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Histologic abnormalities of the left ventricle in a patient with arrhythmogenic right ventricular dysplasia.

Right ventricular histologic abnormalities have been described in association with arrhythmogenic right ventricular dysplasia, but the cause of the disease is unknown. Identical abnormalities were noted in endomyocardial biopsy specimens from the right and left ventricles of a 20-year-old male patient with the clinical syndrome of arrhythmogenic right ventricular dysplasia. Left ventricular function was normal at rest and during bicycle exercise. Eighteen months previously, he had shown clinical and serologic evidence of mycoplasmal myocarditis. Arrhythmogenic right ventricular dysplasia may represent predominant involvement of the right ventricle in a generalized cardiomyopathic process, possibly as a consequence of healed myocarditis.

Adult↗

Current status of myocarditis and endomyocardial biopsy in Brazil.

The results presented here show the inadequate attention that has been paid to myocarditis as a clinical entity and to employing endomyocardial biopsy examination as a valuable tool for diagnostic and research purposes. The results must be interpreted with caution, as otherwise false conclusions may be drawn as far as the epidemiological profile of cardiovascular diseases in Brazil is concerned. The data are likely to be influenced by the striking regional differences in development which are reflected in the scientific output. The high incidence of positive serology in patients in endemic areas of Trypanosoma cruzi infection is a further complication. It is possible that Chagas' disease may be overestimated clinically, decreasing therefore the interest in clarifying diseases with comparable clinical features. Myocarditis (excluding a protozoal etiology) and dilated (congestive) cardiomyopathy may serve as examples. Considering these various points, together with the complex and multi-disciplinary requirements of obtaining and interpreting catheter biopsies of the heart explains the limited clinical application of this technique in Brazil.

Biopsy↗

Fatal hypertrophic cardiomyopathy in an infant of a diabetic mother.

The case is reported of a male infant with hypertrophic cardiomyopathy (HCM), attributed to maternal diabetes, who, contrary to the usually good prognosis of this condition, died at 11 weeks of age. The clinical, echocardiographic, and pathological findings were indistinguishable from familial HCM, except for the lesser degree of myocardial fiber disarray on microscopic examination.

Adult↗

Endomyocardial biopsy in infants and children: experience in 60 patients.

In 60 children, aged between 1 month and 22 years (median 3.54 years) and with a body weight of 3-67 kg (median 12.6 kg), transvascular endomyocardial biopsy (EMB) was performed from the right (35 children) or left ventricle (30 children). The specimens were investigated by light and electron microscopy. There were three indications for biopsy: (1) poorly functioning, dilated left ventricle (seven patients with endocardial fibroelastosis, 16 with dilated cardiomyopathy, six from healing/healed or chronic myocarditis); (2) unexplained left ventricular hypertrophy (10 with hypertrophic cardiomyopathy, four with secondary hypertrophy, three with storage diseases); (3) to answer certain questions in eight children (four with hypoxic and two with cytoxic myocardial damage). Retrospectively, there were five nonindicated biopsies. There were no serious complications. Biopsies were diagnostic in 11.7% of cases, helpful in 71.7%, and of no help in 16.6%. Thus even in childhood endomyocardial biopsy is a diagnostic tool which can add useful information on the etiology or pathogenesis of an underlying myocardial disease.

Adolescent↗

A neuroendocrine-immune interface. The immunostimulatory state of aldosteronism.

Hypertension is an important determinant of heart failure. Ventricular systolic and/or diastolic dysfunction can eventuate in an activation of the renin-angiotensin-aldosterone system (RAAS). Circulating RAAS effector hormones lead to: the appearance of the congestive heart failure syndrome; and a systemic illness that features oxi/nitrosative stress in various tissues, including blood, together with circulating pro-inflammatory cytokines, anorexia and, ultimately, cachexia. In addition to its well-known endocrine properties, expressed in epithelial cells of classic target tissues, aldosterone (ALDO) has an emerging portfolio of actions in nonclassic target tissues, such as circulating lymphocytes and monocytes (or peripheral blood mononuclear cells [PBMC]). This neuroendocrine-immune interface is based on Na(+)-dependent, ALDO-induced iterations in PBMC cytosolic free [Mg(2+)](i) and [Ca(2+)](i). Ca(2+) loading contributes to an induction of oxi/nitrosative stress and activation of PBMC transcriptome. This immunostimulatory state begets a pro-inflammatory/fibrogenic vascular phenotype involving the heart and systemic organs and can be prevented by either ALDO receptor antagonism or antioxidant. The established efficacy of ALDO receptor antagonism as an integral component of the overall management of symptomatic heart failure may include its immunomodulatory properties. This brief review traces studies that led to and then identified the neuroendocrine-immune interface that accompanies aldosteronism.

Aldosterone↗

[Friedreich disease. A neurocardiologic syndrome with uncertain nosologic classification of heart involvement].

Friedreich's ataxia (FA) represents a degenerative, genetically determined disease of the nervous system in combination with myocardial affection and in some cases endocrinological disturbances. Manifestation of myocardial involvement usually follows symptoms of nervous system degeneration later in the course, but seems not to be secondary. These cardiac disturbances are the main cause of death in FA-patients. Therapeutic management of heart disease is possible and interdisciplinary neurologic-cardiologic cooperation should start early in the course of FA.

Adult↗

Dilated cardiomyopathy masquerading as long QT syndrome.

Atrioventricular block has been described in association with cases of long QT syndrome and mortality is increased in this subgroup. We describe an infant with congenital QT prolongation and atrioventricular block with normal cardiac function, leading to the initial diagnosis of long QT syndrome. She subsequently developed dilated cardiomyopathy requiring cardiac transplantation. We postulate that the presenting electrocardiograph abnormalities were early manifestations of the myocardial disease, preceding the development of myocardial dysfunction by several months. The need for heightened surveillance in cases of QT prolongation with atrioventricular block is amplified by the possibility of an evolving cardiomyopathy.

Adult↗

[Radiation-induced cardiac disease].

Radiation-induced effects may damage various cardiac structures chronically and cause heart valve dysfunction as well as occlusive lesions of coronary and other arteries exposed to radiation. A 72-year-old woman with a history of radiation treatment after breast cancer was admitted 25 years later with symptoms of tachycardia and acute dyspnea. We found valvular thickening, medium to severe valvular dysfunction and high grade occlusive coronary artery disease in proximal portions. The left subclavian artery also was affected. Surgical treatment was required immediately. Long-term follow-up cardiac evaluation even in asymptomatic patients is mandatory to uncover cardiac injuries by radiation. To lower the risk and maximize the benefit, early intervention by valvular replacement and myocardial revascularization is indicated. Restrictive myopathy and chronic pericarditis increase risk and have to be clarified. Diagnosis in these radiation exposed patients can be made by typical findings. Echocardiography is of eminent relevancy.

Aged↗