Search

Introduction:The incidence of prosthetic valve implantation is increasing in the paediatric population. Prosthetic valve thrombosis leading to obstruction could potentially be a life-threatening complication. There is a debate regarding optimal management of this complication, and there is limited use of thrombolytic therapy in childhood in the setting of valve thrombosis.Objective:We aim to share our experience of successfully using fibrinolytic therapy in terms of alteplase for paediatric prosthetic mitral valve thrombosis and to propose a management algorithm.Methods:This retrospective analysis of the database was conducted at our hospital including patients who underwent thrombolysis (alteplase) for prosthetic mitral valve thrombosis from June, 2011 to June, 2021. A total of 10 patients with 20 attempts of alteplase infusion were found in our record.Results:Alteplase was successful in 19 attempts to relieve valve thrombosis. The safe and effective dose of alteplase was between 0.1 and 0.3 mg/kg/hour. There were no associated major bleeding complications and alteplase was administered either by central or peripheral line.Conclusion:Thrombolysis by alteplase infusion was found to be successful in relief of prosthetic mitral valve thrombosis in paediatric population without major bleeding complications.

Background: ATP6V1B2 gene mutation is associated with Zimmermann-Laband syndrome 2 (ZLS2), which is a rare developmental disorder characterized by nail hypoplasia and hereditary deafness. Case Presentation: We report a new phenotypic mutation of ATP6V1B2 associated with ZLS 2. The patient has atresia of the left pulmonary artery (LPA) and features of hearing loss and nail hypoplasia. The other interesting part is that the child had two types of mutations inherited from father and mother. He is carrier for GJB2 mutation (inherited from father) and diseased with ATP6V1B2 mutation (inherited from mother). Conclusion: The association of ZLS features with absent LPA was not reported previously in the literature. This finding will add new information to the database of previously reported ATP6V1B2 rare mutations.

Aims: To understand the accuracy of non-invasively obtained blood pressure gradients (cuff vs Doppler gradient) with an invasively measured pressure gradient. Study Design: Retrospective study. Place and Duration of Study: Department of Pediatrics, Section of Pediatric Cardiology, King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia, between Jan, 2010 till Jan, 2020. Methodology: A retrospective study of patients with CoA who underwent cardiac catheterization between Jan, 2010 till Jan, 2020 was performed. Cuff BP gradients and Doppler echocardiography were measured prior to cardiac catheterization and afterwards, when an intervention was performed. Student t test and Bland-Altman analysis were performed. Results: 55 patients with aortic coarctation underwent 92 cardiac catheterizations. Among them 75 needed interventions. This resulted in a total of 162 cardiac catheterizations (cath), from them 67 pressure gradients included in the analysis. There was no statistically significant difference between mean Doppler measurements and invasively derived catheter gradients (p=0.12). In contrast peak Doppler measurement (p < 0.00001) and cuff blood pressure gradients (p=0.03) showed significant differences to the cath gradient. We found that cuff blood pressure gradients accurately reflected cath measurement in native COA (p=0.40) and in those who weigh less than 10 kg (p=0.67). Mean Doppler measurements had a small tendency for underestimation. Peak Doppler gradient and cuff pressure gradient usually overestimated cath measurement. Conclusion: The mean Doppler echocardiography seems to be the most accurate among the other noninvasive methods in use to estimate severity of aortic coarctation. It provided reasonable agreement with the invasively obtained aortic coarctation gradient.

Background Assessment of the shape and dimensions of PDA is usually done angiographically and in the majority of cases need arterial access. Our aim was to evaluate the value of routine 2 D echocardiography (ECHO) in predicting type, dimensions of PDA and to anticipate device size to be used during the intervention. Material and methods The charts of all patients who underwent transcatheter closure of PDA between January 2015 and December 2020 were reviewed. Their pre-procedure ECHO and catheterization details at the time of device closure were analyzed. Results Total of 139 patients were reviewed and 8 were excluded because of lack of adequate echocardiographic or angiographic images. The mean age and weight of the study population were 2.6 ± 2.5 years (range 0.2–14 years) and 11.2 ± 7.8 kg (range: 1.5–57 kg), respectively. There was no statistically significant difference in PDA narrowest diameter (p = 0.99) and predicted device type (p = 0.54) between Echo and angiography. Echo slightly overestimated PDA length (p = 0.01) and aortic ampulla dimension (p = 0.047), while morphology of PDA was correctly identified in the majority of cases (82%). Conclusions Pre-procedure echocardiography correlates well with angiographically obtained measurements and hence can be used to estimate PDA diameter, shape and guide device decide selection. Routine echocardiography can be used successfully to plan the intervention and in some cases to guide transcatheter closure.

Background: Transcatheter closure of secundum atrial septal defect is routinely performed under general anesthesia and transesophageal echocardiography guidance. If patients have good echo windows, the procedure could be performed under transthoracic echo guidance. Aim of study: To evaluate safety and efficacy of the intervention using fluoroscopy and echo guidance. Methods: In a case control study design, 180 patients underwent atrial septal defect closure between January 2010 and December 2016. In 32 patients, the intervention was performed under fluoroscopy and transthoracic echo guidance. Our study group consisted of 22 out of 32 patients (

Background Dilated cardiomyopathy (DCM) is characterized by left ventricular dilation and dysfunction. The association with significant mitral regurgitation worsens the prognosis. Case report A 2-year-old girl presented with DCM and severe mitral regurgitation. She had a history of viral myocarditis at the age of 4 months, necessitating recurrent hospital admissions for management of intractable heart failure, pneumonia, and failure to thrive. The decision was taken to proceed for mitral valve surgery, which ended with mitral valve replacement. Over 3 years of follow-up after surgery, there was significant improvement in her weight gain and she improved clinically. There were still recurrent admissions, but mostly for adjustment of her deranged anticoagulation medications. Conclusion Mitral valve surgery might be indicated in selected patients with DCM.

Management of neonatal native coarctation is debated till now. Surgical therapy remains an option but may be unwarranted in critically sick infants with complex lesions. Balloon dilatation has been employed but with early re-stenosis. Stent angioplasty has also been used but as a bridge towards definitive surgical therapy. Four critically sick infants with complex coarctation and additional co-morbidity factors underwent primary stent therapy as surgical intervention was denied. One patient had died earlier due to reasons unrelated to the procedure. Three survivors underwent multiple dilatations of primary stents as indicated. One of the three survivors did not require any further dilatation after the age of 5 years and remained stable till the time of reporting. High-pressure Cheatham Platinum stents were implanted inside the primary stents in two infants, who developed re-stenosis due to somatic growth. These stents were further balloon dilated at high atmospheric pressure. Femoral arteries in both of them were blocked but were re-canalized after balloon dilatation in one and stent angioplasty in the other. After a follow-up of about 15 years, all of them have been doing fine with acceptable Doppler gradients. They were normotensive and on no cardiac medications. It can be concluded that, though surgical repair remains a standard of care, stent angioplasty in selected infants with complex lesions is feasible and effective. Multiple dilatations can be performed without added risk of stent migration. Bio-absorbable and growth stents hold a promise for future use in such situations.

IntroductionNeonates with severe pulmonary valve stenosis tend to remain oxygen dependent, despite resolution of the transpulmonary gradient. Alpha 2 blockers – phentolamine – and angiotensin-converting enzyme inhibitors – captopril – were reported to improve oxygen saturation.ObjectiveTo describe the role of phentolamine and captopril in the treatment of these patients.MethodsIn a retrospective cohort study, 28 neonates with severe pulmonary valve stenosis underwent balloon valvuloplasty. Among them, 20 remained oxygen or prostaglandin dependent after intervention, and were treated with phentolamine or captopril. Oxygen saturation was monitored before and after intervention and following treatment with these medications. Mean duration of hospitalisation was recorded.ResultsMean age and weight were 25.2 days and 3.1 kg, respectively. Before balloon dilation, 18/20 (90%) neonates were on prostaglandin, whereas after the procedure only 6/18 patients required it. All 20 patients required oxygen after the procedure, and nine patients (45%) were started on phentolamine. Among them, one patient with severe infundibular stenosis did not respond favourably, and 11 patients (55%) were started on captopril. After starting phentolamine or captopril treatment, prostaglandin could be discontinued after a mean time of 15.86 hours. Within ConclusionPhentolamine and captopril seem to have therapeutic roles in neonates with severe pulmonary valve stenosis who remain oxygen dependent after balloon dilation. Both drugs led to vasodilation of the pulmonary and systemic vascularisation and facilitated inflow to the right ventricle. Right-to-left shunt across a patent foramen ovale or atrial septal defect decreased and saturation improved, leading to a significant reduction in the length of hospitalisation.

Ventricular septal defect (VSD) is a life-threatening complication of transmural myocardial infarction. Urgent surgical repair and concomitant revascularization are the standard of care. Percutaneous catheter- based closure techniques have been reserved for patients with a high-risk surgery or a failed surgical procedure with residual shunting. This case report demonstrates the successful transcatheter closure of residual VSD using the Amplatzer muscular VSD device (Amplatzer, Minnesota, USA) after surgical patch dehiscence for postinfarction VSD and 3-and-a-half years' post-intervention follow-up.

We describe the technique of closure of native right ventricular outflow tract by Amplatzer muscular ventricular septal defect device because of severe regurgitation in a patient who had tetralogy of Fallot repair with conduit at 3 years of age followed by percutaneous Melody valve implant 6 years later.

Geleophysic dysplasia is an extremely rare acromelic skeletal dysplasia resembling lysosomal storage disease. It is characterised by characteristic facial phenotype, short stature, micromelia, joint contracture, and early cardiac valvular involvement. It has been described worldwide in

Percutaneous closure of secundum atrial septal defect associated with situs solitus and dextrocardia has not been reported previously. We describe the technical difficulties encountered during transcatheter closure of a secundum atrial septal defect in a 19-month-old girl with situs solitus and dextrocardia.

Percutaneous closure of secundum atrial septal defect (ASD II) is considered the treatment of choice in the majority of cases. Interrupted inferior vena cava with azygos continuation can make delivery of the occluder difficult or not possible. Transjugular, transhepatic approach or surgery can be the alternative. We present the case of a 53-year-old woman with ASD II and interrupted inferior vena cava, and describe successful atrial septal defect closure under transesophageal echocardiography guidance through transfemoral approach using a modification of the standard technique.

Background Neonates with critical pulmonary valve stenosis (PVS) tend to remain oxygen dependentpost successful balloon valvuloplasty. Phentolamine and captopril were observed by us to improve oxygen saturation. Objective Describe the role of phentolamine and captopril in the treatment of these category of patients. Methods In a retrospective cohort study, 28 neonates with critical PVS underwent balloon valvuloplasty. Twenty remained oxygen or/prostaglandin (PGE)-dependent post-interventionand were treated with phentolamine or captopril accordingly. Oxygen saturation was monitored pre and post intervention and following treatment. Results Mean age and weight was 25.2 days and 3.1 kg. Before balloon dilation 18/20 (90%) neonates were on prostaglandin while after the procedure only 6/18 patients required it. A total of 20 patients required oxygen after intervention. Nine patients (45%) werestarted on phentolamine. One with severe infundibular stenosis did not respondfavorably. Eleven patients (55%) were started directly on captopril. After introducing phentolamine or captopril, PGE could be discontinued after a mean time of 15.9 h and mean oxygen saturation increased from 76.7% to 93.0%. Conclusion Phentolamine and/or Captopril have a therapeutic role in neonates with critical PVS who remain oxygen dependent after balloon dilation. Both medicationslead to vasodilatation of pulmonary and systemic vascularity. They facilitate inflowto the right ventricle. Right to left shunt across a PFO or/ ASD minimizesand saturation improves leading to a significantreduction in length of hospitalization.

Infants with post-tricuspid valve shunts (PTS) may benefit from interatrial communication (IAC). The effect of IAC on left ventricular (LV) performance in these patients was studied. IAC was documented prospectively in 55 patients with PTS. Clinical status, echocardiographic dimensions of LV, mitral inflow Doppler, tissue Doppler velocities and time intervals were measured. Creatinine kinase (CK), CKMB, troponin-I and NT pro-brain natriuretic peptide (NT pro-BNP) were measured. Patients were divided into four groups: (A) PTS but no IAC (n = 32); (B) PTS and IAC (n = 23); (C) VSD but no IAC (n = 16); and (D) VSD and IAC (n = 19). Group A had more frequent mitral regurgitation (p = 0.041), larger mitral annulus (1.80 vs. 1.30 cm, p < 0.0001) and larger LV systolic and diastolic dimensions (2.01 vs. 1.40 and 3.28 vs. 2.35 cm, p < 0.001) than group B. The E-wave deceleration time tended to be longer in group A (121.0 vs. 106.8 ms, p = 0.06). By tissue Doppler, group A had E'- and S-waves significantly taller (15.51 vs. 13.14 and 7.69 vs. 6.72 cm, p = 0.04 and p = 0.005, respectively) than group B. Also, NT pro-BNP was significantly higher in group A (1116.15 vs. 458.73 pg/ml, p = 0.028). Group C had significant larger mitral z-score values (1.2 vs. 0.01, p < 0.001), larger LV diameter z-score (p = 0.001) and higher NT pro-BNP level (1477.37 vs. 451.66 pg/ml, p = 0.001) than group D. There was no significant difference in the clinical status between the groups. In children with PTS, the presence of IAC could be beneficial. Their echocardiographic parameters and biomarker show better systolic and diastolic LV performance.

Background Recanalization of arterial duct (AD) is rarely needed. Objective The aim of this study is to report our experience regarding the feasibility and effectiveness of arterial duct recanalization in three infants and review the relevant literature. Methods and results We report on three patients with decreased pulmonary blood flow after initial palliation. The first patient had pulmonary atresia (PA) and an intact ventricular septum. He needed recanalization of the AD after pulmonary valve perforation and dilatation. The second patient had PA and ventricular septal defect (VSD). His AD originated from the left subclavian artery. He required AD recanalization after palliation with a central shunt and clipping of the duct. During intervention he developed a thrombus in the stent, which was treated successfully using thrombolytic treatment. The third patient had PA and VSD. The arterial duct originated from the left subclavian artery and was recanalized after spontaneous closure despite prostaglandin infusion. During the procedure the patient had severe desaturation and bradycardia requiring resuscitation for two minutes. All infants had successful arterial duct recanalization and stenting. Additionally, they were clinically stable during follow-up. Conclusion Arterial duct recanalization and stenting is a feasible and effective procedure in selected cases, and its risks are treatable. Long-term studies are required. (Less)

Percutaneous closure of patent ductus arteriosus (PDA) is done in the cardiac catheterization laboratory, usually under fluoroscopic and angiographic guidance. The aortogram is used for assessing PDAsize and shape the pre-device implantation. Additionally, post-device aortograms are applied for the assessment of device position, profile and residual shunt. Angiograms expose patient to radiation and possible untoward effects of contrast media. Recently, transthoracic echocardiography has been utilized to guide in PDAclosure to avoid radiation exposure as well as contrast material. On two occasions, we were obligated by special circumstances to close the PDAunder echocardiography guidance. First case was a 6-month baby girl with mild signs of heart failure having moderate size PDA. She underwent device closure under transthoracic echocardiogram as the angiographic system had stopped working during the procedure. Second case was a 6-year girl, weighting 16-kg with chronic renal failure. She had moderate size PDAclosed by device under transthoracic echocardiographic, guidance avoiding the use of contrast agent due to chronic renal failure.

Summary The data from the reports we have reviewed showsthat transcatheter closure of the persistently patentarterial duct causes minimal pain, and has a very lowrate of complications. Embolization is becoming veryrare. The procedural and fluoroscopic times are accept-able. If all the devices available for clinical use areaccessible, with rare exceptions, almost every sizeand type of patent duct can be closed successfullyusing the interventional transcatheter approach. Mostdevices produce a very high rate of complete occlusion.The patients can be sent home less than 24 hoursafter intervention. Regular follow-up is still neededto assess long-term safety and efficacy of the devices. Place in clinical management andfuture outlook Studies dealing with the patent arterial duct arerarely prospective, and almost never randomized.With such a malformation, a lower level of evidencethan randomized studies is accepted to alter man-agement. Hence, it is acceptable to rely on consensusmeetings, clinical recommendations of scientificsocieties, and national registries. Recommendationsfor management made by respected societies, how-ever, rarely take economic factors into considera-tions.

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.

Ketamine and midazolam are commonly used in children undergoing cardiac catheterization. However, there is controversy regarding the safety of administering these agents in the absence of an an- esthesiologist. We retrospectively reviewed pediatric cardiac catheterization procedures at our institution between 1996 and 1997. A total of 154 patients (0.3- 192 months) underwent a total of 205 procedures. They received ketamine (n = 79, 1.05 ± 0.88 mg/kg/ hr), midazolam (n = 35, 0.14 ± 0.09 mg/kg/hr), or both (n = 91; ketamine, 1.13 ± 0.84 mg/kg/hr; mi- dazolam, 1.57 ± 1.03 mg/kg//hr). In 18.5% of pa- tients there were complex cardiac lesions. Mean procedure time was 79 ± 36.2 minutes. Pre- and postprocedure systolic and diastolic mean blood procedure 72 ± 14 and 68 ± 12 mmHg, respec- tively. Pre- and postprocedure O2 saturation was 93.19 ± 8.72 and 93.63 ± 8.3, respectively. One patient required intubation, and 15% required oxygen therapy. The mortality rate was zero. The anesthesi- ologist's assistance was requested by the cardiologist in 21 procedures (group A) and not requested in 184 procedures (group B). The two groups were not dif- ferent in relation to the drug used (p = 0.283) or the complexity of the cardiac lesions ( p= 0.051). How- ever, there was significant difference between the two regarding the need for supporting drugs (3/21 vs 3/ 184, p = 0.02) or oxygen treatment (7/21 vs 26/184, p = 0.014). No patients in group B required intu- bation, whereas 14% and 1.6% required oxygen therapy and supporting drugs, respectively. We con- clude that low-dose ketamine and midazolam can be administered safely to most pediatric patients by the cardiologist, who can safely predict the need for an anesthesiologist.

Life-threatening intracardiac and great vessels thrombi are rare in neonates. Recombinant tissue plasminogen activator (rTPA) is used in adults to stimulate fibrinolysis and facilitate thrombus resolution. Its use in neonates, along with heparin, remains controversial because of potential risk of serious bleeding. We aim to present our experience with the use of thrombolytic agents in seven neonates and young infants. In a retrospective study, over a period of 6 years, the medical records of neonates and young infants, who were diagnosed with intracardiac and great vessels thrombi, were reviewed. The following factors were collected: demographic data, primary diagnosis, thrombus site, risk factors, method of diagnosis, thrombolytic and/or anticoagulation agent, route, dose and duration of treatment, complications, and outcome. Six neonates and one 45-day-old infant were analyzed. Age ranged from 5 to 45 days (median age 12 days), and median weight was 2.9 kg (range 0.9-3.8 kg). The thrombi were diagnosed by echocardiography in five and by angiography in two cases. All patients had life-threatening thrombi; four were treated with rTPA (0.5 mg kg(-1) h(-1)) and heparin infusions with complete dissolution of the thrombi, within a median time of 60 h (6-72 h), and without complications. The remaining three patients (two who were premature, at 28 and 34 weeks of gestation, and the third who had a deranged coagulation profile) were treated with unfractionated heparin due to fear of bleeding. The thrombi dissolved in the premature babies (within 2 weeks and 3 months, respectively) but embolized and resulted in the death of the third infant after 2 weeks of treatment. The current case series confirmed the effectiveness and safety of intravenous rTPA infusion, at the dosages used, in neonates and young infants with life-threatening thrombi.

We sought to determine if a higher dose of heparin would reduce arterial complications in patients weighing 10 kg or less undergoing cardiac catheterization to investigate congenital heart disease. Sixty patients were given either 100 (group A) or 150 (group B) IU·kg−1 of heparin in a double-blinded randomized manner. Initial arterial access was established using a 4F cannula in all patients. Mean activated clotting time measured 20 minutes following heparin administration was significantly lower in group A than in group B (199 versus 251 seconds). Only 3 out of 60 patients (5%) required treatment for loss of femoral pulse. The age, weight, activated clotting time, length of catheterization procedure, time taken to establish arterial access, and the duration of arterial cannulation were comparable between the groups. Weight under 4 kg, age under 1 month, and cannula size larger than 4F were identified as independent risk factors for the development of arterial complications. Arterial access using a 4F cannula is a safe procedure in children weighing 10 kg or less. The incidence of significant arterial complications is low, and they do not appear to be preventable by a higher dose of heparin.

We reviewed our 12-year experience of surgical treatment for aortic coarctation in 86 neonates. Twenty-three patients had simple coarctation, 38 had an associated large ventricular septal defect, and 25 had complex intracardiac defects. The surgical techniques included subclavian flap angioplasty in 54 (63%), combined resection with end-to-end anastomosis augmented by a subclavian flap in 22 (26%), resection with extended end-to-end anastomosis in 7 (8%), and patch aortoplasty in 3 (3%). Five patients required additional transverse aortic arch augmentation. Hospital mortality was 14% (12/86) and was not related to the type of repair but associated pathology increased the operative risk. Late mortality was 11% (8/74) within one year of repair. Recoarctation developed in 5 patients (7%) within one year. No recoarctation was observed in the group repaired by end-to-end anastomosis augmented by a subclavian flap (p = 0.04).

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.

Combination of right and left ventricular outflow tracts obstruction is extremely rare. Neonates with combined aortic stenosis (AS) and pulmonary stenosis (PS) present in critical condition and required urgent treatment. The management approach is not well defined. We report five female neonates with combined AS and PS presented to our institute in the last 5 years, age (1–18 days), weight (2.2–3.4 kg). Two had associated muscular ventricular septal defects. The mean Doppler gradient across the aortic valve (AV) was 73 mmHg (53–105 mmHg) and across the pulmonary valve was 62 mmHg (44–76 mmHg). Three had balloon dilatation and one surgical repair. The fifth patient was managed conservatively, but had sudden cardiac death at age of 3 months. One patient arrived in shock and sepsis, underwent emergency balloon dilation of the AV in the ICU. Despite decreasing the gradient, she died next day after the procedure. The surviving children were well at median follow-up age of 3.4 years. This is an extremely rare combination which needs early intervention. The management approach is not well defined. Interventional catheterization is possibly the better option.

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.

A previously healthy 5-month-old girl presented to ouremergency department with history of fever for 2 weeks,skin rash, red and cracked lips and bilateral redness of theeyes appeared 4 days after fever started. On physicalexamination, the general condition was stable includinggood peripheral perfusion. There was no lymphadenopathy.Chest and abdominal examinations were unremarkable.Electrocardiography, apart from mild sinus tachycardia(HR 110 bpm), was normal with no sign of myocardialischaemia or hypertrophy. Chest X-ray showed cardiomeg-aly. Her blood investigation results were as follows: WBC16.2 9 100/lL (neutrophils 36.7%), normal haemoglobinand platelets 1356 9 100/lL. The kidney function and liverfunction tests were normal. Troponin-T 27 lg/L (lab nor-mal value 14 lg/L) and CKMB 42 lg/L (lab normal value25 lg/L). The diagnosis of Kawasaki disease (KD) wasestablished according to the combination of the clinicalpicture and blood investigations. Echocardiogram wasperformed and showed severe dilatation of left anteriordescending coronary artery with giant fusiform aneurysm(Fig. 1) and intracoronary thrombus (Fig. 2). Additionally,normal myocardial function and moderate pericardialeffusion with no sign of cardiac tamponade. Despite thelate presentation, immunoglobulin infusion was startedcombined with heparin, warfarin and low-dose aspirin. Itwas planned after two days to discontinue heparin. Anelective cardiac computerized tomography (CT) with con-trast injection was performed and shows giant aneurysmaldilatation of right coronary artery (RCA; Fig. 3). A repeatedechocardiography, directed on the RCA, confirms the CTfinding (Fig. 4). The critical evaluation of the initial echo-cardiography examination revealed that the RCA aneurysmwas there from the start but was missed as it is very adjacentto and almost as large as right atrium.Kawasaki disease is also known as mucocutaneouslymph node syndrome. Its diagnosis is usually done throughcombination of certain clinical criteria. One of the mostserious complications of KD is dilatation and fatal aneu-rysms or rupture of coronary arteries in untreated patients.The initial echocardiographic study always evaluates theexistence of coronary artery aneurysms. In the presence of aproximal aneurysm, further coronary artery evaluation isusually performed through CT or magnetic resonancetomography combined with contrast injection and/or car-diac catheterization and angiography.Myocardial ischaemia due to thromboembolism is also awell-reported complication. The guidelines for treatment of

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

A 2. days old, 2.7. kg heavy baby boy with critical pulmonary stenosis, underwent successful balloon dilation. After the uneventful procedure, he remained oxygen dependent. The baby was given oral angiotensin converting enzyme inhibitor (ACE inhibitor), instead of an infusion of alpha blocker.Within few hours, in the afternoon of the same day after administration of ACE Inhibitor, the baby could be weaned off oxygen, maintaining on room air, oxygen saturation between 87% and 92%. At follow-up, two months later, his saturation was 99% on room air.We believe that some neonates with critical pulmonary valve stenosis who remain oxygen dependent despite successful balloon dilation, could benefit from such management. © 2011.

Case report Neurocutaneous disorders are characterized by skin lesions (port wine stain, hypopigmented patches) associated with intracranial features (ipsilateral leptomeningeal angiomas, intracranial calcifications and subsequent seizures). Some of these patients will also have opthalmological findings.These disorders are occasionally accompanied by congenital heart disease. Example: PHACE syndrome which is associated with aortic coarctation. On the contrary, Sturge–Weber syndrome, apart from one single case report, is usually not described with congenital heart disease. In the mentioned case report, 13 year old female had along with this syndrome, pulmonary atresia with ventricular septal defect. As the author of this case report questioned whether this might be a new association, we would like to report our patient. This is a 14 months old boy with pulmonary atresia/intact ventricular septum along with intracranial calcification, seizures along with strabismus and tunnel vision. Clinical findings were similar to Sturge–Weber syndrome, though cutaneous features were not present. Brain CT angio with contrast was diagnostic for Sturge–Weber and showed calcifications as well as left parietal hemangioma. As far as we know, this specific association has never been reported before and might be a new constellation as suggested by Huseyin Tan et al. (2003).

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.

Background Very rarely a foreign body in the esophagus may penetrate anteriorly to heart or aorta. Such foreign bodies may cause pericardial effusion and cardiac tamponade. Aim To report that an accidentally swallowed foreign body in the esophagus may penetrate the heart and even cause severe mitral regurgitation. Material and methods A 20 month old girl previously healthy presented to the emergency department with 3 days history of fever, respiratory distress, vomiting and poor feeding. The mother reported that her child had some choking event almost a week before, when the child was admitted to another hospital where diagnosis was made of myocarditis. Anti-failure medications prescribed. On examination she had palpable peripheral pulses, a pansystolic murmur on the cardiac apex, and hepatomegaly. EKG showed sinus tachycardia with left ventricular hypertrophy. Chest X-ray revealed normal cardiac size with lungs congestion and suspicion of possible foreign body. Echocardiography showed dilatation of the left atrium and of the pulmonary veins, severe mitral regurgitation with an echogenic structure in next to the posterior mitral valve leaflet with consensual moving. Chest CT scan revealed a high-density metallic foreign body inside the heart in correspondence of the left atrio-ventricular junction. The foreign body was removed surgically and proved to be a thin shiny metal bar, 2 cm in length. The mitral valve was completely damaged and was replaced by mechanical valve (Carbomedics 18 mm). The postoperative course was uneventful and patient was discharged home with anti-failure medications (frusemide, captopril) and warfarin. She remained asymptomatic one year after surgery. Conclusion The process of diagnosing a foreign body in an infant or a toddler, eroding from the esophagus to the heart was very tricky. The diagnosis was suspected by the history of chocking. The x-ray of the chest showed an abnormal structure on the heart shadow, which was further underlined by the echocardiogram suggesting abnormal structure in the heart. Finally CT angio of heart confirmed a metallic foreign body in the left atrium. Happily, MRI was not done; it may have proved disastrous in such a case. The strategy regarding foreign body management either conservative or by its removal either by cardiac catheterization or surgery, depends on location, size of foreign body as well as on whether the patient is symptomatic and in danger to develop further complications. In our case, as the foreign body was in the left atrium and the patient was very critically ill, we opted to remove it by surgery. Moreover, it was thought that patient might need mitral valve repair but unfortunately the valve was so damaged that we opted for mitral valve replacement.

A 12-year-old boy reported in outpatient department with history of shortness of breath and dyspnoea on moderate exertion. Physical examination was significant for bounding pulses and for a continuous murmur III/VI, best heard at the left upper sternal border. Echocardiography confirmed a large patent arterial duct with shortening fraction of 33%. He underwent successful transcatheter closure of the patent arterial, using Amplatzer duct occluder 12/10. Few hours later echocardiography revealed an unexpected, yet important depression of left ventricular systolic function with shortening fraction decreasing to 24% and then two weeks later decreasing further to 20%. At a follow-up after four months, he had improved clinically but left ventricular dysfunction still persisted with shortening fraction of 24%.

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.

The study aim was to define the long-term outcome of pulmonary balloon valvulotomy (PBV) in adult patients.PBV was performed in 87 patients (46 females, 41 males; mean age 23+/-9 years; range: 15-54 years) with congenital pulmonary valve stenosis (PS). Intermediate follow up catheterization (mean 14.6+/-5.0; range: 6-24 months) was performed after PBV in 53 patients. Clinical and Doppler echocardiography examinations were carried out annually in 82 patients (mean 8.0+/-3.9; range: 2-15 years).There were no immediate or late deaths. The mean catheter peak pulmonary gradient (PG) before and immediately after PBV, and at intermediate follow up was 105+/-39, 34+/-26 (p0.0001) and 17+/-14 (p0.0001) mmHg, respectively. The corresponding values for right ventricular (RV) pressure were 125+/-38, 59+/-21 (p0.0001) and 42+/-12 (p0.0001) mmHg, respectively. The infundibular gradients immediately after PBV and at intermediate follow up were 31+/-23 and 14+/-9 mmHg (p0.0001), whilst cardiac index improved from 2.68+/-0.73 to 3.1+/-0.4 l/min/m2 (p0.05) at intermediate follow up. Doppler PG before PBV and at intermediate and long-term follow up were 91+/-33 (range 36-200) mmHg, 28+/-12 (range 10-60) mmHg (p0.0001) and 26+/-11 mmHg (p = 0.2), respectively. New pulmonary regurgitation (PR) was noted in 21 patients (25%) after PBV. Five patients (6%) with a suboptimal result (immediate valve gradientor =30 mmHg) developed restenosis and underwent repeat valvulotomy 6-12 months later using a larger balloon, and with satisfactory outcome. Moderate to severe tricuspid regurgitation (TR) in seven patients regressed after PBV.The long-term results of PBV in adults are excellent, with regression of concomitant, severe infundibular stenosis and/or severe TR. Hence, PBV should be considered as the treatment of choice for adult patients with PS.

To review the spectrum of double-chambered right ventricle (DCRV) and the outcome of surgical repair in patients diagnosed between February 1988 and March 1999.The charts of patients with DCRV were studied.Tertiary care hospital.A total of 73 patients were identified. Sixty-nine underwent surgical repair, while four are awaiting surgery. The repair was through a transatrial approach in 61 patients, while in eight an additional ventriculotomy was performed.An associated ventricular septal defect (VSD) was present in 56 of 73 patients (77%). These patients were significantly younger (P0.05) than the 17 patients without a VSD. Among patients with a VSD, the 31 requiring patch closure were significantly younger than the 25 patients having direct closure. Five older patients among those with intact septum had impaired right ventricular (RV) function as well as higher intraventricular gradients. At surgery the intraventricular obstruction was relieved by myomectomy. There was no hospital or late mortality. Following surgery, at a mean follow-up of 13.6 months, no increase in the intraventricular gradient was detected by Doppler echocardiography.The development of DCRV is associated with VSD in early life. The probability of the presence of a VSD decreases with age. The disease is progressive, resulting in increased intracavitary gradient within the RV and in RV impairment if it is not treated in a timely fashion. Transatrial repair is safe with excellent midterm results. In the presence of high gradients within the RV, a ventriculotomy may be necessary to obtain acceptable results.

Background Communicating bad news to mothers of children with congenital heart disease (CHD) is a challenging task and an ethical dilemma for the physician. It is never a pleasant task but breaking it at the wrong time or in the wrong way can be even worse, so it’s important to know the best approaches to breaking bad news. Aim To know the preferences of mothers regarding breaking bad news about the diagnosis of congenital heart disease in their children. Methods The study was conducted in our Pediatric Cardiology Department between 1st October 2010 to 1st September 2012. A cross sectional interview was conducted including Saudi mothers whose children had CHD. The questionnaire consisted of 27 questions. Verbal consent was obtained. Results Total 889 mothers were included in the study and 39 refused to participate in the survey. The results revealed that 58.4% mothers were from urban areas while 41.6 from villages and 98.3% were educated. Based on answers to questionnaire we found 20 different preferences. Counseling is preferred: immediately after diagnosis 92.7%, directly in person 99.9%, both parents present 96.4%, single session counseling 92.2%, detailed counseling 93.9%, detailed medical report 98.2%, isolated and quiet place of counseling 64.3%, counseling with help of heart model 85.3%. Most mothers want counseling by their primary physician 92.3%. The mothers accepted to have their nurse present at time of counseling but not relative or friend. Discussion Counseling parents about diagnosis of congenital heart disease in their children is a challenging task for physician and an ethical dilemma. The diagnosis of any congenital heart disease in children leads to parental anxiety. Our results confirm previously reported preferences by mothers from western countries and Egypt to be told early, detailed and in person. We found that mothers want a completely quiet room, presence of nurse and detailed counseling as well as detailed medical report both in paper and electronic form. They want counseling to be done by their primary physician. Conclusion Our study delineates that mothers of patients with CHD in our area have clear preferences for how bad news should be conveyed to them. We believe that following these directions might reduce their anxiety to an accepted level.

Background Geleophysic dysplasia is an extremely rare autosomal recessive acromelic skeletal dysplasia characterized by short stature and short limbs, joint contracture and cardiac involvement. It has been described worldwide in less than 40 patients. We describe the cardiac involvement in three girls of a Saudi family who showed different severity of the cardiac involvement. Clinical description Three sisters referred from general pediatrics over a period 5 years, because of dysmorphic features and heart murmur. The parents are first degree cousins, they have a boy who is now 3 years old and is quite normal in regard of his growth and development. The older sister is 8 years old, the next is six years, and the third is two years old. All three girls were born full term spontanous delivery and had history of respiratory problems with frequent hospital admissions. They are all short for their ages (less than 3rd centile). Furthermore they share dysmorphic features in a form of small hands and feet, hypertelorism, depressed nasal bridge and anteverted narse. They all have contracture at both elbows and knees with inability to flex all to full range of flexion. They are happy and very friendly children, mentally normal and seem to be intellectually appropriate for their age. Genetic studies were done which confirmed the heterogenicity of the ADAMTSL2 gene in the older child. The three girls have associated cardiac lesions with different expression. The older has a mild pulmonary and aortic valve stenosis, the second has thicking of mitral valve and the youngest moderate to severe aortic and pulmonary valve stenosis. Discussion Geleophysics dysplasia (GD) has been described in 1960 as a form of atypical gargoylism by Vanace et al. and described by Spranger et al 1971 as a focal mucopolysacchriodosis. The disease is now considered as a severe form of acromelic skeletal dysplasia which is a rare form of dysplasia including three disorders: geleophysic dysplasia, Weill-Marchesani and acromicric dysplasia. Up to date there are less than 40 reported cases with GD. All of these patients have a combination of cardiac valve involvement. The most frequent cardiac lesions described being the mitral valve, then the aortic valve followed by pulmonary valve and the least is tricuspid valve. Spranger described one girl with atrial septal defect(ASD) and patent arterial duct (PDA) without valvular involvement. Pontz and Santolaya seperately described 5 cases with normal cardiac structure. Until todate there is a believe that the valve involvement show progression over time. Conclusions There are very few case reports describing the cardiac involvement in this condition but none is from the Middle East. Almost most of the cardiac conditions affect the cardiac valves in a form of thickening leading to stenosis in one or more of the cardiac valves. Most of the cases suggest progression of the disease. In our three cases the younger has the most severe form of valve involvement. This suggests that the expression of the gene could determine the severity of the disease, rather than the suggested progression with age.

Fourteen young adult patients between 14 and 29 years of age underwent 15 procedures of transcatheter occlusion of patent ductus arteriosus. In all patients but one, the attempt to implant the device was successful (92%). In one patient the ductus was judged to be too large for occlusion and the patient was sent for surgery. In no patient did the device embolize. A total of 6/13 (46%) patients was discharged home with no residual shunt. Follow-up data (between 3 and 18 months) are available in 10/13 patients. To date, 9/13 (70%) had total occlusion of their duct, in one patient after reocclusion.

Origin of the right pulmonary artery from the ascending aorta is extremely rare. We describe the echocardiographic findings of this lesion in a single case. The absence of an image of the right pulmonary artery in the echocardiographic short axis view at the level of the great arteries, is highly suggestive of this lesion. Differentiation from truncus arteriosus, aorto-pulmonary window and transposition of the great arteries must, and can be made by a comprehensive echocardiographic study.

A newborn is presented with an association of hypoplastic left heart syndrome, cor triatriatum and partial anomalous pulmonary venous connection. The diagnosis was established with echocardiography and further confirmed with computed tomography. To our knowledge the images of such an association have never been reported before. © 2011 .

A neonate is presented with a very rare association consisting of pulmonary atresia with large ventricular septal defect but additionally, combined with a hypoplastic right ventricle and well developed pulmonary arteries. The management strategy is described.

An intrapericardial aneurysm of the left atrial appendage in a 7-year-old girl is reported. Review of the 36 cases described up to now reveals that there is no specific age for this disease. It is therefore questioned whether this defect is really congenital in origin. Systemic embolism and serious rhythm disturbances are the most common complications; the younger the patient, the less likely they are to occur. Systemic embolism has only been described in the case of aneurysms located at the left atrial appendage, not in aneurysms of other parts of the left atrial wall. The risk of the development of rhythm disorders, however, is independent of the location within the left atrium. Clinical findings (including ECG) are nonspecific. Cardiomegaly and unusual configuration of the heart are constantly found, but are not pathognomonic. The diagnosis is usually confirmed by angiocardiography. Operation seems always to be indicated if complications are to be avoided. Operative deaths or complications during the follow-up after operation are not described. Nothing is known about the etiology of the disease.