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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report describes heart disease in a 32-year-old man with the syndrome of chronic progressive external ophthalmoplegia (CPEO). The surface electrocardiogram showed first degree AV block and left bundle-branch block and there was HV prolongation on the His bundle electrogram. Endomyocardial biopsy showed the changes of hypertrophy on light microscopy, and on electron microscopy there were increased numbers of mitochondria which appeared structurally normal. A permanent demand pacemaker was inserted because these patients are prone to develop complete heart block.
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PMID:Cardiac abnormalities in chronic progressive external ophthalmoplegia. 13 70

Intraventricular conduction defects are common following repair of various forms of congenital heart disease. Such defects may affect adversely the long-term prognosis of patients in whom cardiac hemodynamics were adequately restored. Review of previously published studies suggests that the site of the conduction defect may be the reason for the different prognoses reported for patients from different institutions. The so-called "trifascicular block" pattern which sometimes occurs following open heart surgery is probably due to a more extensive lesion to the branching and penetrating parts of the His bundle rather than additional injury to the posterior left bundle branch fibers. Transient complete heart block in the immediate postoperative period seems to be a predictor for late development of complete heart block or sudden death at least as powerful as right bundle branch block and left anterior hemiblock.
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PMID:Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects. 34 55

Ten years ago a clinical method of recording the electrical activity of the His bundle in man with transvenously inserted electrodes was described. His bundle recording has permitted the breakdown of the P-R interval into three conduction intervals, i.e., intraatrial (P-A), A-V nodal (A-H), and His-Purkinje system (H-V). His bundle studies have demonstrated our inability to accurately predict from the surface electrocardiogram the exact location of most A-V blocks. First- and second-degree A-V block can occur in the atrium, A-V node or His-Purkinje system, and third-degree A-V block in the A-V node or His-Purkinje system. However, Mobitz type II block almost always occurs below the A-V node. Intraventricular conduction defects, especially of the so-called bifascicular block, have a high incidence of H-V time prolongation, indicating additional disease of the third fascicle or the main His bundle. The prognostic value of a prolonged H-V time in patients with and without chronic conduction defects remains controversial, with some agreement that patients with unexplained syncope or dizziness, normal sinus rhythm and 1:1 conduction, who show prolonged H-V times, should probably be paced permanently. No long-term studies exist regarding the value of the H-V time in predicting death or A-V block in patients with conduction defects secondary to acute myocardial infarction, congenital heart disease or after cardiac surgery. Electrophysiological studies have been extremely useful in the diagnosis and management of patients with accessory pathways and in the evaluation of ventricular and supraventricular arrhythmias. The most valuable test in diagnosing sinus node dysfunction is the sinus node recovery time. A clearly abnormal test in a patient with unexplained syncope or dizziness predicts an almost one hundred per cent relief of symptoms with permanent pacing.
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PMID:Diagnostic and prognostic value of intracardiac electrophysiological studies. Ten years of experience. 38 29

The topography and severity of lesions of the right branch of the bundle of His have been studied as a function of the electrocardiographie changes and the associated heart disorder in 33 cases with more than 50% of the fibres destroyed. It appears that lesions of the right branch of the bundle of His, while severe and diffuse in cases of chronic complete right branch block, were severe but localised in 5 of the 6 cases with a stable incomplete right block. Total, subtotal or partial destruction of the right branch of the bundle of His was associated with lesions of the A-V node and/or the main truck of the bundle of His in the five cases with a complete atrio-ventricular block. The lesions of the right bundle branch involved the superior, middle and inferior portions in the case of aortic valve lesions, the middle portion in mitral valve disease, and the inferior portion in those with myocardial infarction. Ventricular hypertrophy seems to play an important in deciding whether the axis of the QRS, complex is left or right.
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PMID:[Lesions of the right branch of the bundle of His. Clinical, electrocardiographic and histologic study of 33 cases]. 40 86

Prolonged atrioventricular (A-V) conduction is often an apparently normal finding in children with or without congenital heart disease. The mechanism is probably vagally mediated and appears to be benign in the majority of cases. Eleven children with first-degree and/or second-degree Mobitz type 1 A-V block were studied by intracardiac His bundle electrocardiography with atrial pacing. Six children had congenital heart disease with left-to-right shunts and 5 had normal cardiac anatomy. In all the patients studied, the A-H intervals were longer than normal. Wenckebach periodicity appeared at relatively long cycle lengths, and in 5 of the 6 patients in whom values could be determined, atrioventriculonodal effective and functional refractory periods were prolonged. With atropine and isoproterenol conduction intervals returned to normal. There was no tendency to develop echo beats with atrial extrastimuli, even when A-H intervals became markedly prolonged. The results of this study suggest that vagal tone plays a significant role in the prolongation of A-V conduction, even in congenital heart lesions that are known to be associated with a long PR interval.
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PMID:Prolonged atrioventricular conduction in young children and adolescents. The role of increased vagal tone. 46 89

This case report describes a young man with a 47, XYY karyotype who was convicted of arson. He suffered from a cardiac disorder which may well have been part of the XYY syndrome rather than a chance association. His abnormal karyotype was disclosed in court and used by the defence in a plea in mitigation.
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PMID:An XYY man. 49 32

Electrophysiologic investigation was performed on 61 children and young adults during evaluation of either cardiac dysrhythmia or pre- or postoperative congenital heart disease. The results of these studies were reviewed retrospectively to determine if longitudinal dissociation of the atrioventricular node (AVN) was present. Dual AVN pathways were detected by the atrial extrastimulus technique, using His bundle electrograms. A discontinuous H1H2 response curve indicated the presence of dual AVN pathways. There was a higher incidence of dual AVN pathways in patients with clinically evident paroxysmal supraventricular tachycardia (PSVT) than in those without PSVT. Dual AVN pathways were equally prevalent in children with corrected and uncorrected congenital cardiac defects.
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PMID:Dual atrioventricular nodal pathways: a common electrophysiologic response in children. 49 64

The effects of nitroprusside on His bundle electrograms, when given intravenously for 10 minutes, were studied in 14 patients with heart disease. Recordings were made at varied heart rates using atrial and ventricular pacing. Nitroprusside significantly reduced the AH interval, but it had no effect on the HV interval. Functional and effective refractory periods were measured by the extrastimulus technique. The functional refractory period of the AV node as well as the effective refractory period of the ventriculoatrial conduction system significantly decreased after nitroprusside infusion. This improvement in conduction can probably be explained by the systemic hypotension produced by nitroprusside, which reflexly will increase sympathetic drive to the heart and decrease vagal tone.
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PMID:Electrophysiologic properties of nitroprusside in man. 53 14

Cardiac syncopes appeared in four heavily trained male patients without a history of cerebral or heart disease. Three were young athletes participating in competitive sport, one had trained intensively for years after poliomyelitis complicated by paraplegia. On admission all patients had sinus bradycardia; one had second degree atrioventricular (AV) block at rest, and one had transient sinoatrial (SA) block. His bundle studies demonstrated prolonged recovery time of the SA node (SAN) in two, prolonged atrio-His interval in three, and appearance of second degree AV block at abnormally low pacing rates in two. Refractory periods of the AV node (AVN), determined in three, tended to reach the upper limit of the normal range. The dysfunction of SAN and AVN was temporarily abolished in all patients by 1 mg of atropine i.v., and disappeared during exercise test, which was done by the three young athletes. The patient with paraplegia and one of the young athletes, who had second degree AV block at rest, were given atropine, 0.5 mg six times a day, and all three active sportsmen reduced training activity considerably. After 6--12 months all patients were re-examined. None had cerebral symptoms or other complaints. They were in regular sinus rhythm and in excellent physical condition.
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PMID:Cerebral attacks due to excessive vagal tone in heavily trained persons. A clinical and electrophysiologic study. 71 61

A case of primary myxedema heart disease in an 84-year-old man is presented. His history and physical examination were typical of myxedema. Electrocardiographic changes showing generalized low voltage, nonspecific S-T segment and T-wave changes, and nodal rhythm are characteristic of the disease. The patient showed remarkable improvement after oral liothyronine (Cytomel) therapy.
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PMID:Primary myxedema heart disease. 72 31


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