The conduction system in mitral valve prolapse syndrome with sudden death.
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Biomedical subjects
Publications and source records attributed to M Lev.
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Two dimensional sector scan echocardiography was used to evaluate the morphologic characteristics of the surgically revised atria in 17 patients with d-transposition of the great arteries who had undergone the Mustard operation. Echocardiographic imaging of the atria was obtained from various planar projections. Dimensional measurements of various segments of the systemic and pulmonary venous atria were obtained in each patient. Correlative hemodynamic, angiographic, postmortem and echocardiographic data showed that seven patients (Group I) had no structural abnormalities of the atria. These 7 patients served as controls for 10 other patients with structural abnormalities of the surgically created atria. One patient (Group II) showed stenosis of the junction of the superior vena cava and systemic venous atrium compared with findings in the control group. Three patients (Group III) had significantly reduced echocardiographic dimensions of the junction of the anterior and posterior segments of the pulmonary venous atrium. Six patients (Group IV) had increased echocardiographic dimensions of all components of the pulmonary venous atrium due to tricuspid regurgitation. These data show that qualitative and quantitative two dimensional sector echocardiography can reliably detect structural abnormalities of the surgically revised atria after the Mustard operation.
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A 27-year-old man with primary oxalosis and extensive visceral involvement was maintained on long-term chronic hemodialysis. He had an episode of presyncope associated with electrocardiographic findings of an erratic atrial rhythm, atrioventricular dissociation due to an accelerated junctional rhythm and right bundle branch block. Electrophysiologic studies showed irregular atrial depolarizations probably associated with multiple atrial pacemakers, atrial inexcitability, atrioventricular dissociation and infranodal conduction delay. These findings correlated well with extensive oxalate infiltration of the sinoatrial node and its approaches, the atrial preferential pathways, the approaches to the atrioventricular node, the atrioventricular node, and the His bundle and bundle branches. This is the first reported case of cardiac electrophysiologic abnormalities due to oxalate infiltration in a patient with primary oxalosis.
Detailed pathologic anatomy of tricuspid atresia associated with type I persistent truncus arteriosus is described. These findings are correlated with clinical presentations.
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A 20-year-old man contracted Hodgkin's disease and was treated with mantle radiotherapy. Heart block developed 11 years later. Electrocardiograms revealed predominant atrioventricular (AV) block and occasional AV conduction. Intracardiac electrograms demonstrated that the site of AV block was above the level of the His bundle. A permanent transvenous pacemaker was implanted. Seven months later the patient died of complications from cryptococcal meningitis. Pathological study of the heart revealed marked arteriosclerosis with fibrosis of the epicardium, myocardium, and endocardium. Examination of the conduction system revealed extensive arteriolosclerosis of the sinoatrial node and its approaches. In addition, there was marked fibrosis of the approaches to the AV node, the AV bundle, and both bundle branches. There was no evidence of Hodgkin's disease. This case documents the rare occurrence of AV block due to tissue destruction by radiotherapy. There was a good correlation between block proximal to the His bundle recording site and fibrosis of the approaches to the AV node.
An infant with Ebstein's malformation of the tricuspid valve and severe pulmonic stenosis underwent a 39-day course of prostaglandin E1 infusion, and a histologic study of the ductus arteriosus was undertaken after autopsy. There were marked alterations in the ductal and juxtaductal structures following this prolonged infusion of prostaglandin E1. The internal elastic lamella of the ductus was disrupted in many areas. The media showed widespread areas of disruption with cavity formation. The adventitia adjacent to the junction of the ductus with the pulmonary artery was thickened and infiltrated with mononuclear cells. The nerve trunks in the adventitia were markedly infiltrated with mononuclear cells and showed cavitation as well as considerable surrounding edema. Mucopolysaccharides were increased throughout the ductus. These changes produced increased fragility of the ductal and juxtaductal structures, thus increasing the likelihood of spontaneous aneurysms and rupture, or of tearing or rupture at the aortic and pulmonary junctions at the time of surgical closure of the ductus. Unusual fragility of the ductus, pulmonary artery, and aorta has been observed during ligation of the ductus following prostaglandin E infusions lasting seven and ten days. Additionally, another patient who had received prostaglandin E infusion for six days demonstrated aneurysmal fullness to the ductus arteriosus at autopsy. The histologic findings and intraoperative experience in this study suggest that there may be a real danger of spontaneous or surgically related rupture of the ductus arteriosus after prolonged infusion of prostaglandins.
Two cases are described, one of cardiac sarcoidosis and another of primary cardiac amyloidosis, in which correlation was made between electrophysiologic and postmortem conduction system studies. In Case 1 the electrocardiogram revealed right bundle branch block with first degree and intermittent third degree atrioventricular (A-V) block and recurrent unifocal paroxysmal ventricular tachycardia. Electrophysiologic studies disclosed normal sinus rhythm with prolonged A-H (175 ms) and H-V (60 MS) intervals and extrastimulus induction of repetitive ventricular firing. Postmortem examination revealed a sarcoid aneurysm of the posterior left ventricle and granulomatous infiltration of the A-V node, His bundle and bundle branches. In case 2 the electrocardiogram revealed sinus bradycardia, a prolonged S-T interval and recurrent ventricular fibrillation. Electrophysiologic studies demonstrated a prolonged sinus nodal recovery time (6,080 ms) and H-V (85 ms) interval. Postmortem examination revealed marked amyloid infiltration of the sinoatrial node, atria, proximal bundle branches and left and right ventricular myocardium. There was thus excellent correlation between electrophysiologic and pathologic findings. These cases indicate the importance of making a clinical diagnosis of the disease that might in some cases allow specific therapeutic intervention in addition to antiarrhythmic therapy.
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The uptake of a number of amino acids and dipeptides by cells and spheroplasts of Bacteroides melaninogenicus was stimulated by the presence of glutamine; 50 mM glutamine induced maximum uptake of glycine or alanine, and glutamine stimulated the uptake of glycine over a wide concentration range (0.17 to 170 mM). Glutamine stimulated the uptake of the dipeptides glycylleucine and glycylproline at significantly faster rates compared with glycine and leucine. The amino acids whose uptake was stimulated by glutamine were incorporated into trichloroacetic acid-precipitable material, and the inclusion of chloramphenicol or puromycin did not affect this incorporation. The uptake of glutamine by cells was concentration dependent. In contrast, in the absence of chloramphenicol 79% of the glutamine taken up by cells supplied with a high external concentration (4.4 mM) was trichloroacetic acid soluble. Glutamate and alpha-ketoglutarate were identified in the intracellular pool of glutamine-incubated spheroplasts. The amino acids and peptides were incorporated into cell envelope material, and a portion (30 to 50%) of the incorporated amino acids could be removed by trypsinization or treatment with papain. The effect of glutamine was depressed by inhibitors of energy metabolism, suggesting that glutamine-stimulated incorporation is an energy-mediated effect.
A 10-year-old boy with Marfan's disease suffered an acute dissection of an aortic aneurysm. A dacron sleeve (extending from 3 cm above the sinuses of Valsalva to just distal to the left subclavian artery) was placed around the aorta. 2 years later the child succumbed to aortic dissection and rupture distal to the sleeve. Autopsy showed (1) complete healing of the earlier dissection, and (2) lack of aneurysmal progression within the sleeve. Although the sleeve had provided effective support to the diseased aorta, it did not have sufficient length and should have been extended to include the region of the first and second intercostal arteries. The use of an extensive dacron sleeve is potentially useful as treatment prior to dissection.
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We report three cases of corrected transposition (CT), all with Ebstein's disease, ventricular septal defect (VSD) and ventricular preexcitation. In cases 1 and 2, the ECG revealed sinus rhythm, with type A fusion preexcitation QRS complexes, suggesting left-sided Kent bundles and intact conduction system (CS). Complete serial section (SS) of the CS in both cases revealed an anterior CS and a Kent bundle in the posteroseptal wall of the morphologic right ventricle (MRV). Case 3 had intermittent preexcitation, with periods of complete atrioventricular (AV) block with narrow QRS escape rhythm. The preexcitation complexes suggested the presence of a left lateral Kent bundle. SS of the CS revealed a blind posterior and two anterior AV nodes, one on either side of the pulmonary trunk. The left anterior AV node was blind. The right anterior node formed the anterior bundle, which ended blindly. This bundle emerged again and joined a posterior bundle to form an interrupted sling around the closed VSD. In addition, there was a tenous Kent bundle at the posterolateral wall of the MRV. In summary: (1) preexcitation in CT with Ebstein's disease of the left AV valve is associated with Kent bundles; (2) fusion complexes reflected intact CS; and (3) intermittent preexcitation with AV block was associated with the presence of tenuous Kent bundl and discontinuity of the CS.
A 25-year-old white female had idiopathic hypertrophic subaortic stenosis proved by catheterization. The ECG revealed left bundle branch block. Electrophysiologic studies revealed normal PA and AH intervals, but the HB interval was prolonged (70 msec). The width of the His spike was 20 msec. A clear, reproducible split His potential was demonstrated with the atrial extrastimulus technique. Because the patient was symptomatic, a permanent pacemaker was inserted. One week later the patient died suddenly. Autopsy revealed an enlarged heart with a markedly thickened and sigmoid ventricular septum and a small and coarsely trabeculated lumen. Histologically the common bundle was situated on the right side of the septum. It was intact at its origin, but showed fibrotic changes more distally. The beginning of the left bundle branch was markedly disrupted as it traversed the septum. The right bundle was moderately fibrosed. There was excellent correlation between the electrocardiographic and electrophysiologic findings and the findings in the conduction system. The cause of the sudden death was related either to the outflow obstruction or to an arrhythmia, or both.
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