A rare cause of dilated cardiomyopathy: hypocalcemia.

Dilated cardiomyopathy (DCM) is characterized by systolic dysfunction and is usually idiopathic. A rare cause of reversible DCM is hypocalcemia. Calcium plays a key role in myocardial contraction. Hypocalcemia can lead to a decrease in contraction, left ventricular systolic dysfunction, and heart failure with reduced ejection fraction (EF). Hypocalcemia-related reversible DCM reports are rare. Herein, we present two cases with heart failure caused by hypocalcemia developed due to hypoparathyroidism. The first case presented with severe heart failure and an extremely low serum calcium level (4.4 mg/dL) due to idiopathic hypoparathyroidism. The second case, which was also admitted with heart failure due to hypocalcemia, had iatrogenic hypoparathyroidism due to a subtotal thyroidectomy. In both cases, patients had reduced left ventricular systolic functions (EF was 33% and 42%, respectively). After calcium replacement and heart failure treatment, calcium levels were normalized. A significant and rapid improvement in heart failure was achieved in both cases (EF 60% and 50%, respectively). Serum calcium levels should always be measured in patients with heart failure, and the etiology of hypocalcemia should be sought. In addition to the standard pharmacotherapy of heart failure with reduced EF, calcium supplementation is essential for treating these patients.

Duchenne muscular dystrophy: an overview to the cardiologist.

INTRODUCTION: Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy in children, affecting approximately one in 3,500-5,000 liveborn boys. The main signs and symptoms include gait disturbances beginning in early childhood, with later onset of respiratory and cardiac function disorders, both directly affecting the prognosis. AREAS COVERED: The recent improvement of mechanical ventilation increased the mean DMD survival age; however, there has been little progress in the treatment and prevention of cardiac complications, which currently predominantly impact survival. Cardiological evaluation with imaging methods, such as echocardiography and magnetic resonance imaging, can improve the understanding and detect changes in cardiac function early. EXPERT OPINION: Close monitoring by the cardiologists and early treatment, with adequate heart disease stratification, may be the key to prolong the lives of these patients until more promising therapies are available and can predict DMD prognosis and progression more accurately. The objective of this brief review is to update the cardiologists by highlighting the most relevant aspects of treatment and follow-up, in a practical and concise way.

Review of canine dilated cardiomyopathy in the wake of diet-associated concerns.

Dilated cardiomyopathy (DCM) has been in the literature and news because of the recent opinion-based journal articles and public releases by regulatory agencies. DCM is commonly associated with a genetic predisposition in certain dog breeds and can also occur secondary to other diseases and nutritional deficiencies. Recent communications in veterinary journals have discussed a potential relationship between grain-free and/or novel protein diets to DCM, citing a subjective increase in DCM in dog breeds that are not known to have a genetic predisposition for the disease. This literature review describes clinical presentations of DCM, common sequelae, treatment and preventative measures, histopathologic features, and a discussion of the varied etiological origins of the disease. In addition, current literature limitations are addressed, in order to ascertain multiple variables leading to the development of DCM. Future studies are needed to evaluate one variable at a time and to minimize confounding variables and speculation. Furthermore, to prevent sampling bias with the current FDA reports, the veterinary community should be asked to provide information for all cases of DCM in dogs. This should include cases during the same time period, regardless of the practitioner's proposed etiology, due to no definitive association between diets with specific characteristics, such as, but not limited to, grain-free diets and those containing legumes, novel protein diets, and those produced by small manufacturers to DCM in dogs. In summary, in order to determine if certain ingredients, categories of diets, or manufacturing processes are related to an increased risk of DCM, further studies investigating these variables are necessary.

Taquicardia por reentrada ramo ramo / Bundle-branch reentrant tachycardia

A taquicardia por reentrada ramo a ramo, apesar de ter sido descrita há cerca de 30 anos, é entidade por vezes desconhecida de muitos cardiologistas e, por vezes, não identificada por eletrofisiologistas. Provavelmente subdiagnosticada, em especial nos portadores de cardiomiopatia dilatada, essa entidade acomete pacientes com lesão do sistema elétrico infra-hissiano. O reconhecimento dessa arritmia é importante para poder oferecer tratamento curativo, diminuir a necessidade de antiarrítmicos e minimizar terapias apropriadas após um implante de cardiodesfibrilador implantável

Although the bundle-branch reentrant tachycardia was described about 30 years ago, it is unknown to many cardiologists, and at times it is not identified by electrophysiologists. Probably underdiagnosed, especially in patients with dilated cardiomyopathy, this entity affects patients with lesion of the infra-His system. Identifying this arrhythmia is important to enable curative treatment, decrease the need for antiarrhythmics and minimize appropriate therapies after the implant of an implantable cardiac defibrillator

Prognóstico da Cardiomiopatia dilatada com insuficiência cardíaca grave de acordo com escalas de classificação funcional na infância / Prognosis of dilated cardiomyopathy with severe heart failure according to functional classification scales in childhood

Fundamento: A insuficiência cardíaca é principal manifestação da cardiomiopatia dilatada na infância, e a avaliação sistematizada dos sinais e sintomas permite acompanhar o resultado do tratamento. Objetivo: Avaliar o uso de três escalas de classificação funcional da insuficiência cardíaca na evolução de crianças e adolescentes com cardiomiopatia dilatada. Métodos: Estudo longitudinal e observacional incluindo pacientes de zero a 18 anos com cardiomiopatia dilatada e insuficiência cardíaca inicial grave. Todos foram acompanhados por meio das escalas New York Heart Association (NYHA), The New York University Pediatric Heart Failure Index (The NYU PHFI) e Ross versão 2012. As análises estatísticas foram feitas com uso do Statistical Package for Social Science , versão 14.0, com teste de Mann-Whitney, teste qui quadrado ou de Fisher, aplicação da Curva Característica de Operação, teste de Wilcoxon e coeficiente de Kappa, para comparação das escalas, e curva Kaplan-Meier, para avaliação da sobrevida. O nível de significância adotado foi de 5%. Resultados: Foram incluídos 57 pacientes, com idade de 1 a 200 meses (média de 48,7 ± 55,9) e seguimento de 6 a 209 meses (média de 63,6 ± 48,4). Houve substancial concordância das escalas Ross 2012 e The NYU PHFI com a NYHA (Kappa = 0,71 e 0,82, respectivamente). A análise pareada pelo teste de Wilcoxon, comparando as escalas antes e após o tratamento, foi significativa (p < 0,0001). A sobrevida maior foi encontrada nos pacientes com classes I/II pela NYHA ou pontuação menor que 11 pontos nas outras. Conclusão: O uso das escalas de avaliação funcional da insuficiência cardíaca se mostrou útil no seguimento e na avaliação da resposta terapêutica. Pacientes que permaneceram em classes funcionais III ou IV de NYHA, ou com pontuação igual ou maior que 11 na Ross 2012 ou na The NYU PHFI tiveram pior prognóstico

DOI: 10.5935/2359-4802.20170085 12 International Journal of Cardiovascular Sciences. 2018;31(1)12-21 ARTIGO ORIGINAL Correspondência: Fátima Derlene da Rocha Araújo Rua Indiana, 789, Ap 301. CEP: 30460350, Jardim América, Belo Horizonte, MG ­ Brasil. E-mail: fatima.derlene@hotmail.com, cleverpsi@hotmail.com Prognóstico da Cardiomiopatia Dilatada com Insuficiência Cardíaca Grave de Acordo com Escalas de Classificação Funcional na Infância Prognosis of Dilated Cardiomyopathy with Severe Heart Failure according to Functional Classification Scales in Childhood Fátima Derlene da Rocha Araújo, Rose Mary Ferreira Lisboa da Silva, Henrique de Assis Fonseca Tonelli, Adriana Furletti M Guimarães, Sandra Regina Tolentino Castilho, Zilda Maria Alves Meira Hospital das Clínicas da Universidade Federal de Minas Gerais, Belo Horizonte, MG ­ Brasil Artigo recebido em 14/02/2017; revisado em 17/04/2017; aceito em 05/06/2017 Resumo Fundamento: A insuficiência cardíaca é principal manifestação da cardiomiopatia dilatada na infância, e a avaliação sistematizada dos sinais e sintomas permite acompanhar o resultado do tratamento. Objetivo: Avaliar o uso de três escalas de classificação funcional da insuficiência cardíaca na evolução de crianças e adolescentes com cardiomiopatia dilatada. Métodos: Estudo longitudinal e observacional incluindo pacientes de zero a 18 anos com cardiomiopatia dilatada e insuficiência cardíaca inicial grave. Todos foram acompanhados por meio das escalas New York Heart Association (NYHA), The New York University Pediatric Heart Failure Index (The NYU PHFI) e Ross versão 2012. As análises estatísticas foram feitas com uso do Statistical Package for Social Science , versão 14.0, com teste de Mann-Whitney, teste qui quadrado ou de Fisher, aplicação da Curva Característica de Operação, teste de Wilcoxon e coeficiente de Kappa, para comparação das escalas, e curva Kaplan-Meier, para avaliação da sobrevida. O nível de significância adotado foi de 5%. Resultados: Foram incluídos 57 pacientes, com idade de 1 a 200 meses (média de 48,7 ± 55,9) e seguimento de 6 a 209 meses (média de 63,6 ± 48,4). Houve substancial concordância das escalas Ross 2012 e The NYU PHFI com a NYHA (Kappa = 0,71 e 0,82, respectivamente). A análise pareada pelo teste de Wilcoxon, comparando as escalas antes e após o tratamento, foi significativa (p < 0,0001). A sobrevida maior foi encontrada nos pacientes com classes I/II pela NYHA ou pontuação menor que 11 pontos nas outras. Conclusão: O uso das escalas de avaliação funcional da insuficiência cardíaca se mostrou útil no seguimento e na avaliação da resposta terapêutica. Pacientes que permaneceram em classes funcionais III ou IV de NYHA, ou com pontuação igual ou maior que 11 na Ross 2012 ou na The NYU PHFI tiveram pior prognóstico. (Int J Cardiovasc Sci. 2018;31(1)12-21) Palavras-chave: Cardiomiopatia Dilatada, Prognóstico, Insuficiência Cardíaca, Escalas, Criança, Adolescentes. Abstract Background: Heart failure is the main manifestation of dilated cardiomyopathy in childhood, and the systematic evaluation of signs and symptoms allows monitoring the treatment outcome. Objective: To evaluate the use of three functional classification scales of heart failure in children and adolescents with dilated cardiomyopathy. Methods: Longitudinal and observational study including patients from zero to 18 years with dilated cardiomyopathy and severe initial heart failure. All of them were followed up using the New York Heart Association (NYHA), The New York University Pediatric Heart Failure Index (The NYU PHFI) and Ross version 2012 scales. Statistical analyzes were done using Statistical Package for Social Science, version 14.0, with Mann-Whitney test, Chi-Square test or Fisher's test, application of the Operating Characteristic Curve, Wilcoxon test and Kappa coefficient for comparison of scales and Kaplan-Meier curve for survival evaluation. The level of significance adopted was 5%. Results: A total of 57 patients, aged from 1 to 200 months (mean of 48.7 ± 55.9) and follow-up of 6 to 209 months (mean of 63.6 ± 48.4) were included. There was substantial agreement between the Ross 2012 scales, The NYU PHFI and NYHA (Kappa = 0.71 and 0.82, respectively). Paired analysis by the Wilcoxon test, comparing the scales before and after treatment, was significant (p < 0.0001). The greatest survival was found in patients with class I/II by NYHA or scores lower than 11 points in the others. Conclusion: The use of functional assessment scales of heart failure proved to be useful in the follow-up and evaluation of the therapeutic response and there was no difference between them. Patients who remained in functional classification III or IV NYHA or scores ≥ 11 in Ross 2012 or The NYU PHFI had worse prognosis

Miocardiopatía dilatada reversible asociada a hipotiroidismo severo / Reversible dilated cardiomyopathy in hypothyroidism, case report

Dilated cardiomyopathy is usually not reversible. In a few cases diverse etiologies can be treated and cardiomyopathy may disappear or improve significantly. The case of a young man with hypothyroidism and dilated cardiomyopathy is presented. After thyroid hormone replacement, a severe left ventricular dysfunction became reverted.

Myocardial Response to Milrinone in Single Right Ventricle Heart Disease.

OBJECTIVES: Empiric treatment with milrinone, a phosphodiesterase (PDE) 3 inhibitor, has become increasingly common in patients with single ventricle heart disease of right ventricular (RV) morphology (SRV); our objective was to characterize the myocardial response to PDE3 inhibition (PDE3i) in the pediatric population with SRV. STUDY DESIGN: Cyclic adenosine monophosphate levels, PDE activity, and phosphorylated phospholamban (PLN) were determined in explanted human ventricular myocardium from nonfailing pediatric donors (n = 10) and pediatric patients transplanted secondary to SRV. Subjects with SRV were further classified by PDE3i treatment (n = 13 with PDE3i and n = 12 without PDE3i). RESULTS: In comparison with nonfailing RV myocardium (n = 8), cyclic adenosine monophosphate levels are lower in patients with SRV treated with PDE3i (n = 12, P = .021). Chronic PDE3i does not alter total PDE or PDE3 activity in SRV myocardium. Compared with nonfailing RV myocardium, SRV myocardium (both with and without PDE3i) demonstrates equivalent phosphorylated PLN at the protein kinase A phosphorylation site. CONCLUSIONS: As evidenced by preserved phosphorylated PLN, the molecular adaptation associated with SRV differs significantly from that demonstrated in pediatric heart failure because of dilated cardiomyopathy. These alterations support a pathophysiologically distinct mechanism of heart failure in pediatric patients with SRV, which has direct implications regarding the presumed response to PDE3i treatment in this population.

Should we revisit anticoagulation guidelines during thyroid storm?

Thyroid storm is a rare but potentially catastrophic disease expression of thyrotoxicosis with well-recognized cardiovascular manifestations such as heart failure and atrial fibrillation. Even through some studies have found an increased risk of cardiac thrombus formation and subsequent thromboembolism in these patients, the use of anticoagulation to prevent thromboembolic sequelae of thyrotoxic atrial fibrillation remains unclear. We present a patient presenting with new onset dilated cardiomyopathy and resistant atrial fibrillation with thyroid storm that had a large left atrial appendage clot. Case particulars are discussed and the literature reviewed.

Reserva de fluxo coronariano e padrão de disfunção diastólica empacientes com cardiomiopatia dilatada / Coronary Flow Reserve and Diastolic Dysfunction Pattern In Patients with Dilated Cardiomyopathy

Introdução: Na cardiomiopatia dilatada (CMD) de origem não isquêmica uma reserva coronariana diminuída estáassociada a maior risco de morte e um dos possíveis mecanismos é o aumento das pressões de enchimento doventrículo esquerdo. Objetivo: Avaliar a reserva de fluxo coronariano (RFC) pela Ecocardiografia Transtorácica (ETT) e comparar com graus de função diastólica.Métodos: Foram estudados 156 pacientes com CMD não isquêmica (101 homens, média etária 53 ± 12 anos) e disfunção sistólica importante. A função diastólica foi avaliada pelo fluxo transvalvar mitral, fluxo venoso pulmonar e Doppler tecidual, e classificada em: normal (Grau 0); alteração de relaxamento (Grau I); padrão pseudonormal (Grau II); restritivo com reversão à manobra de Valsalva (grau III); e restritivo sem reversão à manobra de Valsalva (Grau IV). A RFC foi determinada pelo fluxo obtido com o Doppler pulsado na artéria descendente anterior e calculada como a relação entre a velocidade diastólica máxima durante a hiperemia (dipiridamol, 0,84 mg/Kg) e no basal.Resultados: Todos os pacientes apresentavam disfunção sistólica importante, fração de ejeção média de 25,3 ± 5,7%; 86 pacientes (55%) apresentavam função diastólica grau 0 ou I, enquanto 70 pacientes (45%) apresentavam disfunção diastólicagraus II, III e IV. A exequibilidade da medida de RFC foi de 90,4%. A RFC foi significativamente maior nos pacientes com função diastólica 0 ou I (2,2 ± 0,5) do que nos pacientes com disfunção diastólica graus II, II e IV (1,9 ± 0,5; p < 0,001). Conclusões: A RFC apresenta-se reduzida em pacientes com CMD de origem não isquêmica e graus avançados de disfunção diastólica.

Introduction: In patients with nonischemic dilated cardiomyopathy (DCM), decreased coronary flow reserve is associated with increased risk of death and one of the possible mechanisms is the increased left ventricular filling pressures. Objective: To evaluate the coronary flow reserve (CFR) by transthoracic echocardiography (TTE) and compare it with degrees of diastolicfunction in patients with DCM. Methods: We studied 156 patients with DCM (101 men, mean age 53±12 years) and severe systolic dysfunction. Diastolic function was assessed by mitral inflow, pulmonary venous flow and tissue Doppler, and classified as normal (grade 0), impaired relaxation pattern (Grade 1), pseudonormal pattern (Grade 2), reversible restrictive pattern during Valsalva maneuver (Grade 3) and irreversible restrictive pattern during Valsalva maneuver (Grade 4). The CFR was determined by pulsed Doppler in left anterior descending coronary artery and calculated as the ratio of the maximumdiastolic velocity during hyperemia (dipyridamole, 0.84 mg/kg) and baseline. Results: All patients had significant systolic dysfunction, with mean left ventricular ejection fraction of 25.3±5.7%. 86 patients (55%) had grade 0 or 1 diastolic function while 70 patients (45%) had grades II, III or IV of diastolic dysfunction. The feasibility of CFR obtained by TTE was 90.4%. The CFR was significantly higher in patients with diastolic dysfunction 0 or 1 (2.2±0.5) than in patients with diastolic dysfunction grades II, II or IV (1.9±0.5, p<0.001). Conclusion: CFR is reduced in patients with nonischemic DCM and advanced degrees of diastolic dysfunction.

Sindrome de inmunodeficiencia adquirida: compromiso del corazón y de los vasos / AIDS: cardiovascular involvement

Las complicaciones cardiovasculares del SIDA, son a menudo asintomáticas, aunque en ocasiones pueden constituir la causa directa de muerte. Puede comprometerse el pericardio, miocardio, endocardio y los vasos, ya sea como manifestación de la enfermedad de base o como resultado de la terapia antirretroviral y su efecto sobre los factores de riesgo, en el contexto de una patología que es actualmente de evolución crónica y con mejores expectativas de sobrevida gracias a las nuevas drogas empleadas para su control y tratamiento. El objetivo de este artículo es presentar una revisión de los aspectos más relevantes del SIDA que comprometen al corazón y los vasos.

The cardiovascular complications of AIDS, are often asymptomatic, although some may be direct cause of death. Pericardium, myocardium, endocardium, and vessels may be involved as a result of illness or the adverse effects of antiretroviral therapy on risk factors, Today has become a chronic condition with improved life expectancy thanks to the development of new drugs for its treatment and control The aim of this article is to present a review of the most relevant aspects of AIDS involving the heart and vessels.

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

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 subjects 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.