Pathological basis of primary heart block.
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An 18-year old white youth presented with severe right heart failure and was found to have an obliterated and funnel-shaped right ventricle, massive tricuspid regurgitation, and mitral regurgitation. The haemodynamic findings were those of cardiomyopathy with obliteration on the right side. He underwent surgery consisting of decortication and peeling off of a thickened right ventricular endocardium, tricuspid valve replacement, and mitral valve repair. After surgery there was pronounced haemodynamic as well as clinical improvement. The pathological picture was that of constrictive endocarditis. We recommend this method of treatment for patients with obliterate cardiomyopathy on either side of the heart.
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A histopathological index of contractility failure, which was reported to be accurate for the diagnosis of idiopathic dilated or congestive cardiomyopathy in Japan, was used to assess endomyocardial biopsy specimens from 41 Dutch patients with suspected dilated cardiomyopathy. The calculated index had a sensitivity of 62.5% and a specificity of 68% for idiopathic dilated or congestive cardiomyopathy. These disappointing results indicate that the histopathological index of contractility failure does not reliably distinguish between idiopathic dilated cardiomyopathy and other diseases that cause congestive heart failure.
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Twenty-three cases of endomyocardial disease (ED) are presented, studied in Venezuela, a tropical country in northern South America. The diagnosis was confirmed in 18 cases by means of pathological studies, and in 5 cases by angiocardiography which showed the characteristic obliterative ventricular lesions. Eosinophilia was present in 35% of the patients. The most frequent clinical feature was heart failure associated with mitral regurgitation. Systemic embolism was the first clinical feature in 5 cases. In 2 cases, ED was associated with autoimmune haemolytic anaemia or vasculitis. Necropsy revealed a predominance of the left-sided (9/16 cases) and biventricular (6/16 cases) types. The pathological lesions were characterised by fibrous thickening of the endocardium at the apex and the ventricular inflow tracts extending to the myocardium and involving the atrioventricular valves. ED is frequently misdiagnosed as rheumatic valvular cardiopathy. The two-dimensional echocardiogram is a very useful procedure for determining the spatial anatomy of ED. The echo findings were closely correlated with ventriculographic and necropsy findings. Even though ED is widely spread around the world, it is most frequently found in tropical and subtropical countries in Africa, Asia and America, such as Venezuela and Brazil. This suggests that there are aetiological factors in these latitudes, about which little is known.
This study was designed to compare the clinical and cardiological features of endomyocardial disease in temperate and tropical regions. Eleven patients were studied in the U.K., 47 in India and 8 in Brazil. The patients in the U.K. were older, with a male predominance, and they had a systemic illness: the hypereosinophilic syndrome. Half of these patients presented in the early necrotic stage of the disease, and all had biventricular involvement. On the other hand, patients in the tropical countries were younger, with an equal sex incidence, and were from poor, malnourished communities with heavy parasite loads, especially filariasis in India. None presented in the early necrotic stage of the disease and a quarter had isolated right or left ventricular disease. In order to account for these differences between patients in temperate and tropical regions with endomyocardial disease, it was proposed that the nature of the underlying disease and the rate at which endomyocardial lesions develop, determine the clinical features of this disorder. In temperate climates eosinophil granule toxins may produce a rapidly progressive form of the disease in patients with the hypereosinophilic syndrome, whereas the disease may take longer to develop in patients in tropical climates, who have a less marked eosinophilia due to parasitic infections.
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Vascular inflammation was examined as a potential mechanism of aldosterone-mediated myocardial injury in uninephrectomized rats receiving 1% NaCl-0.3% KCl to drink for 1, 2, or 4 wk and 1) vehicle, 2) aldosterone infusion (0.75 microg/h), or 3) aldosterone infusion (0.75 microg/h) plus the selective aldosterone blocker eplerenone (100 mg. kg(-1). day(-1)). Aldosterone induced severe hypertension at 4 wk [systolic blood pressure (SBP), 210 +/- 3 mmHg vs. vehicle, 131 +/- 2 mmHg, P < 0.001], which was partially attenuated by eplerenone (SBP, 180 +/- 7 mmHg; P < 0.001 vs. aldosterone alone and vehicle). No significant increases in myocardial interstitial collagen fraction or hydroxyproline concentration were detected throughout the study. However, histopathological analysis of the heart revealed severe coronary inflammatory lesions, which were characterized by monocyte/macrophage infiltration and resulted in focal ischemic and necrotic changes. The histological evidence of coronary lesions was preceded by and associated with the elevation of cyclooxygenase-2 (up to approximately 4-fold), macrophage chemoattractant protein-1 (up to approximately 4-fold), and osteopontin (up to approximately 13-fold) mRNA expression. Eplerenone attenuated proinflammatory molecule expression in the rat heart and subsequent vascular and myocardial damage. Thus aldosterone and salt treatment in uninephrectomized rats led to severe hypertension and the development of a vascular inflammatory phenotype in the heart, which may represent one mechanism by which aldosterone contributes to myocardial disease.
Shock-induced vulnerability and defibrillation have been mostly studied in structurally normal hearts. However, defibrillation therapy is normally applied to patients with diseased hearts, frequently those with prior myocardial infarction (MI). Shock-induced vulnerability and defibrillation have not been well studied under this condition. We sought to examine the mechanisms of shock-induced arrhythmogenesis and arrhythmia maintenance in a rabbit model of healed MI (4 wk or more postinfarction). Ligation of the lateral division or posterolateral division of the left coronary artery at a level of 40-70% from the apex was performed 53 +/- 21 days before acute experiments. Shock-induced vulnerability was assessed in infarcted (n = 8) and structurally normal (n = 8) hearts by delivering internal monophasic shocks at different shock strengths and delivery phases. Electrical activities from the anterior epicardium during shock application and during shock-induced arrhythmias were optically recorded and quantitatively analyzed. Ligation resulted in a transmural left ventricular free wall infarction mainly located at the apical region with a consistent endocardial border zone (BZ) as confirmed by histological studies. There were significant increases in the incidence, severity, and duration of shock-induced arrhythmias in the infarcted hearts versus controls due to 1) postshock break-excitation wavefronts that frequently originated near the infarction BZ and 2) the existence of an infarction BZ that created an anatomic reentry pathway and facilitated arrhythmia maintenance. In conclusion, the infarction BZ contributes to both increased shock-induced arrhythmogenesis and arrhythmia maintenance in the rabbit model of healed MI.