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

Mid-term results of the lateral tunnel operation at Queen Alia heart institute

To determine midterm results of univentricular repair using intra-atrial lateral tunnel at Queen Alia Heart Institute. Between January 1999 and January 2005, 159 patients [99 males, 60 females] underwent either a fenestrated or non-fenestrated lateral tunnel [Fontan procedure], for a wide range of complex congenital heart disease with a functional single ventricle at Queen Alia Heart Institute. Median age at operation time was 3.8 years [range 1.5-17 years]. Multiple factors were analyzed including: anatomical variations, mean right atrial pressure, pulmonary artery pressure, ventricular end diastolic pressure, aortic saturation, cardiopulmonary bypass time and ischemic time, presence of arrhythmias, pacemaker insertion, thromboembolic complications, early and late mortality. The major anatomic diagnosis was double inlet left ventricle in 72 patients [45.3%] and the least was unbalanced complete atrioventricular septal defect among 8 patients [5%]. Dextrocardia was found in 17 patients. The mean right atrial pressure was 8 +/- 3 mmHg, pulmonary artery pressure 13 +/- 2.6 mmHg, PVR 1.9 +/- 2 Woodunits.m2, ventricular end diastolic pressure 14 +/- 2.2 mmHg, aortic saturation 83 +/- 4%, cardiopulmonary bypass time 121 +/- 9 minutes, ischemic time 47 +/- 3 minutes. Early postoperative supraventricular tachyarrhythmia [SVT] occurred among 10 cases [6.3%] of patients, in whom four needed antiarrhythmic drugs before discharge. Early bradyarrhythmia needing pacemaker in 2 patients [1.26%], whereas 6 patients needed pacemaker at mid term follow-up. There were 10 [6.3%] early deaths and no late deaths occurred after 6 years of follow-up. Seven [4.7%] patients developed protein loosing enteropathy as a late complication and 7 more patients [4.7%] developed neurological complications. Proper selection of patients for the lateral tunnel Fontan procedure results in excellent early and mid-term survival and functional outcome with low incidence of complications

Transcatheter closure of patent ductus arteriosus using the amplatzer duct occluder at Queen Alia Heart Institute: Medium-term follow-up

Transcatheter closure of patent ductus arteriosus is a well-established procedure. The aim of this study was to assess the medium term results of patent ductus arteriosus closure using the Amplatzer Duct Occluder. From January 1998 to January 2005, 204 cases [77 males and 127 females] underwent an attempt of transcatheter closure of their patent ductus arteriosus at Queen Alia Heart Institute using the Amplatzer Duct Occluder[Tm] device. Their median age was 3.5 years [range 0.8-13 years], their median weight 14kg [range 6-32kg], their mean Qp/Qs was 2.3 +/- 0.6, their mean systolic pulmonary artery pressure was 38.44 +/- 7mmHg. The mean narrowest diameter of the pulmonary end of the patent ductus arteriosus angiographically was 4.2 +/- 0.8mm [range 3-8mm]. The devices used were [6-4, 8-6, 10-8 and 12-10mm] delivered antegrade via 5-7 French sheaths. All patients had chest X-ray and color flow echocardiographic follow-up at 24 hours, one, three, six months and yearly thereafter. There was immediate and complete closure of the ductus in 180 [88.24%] of cases. The remaining 24 [11.76%] patients had a trivial residual shunt through the device mesh. Follow-up color flow Doppler echocardiography revealed complete closure of patent ductus arteriosus in 96% of cases at 24 hours, and complete closure at one month follow-up in 100% of cases. One patient developed aortic obstruction where the duct joined the aorta at a more acute angle, which was retrieved surgically. Otherwise no other complications were reported. Neither thromboembolization nor hemolysis or recanalization of the ductus was reported. Furthermore, chest radiographs and Doppler echocardiography follow up revealed no evidence of wire fracture or device disruption or any episodes of infective endocarditis. Since the initial clinical experience in 1998, the transcatheter closure of patent ductus arteriosus using the Amplatzer Duct Occluder has proven to be an easy procedure that could be mastered quickly but with caution and at the same time it is an effective procedure that has almost replaced surgery in our center. Longer follow up will be needed to precisely define the safety and indications of this device