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1/6. Myocardial dysfunction in mitochondrial diabetes treated with Coenzyme Q10.

    Maternally-inherited diabetes and deafness (MIDD) has been related to an A to G transition in the mitochondrial tRNA Leu (UUR) gene at the base pair 3243. Although some previous articles have reported that this mutation may be a cause of cardiomyopathy in diabetes, the degree of cardiac involvement and a specific treatment has not been established. Here, we reported a case of a patient with MIDD who developed congestive heart failure and the therapeutic usefulness of Coenzyme Q10 (CoQ10). In our patient, after the introduction of Coenzyme Q10 150 mg/day, there was a gradual improvement on left ventricular function evaluated by echocardiography. The fractional shortening (FS) and ejection fraction (EF) increased from 26 to 34% and from 49 to 64%, respectively. No side effects were noted. Three months after CoQ10 discontinuation, the parameters of systolic function evaluated by echocardiography decreased, suggesting that CoQ10 had a beneficial effect. Identification of diabetes and cardiomyopathy due to mitochondrial gene mutation may have therapeutic implications and Coenzyme Q10 is a possible adjunctive treatment in such patients.
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2/6. ventricular fibrillation during transesophageal atrial pacing in an infant with wolff-parkinson-white syndrome.

    A complication of transesophageal atrial pacing in an infant with wolff-parkinson-white syndrome (WPW) is reported. A newborn infant born with fetal hydrops had recurrent supraventricular tachycardia (SVT) that required repeated successful conversion by transesophageal atrial pacing. Because of secondary left ventricular dysfunction, digoxin was administered. During repeat transesophageal atrial pacing for recurrent SVT, ventricular fibrillation occurred. Although it is unclear which of several possible contributing factors was responsible for the ventricular fibrillation, recommendations are appropriate to minimize the risk in infants with WPW.
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3/6. propranolol-induced dysfunction of the sinus node in wolff-parkinson-white syndrome.

    We report the findings in a patients with the wolff-parkinson-white syndrome (type A) who initially had recurrent fainting episodes. It appeared that they were caused by prolonged posttachycardiac depression of the sinus node, which was induced by treatment with propranolol. The possibility of covert dysfunction of the sinus node in patients with wolff-parkinson-white syndrome should be considered before commencing therapy with beta-adrenergic blocking agents.
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4/6. angina pectoris in a child with sickle cell anemia.

    A 7-year-old black boy with sickle cell disease, wolff-parkinson-white syndrome, mild left ventricular dysfunction, and normal coronary arteries developed angina pectoris five months after cessation of hypertransfusion therapy. exercise-induced ECG ST segment depression associated with angina disappeared following transfusion therapy.
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5/6. Surgical therapy for wolff-parkinson-white syndrome in patients with bronchial asthma.

    The surgical therapy for wolff-parkinson-white syndrome in patients with bronchial asthma was studied. Between 1974 and 1992, 447 patients with wolff-parkinson-white syndrome were treated, seven of whom had associated severe bronchial asthma. Supraventricular tachyarrhythmias were induced on occasion by bronchodilating agents such as beta-receptor agonists or theophylline preparations. High-dose steroids were required for acute bronchospasm in three patients, despite possibly leading to cardiac dysfunction. Beta-blockers therapy for wolff-parkinson-white syndrome can induce bronchospasm; in patients with wolff-parkinson-white syndrome and bronchial asthma, pharmacological agents used to treat one condition may exacerbate the other. Therefore, a non-pharmacological therapy was performed, namely surgical division of the accessory conduction pathway via an endocardial approach, in all seven asthmatic patients with Wolff-Parkinson- White syndrome. In all cases, division of the accessory conduction pathway resulted in disappearance of the delta wave, and there were no further tachyarrhythmias either at rest or after administration of bronchodilators. Since surgery, bronchial asthma has been effectively controlled with standard drug therapy in all patients. Non-pharmacological therapy is recommended for patients with wolff-parkinson-white syndrome and bronchial asthma.
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6/6. Five-year experience with radiofrequency catheter ablation: implications for management of arrhythmias in pediatric and young adult patients.

    OBJECTIVE: A review of the results of the first 5 years of radiofrequency catheter ablation procedures performed at Children's Hospital, boston, a large tertiary referral center for patients with congenital heart disease and arrhythmias common to the infant, child, and young adult. STUDY DESIGN: A retrospective review of 410 consecutive procedures in 346 patients who underwent at least one application of radiofrequency energy for the treatment of recurrent supraventricular or ventricular tachycardia. RESULTS: The overall final success rate for all diagnoses was 90%, with a higher success rate in patients with an accessory pathway (96%). During the 5-year study period, the success rate improved while the rates of failures and late recurrences declined. The incidence of serious complications was 1.2% (1 late death, 1 ventricular dysfunction, 1 complete heart block, 1 cardiac perforation, and 1 cerebrovascular accident). CONCLUSIONS: This report of a large series of radiofrequency catheter ablation procedures performed at an institution committed to treating congenital heart disease and pediatric arrhythmias confirms the safety and efficacy of this procedure. The pediatric cardiologist/electrophysiologist treating such patients must be aware of specific technical, anatomic, and electrophysiologic variations in the pediatric patient that are critical to the success of this therapy.
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