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Query: UMLS:C0018799 (heart disease)
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Ebstein"s anomaly is the most common congenital heart disease associated with the Wolf-Parkinson-White syndrome. Between November 1973 and March 1993, we surgically treated 42 patients with Wolff-Parkinson-White syndrome and Ebstein's anomaly. The patient's ages ranged from 5 months to 59 years (mean 35.3 +/- 14.0 years). There were a total of 52 accessory pathways, 48 of which were located in the right (65%) or posteroseptal (29%) area. A left-sided accessory pathway was seen in only two patients (3.8%). Division of all right-sided accessory pathways was done during normothermic cardiopulmonary bypass with the heart beating; cryocoagulation was applied together with scalpel dissection of the atrioventricular groove. Division of the left-sided accessory pathways was done with the use cold potassium cardioplegic arrest. Thirty-five of these patients underwent tricuspid valve operation for Ebstein's anomaly and 11 of them underwent tricuspid valve replacement with a bioprosthesis. All 52 accessory pathways were successfully divided, although two patients required reoperation because of tachycardia caused by accessory pathways in different positions. Three hospital deaths (7.1%) occurred. There were no late deaths during the follow-up period (mean 94.3 +/- 52.4 months), but two patients required repeat tricuspid operation because of progression of the tricuspid regurgitation. Because no repeat operations were required during long-term follow-up patients who underwent valve repair or valve replacement, correction should be indicated in some patients.
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PMID:Surgical treatment of patients with Wolff-Parkinson-White syndrome and associated Ebstein's anomaly. 852 83

1995 is the fifth anniversary of the advent of catheter ablation for the treatment of supraventricular tachycardia. Surgery has established the principles of the interventional approaches: 1) identification of the mechanism; 2) localization of the site of the mechanism; 3) identification of the anatomical arrhythmogenic substrate and its localization using preoperative and intraoperative electrophysiological cardiac mapping; 4) ablation of the arrhythmogenic substrate using "surgical" dissection or excision or various forms of energy to neutralize the substrate: cryoablation, laser, etc. Surgical approaches also established the EP interventions as the first line of therapy because they are curative. Currently, surgery for supraventricular tachycardia is essentially confined to atrial fibrillation, and after attempted catheter ablation for the Wolff-Parkinson-White syndrome. Atrial fibrillation is a complex arrhythmia, commonly associated with structural heart disease. To understand atrial fibrillation, a number of premises should be reviewed: atrial functional anatomy, atrial pathology, atrial fibrillation mechanism (s) and clinical presentation. The role of atrial fibrillation in terms of symptoms, morbidity and mortality is not clear because it is difficult to determine if atrial fibrillation is a symptom, a marker, an autonomous disease albeit it is in most cases an aggravating factor. Surgical rationales for atrial fibrillation are based on three concepts: exclusion, fragmentation and channelling. The Corridor operation was the first used direct surgical approach. The Maze operation and other techniques (fragmentation, spiral) have been reported. All surgical techniques have been reported with good results in terms of sinus node function and exercise tolerance, and to various degrees, in terms of atrial contraction. Currently, there is a trend to combine direct atrial fibrillation surgery with surgery for mitral valve albeit beneficial effects are not documented.
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PMID:Surgery for supraventricular tachycardia. 873 73

The number of patients with congenital cyanotic heart disease who reach child-bearing age is increasing. This is partly a consequence of the high long-term survival and the haemodynamic benefits resulting from the Fontan procedure, which is used for the definitive palliation of such cyanotic heart disease as tricuspid atresia and single ventricle. However, so far little experience has been recorded with pregnant patients who have undergone right ventricular exclusion procedures. The particular physiology of a univentricular heart and a passive, non-pulsatile blood flow through the lungs has significant implications for the anaesthetic obstetric management of these patients. We report a case of successful pregnancy and caesarean delivery after a modified Fontan procedure. CASE REPORT. The patient was a 30-year-old pregnant woman with a singleton pregnancy. At the age of 20, after four palliative shunt operations, she had undergone a modified Fontan operation due to tricuspid atresia with a single ventricle, d-transposition of the great arteries, pulmonary atresia and a single atrium. Following the Fontan repair, she initially suffered from intermittent Wolff-Parkinson-White syndrome and isorhythmic AV dissociation. The pregnancy was uneventful, and caesarean section was scheduled for 32 weeks' gestation. Because of the increased risk of thrombosis, the patient was treated with s.c. heparin preoperatively; for this reason, epidural anaesthesia was excluded, though it may otherwise be preferred for such patients. Amoxicilline was used to prevent endocarditis. At the date of caesarean delivery her body weight was 54 kg and boy height, 155 cm. Before induction of anaesthesia, a central venous and a radial artery catheter were placed for invasive pressure monitoring. An exaggerated left lateral tilt position was used to avoid aortocaval compression. After careful preoxygenation, anaesthesia was induced with 24 mg etomidate, 1.5 mg norcuronium, and 75 mg succinylcholine. Halothane 0.5-0.7% in oxygen was used during the first few minutes of surgery. Central venous pressure under mechanical ventilation was 20 mmHg, while the heart rate varied between 70 and 90 bpm. Delivery was accomplished 8 min after the induction of anaesthesia. The Apgar scores after 1 and 5 min were 9 and 10, respectively. Anaesthesia was continued with fentanyl, midazolam and nitrous oxide 50%. The remainder of surgery was unevenful. The child is now 5 years old and healthy. The mother has a near-normal activity level and does not need any help to care for her child. DISCUSSION. After a modified Fontan repair, i.e. atriopulmonary or total cavopulmonary anastomosis, the pulsatile pulmonary blood flow is converted to a passive, non-pulsatile blood flow that depends critically both on the pressure gradient between right (RAP) and left atrial pressure (LAP) and on pulmonary vascular resistance (PVR). Thus, the maintenance of an adequate transpulmonary pressure gradient and avoidance of an increase in PVR are of major importance for the obstetric anaesthetic management in patients who have undergone right ventricular exclusion procedures. Impairment of venous return caused by slight caval compression or high airway pressure may reduce cardiac output more critically than in patients with a normal circulation. CONCLUSION. This case demonstrates that the haemodynamic consequences of pregnancy and of caesarean delivery under general anaesthesia can be tolerated in post-Fontan patients despite the absence of a contractile pulmonary ventricle.
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PMID:[Anesthesiologic aspects of pregnancy and delivery in a patient following a modified Fontan procedure ]. 876 69

The authors report a case of paroxysmal supraventricular reciprocal tachycardia, associated with transient Wolff-Parkinson-White syndrome in a 25-year-old woman, without heart disease, at 31 weeks gestation. The arrhythmia was successfully converted to sinus rhythm by intravenous flecainide. After delivery the arrhythmia and pre-excitation syndrome spontaneously disappeared and were not observed yet during the 6 months follow-up. Pregnancy may predispose patients with Wolff-Parkinson-White syndrome toward paroxismal supraventricular reciprocal tachycardia. The management of a similar case is discussed.
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PMID:[Reciprocal supraventricular paroxysmal tachycardia in pregnancy. Apropos of a case]. 896 6

The management of unexplained syncope begins with the patient's history and physical examination, which are oriented to help separate benign from serious causes. Malignant etiologies are more likely to occur with exertional syncope. Cardiac causes should be considered, particularly cardiomyopathy, postoperative congenital heart disease, right ventricular dysplasia, anomalous coronary artery, pulmonary artery hypertension, myocarditis, long QT syndrome, and Wolff-Parkinson-White syndrome. Neurological and metabolic disorders may underlie a syncope episode. After malignant causes of syncope have been excluded and the diagnosis of neurocardiac syncope has been established, treatment strategies include behavior modification, salt and increased fluids, and pharmacological agents. Efficacious agents include beta-blockers, dysopyramide, fludrocortisones, and alpha agents. Yet, behavior modification alone may be as effective as salt or pharmacological therapy. Because the natural history of neurocardiac syncope in children is spontaneous resolution, it is appropriate to try the simple measures before introducing drug therapy.
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PMID:Unexplained syncope: clinical management. 927 5

In 50% of the patients presenting with a syncope, the cause is cardiac. The incidence of sudden death with 24% is high in this group. Since most of the tachycardia-induced syncopes are due to ventricular tachycardia (VT), a careful diagnostic approach must be used. The possibility of a VT to end in a fibrillation is great, especially in the presence of a organic heart disease, which leads to a bad prognosis in such patients. The aim of a careful anamnesis and clinical history is to establish the presence of a cardiac disease. A Wolff-Parkinson-White syndrome, a long QT, an old myocardial infarction or a coronary artery disease (CAD) can be assessed by echocardiography (ECG). Stress testing is useful in evaluating a CAD and can possibly lead to a diagnosis when a VT or a supraventricular tachycardia (SVT) is induced. ECG is used to assess the cardiac ejection fraction and in the evaluation of a suspected right or left cardiomyopathy. The ambulatory ECG allow a diagnosis only in 2 to 3% of the cases. Nevertheless, the presence of more than 10 PVC/h and/or asymptomatic nonsustained VT is a predictor for sudden death in syncopy patients. Detection of late potentials has a sensitivity of 50 to 83% and a sensibility of 89 to 91% for the prediction of inducible sustained VT during electrophysiological studies (EPS) in patients with syncope. However, the usefulness of this technique is not fully established, since there is no significant difference in survival or recurrence of syncope between patients with and without late potentials. The EPS is an invasive technique and therefore used at the end of the investigations. The cardiovascular mortality is low (4%) in patients with a negative EPS. A treatment is mandatory in tachycardia-induced syncopes even when the cause is a SVT. Antiarrhythmic drugs are useful for the treatment of SVT. However, radiofrequency ablation of the accessory pathway is preferable, since the success rate is over 90%, and the side effects of chronic ingestion of antiarrhythmic drugs can therefore be avoided. Some VT can be treated successfully with drugs under the control of an EPS, but most of the patients must have the implantation of an internal cardiac defibrillator (ICD).
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PMID:[Tachycardia-induced syncopes]. 933 80

Atrial fibrillation (AF) is in most patients (approximately 70%) associated with organic heart disease including valvular heart disease, coronary artery disease, hypertension, hypertrophic cardiomyopathy, dilated cardiomyopathy, and congenital heart disease, mostly atrial septal defect in adults. In many chronic conditions, determining whether AF is the result or is unrelated to the underlying heart disease, remains unclear. The list of possible etiologies also include cardiac amyloidosis, hemochromatosis and endomyocardial fibrosis. Other heart diseases, such as mitral valve prolapse (without mitral regurgitation), calcifications of the mitral annulus, atrial myxoma, pheochomocytoma, and idiopathic dilated right atrium may present with AF. Atrial fibrillation may occur in the absence of detectable organic heart disease, the so-called "lone AF", in about 30% of cases. The term "idiopathic AF" implies the absence of any detectable etiology including hyperthyroidism, chronic obstructive lung disease, overt sinus node dysfunction, and overt or concealed preexcitation (Wolff-Parkinson-White syndrome), only to mention a few of other uncommon causes of AF. The autonomous nervous system may contribute to the occurrence of AF in some patients. AF occurs commonly. In patients with valvular heart disease, AF is common, particularly when the mitral valve is involved. The occurrence of AF is unrelated to the severity of mitral stenosis or mitral regurgitation but is more common in patients with enlarged left atrium and congestive heart failure. In patients with coronary artery disease, AF occurs predominantly in older patients, males, and patients with left ventricular dysfunction, Important predictive factors of AF include hypertension, left ventricular hypertrophy and diabetes. The risk of the development of AF, in an individual patient, is often difficult to assess. Increasing age, presence of valvular heart disease, and congestive heart failure increase the risk of atrial fibrillation.
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PMID:Factors predisposing to the development of atrial fibrillation. 935 13

Closed-chest transcatheter electrical ablation (catheter ablation) has been applied to various supraventricular and ventricular tachyarrhythmias as a radical therapeutic technique since its introduction in 1982. Currently, it has become a first line therapy for supraventricular tachyarrhythmias except atrial fibrillation and uncommon types of atrial flutter. We first carried out the ablation procedure in 1991 for the treatment of ventricular tachycardia. Up to February 1997, a total of 187 patients underwent catheter ablation in our institution. The aims of this study are to demonstrate our results of catheter ablation in the early 5 years and to show the usefulness of this new curative method. Successful results were obtained in 168 of 187 patients (overall final success rate: 89.8%). The success rates of each category of tachyarrhythmias were 100/105 patients (95%) with WPW syndrome, 41/46 (89%) with atrioventricular nodal reentrant tachycardia, 7/10 (70%) with atrial flutter, 4/4 (100%) with atrial tachycardia, 2/2 (100%) with medically refractory atrial fibrillation, 13/15 (85%) with idiopathic ventricular tachycardia and 3/7 (43%) with sustained ventricular tachycardia associated with structural heart disease, respectively. Complications that required invasive treatments were observed in 3 patients (2 hemopericardium and 1 complete atrioventricular block). Our results indicate that catheter ablation is highly effective in most categories of tachyarrhythmias and can be applied safely without lethal complications.
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PMID:Curative percutaneous catheter ablation for various supraventricular and ventricular tachyarrhythmias. Results in 187 consecutive patients during the first five years. 943 76

This study was designed to assess adverse effects of oral propafenone in a large number of pediatric patients. Retrospective data from 27 European centers covering 772 patients treated with oral propafenone were analyzed. The following arrhythmias were treated: reentrant supraventricular tachycardia in 388 patients, atrial ectopic tachycardia in 66, junctional ectopic tachycardia in 39, atrial flutter in 21, ventricular premature complexes in 140, ventricular tachycardia in 78, and other arrhythmias in 39 patients. Two hundred forty-nine patients (32.3%) had structural heart disease. Significant electrophysiologic side effects and proarrhythmia were found in 15 of 772 patients (1.9%): sinus node dysfunction in 4, complete atrioventricular block in 2, aggravation of supraventricular tachycardia in 2, acceleration of ventricular rate during atrial flutter in 1, ventricular proarrhythmia in 5, and unexplained syncope in 1 patient. Cardiac arrest or sudden death occurred in 5 of 772 patients (0.6%): 2 patients had supraventricular tachycardia due to the Wolff-Parkinson-White syndrome and a normal heart; the remaining 3 patients had structural heart disease. Overall, adverse cardiac events were more common in the presence (12 of 249 patients, 4.8%) than in the absence (8 of 523 patients, 1.5%) of structural heart disease (p <0.01). There was no difference between patients treated for supraventricular and ventricular arrhythmias. Thus, propafenone is a relatively safe drug for the treatment of several pediatric tachyarrhythmias. Proarrhythmic effects seem to be less frequent than those reported for encainide or flecainide and occur predominantly in patients with structural heart disease.
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PMID:Safety of oral propafenone in the treatment of arrhythmias in infants and children (European retrospective multicenter study). Working Group on Pediatric Arrhythmias and Electrophysiology of the Association of European Pediatric Cardiologists. 960 53

The morphological and functional cardiac adaptations induced by physical training may be reflected in several athlete's electrocardiographic variants. Rhythm and heart rate disturbances are the most common findings, and sinus bradycardia is the most frequent adaptation. Non-specific intraventricular conduction delay and incomplete right bundle branch block are also frequent, but other bundle branch and fascicular blocks are extremely rare. While the atrioventricular conduction may be prolonged, the occurrence of first degree and type I second degree atrioventricular blocks depends on the individual's susceptibility. Advanced second and third degree atrioventricular blocks are exceptional, and when present, the possibility of underlying heart disease must be excluded. High QRS voltage is more frequent in male athletes, but its correlation with left ventricular hypertrophy is low. The ST segment elevation in the so called "early repolarization" pattern is typical of the athlete's electrocardiogram. Vagotonic or high T wave voltages and U waves are also frequent when sinus bradycardia is present. Tachyarrhythmias and increased automatism arrhythmias are rare and usually benign. The increased vagal tone is responsible for the suppression of the physiological and ectopic pacemakers. While Wolff-Parkinson-White syndrome per se does not exclude an athlete from sports activity, the risk of a sudden death makes it mandatory to perform an exhaustive cardiac evaluation. We may conclude that no sport can be considered arrhythmogenic or as a predisposing factor for malignant ventricular arrhythmias.
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PMID:["The athlete's heart": most common electrocardiographic findings]. 964 59


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