Isolated paradoxical embolus to the right coronary artery.
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Biomedical subjects
Publications and source records attributed to J K Perloff.
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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.
Acute severe aortic regurgitation is a relatively unfamiliar, though life-threatening, disease. We review its diverse causes, anatomic faults, and hemodynamic sequelae and set the stage for an understanding of the clinical manifestations in light of their physiologic mechanisms. Clinical information includes the natural history, physical signs (physical appearance, systemic arterial pulse, jugular venous pulse, precordial palpation, auscultation), electrocardiogram, and chest roentgenogram. Echocardiographic features are especially emphasized and the need for prompt diagnosis and surgical intervention underscored, even in the setting of active infective endocarditis.
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Dextroversion of the heart is an uncommon congenital anomaly characterized by situs solitus (normal position) of thoracic and abdominal viscera with right cardiac apex. Isolated dextroversion, i.e., without associated congenital heart disease, is rare, but its occurrence permits adult survival, setting the stage for late development of acquired heart disease. The patient herein reported was known since childhood to have a right thoracic heart that represented isolated uncomplicated dextroversion. He presented in the sixth decade with a new murmur that proved to be due to asymmetric septal hypertrophy (ASH) with obstruction. This combination of anomalies is possible only if dextroversion exists without ventricular inversion, since aortic-anterior mitral leaflet continuity is obligatory for obstructive ASH. This paper presented clinical, phonocardiographic, vectorcardiographic, echocardiographic, hemodynamic, angiocardiographic, and intracardiac electrophysiologic information on the unique combination of isolated dextroversion of the heart with obstructive asymmetric septal hypertrophy.
Though ethyl alcohol is known to be one of the exogenous toxins that can directly damage the myocardium, nonalcoholic additives to alcoholic beverages (as well as poor nutrition or viral infection) may also play a role in cardiomyopathy. The authors note the interesting fact that, unlike the liver, the heart does not contain an alcohol dehydrogenase and thus lacks a possible protective mechanism against alcohol's toxicity.
For half a century the systolic click and late systolic murmur lay dormant as innocent auscultatory curiosities. The thirteen years since Barlow related these phenomena to mitral leaflet prolapse have witnessed an astonishing information explosion. We have sought to bring together the accumulated data in this review. An Historical Perspective traces the evolution from the now abandoned "pericardial" or "extracardiac" phases, through the leafletchordal phase (redundancy), the myocardial phase (segmental left ventricular contraction abnormalities), to the anular phase (dilatation and faulty systolic contraction). Functional Anatomy is dealt with in terms of pathology, pathophysiology, hemodynamics, angiocardiography, echocardiography, and physical and pharmacological interventions. Clinical Manifestations are concerned with prevalence, natural history, symptoms, physical signs, electrocardiographic abnormalities and roentgen fingings. The four Major Complications- sudden death, infective endocarditis, spontaneous rupture of chordae tendineae, and progressive mitral regurgitation- are examined. Associated Cardiac Diseases, i.e., Marfan's syndrome, ostium secundum atrial septal defect and atherosclerotic coronary artery disease, are discussed, and a section on Treatment deals chiefly with prophylaxis for infective endocarditis and the management of arrhythmias and chest pain. A final section on Evolving Information considers etiologic concepts, the nature of left ventricular contration abnormalities, the cause of chest pain, the relationship to Marfan's syndrome and ostium secundum atrial septal defect, and the effect of aging and sex differences on leaflet chordal redundancy.
In addition to recording the motion of the mitral, tricuspid, aortic, and pulmonic valves, echocardiography can identify right and left ventricular cavities and the interventricular septum. Disorders such as atrial-septal defect, valvular and subvalvular aortic stenosis, pulmonic stenosis, Ebstein's anomaly of the tricuspid valve, and the hypoplastic left-heart syndrome can readily be evaluated by echocardiography. In tetralogy of Fallot and truncus arteriosus, discontinuity between the anterior aortic wall and septum with overriding aorta has been demonstrated. Doubleoutlet right ventricle is associated with posterior aortic wall and mitral valve discontinuity. In disorders such as single ventricle, tricuspid atresia, and endocardial cushion defect with common A-V canal, echocardiographic demonstration of the absence of the interventricular septum has provided the clinician with valuable information. Newer techniques such as compound-B ultrasonography, which produces a two-dimensional cross-sectional image of intracardiac structures, and multiscan echocardiography will enhance the use of conventional echocardiography by providing a more accurate anatomic display of cardiac chambers and outflow vessels.
Electrophysiologic characteristics of five patients with Ebstein's anomaly of the tricuspid valve were defined with studies using luminal intracardiac electrode catheters. The diagnosis was made in each case from clinical data and confirmed at cardiac catheterization by the presence of an atrialized right ventricular chamber with atrial mechanical activity and ventricular electrical activity. In three cases intra-right atrial conduction was prolonged (P-A intervals of 50, 50, and 65 msec), a finding which reflected the presence of a characteristically large right atrium. The bundle of His electrogram was recorded in its usual anatomical location. Atrioventricular nodal conduction was prolonged in only one case. Intra-His delay was observed in two cases (bundle of His duration of 30 and 30 msec). Infranodal conduction was prolonged in four cases with H-V intervals of 60, 65, 65, and 80 msec. The anatomical abnormalities were least severe in the only patient with a normal H-V interval (50 msec). The prolonged H-V interval was thought to result from stretching of the conduction system over the atrialized right ventricle (ARV). The late depolarization during the splintered R' of the electrocardiogram found during intracardiac mapping of the ARV in three patients confirms the theory that the ARV produces the "second QRS" typically seen in this anomaly. The ARV was particularly irritable, and ventricular fibrillation was produced in two patients during catheter manipulation in this area. In one case the ARV had a shorter refractory period than the body of the right ventricle. Re-entrant supraventricular tachycardia was induced in the only patient with Wolff-Parkinson-White syndrome. In addition to the previously recognized electrophysiologic features reconfirmed here, patients with Ebstein's anomaly of the tricuspid valve usually have: normal position of the bundle of His, prolonged intra- right atrial conduction, prolonged infranodal conduction, and irritable ARV with delayed activation.