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Background: The objective was to study the ductus arteriosus morphology in duct-dependent pulmonary circulation and its pattern in different ventricle morphology using CT angiography. Method: From January 2013 to December 2015, patients aged 6 months and below with duct-dependent pulmonary circulation underwent CT angiography to delineate the ductus arteriosus origin, tortuosity, site of insertion, and pulmonary artery anatomy. The ductus arteriosus were classified into type I, IIa, IIb, and III based on its site of origin, either from descending aorta, distal arch, proximal arch, or subclavian artery, respectively. Results: A total of 114 patients and 116 ductus arteriosus (two had bilateral ductus arteriosus) were analysed. Type I, IIa, IIb, and III ductus arteriosus were seen in 13 (11.2 %), 71 (61.2%), 21 (18.1%), and 11 (9.5%), respectively. Tortuous ductus arteriosus was found in 38 (32.7%), which was commonly seen in single ventricular lesions. Ipsilateral and bilateral branch pulmonary artery stenosis was seen in 68 (59.6%) and 6 (5.3%) patients, respectively. The majority of patients with pulmonary atresia intact ventricular septum had type I (54.4%) and non-tortuous ductus arteriosus, while those with single and biventricular lesions had type II ductus arteriosus (84.9% and 89.7%, respectively). Type III ductus arteriosus was more common in biventricular lesions (77.8%). Conclusions: Ductus arteriosus in duct-dependent pulmonary circulation has a diverse morphology with a distinct origin and tortuosity pattern in different types of ventricular morphology. CT may serve as an important tool in case selection and pre-procedural planning for ductal stenting.

Background:Vascular compression of the airway often complicates CHD management. This study evaluated the use of CT in determining cardiovascular causes, clinical manifestations, and outcome of tracheobronchial compression among children with CHD.Methods:A retrospective review of clinical records of all patients with CT scan evidence of tracheobronchial compression from January 2007 to December 2017 at National Heart Institute. Cardiovascular causes of tracheobronchial compression were divided into three groups; group I: vascular ring/pulmonary artery sling, II: abnormally enlarged or malposition cardiovascular structure due to CHD, III: post-CHD surgery.Results:Vascular tracheobronchial compression was found in 81 out of 810 (10%) patients who underwent CT scan. Group I lesions were the leading causes of vascular tracheobronchial compression (55.5%), followed by group II (34.6%) and group III (9.9%). The median age of diagnosis in groups I, II, and III were 16.8 months, 3 months, and 15.6 months, respectively. Half of group I patients are manifested with stridor and one-third with recurrent chest infections. Persistent respiratory symptoms, lung atelectasis, or prolonged respiratory support requirement were clues in groups II and III. Higher morbidity and mortality in younger infants with severe obstructive airway symptoms, associated airway abnormalities, and underlying complex cyanotic CHD.Conclusions:Vascular ring/pulmonary artery sling and abnormally enlarged or malposition cardiovascular structure were the leading causes of cardiovascular airway compression. A high index of suspicion is needed for early detection due to its non-specific presentation. The outcome often depends on the severity of airway obstruction and complexity of cardiac lesions.

OBJECTIVES This study aims to review our institutional experience of ductal stenting (DS) on the growth of pulmonary arteries (PAs) and surgical outcomes of PA reconstruction in this subset of patients. METHODS This is a retrospective study done in neonates and infants up to 3 months of age with duct-dependent pulmonary circulation who underwent DS from January 2014 to December 2015. Post-stenting PA growth, surgical outcomes of PA reconstruction, post-surgical re-interventions, morbidity and mortality were analysed. RESULTS During the study period, 46 patients underwent successful DS, of whom 38 underwent presurgery catheterization and definite surgery. There was significant growth of PAs in these patients. Biventricular repair was done in 31 patients while 7 had univentricular palliation. Left PA augmentation was required in 13 patients, and 10 required central PA augmentation during surgery. The mean follow-up period post-surgery was 4.5 ± 1.5 years. No significant postoperative complications were seen. No early or follow-up post-surgery mortality was seen. Four patients required re-interventions in the form of left PA stenting based on the echocardiography or computed tomography evidence of significant stenosis. CONCLUSIONS DS provides good short-term palliation and the growth of PAs. However, a significant number of stented patients require reparative procedure on PAs at the time of surgical intervention. Acquired changes in the PAs following DS may be the reason for reintervention following PA reconstruction.

Background Patients with single‐ventricle physiology who undergo the Fontan procedure are at risk for thrombotic events associated with significant morbidity and mortality. The UNIVERSE Study evaluated the efficacy and safety of a novel liquid rivaroxaban formulation, using a body weight–adjusted dosing regimen, versus acetylsalicylic acid (ASA) in children post‐Fontan. Methods and Results The UNIVERSE Study was a randomized, multicenter, 2‐part, open‐label study of rivaroxaban, in children who had undergone a Fontan procedure, to evaluate its dosing regimen, safety, and efficacy. Part A was the single‐arm part of the study that determined the pharmacokinetics/pharmacodynamics and safety of rivaroxaban in 12 participants before proceeding to part B, whereby 100 participants were randomized 2:1 to open‐label rivaroxaban versus ASA. The study period was 12 months. A total of 112 participants were enrolled across 35 sites in 10 countries. In part B, for safety outcomes, major bleeding occurred in one participant on rivaroxaban (epistaxis that required transfusion). Clinically relevant nonmajor bleeding occurred in 6% of participants on rivaroxaban versus 9% on ASA. Trivial bleeding occurred in 33% of participants on rivaroxaban versus 35% on ASA. For efficacy outcomes, 1 participant on rivaroxaban in part B had a pulmonary embolism (2% overall event rate); and for ASA, 1 participant had ischemic stroke and 2 had venous thrombosis (9% overall event rate). Conclusions In this study, participants who received rivaroxaban for thromboprophylaxis had a similar safety profile and fewer thrombotic events, albeit not statistically significant, compared with those in the ASA group. Registration URL: https://www.clinicaltrials.gov . Identifier: NCT02846532.

Bioresorbable stent has the theoretical advantage in the pediatric age group; however, experience in the literature is very limited.This pilot study sought to evaluate the feasibility, safety, and performance of iron bioresorbable scaffold (IBS Angel™) as short-term palliation in lesions with noncomplex patent ductus arteriosus (PDA).Nine neonates with duct dependent but dual-source pulmonary blood flow (PBF) were included. Major stent-related complications, in-hospital course, stent patency up to 6 months, and unplanned re-interventions were studied, as well the percentage of stent material resorbed in patients in whom the stents were explanted at the surgery.IBS Angel™ was successfully implanted in all nine patients (mean weight range 3.4 ± 0.4 kg). Six were pulmonary atresia with an intact ventricular septum and 1 critical pulmonary stenosis patients where PDA stenting was done together or after balloon dilation. The mean procedure and fluoroscopy times were 89 ± 39 min and 16.3 ± 6.9 min, respectively. There were no major complications such as stent thrombosis or embolization and there were no in-hospital deaths. Post procedure overshunting and prolonged ventilatory support was a prominent feature. The median ventilation days was 3 (1-11 days). One patient died after 1 month due to respiratory syncytial virus pneumonia at the referring hospital. At 6 months follow-up, four had patent stents and four had blocked or restrictive stent flow. One patient received re-stenting at 4 months for restrictive stent flow. In three patients where microcomputed tomography of explanted stent was available, resorption of iron was 15% at 6 months and65% at 16 months.Ductal stenting with IBS Angel™ is feasible and safe in selected patients. Because of early luminal loss, its use may be recommended for lesions with dual-source PBF with noncomplex PDA morphology that requires a short duration of palliation.

Ductal stenting in patients with duct-dependent pulmonary circulation has allowed growth of pulmonary arteries prior to definitive surgical procedures. Intraprocedural stent thrombosis (IPST) of the arterial duct is a life-threatening complication as it leads to total circulatory collapse. Previous reports have described use of tissue plasminogen activators in infants for less emergent settings. We report three infants with IPST and the use of tenecteplase to overcome this. Also discussed are the predisposing mechanisms in each scenario and a possibility of direct catheter-guided tenecteplase administration. Judicious use of tenecteplase can be life-saving and rapid access to this drug may obviate the need for emergency extracorporeal life support.

Long QT syndrome (LQTS) may be a cause of foetal bradyarrhythmia and an important cause of death in children with arrhythmia. We present the case of a patient of Kadazan Iban descent with LQTS. He was detected prenatally to have foetal 2:1 atrioventricular (AV) block and tetralogy of Fallot. His postnatal electrocardiogram revealed a functional 2:1 AV block with QTc interval of 690 ms. Dysmorphism and cutaneous syndactyly of both hands and feet pointed to a diagnosis of classical Timothy syndrome (TS) type 1. This diagnosis was confirmed molecularly with a heterozygous mutation c.1216G>A. p. (Gly406Arg) at exon 8A in the CACNA1C gene. To the best of our knowledge, this is the first reported case of TS in a Kadazan Iban child. JRCD 2019; 4 (2): 42-46.

Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is caused by deficient activity of the enzyme, N-acetylgalactosamine-4-sulfatase, resulting in impaired degradation of the glycosaminoglycan dermatan sulfate. Patients experience a range of manifestations including joint contractures, short stature, dysostosis multiplex, coarse facial features, decreased pulmonary function, cardiac abnormalities, corneal clouding and shortened life span. Recently, clinicians from institutions in the Asia-Pacific region met to discuss the occurrence and implications of delayed diagnosis and misdiagnosis of MPS VI in the patients they have managed. Eighteen patients (44% female) were diagnosed. The most common sign presented by the patients was bone deformities in 11 patients (65%). Delays to diagnosis occurred due to the lack of or distance to diagnostic facilities for four patients (31%), alternative diagnoses for two patients (15%), and misleading symptoms experienced by two patients (15%). Several patients experienced manifestations that were subtler than would be expected and were subsequently overlooked. Several cases highlighted the unique challenges associated with diagnosing MPS VI from the perspective of different specialties and provide insights into how these patients initially present, which may help to elucidate strategies to improve the diagnosis of MPS VI.

Purpose Patent Ductal Arteriosus (PDA) stenting in the setting of pre-existing branch pulmonary artery (PA) stenosis is controversial. Current American Heart Association guidance is to avoid ductal stenting in this setting. During fellowship training I spent time at an institution which has undergone a learning curve in complex ductal stenting. CT imaging enabled better understanding of complex ducts, aided patient selection, planning and consideration of access routes. Methods Prospective review of patients ( 2.5 kg) undergoing PDA stenting with ductus related branch PA stenosis between Jan 2014 – Dec 2015. The growth of the jailed stenotic PA branch with the contralateral PA was compared angiographically 6–12 months later. Results 46 patients underwent ductal stenting. 37% had ToF-PA (17/46). There was no in-hospital mortality and 2 early deaths occurred. 4 patients required early modified Blalock Taussig Shunt (mBTS) and were further excluded. The unaffected PA showed good growth (mean Z score from −0.6 to +2.2). The jailed PA also showed proportionate growth (mean Z score from −1.4 to +1.1) in 93% (37/40). 3 patients showed poor PA growth (2 underwent restenting, 1 mBTS to affected side). Conclusion In an era where neonatal mBTS continues to have significant morbidity and mortality, we feel that ductal stenting in complex PDA’s with pre-existing PA stenosis is a feasible alternative. The stenosed, jailed PA still has potential for proportionate growth. Patient selection and close follow up is vital. Stent removal with surgical repair within 1 year of age is our current recommendation.

Objective: We introduced primary arterial switch operation for the patient with transposition of great arteries and intact ventricular septum (TGA-IVS) who had more than 3.5 mm of posterior left ventricle (LV) wall thickness. Methods: Between January 2013 and June 2015, a total of 116 patients underwent arterial switch operation. Of the 116 patients, 26 with TGA-IVS underwent primary arterial switch operation at more than 30 days of age. Results: The age and body weight (mean ± SD) at the operation were 120.4 ± 93.8 days and 4.1 ±1.0 kg, respectively. There was no hospital mortality. The thickness of posterior LV wall (preoperation vs postoperation; mm) was 4.04 ± 0.71 versus 5.90 ± 1.3; P < .0001; interval: 11.8 ± 6.5 days. The left atrial pressure (mm Hg; postoperative day 0 vs 3) was 20.0 ± 3.2 versus 10.0 ± 2.0; P < .0001; and the maximum blood lactate level (mmol/dL) was 4.7 ± 1.4 versus 1.4 ± 0.3; P < .0001, which showed significant improvement in the postoperative course. All cases had delayed sternal closure. The patients who belonged to the thin LV posterior wall group (Conclusions: The children older than 30 days with TGA-IVS can benefit from primary arterial switch operation with acceptable results under our indication. However, we need further investigation for LV function.

Heart arrhythmia is a serious health problem that is commonplace among patients of different ages, from new born infants to the elderly. For such patients, an artificial pacemaker may be implanted for hear regulation by sending electrical signals to the patient's heart chambers. The advances in the electronics technology, has the potential to improve the artificial pacemakers in terms of price, energy consumption, and miniaturization to eliminate the leads. A microcontroller pacemaker, along with a PC-based programmer is proposed for bradycardia pacing. The algorithm is implemented in Arduino microcontrollers, which is programmed using PC and MATLAB. A ventricular signal with random or frequent lost beats is studied, and a pacing system is proposed for regulation of the simulated heart signal as in VVI mode. The experimental results show the ability of the system in detecting absence of the heartbeat in the expected times, and composing the required pacing pulses.

The major drawbacks of current pacemaker are the battery replacement. Patient will need additional surgery to replace the pacemaker unit with the new one. Utilization of rechargeable battery has been proposed to overcome this problem. However, recharging a battery within the body is not feasible due to tissue-heating and battery’s charge-and-discharge life-time. By those reasons, the utilization of piezo-polymer is suitable for a self-powered pacemaker as energy harvester. Piezo-polymer has been widely used as energy harvesting but none for cardiothoracic implantable device. This study focuses on specific implementation of piezo-polymer for epicardial pacemaker. The proposed fundamental research aims to identify the optimum location on the heart to put the piezo-polymer. This research will be conducted by simulation of left ventricle of heart via ANSYS. Heart stress-strain Finite Element Analysis (FEA) will be employed to obtain the maximum harvested power. The result shows the location of myocardial contraction that produces sufficient kinetic energy for the placement of the pacemaker. The heart 3-dimensional images are taken from cardiac-CT or cardiac-MRI to search the optimum location on the heart for energy harvesting and minimize pacing energy. In conclusion, the left ventricular wall motion and deformation caused by the cardiac wall movements have been analysed based on the left ventricle model in the simulation to get the location of the maximum kinetic energy formed.

ObjectiveTo determine the feasibility and safety of the Conquest Pro wire as an alternative to radiofrequency wire for perforation of atretic pulmonary valve and subsequent balloon dilatation and patent ductus arteriosus stenting in patients with pulmonary atresia with intact ventricular septum.BackgroundRadiofrequency valvotomy and balloon dilatation has become the standard of care for pulmonary atresia with intact ventricular septum in many institutions today.MethodsWe report eight consecutive patients in whom we used the Conquest Pro coronary guidewire, a stiff wire normally reserved for revascularisation of coronary lesions with chronic total occlusion, for perforation of atretic pulmonary valve and subsequent balloon dilatation, and stenting of the patent ductus arteriosus.ResultsPerforation of atretic pulmonary valve was successful in seven out of eight cases. Radiofrequency valvotomy was employed after failure of perforation by the Conquest Pro wire in one case where the right ventricular outflow tract was broad based and tapered towards the pulmonary valve, and was heavily trabeculated. Failure of the Conquest Pro wire to perforate the pulmonary valve plate was mainly attributed by the failure to engage the wire at the correct position.ConclusionThe Conquest Pro wire for perforation and subsequent interventions in the more straightforward cases of pulmonary atresia with intact ventricular septum is effective and safe, simplifying the entire procedure. However, the radiofrequency generator and wires remain essential tools in the paediatric interventional catheter laboratory.

Objectives Our objective was to determine the feasibility and early to medium-term outcome of stenting the patent ductus arteriosus at the time of radiofrequency valvotomy in the subgroup of patients with pulmonary atresia with intact ventricular septum and intermediate right ventricle. Background Stenting of the patent ductus arteriosus and radiofrequency valvotomy have been proposed as the initial intervention for patients with intermediate right ventricle inasmuch as the sustainability for biventricular circulation or 1½-ventricle repair is unclear in the early period. Methods Between January 2001 and April 2009, of 143 patients with pulmonary atresia and intact ventricular septum, 37 who had bipartite right ventricle underwent radiofrequency valvotomy and stenting of the patent ductus arteriosus as the initial procedure. The mean tricuspid valve z-score was −3.8 ± 2.2 and the mean tricuspid valve/mitral valve ratio was 0.62 ± 0.16. Results Median age was 10 days (3–65 days) and median weight 3.1 kg (2.4–4.9 kg). There was no procedural mortality. Acute stent thrombosis developed in 1 patient and necessitated emergency systemic–pulmonary shunt. There were 2 early in-hospital deaths owing to low cardiac output syndrome. One late death occurred owing to right ventricular failure after the operation. Survival after the initial procedure was 94% at 6 months and 91% at 5 years. At a median follow-up of 4 years (6 months to 8 years), 17 (48%) attained biventricular circulation with or without other interventions and 9 (26%) achieved 1½-ventricle repair. The freedom from reintervention was 80%, 68%, 58%, and 40% at 1, 2, 3, and 4 years, respectively. Conclusions Concomitant stenting of the patent ductus arteriosus at the time of radiofrequency valvotomy in patients with pulmonary atresia with intact ventricular septum and intermediate right ventricle is feasible and safe with encouraging medium-term outcome.

Background: Transcatheter closure of atrial septal defects (ASD) has been accepted world-wide as an alternative to surgical closure with excellent results. This interventional, non-surgical technique plays an important role in the treatment of ASD mostly in the developing world where resources are limited. Objectives: To report the outcomes and short term follow-up of transcatheter closure of ASD over a 12-year period at our institution with limited resources. Patients and Methods: This retrospective study included all patients with the diagnosis of secundum ASD and significant shunting (Qp/Qs > 1.5:1) as well as dilated right atrium and right ventricle who had transcatheter closure at Integrated Cardiovascular Center (PJT), Dr. Cipto Mangunkusumo Hospital between October 2002 and October 2014. One hundred fifty-two patients enrolled in this study were candidates for device closure. Right and left heart cardiac catheterization was performed before the procedure. All patients underwent physical examination, ECG, chest X-ray and transthoracal echocardiography (TTE) prior to device implantation. Results: A total of 152 patients with significant ASD underwent device implantation. Subjects’ age ranged from 0.63 to 69.6 years, with median 9.36 years and mean 16.30 years. They consisted of 33 (21.7%) males and 119 (78.3%) females, with mean body weight of 29.9 kg (range 8 to 75; SD 18.2). The device was successfully implanted in 150 patients where the majority of cases received the Amplatzer septal occluder (147/150; 98%) and the others received the Heart Lifetech ASD occluder (3/150, 2%), whereas two other cases were not suitable for device closure and we decided for surgical closure. The mean ASD size was 19.75 (range 14 - 25) mm. During the procedure, 5 (4.9%) patients had bradycardia and 3 (2.9%) patients had supraventricular tachycardia (SVT), all of which resolved. Conclusions: In our center with limited facilities and manpower, transcatheter closure of atrial septal defect was effective and safe as an alternative treatment to surgery. The outcome and short-term follow-up revealed excellent results, but long-term follow-up is needed.

Technique From November 1997 to June 2002, percutaneous transcatheter closure of muscular ventricular septal defects was attempted in seven patients. Four patients had single and three had multiple defects. Surgical closure was performed in two patients in an attempt to close a perimembranous defect, leaving behind a large apical muscular defect, which was successfully closed using a device in one patient, whilst the second patient succumbed to septicemia/endocarditis 3 weeks after failure of device implantation. One patient had previous pulmonary artery banding and in another intraoperative placement of two Clamshell devices followed by additional transcatheter closure using Gianturco coils in two different sessions was performed. Results Transcatheter closure of ventricular septal defect was successful in six patients. Three patients underwent closure of their ventricular septal defects using the Amplatzer Septal Occluder and in the remaining three patients. Amplatzer Muscular Ventricular Septal Defect Occluder was used. The median age was 15 months (range 14 months to 10 years) and the median weight was 7.8 kg (range 7–16 kg). The devices were deployed antegradely in all patients. Immediate complete closure was obtained in three patients while two patients had small and one had a large residual following the procedure. The later was due to another multiple muscular septal defect. Transient heart block occurred in one patient during the procedure and another patient developed heart block on day 3 post-procedure. The latter required temporary pacemaker. During the follow up, one patient who had residual multiple muscular defects underwent a successful redo transcatheter closure using two Amplatzer Muscular Ventricular Septal Occluder devices. Conclusions We conclude that transcatheter closure of muscular ventricular septal defect using Amplatzer devices is feasible and effective.

Some patients with pulmonary atresia with an intact ventricular septum, mild to moderate right ventricular hypoplasia, and a patent infundibulum remain duct dependent on the flow of blood through the arterial duct despite adequate relief of the obstruction within the right ventricular outflow tract.The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve.We reviewed the data from 53 patients seen between November 1995 and December 2001. Of the 47 patients who survived, 6 required stenting of the patent arterial duct, while 4 needed construction of a modified Blalock-Taussig shunt to augment the flow of blood to the lungs at a mean of 7 plus or minus 5.7 days following the initial intervention. The remaining 37 patients required no additional procedures. We compared the findings in these two groups.The mean diameter of the tricuspid valve in the patients requiring early reintervention was 8.5 plus or minus 3.7 millimetres, giving a Z-score of −1.1 plus or minus 1.47, whilst those in the group without early reintervention had values of 10.7 plus or minus 2.2 millimetres, giving a Z-score of −0.58 plus or minus 1.18 (p equal to 0.003). No statistically significant differences were found in right ventricular morphology, McGoon ratio, or residual obstruction across the right ventricular outflow tract after decompression of the right ventricle.The diameter of the tricuspid valve, therefore, appears to be the only factor predicting the need for augmentation of flow of blood to the lungs. As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve.

Objectives This study was designed to assess the safety, efficacy, medium-term outcome, and complications of patent ductus arteriosus (PDA) stenting in duct-dependent pulmonary circulation. Background Patent ductus arteriosus stenting has been proposed as an alternative to surgical shunt on account of postoperative morbidity and complications of surgical shunting. Methods Between April 2000 and February 2003, 69 patients with duct-dependent pulmonary circulation underwent cardiac catheterization with the intent of PDA stenting as first palliative procedure. Patients with critical pulmonary stenosis and pulmonary atresia with intact ventricular septum post-radiofrequency valvotomy who had PDA stenting were excluded. Thirteen more patients were excluded because of branch pulmonary artery (PA) stenosis. The follow-up was by clinical examination, echocardiography, and repeat cardiac catheterization at six to nine months following the procedure. Results Patent ductus arteriosus stenting was successful in 51 patients (91.1%) and failed in 5 patients (8.9%). The mean narrowest PDA diameter was 1.9 ± 0.6 mm. The mean procedure and fluoroscopy time were 95.7 min and 29.4 min, respectively. In one patient the stent dislodged and migrated to the left femoral artery and another patient developed transient intravascular hemolysis. There was no procedure-related mortality. Three patients (5.9%) died one day to two months after the procedure. At follow-up (3.2 months to 2.4 years), 8 patients developed significant stent stenosis requiring reintervention. Seven patients developed worsening of preexisting branch PA stenosis. The freedom from reintervention was 89% and 55% at 6 months and 1 year, respectively. Conclusions Patent ductus arteriosus stenting is an attractive alternative to surgical shunt in a majority of patients with duct-dependent circulation. An absolute contraindication to this technique is the presence of branch pulmonary stenosis.

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer® Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer® device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 ± 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.

Background Morquio A syndrome is an autosomal recessive lysosomal storage disease often resulting in life-threatening complications. Early recognition and proficient diagnosis is imperative to facilitate prompt treatment and prevention of clinical complications. Methods Experts in Asia Pacific reviewed medical records focusing on presenting signs and symptoms leading to a diagnosis of Morquio A syndrome. Results Eighteen patients (77% female) had a mean (median; min, max) age of 77.1 (42.0; 0.0, 540.0) months at symptom onset, 78.9 (42.0; 4.5, 540.0) months at presentation and 113.8 (60.0; 7.0, 540.0) months at diagnosis. Orthopedic surgeons and pediatricians were most frequently consulted pre-diagnosis while clinical geneticists/metabolic specialists most frequently made the diagnosis. Delayed diagnoses were due to atypical symptoms for 5 patients (28%), while 4 patients (22%) experienced each of subtle symptoms, symptoms commonly associated with other diseases, or false-negative urine glycosaminoglycan analysis. Two patients (11%) each experienced overgrowth within the first year of life. Two patients with Morquio A syndrome (11%) were diagnosed with craniosynostosis and 1 (6%) for each of Legg-Calvé-Perthes disease, Leri-Weill syndrome, and pseudoachondroplasia. Early radiographic features of Morquio A syndrome led to more efficient diagnosis. Conclusions Increased awareness of clinical symptomology overlapping with Morquio A syndrome is essential. Clinicians encountering patients with certain skeletal dysplasia should consider Morquio A syndrome in their differential diagnosis. Atypical or subtle symptoms should not eliminate Morquio A syndrome from the differential diagnosis, especially for patients who may have non-classical phenotype of Morquio A syndrome.

Background: The Rashkind Umbrella device has proven effective in closing small to moderate size patent ductus arteriosus. A comparable early occlusion rate was also seen in PDA occlusion using Gianturco coils. This study addresses the long-term outcome of both techniques in a single centre. Methods: Review was undertaken of 522 patients with PDA who underwent transcatheter occlusion before January 1997; 244 patients (mean: age 8.0±6.7 years, weight 21.8±12.9 kg, PDA size 3.2±1.0 mm) were intended to receive the Rashkind Umbrella device and the remaining 278 patients (mean: age 6.4±5.6 years, weight 18.6±11.2 kg, PDA size 2.5±1.0 mm) would receive Gianturco coils. Deployment failure occurred in 8 patients (3%) and 6 (2%) during the initial learning curve of the Rashkind Umbrella and Gianturco coil, respectively. In the Rashkind Umbrella group, haemolysis occurred in 6, device embolisation in 3, and 1 patient died from anaesthetic sequelae. Peripheral pulmonary embolisation of coils occurred in 7 patients and all were successfully retrieved. Results: Results were analysed from 228 and 258 patients who had successful Rashkind Umbrella device and Gianturco coil deployment respectively. All had completed at least 1 year follow-up. They were evaluated clinically for residual ductal murmur and echocardiographically for a residual ductal shunt and presence of pulmonary artery or aortic obstruction. The Rashkind Umbrella group had significantly higher residual shunt compared to the Gianturco coil group at various stages of follow-up (p Conclusions: Transcatheter occlusion of PDA using the Gianturco coil is safer and more effective than the Rashkind Umbrella device. It has become the first choice for treating small to moderate size native PDA at our institution.

Transesophageal echocardiography (TEE) is a trusted method of sizing atrial septal defect (ASD) prior to percutaneous closure but is invasive, uncomfortable, and may carry a small risk of morbidity and mortality. Magnetic resonance imaging (MRI) may be useful non-invasive alternative in such patients who refuse or are unable to tolerate TEE and may provide additional information on the shape of the A0SD. To validate the accuracy of ASD sizing by MRI compared with TEE. Twelve patients (mean age 30 years; range 11–60 years) scheduled for ASD closure underwent TEE, cine balanced fast field echo MRI (bFFE-MRI) in four-chamber and sagittal views and phase-contrast MRI (PC-MRI) with reconstruction using the two orthogonal planes of T2-weighted images as planning. The average of the three longest measurements for all imaging modalities was calculated for each patient. Mean maximum ASD length on TEE was 18.8 ± 4.6 mm, mean length by bFFE-MRI was 20.0 ± 5.0 mm, and mean length by PC-MRI was 18.3 ± 3.6 mm. The TEE measurement was significantly correlated with the bFFE-MRI and PC-MRI measurements (Pearson r = 0.69, p = 0.02 and r = 0.59, p = 0.04, respectively). The mean difference between TEE and bFFE-MRI measurements was −1.2mm (95% CI: −3.7, 1.3) and between TEE and PC-MRI was 0.5 mm (95% CI: −1.9, 2.9). Bland–Altman analysis also determined general agreement between both MRI methods and TEE. The ASDs were egg-shaped in two cases, circular in 1 patient and oval in the remaining patients. ASD sizing by MRI using bFFE and phase-contrast protocols correlated well with TEE estimations. PC-MRI provided additional information on ASD shapes and proximity to adjacent structures.

Background: From January, 1997, as part of an international multicentric trial, we have been closing small-to-moderate atrial septal defects within the oval fossa using the Amplatzer Septal Occluder (ASO, AGA Medical). Methods: All patients with defects within the oval fossa deemed potentially suitable for transcatheter closure were investigated by transesophageal echocardiography with the aim of gaining extra information that might alter the decision to use the device to close the defect. Views were obtained in transverse and longitudinal planes, permitting measurements of the diameter of the defect, and its distance from the atrioventricular valves, coronary sinus, and pulmonary veins. Additionally, we sought to identify multiple defects, and to exclude sinus venosus defects. Results: Of 56 patients with left-to-right shunts, 41 (73.2%) were deemed suitable for closure with the Amplatzer Septal Occluder. All underwent the procedure successfully, with no complications. This includes 5 patients with multiple small defects that were sufficiently close to the main defect to be closed with a single device. Only two of these had been detected on the transthoracic study. In the remaining 15 of 56 patients, transcatheter closure was deemed unsuitable. In 9 patients, this was due to the limitation of the size of the device available during the period of study, this representing a relative contraindication. In the remaining 6 (10.7%), transcatheter closure was not performed because multiple defects were too far apart to be closed with a single device in 3 patients, two patients were noted to have a sinus venosus defect, and another was noted to have anomalous connection of the right upper pulmonary vein to the right atrium. Excluding patients contraindicated due to the size of the defect alone, transesophageal echocardiography provided extra information in one-tenth of our patients, which altered the decision regarding management. Conclusion: Transesophageal echocardiography is indispensable in the evaluation of patients undergoing transcatheter closure of atrial septal defect.

Two hundred eleven patients with small- to moderate-sized native patent ductus arteriosus underwent closure using Gianturco coils, employing the transvenous multiple catheter approach. Short-term results showed a high rate of complete occlusion and a potential long-term complication of mild left pulmonary artery stenosis in a small number of patients.

A 10-month-old boy underwent operation to close a large secundum atrial septal defect and multiple muscular ventricular septal defects. Closure of the ventricular septal defects was unsuccessful and led to worsening cardiac failure and inability to wean the patient from mechanical ventilation. Transcatheter closure of the ventricular septal defects using Gianturco coils was undertaken. This technique is an effective alternative for closure of multiple muscular ventricular septal defects in infants and small children.