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)

Eleven patients with short P-R intervals and narrow QRS complexes had ventricular tachycardia due to organic heart disease: mitral valve prolapse with mitral insufficiency (2 patients); alcoholic (?) cardiomyopathy (2 patients); and coronary artery disease (7 patients). Intracardiac studies showed short A-H intervals during sinus rhythm in all cases. The onset of ventricular fibrillation (which, to our knowledge, has not been observed in patients having short P-R and A-H intervals coexisting with narrow QRS complexes) was documented in 4 cases. Only 1 patient (with quinidine syncope) had been premedicated. In the 3 other patients the episodes of ventricular fibrillation appeared during bouts of atrial fibrillation with rapid ventricular rates which could have been an exprerssion of the "enhanced A-V conduction" that had been manifested in sinus beats by short P-R and A-H intervals. In clinical settings and physiological conditions proven to be hemodynamically unstable (such as transient ischemia or acute myocardial infarction) these rapid ventricular rates could have led to ventricular fibrillation; directly because of the R-on-T phenomenon, and/or indirectly due to decreased coronary perfusion. Ventricular tachycardia and ventricular fibrillation due to organic heart disease probably occur more often than suggested by the few reported cases in the literature. Its significance, however, has to be clarified by further prospective studies.
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PMID:Ventricular tachycardia and ventricular fibrillation in patients with short P-R intervals and narrow QRS complexes. 9 18

Many patients with hypertrophic cardiomyopathy die suddenly and unexpectedly, a significant number perhaps due to arrhythmia. Of 100 patients initially evaluated for signs or symptoms suggestive of heart disease or a family history of hypertrophic cardiomyopathy, 51 were selected solely because they met the echocardiographic criteria for the disease, and 49 patients were selected primarily because they had: 1) normal sinus rhythm despite left atrial enlargement, 2) a history of syncope, 3) a family history of premature death, or 4) a history of paroxysmal atrial fibrillation. All 100 patients were studied by 24-hour ambulatory electrocardiographic monitoring and 74 of them also underwent treadmill exercise testing. More than 50% of patients in repetitive ventricular premature depolarizations, including 19% who had ventricular tachycardia. Monitoring was superior to exercise testing for exposing these arrhythmias. Two patients experienced cardiac arrest within 2 months of monitoring; in each, monitoring had revealed ventricular tachycardia. Two patients with paroxysms of supraventricular tachycardia during monitoring developed fixed atrial fibrillation within 1 year. These preliminary observations suggest that monitoring may help identify patients at increased risk for significant arrhythmic events.
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PMID:Prevalence of arrhythmias during 24-hour electrocardiographic monitoring and exercise testing in patients with obstructive and nonobstructive hypertrophic cardiomyopathy. 15 81

In this series of one hundred and twenty-eight adults with Down's syndrome nearly half (i.e. 42.2 per cent) developed a normal EEG. This would appear to bear out the findings of Gregoziades and Pampiglione (1966) that older children with this syndrome tended to have tracings similar to the normal child. The youngest age group of fifteen to twenty-four years developed a normal tracing in 38.9 per cent of cases. The most frequent abnormality was an excess of theta, in keeping with the suggestion of Godinova and Hirai and Izawa that this was due to immaturity. Neither the presence of congenital heart disease nor diabetes nor intercurrent illness appeared to have any effect on the development of seizures. Epilepsy developed at any time during adult life but, not surprisingly, the five cases developing it had shown sharp or paroxysmal activity previously. Two had suffered from fainting attacks. In one, the diagnosis was confirmed later by a typical grand mal seizure and the other by response to anticonvulsants. Neither suffered from congenital heart disease.
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PMID:The EEG and incidence of epilepsy in Down's syndrome. 15 92

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

Idiopathic hypertrophic subaortic stenosis (IHSS) occurs more commonly in the elderly than is generally believed, and is often unsuspected. In 26 patients above the age of 60, the diagnosis was recognized in only 7 (27 percent) prior to echocardiography. Symptoms included dyspnea in 17, chest pain in 16, and dizziness or syncope in 8 patients. In 10 patients, establishing the correct diagnosis led to therapy with propranolol, with or without discontinuation of digitalis; in 7 of these, the chest pain was significantly reduced. An accurate diagnosis is particularly important because drugs that are useful in other forms of heart disease may have adverse effects in IHSS. Echocardiography is the diagnostic procedure of choice and is indicated in the presence of an unexplained systolic murmur, especially when it is associated with chest pain, syncope or left ventricular hypertrophy.
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PMID:Unsuspected hypertrophic subaortic stenosis in the elderly diagnosed by echocardiography. 57 Sep 83

Thirty children and young adults with electrocardiographic and clinical evidence of sinus node dysfunction (SND) are described. Ages ranged from 3 days to 25 years. Of these, 10 patients had inappropriate sinus bradycardia, 16 had periods of sinus arrest, and 9 had sinoatrial exit block. A total of 22 patients had significant heart disease and SND developed in 13 after cardiac surgery. Eight patients with SND had no obvious heart disease. Seven patients were symptomatic, having either syncopal (three) or near-syncopal episodes (four). The three with syncope have received permanent demand pacemakers. Although significant SND occurs most frequently in children with congenital heart disease, particularly following corrective cardiac surgery, it may occur in otherwise normal individuals.
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PMID:Sinus node dysfunction in children, adolescents, and young adults. 66 85

The oto-vertebral syndrome is a rare combination of malformations of the ears, spinal anomalies, and congenital heart disease. The syndrome appears to be caused by early embryonic exogenous damage during the 6th or 7th week of embryonic development. The case is reported of a 28-year-old man with bilateral aplasia of the external ear, bilateral aplasia of the ear canal, hypoplasia of the mandibula, severe thoracic scoliosis, and ventricular septal defect with pulmonary hypertension. He was admitted with dyspnea on exertion, syncope, and severe cyanosis. Cardiac catheterization revealed severe pulmonary hypertension with moderate right-to-left and slightly left-to-right shunt (Eisenmenger syndrome). Right and left ventricular function, as evaluated by angiocardiography, was slightly reduced. Because of the severe hemodynamic alterations, symptomatic therapy with digitalis, repeated venesection, and anticoagulation was initiated.
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PMID:[The oto-vertebral syndrome]. 70 8

The basic features of Goldenhar syndrome are preauricular appendices, epibulbar dermoids, vertebral and cardiac anomalies, and hypoplasia of the lungs. The syndrome appears to be caused by early damage during the first or second month of embryonic development. Prognosis is most often determined by the underlying heart disease. In the present study a 24-year-old female student is reported who exhibited a preauricular appendix on the right side, severe thoracic scoliosis, aplasia of the middle and lower lobes of the right lung, dextropositio cordis and an atrial septal defect with severe pulmonary hypertension. Cineangiocardiography revealed a mitral valve prolapse with slight mitral regurgitation and a dilated right ventricle with severe tricuspid regurgitation. Left and right ventricular function was slightly to moderately reduced. Selective coronary arteriography revealed a coronary fistula from an atrial branch of the left coronary artery to the right atrium. Symptomatic therapy with digitalis, anticoagulation and repeated venesection was initiated because of the severe hemodynamic findings. However, the patient developed syncope at increasingly frequent intervals and died 3 months after the initial examination.
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PMID:[Goldenhar syndrome]. 76 Jan 85

Ventricular arrhythmias are rare in childhood but may be associated with syncope and sudden death. This report describes 8 children with ventricular arrhythmias, 6 of whom suffered syncopal episodes. Ventricular tachycardia was documented in 5. One boy died suddenly. Complete cardiac investigation was carried out with exercise testing, Holter monitoring, echocardiography, cardiac catheterization, angiography, and electrophysiological studies. The spectrum of abnormalities related to the arrhythmias included prolapsing mitral valve, prolonged QT syndrome, sick sinus syndrome, congenital heart disease, cardiomyopathy, and idiopathic ventricular tachycardia. Exercise testing and Holter monitoring were particularly useful in documenting the arrhythmias and monitoring response to therapy.
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PMID:Evaluation of children with ventricular arrhythmias. 86 64

Twenty-four children and young adults with electrocardiographic evidence of sinus node dysfunction (SND) are described. The patients, whose ages ranged from three days to 25 years, were divided into three groups based on the type of SND. Ten patients had persistent and inappropriate sinus bradycardia (group I): twelve patients had episodes of prolonged sinus arrest (group II); and seven patients had repeated episodes of Mobitz type I or II sinoatrial exit block (group III). Eighteeen patients had significant heart disease and in 11 of these the SND followed corrective cardiac surgery. Seven were operations for atrial septal defect and transposition repair. Seven patients with SND were symptomatic, having either syncopal (3) or near syncopal episodes (4). All three with syncope have received permanent demand pacemakers. These data show that sinotrial exit block occurs in many young patients with SND (29% in this series). Its recognition, however, requires careful electrocardiographic analysis in order to differentiate sinus bradycardia and sinus arrhythmia. Although SND most commonly occurs in children with congenital or acquired heart disease, particularly following corrective cardiac surgery, it may occur in the absence of other cardiac abnormalities.
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PMID:Electrocardiographic recognition of sinus node dysfunction in children and young adults. 87 15


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