Chronic post-rheumatic fever arthropathy of Jaccoud.
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Biomedical subjects
Publications and source records attributed to J K Perloff.
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Examination of the heart and circulation includes five items: 1) the patient's physical appearance, 2) the arterial pulse, 3) the jugular venous pulse and peripheral veins, 4) the movements of the heart--observation, palpation and percussion of the precordium, and 5) auscultation. This report deals with specific examples that relate cardiac and vascular physical signs to their mechanisms, focusing on each of the five sources. It draws liberally on early accounts, emphasizing that modern investigative techniques often serve chiefly to verify hypotheses posed in the past.
An edited summary of an Interdepartmental Conference arranged by the Department of Medicine of the UCLA School of Medicine, Los Angeles. Director of Conferences: William M. Pardridge, MD, Associate Professor of Medicine.In the past two decades we have witnessed the maturing of diagnostic and surgical skills in the management of congenital heart disease. Although longevity and quality of life have improved, cures are few; varying degrees of postoperative medical supervision are therefore needed. This new patient population of adults requiring long-term medical care continues to increase. Proper management of such patients can be taxing, requiring knowledge not only of the preoperative disease but also of the nature and effects of surgical intervention and of the presence, type and extent of late postoperative residua and sequelae. The tetralogy of Fallot is taken as a model because it is well known in both pediatric and adult medicine, because intracardiac repair includes a wide range of techniques and because postoperative residua and sequelae comprise a broad spectrum of patient care concerns.
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This report deals with increased cardiac mass in the light of the following variables: normal ventricular growth (embryo, fetus, neonate and child), the response to work loads (hemodynamic stress) and hypoxia, the cell responses of hyperplasia (increase in cell number), hypertrophy (increase in cell size) and the type of cell (muscle or connective tissue), the age or maturity of the myocardium at the time the hemodynamic or hypoxic stress is imposed, and the biochemistry, ultrastructure and functional morphology (modeling) of the ventricles in response to volume or pressure overload. The desirable physiologic adaptations to work loads are characterized, and the transition from physiologic to pathologic states is examined, comparing and contrasting increased ventricular mass in patients and in trained athletes. Regression of increased ventricular mass is then discussed, first at the cell level (hypertrophy/hyperplasia; muscle cell/connective tissue cell), then at the organ level. The requirements for maintaining or establishing normal ventricular function after removal of overload are reviewed, together with such variables as the type and duration of preoperative hemodynamic stress, the right versus the left ventricle and the relative rates of contractile protein synthesis and degradation.
Increased mitral valve E point to ventricular septal separation (EPSS) is widely used as an echocardiographic index of depressed left ventricular (LV) ejection fraction (EF), yet LV size has not been examined as an independent variable potentially affecting EPSS. Accordingly, we studied end-diastolic dimensions (EDD). Twenty normal controls had EPSS 3.2 +/- 2.2 mm (mean +/- SD), EDD 47 +/- 5 mm, EPSS/EDD ("normalized" EPSS) 0.07 +/- 0.04, and fractional shortening (FS%) 38 +/- 6%. Nine patients with pure chronic mitral regurgitation had dilated LV (EDD = 65 +/- 7 mm) with normal LV function (FS% 41 +/- 5%; angiographic EF 62 +/- 9%); eight patients had dilated cardiomyopathy (EDD 69 +/- 8 mm) with decreased LV function (FS% 16 +/- 7%; angiographic EF 32 +/- 8%); and eight patients with amyloid cardiomyopathy had nondilated LV (EDD 42 +/- 5 mm) with decreased LV function (FS% 19 +/- 6; angiographic EF 35 +/- 7%). Mitral E point to ventricular septal separation and EPSS/EDD accurately separated individuals with normal and abnormal LV function irrespective of LV size (chi 2 = 36.7; p less than 0.00001). Increased internal dimensions per se did not affect EPSS unless depressed LV function coexisted. EPSS is therefore a valid predictor of depressed ejection phase indices independent of LV size.
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In this report we show that coronary arterial and esophageal spasm are sometimes clinically indistinguishable. Pain patterns can be identical, nitroglycerin can bring relief, interval electrocardiograms and exercise electrocardiograms generally disclose no abnormalities, coronary arteriograms may be within normal limits or nearly so, and, importantly, an ergonovine provocative test can provoke esophageal spasm accompanied by pain mistaken for Prinzmetal's angina. Accordingly, chest pain in response to the administration of ergonovine is not evidence of coronary arterial spasm without simultaneous arteriographic proof together with changes in the monitoring electrocardiogram. Conversely, gastroenterologists should not expose patients to the risk of ergonovine without prior assurance that the coronary arterial response is normal. Our observations illustrate these points and identify a patient with symptomatic esophageal spasm previously diagnosed as Prinzmetal's angina.
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Discrete fibromembranous subaortic stenosis may be severe at birth, progress in severity irrespective of the initial degree of obstruction, and morphologically change during the course of its natural history. Initial status, progression of the lesion and perhaps later development of fibromuscular (tunnel) and/or asymmetric septal hypertrophy may all contribute to a shortened life span and account for the different incidence of fibromembranous subaortic stenosis in children and adults. In the case reported here, a patient survived into the sixth decade with a discrete fibromembranous subaortic stenosis, which led to typical as well as unusual echocardiographic findings.
This report deals with the ramifications of the concept of left axis deviation. In early life, the leftward shift of the frontal plane QRS axis is determined chiefly, if not solely, by the relative weights of the ventricles. Once adult ventricular weight ratios are reached, there is a long period of axis stability, then a gradual leftward drift of the QRS, governed principally by left anterior fascicular conduction. Thus, the normal QRS axis is age-dependent, and left axis deviation must be considered accordingly.
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Clinical and morphologic features are described in a 13 week old male infant with ventricular inversion without associated transposition of the great arteries but with concomitant atresia of the left-sided (tricuspid) atrioventricular valve. Isolated ventricular inversion without situs inversus or visceral heterotaxy has previously been reported in English in only three patients, none with tricuspid atresia.