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

Probable right ventricular dysplasia and a patent foramen ovale resulted in cyanotic heart disease in a patient with some characteristics of Noonan syndrome.
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PMID:Probable right ventricular dysplasia and patent foramen ovale presenting with cyanosis and clubbing in a patient with characteristics of Noonan syndrome. 748 69

Problems of arrhythmogenic sudden death (ASD) in athletes have been re-assessed on the clinicopathological plane, encompassing the emerging, unsolved, question of so-called idiopathic ventricular tachycardia, and its debated diagnostics versus arrhythmogenic right ventricular dysplasia-cardiopathy. Ischemic-infarction ASD from coronary artery pathology in young athletes has been seen to present with atherosclerotic "soft" subintimal plaques, rich in newly formed smooth myocytes, often attended by adventitial mast cell, as suspect microscopic markers of spasm, relevant to reperfusion; these features can be found also in precociously intramural arteries, responsible for ASD. Rare congenital abnormalities of the coronary ostia occasionally underlie ASD, together with the acquired aneurysmic coronaritis of chronic Kawasaki disease. Ischemic ASD can also be due to coronary arteriolopathy attending hypertrophic cardiomyopathy, a not uncommon disease in athletes, to be carefully discriminated from training heart hypertrophy. Young South-American sportsmen with Chagas' chronic cardiopathy seem to be at particular risk of ASD. Minor, but specific arrhythmogenic cardiac malformations such as accessory AV pathways have been detected in athletes succumbing to otherwise unexplained ASD, undergone careful post-mortem investigation. The need of more attentive and extended histopathologic control emerges from the hitherto ignored cardiac neuropathological substrates of reflexogenic ASD, which is cogent to problems of ASD in competing athletes. The thorough examination of the cardiac vascular centers in the brain stem, and of the peripheral cardiac innervation, at either abutments of the arc of dive- and/or Bezold-Jarisch cardioinhibitory-vasodepressor reflex, made it possible to suggest novel clinicopathological explanations in controversial cases of athletes' ASD, safeguarding from grave leval misjudgements due to sport's forensic medical mistakes.
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PMID:Structural and non-structural disease underlying high-risk cardiac arrhythmias relevant to sports medicine. 750 Jun 31

The authors describe, in a series of 132 pediatric autopsies in the period January 1979-December 1994, the associations between congenital cardiopathies and pulmonary malformations. These latter were present in 13 of 46 cases (34.8%) in which cardiopathy was associated to a malformation of other discricts. In 9 of cases was exclusively interested the lung, while in the remaining 4 cases also other organs presented anomalies. The lung malformations observed were represented in 12 cases by supernumerary scissures with conjuncted cardiac septal system defects; in one case an alveolar dysplasia was associated to a previous Botallus duct. None of the 8 malformative syndromes, due to chromosomopathies, showed lung malformations. Examining the sequential chronological phases of the development of the two apparatus, the Authors hypothesize a common etiopathogenesis in both malformations, due to the isochronous action of the same intra-uterine pathogenic noxa, favoured by the topographic relationships of the two systems. Conversely, seems unlike the action of genetic damages, although either in genetically determined, or intra-uterine acquired, malformative syndromes, the pulmonary defects are almost exclusively represented by anomalies in the scissures formation.
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PMID:[Pulmonary malformations associated with congenital cardiopathy]. 756 61

Ventricular tachycardia (VT) is found usually in patients with structural heart disease. Its symptomatology depends on haemodynamic manifestations. ECG criteria for the diagnosis of VT are known. For the classification of VT we use morphological criteria (monomorphous and polymorphous), duration of arrhythmia (non-and sustained VT) and the pathomechanism of VT (re-entry, increased automation and triggered activity). The clinical impact of VT and the therapeutic approach depend to a great extent on the basic disease. The therapeutic results and prognostic estimates assembled in ischaemic heart disease cannot be mechanically applied in non-ischaemic heart disease. The authors mention the prevalence of VT and the approach to its treatment in dilatative cardiomyopathy, in prolapse of the mitral valve, in hypertrophic cardiomyopathy, in arrhythmogenic right ventricular dysplasia and in patients with a "normal" heart. Only collection of the necessary data and their analysis will help us to achieve better therapeutic results. In the treatment authors focus attention first of all on the pharmacological approach. They emphasize the need of thorough and comprehensive examination of the patient, draw attention to proarrhythmia. In the prevention of relapses of VT most frequently beta-blockers and amiodarone are used (either alone or combined).
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PMID:[Ventricular tachycardia in non-ischemic heart disease]. 757 85

The literature published of the Ebstein anomaly of the tricuspid valve have been given little attention to the study of the trabecular portion of the right ventricle. This motivate us to study the morphology of twenty-three hearts by the segmentary sequential method determining with special interest the extension and grade of valvular attachment, dysplasia, characteristics of the trabecular portion of the right ventricle and associated anomalies. The three valves had some grade of attachment but this was less frequently in the anterior valve. However in our material this occurred in 43% which is consider high comparing with the rest of the literature; the trabecular portion had several bands at this zone and it was covered by the valve attachment. The downward displacement of the functional ring was directly proportional to the valve attachment. The structural anomalies of the right ventricle related to the valve malformation lead us to consider that the primary damage that causes this cardiopathy occurred at the right ventricle during the embryonic development altering the morphogenesis of the tricuspid valve.
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PMID:[A morphological study of 23 hearts with Ebstein's anomaly]. 757 19

The authors undertook a prospective study comparing exercise testing and programmed ventricular stimulation in order to assess the diagnostic value and risks of exercise stress testing in patients with sustained ventricular arrhythmias. Fifty-five consecutive patients (47 men and 8 women) with an average age of 47 +/- 15 years were included. The initial condition requiring investigation was sustained ventricular tachycardia (47 cases) and ventricular fibrillation (8 cases). The patients had ischaemic heart disease (N = 18), dilated cardiomyopathy (N = 9), valvular heart disease (N = 4) and congenital heart disease (N = 3). Six patients presented with arrhythmogenic right ventricular dysplasia and 15 patients had apparently normal hearts. Four episodes of sustained ventricular tachycardia (7.3%) and 10 non-sustained ventricular tachycardia (18.2%) were induced by exercise testing. One poorly tolerated episode of ventricular tachycardia required cardioversion. No signs of myocardial ischaemia were observed in association with the induced ventricular tachycardia Patients with ventricular tachycardia induced by exercise were significantly younger (39.5 +/- 12.5 vs 49.5 +/- 15.4 years; p = 0.04) and attained a lower predicted maximal heart rate (82.2 +/- 14.6 vs 91.4 +/- 14.4%; p = 0.04), but the underlying pathologies were the same, as were the initial arrhythmias and the level of exercise attained. With respect to the clinical circumstances of induction of the initial ventricular arrhythmia, patients with ventricular tachycardia induced by exercise had an increased tendency to arrhythmias during effort or stress (7/14 vs 8/41; p = 0.06). Programmed ventricular stimulation induced more ventricular tachycardias than exercise testing (36/55 vs 14/55; p < 0.0001). The results of exercise testing were not correlated to those of programmed stimulation (p = 0.38).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Diagnostic value of exercise test in the evaluation of sustained ventricular arrhythmia]. 770 18

The differential diagnosis of VTs with LBBB morphology includes several well-defined syndromes. Although the majority of cases are attributable to acquired structural heart disease, including ischemia, prior infarction, or dilated cardiomyopathy, consideration of specific right ventricular processes is essential to proper evaluation and treatment. The approach to older patients or those with evidence for heart disease should begin with an evaluation for coronary artery disease and an assessment of biventricular function. Careful evaluation for bundle branch reentry should be performed during electrophysiological study, especially when there is underlying conduction system disease. Younger patients, those without overt heart disease, or those with isolated right ventricular disease, should receive a complete noninvasive evaluation of right and left ventricular size and function. An abnormal SAECG or identification of intracardiac late potentials suggest right ventricular dysplasia or cardiomyopathy, whereas responsiveness to adenosine and absence of detectable heart disease support the diagnosis of idiopathic right VT. Newer techniques, including MRI, show promise in identifying subtle right ventricular disease not otherwise detectable even in the setting of presumed idiopathic right VT. Following surgical repair of selected congenital heart defects, particularly tetralogy of Fallot, symptoms of recurrent palpitations, near syncope, syncope, or aborted sudden death may be attributable to recurrent VT, and diagnostic electrophysiological study should be considered for these patients. Finally, SVTs with LBBB morphology, particularly cases associated with right-sided or septal accessory pathways, should always be considered in this differential diagnosis.
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PMID:Ventricular tachycardias with left bundle branch block morphology. 773 82

Eighty-nine cases of ventricular tachycardia, resistant to antiarrhythmic therapy, were treated over a 10 year period by high energy D ablation (fulguration). This series included 37 cases of myocardial infarction with a mean ejection fraction of 30%. The mean follow-up period of the survivors was 61 months and clinical efficacy was 87.9%. Twenty-three cases of arrhythmogenic right ventricular dysplasia, aged 40 years, and with an ejection fraction of 57%, followed up for 71 months, had a clinical efficacy of 83%. Twelve patients had verapamil sensitive (fascicular) ventricular tachycardia. Their age was 30, their ejection fraction 65%, the follow-up period 55 months, and the clinical efficacy was 100%. Ten patients had primary dilated cardiomyopathy. Their age was 35, their ejection fraction 23%, the follow-up period of 38 months with a clinical efficacy of 80%. Four patients, aged 21, had operated congenital heart disease with an ejection fraction of 60%, a follow-up of 36 months and a clinical efficacy of 100%. Finally, 3 patients had idiopathic infundibular ventricular tachycardia. Their age was 36, the ejection fraction 62%, the follow-up period was 72 months and the clinical efficacy was 67%. Non lethal complications were observed in 16% of cases, mainly haemopericardium requiring pericardocentesis in 4.5% of cases. The operative mortality and in the month following ablation was 9.2%, observed mainly during the learning period. These results show that fulguration is not without risk, but in skilled hands, it gives remarkable results in the majority of cases.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Fulguration and radiofrequency in ventricular tachycardia]. 777 7

Clinical and electrophysiological features of 20 patients presenting with ventricular tachycardia (VT) of left bundle branch block (LBBB) morphology without evidence of coronary artery disease were studied. The mean age of the patients was 35.2 +/- 12 (range 15-57 years). The rate of VT varied between 140-240/min (182 +/- 80). Six (30%) patients experienced giddiness or syncope during palpitations. Structural heart disease was found in 10 (50%) of these patients, which included arrhythmogenic right ventricular dysplasia in five, submitral left ventricular (LV) aneurysm in one, anterolateral LV dyskinesis in one, dilated cardiomyopathy in one, endomyocardial fibrosis in one and nonobstructive hypertrophic cardiomyopathy in one case. Ten patients were free of structural heart disease. Electrophysiological study was done in all patients. VT with same morphology as spontaneous VT was inducible in only 14 patients. Seventeen patients were treated medically with total or partial amelioration of symptoms. In three patients, two with arrhythmogenic right ventricular dysplasia and one with structurally normal heart, who were unresponsive to drug therapy, the VT focus could be mapped in right ventricular outflow tract and successful electrical ablation was done. Thus in patients who present with VT with LBBB morphology, the heart is often structurally normal but organic disease is not uncommon, and should be carefully searched.
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PMID:Ventricular tachycardia with left bundle branch block morphology in the absence of ischaemic heart disease--clinical and electrophysiological observations. 779 12

The number of pediatric heart transplantations for complex congenital heart disease has increased over the last years, but little experience has been reported in adolescent and adult populations. Between 1987 and 1992, 14 patients (mean age 33.2 years, range 14 to 51 years) were transplanted in our institution because of structural congenital heart disease (n = 9) or other rare disorders of the endomyocardial morphogenesis (n = 5). The main diagnosis included transposition of the great arteries, congenitally corrected transposition of the great vessels, left superior vena cava, tricuspid atresia with right ventricular hypoplasia, double outlet right ventricle with transposition, left ventricular sinusoidal malformation and right ventricular dysplasia. In several cases there were additional intracardiac malformations, including ventricular septum defect, atrial septum defect as well as different forms of pulmonary stenosis. Seven patients had undergone one or more palliative repairs that consisted of modified Blalock-Taussig shunts, Glenn's cavopulmonary anastomosis, Waterstone shunt, Blalock-Hanlon atrioseptectomy and Brock pulmonary valvotomy. Two patients had undergone Senning procedure for transposition of the great arteries. The donor cardiectomy was modified in order to include complete inflow and outflow tissue in the explant and transplantation could be performed without prosthetic material in all patients; deep hypothermic cardiac arrest was never necessary in this series. There was no early or late mortality after a mean follow-up of 37 months (range 4 to 74 months); postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results in all patients. Adolescent and adult patients with complex congenital cardiac diseases can be transplanted with a very low perioperative risk, even after several prior operative procedures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Should cardiac transplantation for congenital heart disease be delayed until adult age? 781 78


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