1/752. 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 = heart (Clic here for more details about this article) |
2/752. 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 = 1.6666666666667keywords = heart (Clic here for more details about this article) |
3/752. 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 = 2keywords = heart (Clic here for more details about this article) |
4/752. 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 = 1.6666666666667keywords = heart (Clic here for more details about this article) |
5/752. Complete atrioventricular block during anesthesia.PURPOSE: To describe a case of asymptomatic first degree atrioventricular block with a bifascicular block that progressed to complete atrioventricular block during anesthesia. This potentially fatal block was successfully treated with transesophageal ventricular pacing. CLINICAL FEATURES: A 67-yr-old man was scheduled for microvascular decompression of the right trigeminal nerve under general anesthesia. His preoperative ECG showed first degree atrioventricular block with complete right bundle branch block and left anterior hemiblock, but he had experienced no cardiovascular symptoms. anesthesia was induced with sevoflurane 5%, and maintained with isoflurane 1.5-2% in oxygen. Fifteen minutes later in the left lateral decubitus position, the systolic arterial blood pressure suddenly decreased from 80 mmHg to 0 mmHg. Then, the ECG abruptly changed from sinus rhythm to complete atrioventricular block. The heart was unresponsive to drug therapy such as atropine 1.3 mg and isoproterenol 0.5 mg, or transcutaneous pacing but transesophageal pacing was successful. CONCLUSION: Asymptomatic first degree atrioventricular block with bifascicular block advanced to complete atrioventricular block during anesthesia. The block was successfully managed with transesophageal pacing.- - - - - - - - - - ranking = 0.33333333333333keywords = heart (Clic here for more details about this article) |
6/752. bacillus popilliae endocarditis with prolonged complete heart block.bacillus popilliae, a fastidious, aerobic, gram-positive, spore-forming bacillus, has never been reported as a pathogen in human infectious diseases. We report the first case of a human infected by the pathogen B. popilliae, which presented as endocarditis involving the bicuspid aortic valve and complicated with prolonged (> 30 days; to our knowledge, the longest in the literature) complete heart block. Although surgery may be warranted by previous reports, the patient was successfully managed by medical treatment instead, because of the absence of evidence from various approaches that support the existence of perivalvular extension of infection.- - - - - - - - - - ranking = 1.6666666666667keywords = heart (Clic here for more details about this article) |
7/752. Radiofrequency catheter ablation of atrial flutter after orthotopic heart transplantation: insights into the redefined critical isthmus.We report a case of successful radiofrequency catheter ablation of recurrent atrial flutter in a heart transplant recipient and discuss technical aspects of the procedure. A counterclockwise flutter circuit was defined during endocardial mapping of the donor atrium. Termination of atrial flutter was achieved by creating lines of radiofrequency lesions from the tricuspid ring to the suture line between donor and recipient atria. Creation of bidirectional conduction block in the tricuspid ring-suture line isthmus resulted in abolition of atrial flutter.- - - - - - - - - - ranking = 1.6666666666667keywords = heart (Clic here for more details about this article) |
8/752. In utero treatment of fetal complete heart block with terbutaline. A case report.BACKGROUND: Isolated fetal complete heart block is an uncommon finding, with a mortality rate of 20-30%. Various treatment modalities have been reported, with no consistent success. CASE: Fetal complete heart bock was diagnosed in a 30-year-old woman at 29 weeks' gestation. She had an elevated antinuclear antibody and anti-SSA antibody titer. Fetal cardiac decompensation was detected at 32 weeks. After a trial of intravenous isoproterenol without significant side effects, she was treated with oral terbutaline. An increase in the fetal ventricular rate and complete resolution of hydrops fetalis occurred. A male infant was delivered by cesarean section, at term, and underwent cardiac pacemaker implantation at 4 days of age. CONCLUSION: The treatment of fetal complete heart block is controversial. Premature delivery with cardiac pacing is associated with high morbidity and mortality. We report prenatal treatment with oral sympathomimetic medication, which allowed delivery to be delayed until term, with a successful outcome.- - - - - - - - - - ranking = 2.3333333333333keywords = heart (Clic here for more details about this article) |
9/752. role of fetal echocardiography in the management of isolated fetal heart block with ventricular rate <55 bpm.Persistent bradycardia is an uncommon cardiac problem in fetuses but carries a high mortality in those with a ventricular rate <55 bpm. fetal heart block is one of the most common causes of persistent fetal bradycardia (PFB). An optimal method for assessing and monitoring cardiovascular compensation in the setting of PFB due to heart block has not been fully established. We report the application of two-dimensional and Doppler echocardiography in close monitoring of cardiac function and hemodynamics in a third-trimester fetus with a ventricular rate <55 bpm due to heart block, which assisted in successful management of the pregnancy to term. Hemodynamic and cardiac adaptive changes in compromised fetuses, particularly due to heart block, are discussed.- - - - - - - - - - ranking = 2.6666666666667keywords = heart (Clic here for more details about this article) |
10/752. Perinatal monitoring of fetal well-being in the presence of congenital heart block.We present a case of congenital complete heart block associated with maternal autoantibodies in which a normal labor and delivery could safely be allowed to proceed despite the absence of the usual tool of electronic fetal heart rate monitoring for fetal distress, by the technique of rupturing membranes and using a fetal scalp electrode electrocardiographic tracing to assess the fetal atrial rate.- - - - - - - - - - ranking = 2keywords = heart (Clic here for more details about this article) |
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