1/1540. Variable clinical expression of Holt-Oram syndrome in three generations.Holt-Oram syndrome is a distinct autosomal dominant entity presenting with upper limb defects and cardiac abnormality. No correlation between the severity of the heart and the limb defects has been established. Here we report variable clinical expression of Holt-Oram syndrome in three generations. The grandfather presented with typical upper limb defects: phocomelia of arms with three digits on each hand, congenital heart defect and narrow shoulders. His son manifested cardiac conduction disturbance with no congenital heart or skeletal defect. The granddaughter showed ventricular septal defect and moderate radial deviations of both hands with no obvious hypoplasia of the extremities. Clinical data of the presented family suggests lack of penetrance with respect to skeletal and structural cardiac abnormalities in the Holt-Oram syndrome.- - - - - - - - - - ranking = 1keywords = de (Clic here for more details about this article) |
2/1540. Unusual evidence of myocardial involvement during a hypersensitivity reaction to oral penicillin.A hypersensitivity reaction to orally administered phenoxymethyl penicillin is reported. The manifestations of the reaction included fever, arthralgia, urticaria and an irregular pulse. Serial ECG showed second-degree atrioventricular block with junctional escape beats, an atypical Wenckebach pattern and, finally, first-degree atrioventricular block with gradually decreasing PR intervals. A normal tracing was recorded on the sixth day despite the persistence of the rash and joint pains.- - - - - - - - - - ranking = 1.1111111111111keywords = de (Clic here for more details about this article) |
3/1540. Junctional ectopic tachycardia evolving into complete heart block.Transition from congenital junctional ectopic tachycardia to complete AV block was observed in an 8 month old girl, over a 36 hour period, during initial hospital admission. Two years later she had evidence of a rapidly increasing left ventricular end diastolic diameter, associated with lowest heart rates during sleep of < 30 beats/min. A transvenous permanent pacemaker was therefore implanted. This finding supports the idea that a pathological process in the area of the AV junction, initially presenting as junctional ectopic tachycardia may later extend to sudden complete atrioventricular block.- - - - - - - - - - ranking = 0.33333333333333keywords = de (Clic here for more details about this article) |
4/1540. atrioventricular block occurring several months after radiofrequency ablation for the treatment of atrioventricular nodal re-entrant tachycardia.Atrioventricular (AV) block following radiofrequency (RF) ablation for the treatment of AV nodal re-entrant tachycardia (AVNRT) is a rare but well recognised complication of the procedure--the reported incidence ranges from 1% to 21%. Almost all cases of AV block occur during or shortly after the procedure, are transient, and recover quickly. Two patients (a 22 year man and a 72 year old woman) with symptomatic AV block occurring several months after slow pathway RF ablation, requiring permanent pacemaker implantation, are described. Both patients had had several 24 hour Holter recordings before the procedure, and in neither case was there any evidence of intermittent or persistent AV block. This is a rare complication with no definitive predictors; however, all efforts should be made to exclude AV block in patients presenting with suggestive symptoms following RF ablation. With the wide use of RF ablation for the treatment of AVNRT, more cases are likely to occur. A registry should allow documentation of the incidence of this complication.- - - - - - - - - - ranking = 0.88888888888889keywords = de (Clic here for more details about this article) |
5/1540. Pacemaker therapy in a pediatric patient with hypertrophic obstructive cardiomyopathy and rapid intrinsic atrioventricular conduction.A 13-year-old boy with hypertrophic obstructive cardiomyopathy was treated with dual-chamber pacing after severe progression of left ventricular outflow tract obstruction and of clinical symptoms despite drug therapy. Rapid intrinsic atrioventricular conduction was overcome and complete preexcitation of the septum achieved by omitting atrial sensing and programming constant atrial pacing with a short atrioventricular delay of 70 msec. After 8 weeks of therapy, a reduction of the left ventricular outflow tract gradient from 125 to 16 mmHg and remodeling of the left ventricle were demonstrated.- - - - - - - - - - ranking = 0.44444444444444keywords = de (Clic here for more details about this article) |
6/1540. Reversion to sinus rhythm 11 years after surgically induced heart block.A patient is presented in whom the heart reverted spontaneously to sinus rhythm 11 years after surgical closure of a ventricular septal defect complicated by complete heart block. It seems unlikely that regeneration of fibres in the bundle of his, if these had indeed been destroyed, could account for the restoration of sinus rhythm after so long an interval.- - - - - - - - - - ranking = 0.33333333333333keywords = de (Clic here for more details about this article) |
7/1540. Pseudo second degree atrioventricular block with bradycardia. Successful treatment with quinidine.Pseudo second degree atrioventricular block resulting from blocked His premature beats was successfully treated with quinidine. The diagnosis was proved by His bundle electrogam which showed both blocked and conducted His premature beats. The blocked His prematures produced second degree atrioventricular block by making the atrioventricular junction refractory. quinidine abolished both conducted and blocked His extrasystoles. There has been no recurrence of arrhythmia during a one-year follow-up.- - - - - - - - - - ranking = 0.66666666666667keywords = de (Clic here for more details about this article) |
8/1540. Recurrent miscarriage, congenital heart block and systemic lupus erythematosus.We report the obstetric history of a woman, who between 15 spontaneous abortions, gave birth to a child with congenital heart block. She later developed systemic lupus erythematosus, had antibodies to SS-A/Ro and SS-B/La but was repeatedly negative for antiphospholipid antibodies.- - - - - - - - - - ranking = 0.11111111111111keywords = de (Clic here for more details about this article) |
9/1540. Single-lead VDD pacemaker implantation via persistent left superior vena cava: an improved technique and a new modality.Persistent left superior vena cava (PLSVC), which occurs in approximately 0.5% of the general population, may complicate pacemaker implantation by making lead insertion into the right ventricle more difficult and increasing lead instability when the transvenous approach is attempted. We describe our experience with four PLSVC patients with pacemakers. We developed an open J-loop technique in which the stylet tip is directed toward the orifice of the tricuspid valve anteroinferiorly. The lead was implanted into the right ventricular apex without difficulty. Three of our patients had high-degree atrioventricular block and received VVI pacemakers with the new technique. One patient with complete atrioventricular block received a single-lead VDD pacemaker by means of the same technique, with the paired electrodes positioned in the lower right atrium. Excellent results were obtained on exercise tolerance testing, 24-hour Holter monitoring, and echocardiographically determined systolic and diastolic function. This improved technique can simplify pacemaker implantation in patients with PLSVC. The VDD device is a new way to maintain systolic and diastolic function and is an appropriate option in patients with high-degree atrioventricular block and PLSVC who require pacemaker implantation.- - - - - - - - - - ranking = 0.77777777777778keywords = de (Clic here for more details about this article) |
10/1540. adenosine-induced cardiac pause for endovascular embolization of cerebral arteriovenous malformations: technical case report.OBJECTIVE: Extremely high flow through arteriovenous malformations (AVMs) may limit the safety and effectiveness of endovascular glue therapy. To achieve a more controlled deposition of glue, we used transient but profound systemic hypotension afforded by an intravenously administered bolus of adenosine to induce rapidly reversible high-degree atrioventricular block. methods AND CASE REPORT: A patient with a large high-flow occipital AVM fed primarily by the posterior cerebral artery underwent n-butyl cyanoacrylate glue embolization. nitroprusside-induced systemic hypotension did not adequately reduce flow through the nidus, as determined by contrast injection in the feeding artery. In a dose-escalation fashion, boluses of adenosine were administered to optimize the dose and verify that there was no flow reversal in the AVM and no other unexpected hemodynamic abnormalities by arterial pressure measurements and transcranial Doppler monitoring of the posterior cerebral artery feeding the AVM. Thereafter, 64 mg of adenosine was rapidly injected as a bolus to provide 10 to 15 seconds of systemic hypotension (approximately 20 mm Hg). Although there were conducted beats and some residual forward flow through the AVM during this time, the mean systemic and feeding artery pressures were roughly similar and remained relatively constant. A slow controlled injection of n-butyl cyanoacrylate glue was then performed, with excellent filling of the nidus. CONCLUSION: adenosine-induced cardiac pause may be a viable method of partial flow arrest in the treatment of cerebral AVMs. Safe, deep, and complete embolization with a permanent agent may increase the likelihood of endovascular therapy's being curative or may further improve the safety of microsurgical resection.- - - - - - - - - - ranking = 1.8888888888889keywords = de (Clic here for more details about this article) |
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