopathologic and corresponding cardiac MR imaging findings of hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, cardiac sarcoidosis, giant cell myocarditis, and cardiac amyloidosis-mainly those seen on LGE MR images-as assessed by using whole-heart specimens obtained from autopsy or transplantation. ©RSNA, 2017.

Significance and Value of Endomyocardial Biopsy Based on Our Own Experience.

Endomyocardial biopsy (EMB) has been established in parallel with the development of percutaneous catheter technology for the diagnosis of cardiac diseases. It was developed in the early 1960 s in Japan by Drs. Konno, Sakakibara and Sekiguchi of Tokyo Women's Medical University. EMB is a valuable and useful, but invasive, modality for making a definite diagnosis in diseases such as myocarditis and secondary cardiomyopathies, which are often difficult to diagnose by imaging modality alone. In the field of heart transplantation, the histology of EMB helps monitor rejection to allografts. In cases of chronic heart failure, fibrosis and degeneration of cardiomyocytes are very important findings of heart remodeling. Recently, molecular biology technology has been applied to EMB specimens to get more detailed information. However, we must also recognize that EMB is an invasive examination that should not be performed without skillful cardiac catheterization experience to avoid complications. In this review as a message from pathologists, we present key cardiac histopathology using EMB, in a way that allows one to imagine whole cardiac pathological conditions. We also describe the current role of EMB and its significance in order to encourage young cardiologists to perform EMB to see another world of pathology.

Cardiac failure secondary to hypocalcemia

Hypocalcemic cardiomyopathy due to hypoparathyroidism is a very atypical and rare circumstance, which is usually intractable to conventional therapy for cardiac failure, but responds satisfactorily to restoration of normocalcemia. We describe a young woman who developed clinical signs of hypocalcemia due to hypoparathyroidism, reduced left ventricular ejection fraction and polymorphic ventricular tachycardia as consequences of hypocalcemia. This case underscores the importance of biochemical abnormalities like hypocalcemia as a rare cause of secondary cardiomyopathy and emphasizes on the need for effective and immediate treatment of hypocalcemia and its related causes.

Застосування левокарнітину для лікування вторинної кардіоміопатії у дітей раннього віку

Застосування левокарнітину для лікування вторинної кардіоміопатії у дітей раннього віку

Is coronary angiography needed in all cases of secondary Takotsubo cardiomyopathy?

Is coronary angiography needed in all cases of secondary Takotsubo cardiomyopathy?

Abstract 14688: Paradox of Right Ventricular Function: Lower the Better? -- Right Ventricular Dysfunction May Predict the Diagnosis of Tachycardia Induced Cardiomyopathy

Background: Differentiating tachycardia induced cardiomyopathy (TIC) from other dilated cardiomyopathies (DCM) remains challenging in patients presenting with LV dysfunction and AF tachycardia. While sustained rapid pacing in animals produces biventricular dysfunction, left-sided heart failure patients develop right ventricular (RV) dysfunction in advanced stages following increase in pulmonary vascular resistance. We hypothesized that RV dysfunction at presentation may predict the diagnosis of TIC. Methods: We studied the RV function using CMR in 102 consecutive patients with LVEF&lt;45% and uncontrolled AF tachycardia (defined as heart rate over 100 at rest or over 160 on exercise). Patients with coronary, valvular, congenital, or other secondary cardiomyopathies were not included. CMR was performed after heart rate stabilization, and ventricular functions assessed by Argus (Siemens, Germany), endomyocardial biopsies and hemodynamic data from Swan-Ganz catheter were analyzed. Patients who had normal EF after 1 year treatment were classified as TIC group (n=45), and patients who did not as DCM group (n=57). Results: LVEF at presentation by echocardiogram were similar between the 2 groups (29.2±9.7 vs.28.1±8.0%, p=0.79). At CMR, TIC group had higher LVEF (33.5±9.8 vs. 24.8±7.6%, p&lt;0.001) but tended to have lower RVEF (33.0±7.4 vs. 35.0±6.7%, p=0.11). No significant differences were observed between TIC and DCM in myocardial fibrosis, hypertrophy or hemodynamic parameters including pulmonary vascular resistance. RVEF/LVEF ratio was significantly lower in TIC (1.03±0.25 vs. 1.51±0.45, p&lt;0.001), and by ROC analysis, RVEF/LVEF ratio&lt;1.1 predicted TIC with a sensitivity of 76% and specificity of 87% (AUC=0.85). Logistic regression analysis showed RVEF/LVEF ratio is a strong independent predictor of TIC (OR=0.62 per 0.1, p&lt;0.001). Conclusions: RV dysfunction assessed by RVEF/LVEF ratio was highly predictive of TIC. Ventricular impairment in TIC occurs equally in both ventricles, and RV dysfunction in TIC develops without elevation of pulmonary vascular resistance. While RV dysfunction generally indicates advanced heart failure with worse prognosis, it may also indicate TIC, which is generally considered to have good prognosis.

Abstract 14706: Cardiac Phenotype in Children Undergoing Screening for Familial Hypertrophic Cardiomyopathy: Is it Time to Change Screening Guidelines?

Introduction: Current hypertrophic cardiomyopathy (HCM) screening guidelines recommend that clinical and/or genetic screening of children with family history of HCM be initiated from age 10 years (European Society of Cardiology 2014) or 12 years (American Heart Association 2011) onward and earlier only in the presence of malignant family history, presence of symptoms and/or participation in competitive sports. We assessed the proportion of asymptomatic children undergoing HCM screening that developed HCM phenotype or major adverse cardiac events (MACE) prior to age 12 years. Methods: This was a single centre retrospective study of HCM kindreds referred for screening to our cardiomyopathy clinic. Index cases with HCM, and cases with secondary cardiomyopathy (metabolic, neuromuscular, syndromic) were excluded. Genotype and phenotype status at initial screening and last follow-up were analyzed. MACE was defined as occurrence of death, transplant, resuscitated cardiac arrest, implantation of ICD or myectomy. Results: From 2005-2014, 427 children were referred for cardiac screening secondary to a family history of HCM. Of these, 379 (88.8%) had first-degree relatives with HCM and 146 (34.1%) had a family history of sudden cardiac death. The median age at first screening was 7.4 years (range 1 day-17 years), and median follow-up duration was 3 years. 45% (13/29 tested) phenotype-positive children, and 53% (32/60 tested) phenotype-negative children has positive genetic testing. 27/427 (6.3%) were phenotype-positive (maximal LV wall thickness Z-score &gt;2.0) at initial screening and an additional 13 (3%) became phenotype-positive during follow-up. The median age at positive phenotypic diagnosis of HCM was 8 years (range 5 days-17 years) with 24 children being &lt;12 years old (5.6%). 11/427 (2.6%) suffered a MACE during follow-up. Median age at MACE occurrence was 10.9 years (range 6-15 years) with 6 children being &lt;12 years old at occurrence of MACE (1.4%). Conclusion: Overall, more than 5% children being screened for HCM were phenotype-positive and 1.4% suffered a MACE before 12 years of age. These data suggest the importance of revising existing guidelines to recommend clinical and/or genetic screening of HCM of all family members independent of age.