O coração na obesidade: Mecanismos e consequências / Heart in obesity: Mechanism and consequences

A obesidade está associada a risco aumentado de doença cardiovascular, tanto pela associação a múltiplos fatores de risco que frequentemente a acompanham como pelos efeitos diretos do excesso de peso sobre a estrutura e a dinâmica cardíacas. A patogênese da disfunção miocárdica na obesidade é complexa e multifatorial. Alterações hemodinâmicas, neuro-humorais e no metabolismo dos substratos energéticos estão implicadas no desenvolvimento da miocardiopatia da obesidade. Acredita-se que as mudanças estruturais e funcionais no miócito cardíaco representem uma “má adaptação” às alterações metabólicas descritas na obesidade,levando à disfunção miocárdica progressiva e, finalmente, à insuficiência cardíaca. A perda de peso induz significativas mudanças tanto na estrutura miocárdica quanto na disfunção diastólica relacionada à obesidade. Abordagens farmacológicas que atuem sobre o remodelamento cardíaco, bloqueando a fibrogênese, tais como TGF-β1, espécies reativas de oxigênio ou endotelina-1, têm apresentado resultados promissores em estudos experimentais.

Obesity is associated with increased risk of cardiovascular disease, both by the presence of multiple well documente drisk factors that often accompany this condition, and the direct effects of excess of weight on cardiac structure and dynamics. The pathogenesis of myocardial dysfunction in obesity is complex and multifactorial. Neurohumoral, hemodynamic, and metabolism changes of energy substrates are implicated in the development of the cardiomyopathy of obesity. It is believed that the structural and functional changes in cardiac myocytes represent a “maladaptive”metabolic alteration described in obesity, leading to progressive heart failure and ultimately to myocardial dysfunction. Weight loss can induce significant changes inboth myocardial structure and diastolic dysfunction relatedto obesity. Pharmacological approaches that act on cardiac remodeling blocking fibrogenesis, such as TGF-β1, reactive oxygen species and endothelin-1, have shown promising results in experimental research.

Clinical recommendations for postoperative care after heart transplantation in children: 21 years of a single-center experience.

Heart transplantation is an option for children with complex congenital heart disease and cardiomyopathies. A patient's quality of life and long-term survival depend on successful management of the surgical complications and adverse side effects of immunosuppression. The purpose of this review was to summarize the practical management of postoperative care in this patient population and to make recommendations for the future.

Clinical recommendations for postoperative care after heart transplantation in children: 21 years of a single-center experience

Heart transplantation is an option for children with complex congenital heart disease and cardiomyopathies. A patient's quality of life and long-term survival depend on successful management of the surgical complications and adverse side effects of immunosuppression. The purpose of this review was to summarize the practical management of postoperative care in this patient population and to make recommendations for the future.

Long-term survival of a patient with isolated noncompaction of the ventricular myocardium.

Isolated noncompaction of the myocardium (INCM) is a rare, congenital, unclassified cardiomyopathy. It is caused by a disorder in endomyocardial morphogenesis in the absence of other structural disease. INCM is characterized by numerous prominent trabeculations and deep intertrabecular recesses in the myocardium. Frequently, INCM is associated with an increased incidence of heart failure, arrhythmias, and cardioembolic events with high morbidity and mortality. We describe a 28-year-old woman experiencing symptoms of heart failure since she was 4 years old. She had been intensively investigated and misdiagnosed as having dilated, restrictive, and apical hypertrophic cardiomyopathies. Cardiac magnetic resonance and echocardiography recently revealed the actual diagnosis of INCM. The patient is alive and well, taking vasodilators and warfarin. Herein, we describe the long-term follow-up of this patient and demonstrate that some patients have a favorable prognosis. In addition, the improvement in noninvasive cardiac imaging has revealed a higher prevalence of INCM, previously undetected.

[Spongy cardiomyopathy in an elderly woman. Echocardiographic description]. / Miocardiopatía espongiforme en una mujer anciana. Descripción ecocardiográfica.

Isolated left ventricular noncompaction, also known as spongy myocardium or spongy cardiomyopathy, is a recently described congenital disease caused by an arrest in the left ventricular myocardial embriogenesis that makes the ventricular wall to persist thickened with multiple trabecular formations and deep sinusoidal recesses. It is clinically characterized by heart failure, cardiac arrhythmia and systemic embolic events. Most of the affected subjects are detected during childhood or adolescence, others in the adult life but very few elderly patients have been reported in the worldwide medical literature. We here report the case of a 75-year-old woman that is one of the oldest patients ever reported, whose clinical picture and echocardiographic findings are typical of this modality of cardiomyopathy. We do comments on this case in regard to the most relevant facts that appear in the limited medical literature about this interesting disease.

Miocardiopatía espongiforme en una mujer anciana. Descripción ecocardiográfica / Spongy cardiomyopathy in an elderly woman, echocardiographic description

La miocardiopatía espongiforme, también conocida como ausencia aislada de compactación del ventrículo izquierdo, es una anomalía congénita recientemente descrita y que resulta de la interrupción en la morfogénesis embrionaria del miocardio ventricular izquierdo el cual permanece engrosado por grandes trabéculas y sinusoides y que suele expresarse clínicamente con manifestaciones de insuficiencia cardíaca, arritmias y eventos embólicos. Esta enfermedad suele detectarse en la infancia o en la adolescencia, menos frecuentemente en la vida adulta y muy excepcionalmente en la senectud. Presentamos aquí el caso de una mujer de 75 años de edad que corresponde a uno de los casos de miocardiopatía espongiforme de mayor edad entre los que se han publicado; sus manifestaciones clínicas y ecocardiográficas son típicas de esta variedad de miocaridopatía. El caso se comenta a la luz de los hechos más relevantes que existen en la escasa literatura publicada sobre esta interesante enfermedad.

Isolated left ventricular noncompaction, also known as spongy myocardium or spongy cardiomyopathy, is a recently described congenital disease caused by an arrest in the left ventricular myocardial embriogenesis that makes the ventricular wall to persist thickened with multiple trabecular formations and deep sinusoidal recesses. It is clinically characterized by heart failure, cardiac arrhythmia and systemic embolic events. Most of the affected subjects are detected during childhood or adolescence, others in the adult life but very few elderly patients have been reported in the worldwide medical literature. We here report the case of a 75-year-old woman that is one of the oldest patients ever reported, whose clinical picture and echocardiographic findings are typical of this modality of cardiomyopathy. We do comments on this case in regard to the most relevant facts that appear in the limited medical literature about this interesting disease. (Arch Cardiol Mex 2005; 75: 184-187.

Miocardiopatias / Miocardiopathy

O grupo-tarefa constituído pela Organização Mundial da Saúde/Federação e Sociedade Internacional de Cardiologia (OMS/SIFC) define miocardiopatia (ou cardiomiopatia), como doença miocárdica associada à disfunção cadíaca. Esta definição, excessivamente genérica, encerra o inconveniente de ampliar em demasia o campo de estudo, de sorte que, no presente capítulo, o termo será empregado em sentido mais restrito, englobando apenas as afecções miocárdicas associadas à disfunção cardíaca não decorrentes de aterosclerose coronária significativa, disfunção valvar ou obstáculo fixo à ejeção das câmaras ventriculares, shunts dentro ou fora do coração e de hipertensão arterial sistêmica ou pulmonar passada ou presente...

Miocardiopatias / Cardiomiopathies

O grupo-tarefa constituído pela Organização Mundial da Saúde/Federação e Sociedade Internacional de Cardiologia (OMS/SIFC) define miocardiopatia (ou cardiomiopatia), como doença miocárdica associada à disfunção cadíaca. Esta definição, excessivamente genérica, encerra o inconveniente de ampliar em demasia o campo de estudo, de sorte que, no presente capítulo, o termo será empregado em sentido mais restrito, englobando apenas as afecções miocárdicas associadas à disfunção cardíaca não decorrentes de aterosclerose coronária significativa, disfunção valvar ou obstáculo fixo à ejeção das câmaras ventriculares, shunts dentro ou fora do coração e de hipertensão arterial sistêmica ou pulmonar passada ou presente...

Focal giant-cell cardiomyopathy.

A newborn with supraventricular arrhythmia diagnosed during fetal life was found at autopsy to have a small nodule with hypertrophic and multinucleated myocardial cells in the atrial septum, near the atrioventricular node. There were no stigmata of Beckwith-Wiedemann syndrome or tuberous sclerosis. This focal giant-cell cardiomyopathy has been described only once, in an infant with Beckwith-Wiedemann syndrome. It is suggested that these dysplastic myocardial cells may be responsible for the atrial arrhythmia and that this hamartomatous lesion has the potential for malignant transformation.