Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seven out of 829 pediatric cardiac patients (0.84%) were found to have sinus node dysfunction (SND) over the past seven years. Of the seven patients, three had structurally normal hearts. One of these three patients had long QT syndrome. In four patients, structural heart disease was noted. In three of these four patients the sinus node dysfunction was attributed to cardiac surgery. The age of onset of SND ranged from four months to eight years. Presenting symptoms and signs included syncope, near-syncope, seizure and congestive heart failure. Two patients were asymptomatic. Five patients had episodic sinus pause. Sinus or junctional bradycardia was noted in four patients. Three had tachy-bradycardia. High grade atrioventricular block was noted in one patient. Treadmill exercise test revealed a nonsustained ventricular tachycardia in two patients. All seven patients were found to have prolonged maximal corrected sinus node recovery time. Prolonged intra-atrial conduction time was found in three, prolonged AV nodal conduction time in one, and prolonged His-Purkinje conduction time in one patient during the electrophysiologic study. All seven patients showed abnormal results in intrinsic heart rate study. Anti-arrhythmic drugs were prescribed. During the follow-up study, no patient died, but two patients received a pacemaker implantation. Because of the extent of their conduction system diseases, it is recommended that patients with SND should be thoroughly investigated.
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PMID:Sinus node dysfunction in children. 151 8

Genetic factors contribute to heart disease. In this study, linkage analyses have been performed in a family that is predisposed to sudden death from cardiac arrhythmias, the long QT syndrome (LQT). A DNA marker at the Harvey ras-1 locus (H-ras-1) was linked to LQT with a logarithm of the likelihood ratio for linkage (lod score) of 16.44 at theta = 0, which confirms the genetic basis of this trait and localizes this gene to the short arm of chromosome 11. As no recombination was observed between LQT and H-ras-1, and there is a physiological rationale for its involvement in this disease, ras becomes a candidate for the disease locus.
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PMID:Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras-1 gene. 831 39

The long-QT syndrome (LQT; Ward-Romano syndrome) is a cardiac disorder that is inherited as an autosomal dominant trait. Affected family members suffer from recurrent syncope and sudden death due to ventricular arrhythmias. Recently, we identified a DNA marker on the short arm of chromosome 11 (the Harvey ras-1 locus [H-ras-1]) that was completely linked to the LQT locus in one large family. In the study presented here, we performed linkage investigations on six new and unrelated families with LQT. The LQT locus was again completely linked to the H-ras-1 locus in all families examined, with a combined lod score of 5.25 at a recombination fraction of 0. This work confirms our previous assignment of the LQT locus to chromosome 11p and supports the hypothesis that LQT is genetically homogeneous. As no obligate recombinants were identified in either this or our previous study, the H-ras-1 protooncogene remains a candidate for the LQT disease gene. Identification of LQT families with locus homogeneity is an important step in the development of a refined genetic map of this locus and will help determine whether the H-ras-1 marker would be of general use for presymptomatic diagnosis of this potentially fatal, but treatable, disorder.
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PMID:Consistent linkage of the long-QT syndrome to the Harvey ras-1 locus on chromosome 11. 174 60

An international survey identified 40 patients less than 20 years old who underwent surgical implantation of an automatic implantable cardioverter-defibrillator (AICD). There was a history of aborted sudden cardiac death or sustained ventricular tachycardia in 92.5% of these patients. Twenty-two patients (55%) had structural heart disease; dilated and hypertrophic cardiomyopathy were the most common diagnoses. Eighteen patients (45%) had primary electrical abnormalities including seven with the congenital long QT syndrome. There were no perioperative deaths associated with device implantation. Concomitant drug therapy was administered to 75% of the patients. Defibrillator discharge occurred in 70% of the patients, with 17 patients (42.5%) receiving at least one appropriate shock. There were two sudden and two nonsudden deaths at 28.2 months' median follow-up. Sudden death-free survival rates by life table analysis at 12 and 33 months were 0.94 and 0.88, respectively. Total survival rates at 12 and 33 months were 0.94 and 0.82, respectively. The AICD represents an effective treatment approach for young patients with life-threatening ventricular tachyarrhythmias.
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PMID:The automatic implantable cardioverter-defibrillator in young patients. 221 70

Our study group included 12 patients (4 males, 8 females), mean age 60 yr, with symptomatic or threatening tachyarrhythmias (Lown classes IV A, B, V); 2 patients were suffering from mitral valve prolapse syndrome, 2 from ischemic heart disease; 4 from cardiac insufficiency caused by hypertensive or ischemic heart disease; 4 had no evident clinical signs of cardiopathy. Patients suffering from: cardiac insufficiency (F.C. III e IV NYHA); II and III degree BAV; atrial flutter and fibrillation; long QT syndrome; acute ischemic heart disease were excluded from the study. During short-term treatment, patients received placebo for four days and subsequently flecainide 200 mg daily for four days. During medium-term treatment patients received flecainide 200 mg daily (for six months). Several Holter/24-hour monitorings were performed for evaluation of therapy. No significant reduction in the number of ectopic ventricular beats (B.E.V.) was found with placebo whereas reductions of B.E.V. number (97% and 95%, respectively) were found during short and medium-term treatment with flecainide. Flecainide produced: changes in Lown class: from IV A, B and V to II and I; a marked reduction of subjective symptoms (dyspnea, giddiness syncope, precordial pain); ECG changes: increases in: PR: 5-25%; QRS: 11-12%; QT: 11-22%. Flecainide produced no pro-arrhythmic effects or changes in echocardiographic ventricular function index. Flecainide can be considered one of the most effective new antiarrhythmic drugs.
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PMID:[Short- and medium-term treatment of ventricular hyperkinetic arrhythmia with flecainide]. 252 12

To evaluate the mechanism of sudden death in childhood and the physical activity levels at the onset of sudden death, we studied the following items: (1) the incidence and the circumstances surrounding sudden death at school in Kanagawa Prefecture, (2) high risk heart diseases detected among healthy school children by heart disease screening, (3) sudden cardiac death or near miss seen in outpatients with heart disease except congenital heart disease. Among total 15,156,346 school children, sudden death was observed in 97 subjects (M:77, F:20). Annual incidence of sudden death was 6.4 per 10(6). Of the 97 subjects, acute heart failure of unknown etiology was found in 60 (62%), cardiovascular disease in 18 (19%), cerebral vascular accidents in 14 (14%) and heat stroke in 5 (5%). Of the 78 subjects (M:64, F:14) considered as sudden cardiac death, 62 (79%) died during sports activities, and 16 (21%) died at rest. Of the 62 subjects, 29 died during track and field activities and 7 while swimming, both in physical education classes. Eighteen died during athletic club activities and 8 during extracurricular activities. Consequently, 54 subjects (87%) died in the presence of a school teacher. Of the 18 subjects with cardiovascular disease, 9 (hypertrophic cardiomyopathy in 3, myocarditis in 3, Kawasaki disease in 2 and long QT in one) were diagnosed initially by the autopsy study. Latent high risk heart diseases, detected among presumably healthy school children by the heart disease screening program, were the following: hypertrophic cardiomyopathy, long QT syndrome, Kawasaki disease and some arrhythmias (ventricular tachycardia, sick sinus syndrome, A-V block and atrial fibrillation). Follow-up observations of outpatients with heart disease revealed the same results as the heart disease screening program. In order to prevent sudden death at school, the following recommendations should be observed: 1) sports directors should learn "sports medicine in childhood", including primary cardiovascular resuscitation, 2) an accurate heart disease screening program should be operated to detect latent high risk heart diseases, advise on adequate medical treatment, and help ensure an appropriate selection of sports activities, 3) comprehensive autopsy studies should be performed.
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PMID:Sudden cardiac death in childhood. 263 28

The purpose of this study was to determine left ventricular activation, dispersion of refractoriness, and total recovery time in patients with coronary artery disease and ventricular tachycardia and in patients with the long QT syndrome and to compare these patients with a group of normal patients. Left ventricular endocardial catheter mapping and left ventricular refractory period determination were performed in 18 patients. Group 1 consisted of seven patients with no heart disease and no arrhythmia; group 2 consisted of six patients with previous infarction and sustained ventricular tachycardia; and group 3 consisted of five patients with prolonged QT interval and previous cardiac arrest. Total left ventricular endocardial activation was significantly longer in group 2 (75 +/- 23 msec, mean +/- SD) compared with group 1 (34 +/- 9 msec, p less than 0.01) and group 3 (42 +/- 5 msec, p less than 0.05). Dispersion of refractoriness was significantly greater in group 3 (87 +/- 27 msec) than in group 1 (40 +/- 14 msec, p less than 0.01) and group 2 (53 +/- 14 msec, p less than 0.05). Dispersion of total recovery time was significantly greater in group 2 (90 +/- 30 msec) than in group 1 (52 +/- 14 msec, p less than 0.05) as well as group 3 (114 +/- 43 msec) compared with group 1 (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nonuniform recovery of excitability in the left ventricle. 319 91

Ambulatory electrocardiographic monitoring has become an important clinical tool for the diagnosis and management of patients with symptoms suggesting cardiac arrhythmias or ischemic heart disease. Holter monitoring (H) is widely used in the evaluation of patients with recent myocardial infarction, angina pectoris, hypertrophic cardiomyopathy, dilated cardiomyopathy, long QT syndrome, sinus node dysfunction. The role of H monitoring in defining prognosis in many of these heart disease is not well established. Holter monitoring is also used to evaluate the results of antiarrhythmic and antianginal drug therapy. Application of quantitative H monitoring to define drug efficacy has revealed that antiarrhythmic drugs may have a proarrhythmic effect and that a withdrawal syndrome can follow the abrupt cessation of antianginal and antiarrhythmic drugs. There are no consistent data about the optimal duration of monitoring to detect and quantify ventricular arrhythmias and ischemic attacks. We think that the appropriate duration of monitoring must be adjusted to the single patient, considering: a) the frequency and severity of symptoms; b) the cardiac lesion (s) and functional class; c) the goal of H. When used appropriately H can be a cost-effective tool and may prevent hospitalizations.
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PMID:[Role of ambulatory ECG in a cardiology department]. 350 1

The most important characteristics of arrhythmias that determine the need for treatment are the prevalence and clinical significance of the ventricular arrhythmia. Ventricular arrhythmias occur more frequently with advancing age, severity of heart disease and ventricular hypertrophy. Malignant ventricular arrhythmias are of 3 forms: out-of-hospital ventricular fibrillation (VF), recurrent sustained ventricular tachycardia and torsades de pointes ventricular tachycardia in the long QT syndrome. Each condition has a high 1-year mortality rate. Potentially malignant ventricular arrhythmias are ventricular premature complexes (VPCs) of greater than 10 per hour 10 to 16 days after acute infarction and repetitive VPCs. The most malignant arrhythmias occur with severely depressed ventricular function, but VPCs alone have independent prognostic significance. Benign ventricular arrhythmias occur in patients without known heart disease and do not require treatment. The exact effect of frequent and complex VPC in these patients needs further definition.
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PMID:Definition of benign versus malignant ventricular arrhythmias: targets for treatment. 619 81

Ventricular beats are abnormal rhythms which are frequently detected by modern recording techniques in healthy subjects as well as in patients with heart disease. In the first case, analysis of the literature enables us to exclude any pejorative prognostic implication associated with V.E.B., in the absence of any major risk factor for coronary artery disease. However, when there is an underlying heart disease, a number of subgroups can be distinguished with a high risk of sudden death: coronary insufficiency associated with ischaemic cardiomyopathy, especially in the early post-hospital phase after a myocardial infarction; hypertrophic cardiomyopathy with ventricular tachycardia on the Holter monitor, family history of sudden death, personal past history of syncope; mitral prolapse with clinical symptoms and auscultatory signs; idiopathic long QT syndrome. In contrast, V.E.B.s do not appear to have prognostic significance in idiopathic hypokinetic cardiomyopathies and aortic valvular disease. In general, it is more the clinical setting than the actual morphology which determines the prognostic implications of ventricular extrasystoles. The sub-groups at high risk should be treated with anti-arrhythmics, but the evaluation of the effectiveness of such treatment remains uncertain and the authors believe that the development of studies of ventricular stimulation prior to and during treatment are justified.
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PMID:[Ventricular extrasystole: prognostic value and therapeutic indication]. 620 Nov 23


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