Initial experience of atrial septal defect closure with occlutech figulla occluder device at queen alia heart institute

To evaluate the effectiveness of the Occlutech Figulla occluder device in the treatment of ostium secundum atrial septal defect. This is a retrospective review which was conducted between June 2008-June 2011 involving 54 cases with ostium secundum atrial septal defect who underwent transcatheter closure using the Occlutech Figulla device at Queen Alia Heart Institute. All procedures were performed under general anaesthesia with continuous Transesophageal Echocardiographic monitoring. Clinical and echocardiographic assessments were performed after 24 hours and then after one, three and six months respectively. The results are presented as means, standard deviations and percentages. From the 54 cases, 33 patients [61%] were females. Their mean age and weight were 16.47 +/- 11.8 year, 38.4 +/- 20.7kg respectively. The mean atrial septal defect diameter by Transesophageal Echocardiogram was 13.2 +/- 4.2mm, and the mean atrial septal defect size of the implanted device was 15.97 +/- 4.5mm, ranging from 10.5 to 27mm. Two cases were sent for surgery due to inadequate postero-inferior rim. The procedure success rate was 96.3%. In five cases [9.6%] there was trivial residual shunt after 24 hours of the procedure which had disappeared at follow-up. The Occlutech Figulla occluder device is a safe and effective for transcatheter closure of secundum atrial septal defect. Longer term follow-up studies to evaluate the safety of the device are needed

Jervell and Lange-Nielsen syndrome in four Jordanian families

To report the importance of the cardiologic aspect in the evaluation of syncope in children with congenital deafness. A total of 16 deaf-mute siblings in four Jordanian families were studied. Each family had at least one sibling with congenital deafness and syncopal attacks. Audiogram confirmed the profound sensorineural hearing loss in all the siblings. Electrocardiogram was done in all symptomatic siblings and corrected QT interval was calculated. Screening for corrected QT interval prolongation was performed in the asymptomatic siblings and parents. Eight siblings had abnormal prolongation of corrected QT interval and were diagnosed as Jervel-Lange-Nielsen syndrome [congenital sensorineural deafness and abnormal prolongation of corrected QT interval]. The syncope in all symptomatic siblings was controlled with the use of propranolol while the asymptomatic siblings were given propranolol for prophylaxis. Electrocardiogram is an important diagnostic test in congenitally deaf children, especially those with syncopal episodes