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

This clinical review details our 15 year experience with permanent cardiac pacemakers in 81 infants and children. Pacing was found inappropriate in one infant. The other 80 patients were paced because of congenital heart block [24], post-operative block [50], or sick sinus syndrome [6]. Maintenance of long-term pacing requires all too frequent re-operation for battery depletion [37%], lead related problems [32%] or wound dehiscence [31%]. Problems related to pacemaker size and the presence of a high myocardial threshold are particularly important in the pediatric patient. In spite of these problems, children requiring cardiac pacemakers can be extremely well, their prognosis depending almost entirely on the presence of underlying heart disease.
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PMID:Permanent cardiac pacing in infants and children. 9 36

Consecutive autopsy of 1,000 cases of the aged disclosed coronary sclerosis in 428 cases, myocardial infarction in 137, cardiac hypertrophy in 237, valvular heart disease in 120, conduction disturbances in 96, cor pulmonale in 41, pericarditis in 39, and congenital heart disease or anomalies in 28. There were 343 morphologically normal hearts. A total of 1,022 heart diseases were found in 657 cases, corresponding 1.6 heart diseases per one heart. Heart diseases in the aged were divided into (1) those continuing from the younger period and (2) those specifically found in the aged. The latter were non-inflammatory valvular diseases and chronic conduction disturbances. Mitral regurgitation, including mitral ring dilatation (10), mitral ring calcification (9), spontaneous rupture of the chordae tendineae (2) and calcified aortic stenosis (12) and degenerative aortic regurgitation of prolapsed cusp (35) were produced by various degeneration of the connective tissue of the valves. A total of 59 cases of conduction disturbances consisted of complete or advanced heart block (15), right bundle branch block (RBBB) (16), RBBB with left axis deviation (17), and left bundle branch block (LBBB) (11). Forty-seven cases showed main lesions in the branching portion of the AV bundle and origin of the bilateral bundle branches, which were closely related to the degeneration of the central fibrous body and fibrosis at the summit of the ventricular septum. The common field, where the valvular diseases and conduction disturbances occurred, was the fibrous trigone of the heart.
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PMID:A clinicopathological study of the heart diseases in the aged. The morphological classification of the 1,000 consecutive autopsy cases. 12 9

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

Clinical and necropsy findings in 10 dogs with a spontaneous primary hypertrophic cardiomyopathy are described. Each dog had marked cardiac hypertrophy, and 8 dogs had disproportionate thickening of the ventricular septum with respect to the left ventricular free wall (compared with dogs with normal hearts or with cardiac hypertrophy due to acquired or congenital heart disease). Septal:free wall thickness ratios in the 10 dogs ranged from 1.1 to 1.5; 6 had ratios greater than or equal to 1.3. However, marked cardiac muscle cell disorganization in the ventricular septum, characteristic of patients with hypertrophic cardiomyopathy, was present in only 2 of the 10 dogs. Death occurred most commonly while the dogs were under anesthesia during the course of operative procedures (5 dogs) or suddenly and unexpectedly in animals without previous symptomatic manifestations of cardiac disease (3 dogs). Four dogs had clinical signs of congestive heart failure, including 2 with marked cardiac decompensation. In addition, 2 of these 4 dogs with heart failure and 1 dog without previous symptoms (that died during a noncardiac operation) manifested complete heart block. It is conceivable that dogs with spontaneous hypertrophic cardiomyopathy may prove useful in the future investigations of the clinical, hemodynamic, and pathologic features of this disease in humans.
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PMID:Hypertrophic cardiomyopathy in the dog. 15 45

Problems associated with the early evolution of corrective operation (septation) for univentricular heart (single or common ventricle) are illustrated in a total experience of 36 such operations, 30 of which were recent. Complete heart block existed preoperatively in five of the 30 patients and developed at operation in another 10; only two of these 15 patients attained a satisfactory result. Associated pulmonary stenosis was present in 16 patients, and this seemed to influence the result favorably. An extracardiac conduit between the right-sided ventricular chamber and the distal pulmonary trunk was employed in 20 patients. The presence of associated anomalies, including function of the atrioventricular valves in seven, had a highly unfavorable influence on the result. Although all seven of the most favorable candidates attained a satisfactory result, only 13 (43 percent) in the total group of 30 attained a satisfactory result. Further evolution of this operation, as in previous operations for complex congenital heart disease, should provide more results that are satisfactory.
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PMID:Correction of the univentricular heart having two atrioventricular valves. 32 8

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

In 18 patients who presented in less than 2 years with heart disease characterized by arrhythmias (including atrial fibrillation, ventricular arrhythmias and heart block), atypical chest pain, pericarditis and cardiac failure, extensive investigation revealed no cause for the disease except for evidence of toxoplasmic infection. One patient had acute toxoplasmosis; the other 17 patients had chronically increased titers, higher than the expected level in the community and also higher than in a control series of patients with well defined heart disease. Toxoplasmosis is probably a fairly common cause of heart disease in this community. The source of infection appeared to be cats, uncooked meat and congenital infection. Patients received chemotherapy with either pyrimethamine and sulfadiazine or tetracycline. Serious relapse occurrred in three patients and embolic complications in two. Experimental myocarditis occurs when toxoplasmic cysts rupture within the heart; therefore clinical symptoms may occur sporadically during a chronic infection.
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PMID:Toxoplasmic infection in cardiac disease. 42 23

Necropsy findings in 10 dogs with naturally occurring cardiac disease closely resembled hypertrophic cardiomyopathy in human beings and cats. Each dog had marked cardiac hypertrophy, and 8 dogs had disproportionate thickening of the ventricular septum with respect to the left ventricular free wall (compared with dogs with normal hearts or with cardiac hypertrophy due to acquired or congenital heart disease). Ratios of septum to free wall thickness in the 10 dogs ranged from 1.1 to 1.5, and 6 had ratios greater than or equal to 1.3. Marked cardiac muscle cell disorganization in the ventricular septum, characteristic of human patients with hypertrophic cardiomyopathy, was found in only 2 of the 10 dogs. Death occurred while the dogs were under anesthesia during the course of operative procedures (5 dogs) or unexpectedly in animals without previous manifestations of cardiac disease (3 dogs). Four dogs had clinical signs of congestive heart failure, including 2 with marked cardiac decompensation. Two of these 4 dogs with heart failure and 1 dog that died during unrelated surgery, but without prior signs of heart disease, had electrocardiographic evidence of complete heart block.
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PMID:Canine hypertrophic cardiomyopathy. 42 33

A three-year-old girl with congenital complete heart block presented with repeated bouts of ventricular tachycardia and ventricular fibrillation. The ECG was remarkable for both complete heart block and a long Q-T interval, when corrected for rate. The Q-T interval was longer than the Q-T interval of children with congenital complete heart block and of children without heart disease. Overdrive ventricular pacing was necessary to control the arrhythmias. A prolonged Q-T interval in patients with complete heart block, even in the presence of a normal QRS duration, may predispose the patient to sudden death. Permanent pacing can suppress these arrhythmias by overdriving.
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PMID:Congenital complete heart block and long Q-T syndrome requiring ventricular pacing for control of refractory ventricular tachycardia and fibrillation. 46 47

Twenty-six children who had permanent pacemakers implanted at 6 hours to 11 years of age have been followed for up to 163 months. There were 14 children with surgical heart block, 9 with congenital heart block, 2 with postcatheterization complete heart block, and 1 with bradytachydysrhythmia syndrome. Eighteen of the 26 patients (69%) are still being paced with their original electrodes, some for more than eight years. Sixty pulse generators lasted an average of 17 months (range, 1 to 55 months). Five of the 26 patients (19%) are dead. Three died of noncorrectable heart disease, but there were 2 sudden unexplained deaths at home, both apparently due to sudden arrhtthmias. Both of these patients had received fixed-rate pulse generators, and 1 had a known potential for competing rhythms. The current optimal choices of equipment and techniques, including the role of synchronized, demand, lithium-powered, and nuclear-powered pulse generators, are discussed.
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PMID:Fourteen years of implanted pacemakers in children. 62 35


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