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Williams-Beuren syndrome (WBS) affects young infants and children. The underlying etiopathogenesis of this rare disease is due to the mutation of the elastin gene that is responsible for the elasticity of the arterial wall. As a result of inadequate elastin production, the major systemic arteries become abnormally rigid and can be manifested by an impediment to the blood flow. The most common cardiovascular abnormalities encountered in WBS are supravalvular aortic stenosis, pulmonary arterial stenosis, and mitral valve prolapse. Less frequently observed cardiovascular abnormalities include coarctation of the aorta, ventricular septal defect, patent ductus, subaortic stenosis, and hypertrophic cardiomyopathy. Coronary artery stenosis and severe impediment to the bi-ventricular outflow as a result of supravalvular aortic and pulmonary artery stenosis predispose patients to sudden death. Patients with progressed arterial stenosis and severe stenosis are likely to require intervention to prevent serious complications. Rarely, imaging findings may precede clinical presentation, which allows the radiologist to participate in the patient care. However, to be more prudent, the radiologist must be accustomed to the imaging characteristics of WBS as well as the patient's clinical information, which could raise the suspicion of WBS. We performed a retrospective analysis of all the available images from patients diagnosed with WBS in last 4 years at our institution, and present key imaging findings along with a review of the literature to summarize the clinically relevant features as demonstrated by multidetector computed tomography in WBS. Cross-sectional imaging plays a vital role in the diagnosis of WBS cases with equivocal clinical features. MDCT evaluation of complex cardiovascular abnormalities of WBS including coronary artery disease is feasible with modern MDCT scanners and in the future, this approach could provide accurate information for planning, navigation, and noninvasive assessment of the secondary arterial changes in WBS and thus reducing the dependence upon invasive contrast catherization techniques.

Background Coarctation of aorta may present as severe heart failure in infants and may lead to myocardial dysfunction. Current evidence supports surgical management of neonatal coarctation. However, it can be precarious in critically sick infants. Aim and objective To investigate the safety, efficacy, immediate and mid-term outcome of percutaneous balloon angioplasty (BAP) for infantile aortic coarctation in critically sick patients. Methods and patients Data of all patients under age of 6 months who underwent balloon angioplasty for coarctation were reviewed. Results Between January 2008 and April 2014, 15 infants were identified with coarctation with a mean weight of 3.4 kg (1.4–5 kg) and median age of 54 days (4–142 days). All patients were critically sick, needed admission in intensive care unit for inotropic or ventilatory support. All underwent successful percutaneous balloon angioplasty with no major complications. The clinical condition and left ventricular function improved leading to discontinuation of prostaglandin and weaning from inotrope and ventilator support. Seven patients underwent elective surgical repair after a mean time of one month, three needed re-dilation and five continued without any further intervention. Conclusions Surgical repair for native neonatal and infantile coarctation is a preferred choice of treatment but it can be challenging in critically sick patients. However, balloon dilation remains a safe and effective temporary palliation for the critically sick patients. Despite of high incidence of restenosis, some patients do not need for mid-term further intervention.

Background Incidence of congenital heart disease in patients with Down syndrome (DS) is 40%. In the past some have advocated that the cardiac defects in DS should not be repaired, reports had shown outcome comparable to individuals without DS. The aim of the study is to analyze the outcome of cardiac surgery in DS. Methodology We conducted a retrospective study in the last 2 years at our institution. Patients involved were DS less than 13 years who had cardiac surgery. DS with chronic lung disease or unreactive pulmonary hypertension were excluded. We analyzed their demographic data, cardiac lesion, type of surgery, ICU stay, morbidities and mortality. Results 23 patients with DS (11 Females, 13 Males), with median age of 7 months, mean age of 32month. AVSD were ( n = 15) 65%), VSD were ( n = 5) 21%. Postoperatively, 21% were extubated on the same day. 27% had intubation more than 9 days. Almost 30% had a hospital stay of >15 days. Complications were respiratory problems in 45%, arrhythmias in 8% and residual AV valve regurgitation in 8%. Hospital mortality was ( n = 2/23) 8%. Conclusion Whether to operate Down syndrome patients or not remains unclear. Our study showed, significant postoperative complications, lengthy hospital stay and relatively high mortality. Despite of this we feel they should be given the chance of surgery to improve their life quality.

Background: Central line insertion is a routine procedure in medical practice. Dislodgement of lines into the vascular system is a rare complication. We noticed that paediatric health care providers (PHCP) contact the cardiac or general paediatric surgeon for extraction of dislodged lines more frequently than using the less invasive percutaneous approach. Aim: To study the referral preference of PHCP for patient with embolised intravascular foreign bodies. Methods: A questionnaire with three questions was distributed to PHCP of all paediatric subspecialties, including surgery, in two tertiary care centres. The questions were about the total number of patients seen with central line, experience with complications, and preferred specialty for removal of dislodged central lines. Results: The questionnaire was distributed to 128 professionals. The response rate was 79% (n=101). Incomplete answers (n=14) were excluded. The grades of responders were senior consultants 18%, junior consultants 38%, and residents 43%. Thirty nine percent of care providers experienced dislodgement or fragmentation of central lines. The majority (82%) prefer to refer the patients for surgical removal. Conclusions: Most PHCP in the selected hospitals prefer to refer patients with embolised foreign bodies in the vascular system for surgical removal. The local health policy should be updated for the use of the alternative percutaneous approach. Keywords: cardiac surgery; clinical practice; transcatheter retrieval (Published: 5 March 2013) Citation: Libyan J Med 2013, 8 : 20495 - http://dx.doi.org/10.3402/ljm.v8i0.20495

Congenital coronary artery fistulas are rare anomalies. Most of these fistulas drain to the right side of the heart. They seldom connect to either left atrium or left ventricle. We report a three years old girl in whom an atrial septal defect (ASD) was diagnosed. Echocardiography suggested a possible additional coronary artery fistula to the left ventricle (LV). Before closing the ASD, coronary angiography identified multiple coronary artery fistulas to the left ventricle. Transcatheter closure of the ASD was done successfully. The hemodynamic effect of coronary artery fistula to LV after ASD closure is discussed along with literature review of this extremely rare association.

An 18-year-old pregnant woman with severe pulmonary valve stenosis and exertional dyspnea underwent balloon dilation during pregnancy using sole echocardiographic guidance to protect the baby from radiation. The main technical difficulty encountered was during advancement of the catheter across the right ventricular outflow tract into the pulmonary valve. This was overcome using a wedge balloon catheter over a percutaneous transluminal coronary angioplasty (PTCA) wire. Using echo guidance, the balloon was positioned across the pulmonary valve and inflated. Pulmonary balloon valvuloplasty can be performed safely using sole transthoracic echocardiography guidance without fluoroscopy.

Northwest Armed Forces Hospital Tabuk, Saudi Arabia We present a case of right sided diphragmatic hernia in a three years old girl.The child had a Ventricular Septal defect (VSD) closure via median sternotomy one year ago. The child also had a permanent pacemaker implanted for heart block after VSD closure on bypass. She presented one year later with some shortness of breath. Chest X-ray at that stage was interpreted as ‘‘eventeration’’ of left Hemidiaphragm. In the subsequent months she was readmitted with respiratory symptoms.Herniation of gut loops in left pleural cavity were confirmed on X-rays, and CT with Contrast. The child under went successful repair of the diaphragmatic hernia via laparotomy. Review of literature showed two previous case reports of Iatrogenic Diaphragmatic Hernia in children after cardiac operations via median sternotomy. Conclusion: diaphragmatic hernia should be kept in mind while interpreting unusual looking chest xrays in children atier open heart surgery. doi:10.1016/j.jsha.2011.02.077 SHA 077. Effect of age of repair of Tetralogy of Fallot on the short term outcome Sameh Rabie Ismail, Mohamed S. Kabbani, MD, Hani K. Najm, MD, Riyadh M. Abu-sulaiman Department of Cardiac Sciences (MC: 1420), King Abdulaziz Medical City, National Guard Hospital, P.O. Box 22490, Riyadh 11426, Saudi Arabia E-mail address: samis93@gmail.com (S.R. Ismail) Introduction: All patients with Tetralogy of Fallot (TOF) require surgical intervension. Significant advances in the perioperative care, anaesthesia, and cardiopulmonary bypass techniques have spurred earlier correction. Aim of the study: To study the effect of age of repair on the early post operative course and outcome of children undergoing TOF repair. Method: A retrospective study of all cases of TOF repair in King Abdulaziz Cardiac Center between March 2002 and December 2007 was conducted. We excluded cases with pulmonary atresia and neonatal repair. Cases were divided into three groups. Group A included cases operated at the age below 6 months, group B included cases operated between 6 and 12 months, while group C included cases operated above 12 months. We compared demographic, risk category, ICU parameters, ECHO data and short-term outcome of both groups. Results: 83 patients fulfilled the study criteria. We identified 24 Cases (29%) in-group A, 32 cases (39%) in group B,and 27 cases (33%) in group C. There was a tendency toward having a smoother course post operatively in the group B which was operated between 6 and 12 months, but We did not appreciate any statistically significant effect for age on the early post operative outcome of Tetralogy of Fallot repair. Conclusion: In general, children undergoing TOF repair had excellent short-term outcome. Contrary to some previous reports, in our series of TOF repair there was no effect for the age of repair on the early post operative outcome. doi:10.1016/j.jsha.2011.02.078 SHA 078. Chylothorax after pediatric cardiovascular surgery Sameh Rabie Ismail, Mohamed S. Kabbani, MD, Hani K. Najm, MD, Ghassan A. Shaath, MD, Abdulraouf M.Z. Jijeh, MD National Guard Hospital, Cardiac Intensive Care, Riyadh, Saudi Arabia E-mail address: samis93@gmail.com (S.R. Ismail) Background: Chylothorax is the accumulation of chyle in the pleural cavity, usually develops after disruption of the thoracic duct along its intra-thoracic route. In the majority of cases this rupture is secondary to trauma (including cardio thoracic surgeries). Chylothorax is a potentially serious complication after cardiovascular surgeries that require early diagnosis and adequate management. Methods: A retrospective study of all cases complicated with chylothorax after pediatric cardiac surgery in King Abdulaziz Cardiac Center between January 2007 and December 2009 was conducted. Chylothorax was certainly suspected if milky fluid drainage from the pleura in the post-operative period, it is also suspected if excessive drainage of non-milky fluid, and in all cases the diagnosis was confirmed by laboratory analysis of the pleural fluid. The study aim to determine the risk factors, the impact on Abstract for SHA22 193