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Background: When young patients with an oval fossa defect present early with symptoms of heart failure and pulmonary hypertension, surgical treatment is recommended in small bodyweight rather than transcatheter closure., Methods: Outcomes of device closure in consecutive symptomatic children weighing under 10 kg were compared with young children aged below 4 years but weighing above 10 kg. Transthoracic echocardiography under conscious sedation guided the procedure in all without need for balloon sizing, transesophageal echocardiogram, and intubation anaesthesia. Symptoms, anthropometry, shunt ratio, pulmonary pressures, defect and device size, percentage oversizing, device/body weight ratio, complications, and post-procedural growth spurt were compared., Results: Ninety-six patients weighing under 10 kg were compared with 160 patients weighing above 10 kg. In total, 83.3% of patients in the study group and 25% of controls were severely malnourished. The median indexed defect size was 35.2 mm/sq.m and 27.4 mm/sq.m, and the device was oversized by 8.7% and 14.2% in the study group and controls, respectively. The device/body weight ratio was 1.93 in study group and 1.4 in controls. Procedure was successful in all except one patient weighing under 10 kg who had a device embolisation. Both groups showed significant growth spurts and proportion, with severe malnutrition reduced to 42% and 11% in the two groups., Conclusions: Device closure was feasible and safe in patients under 10 kg. Transthoracic echocardiographic imaging on conscious sedation provided adequate guidance. Symptoms and growth significantly improved after intervention. Despite a larger defect size, smaller patients had comparable outcomes. In symptomatic children under 10 kg needing early closure, transcatheter intervention should not be deferred.

While infradiaphragmatic total anomalous pulmonary venous drainage to portal vein is well described, hemianomalous drainage of right pulmonary veins to portal vein in Scimitar syndrome has not yet been reported.

Background: Device embolisation is a serious adverse event during transcatheter duct closure. This study analyses risk factors for embolisation., Methods: Demographic parameters, echocardiographic anatomy, haemodynamics, and procedural characteristics of consecutive duct closures in a tertiary centre over 8 years were analysed. Procedures complicated by embolisation were compared to uncomplicated procedures., Results: Fifteen embolisations occurred during 376 procedures. All except one embolisation were in infants. The pulmonary artery: aortic pressure ratio was 0.78 ± 0.22. Embolisation was seen significantly more commonly in Type C tubular ducts. Vascular plugs were more significantly associated with embolisations. Logistic regression analysis showed device embolisation was significantly higher in age group of < 6 months compared to 6-12 months (p = 0.02), higher in those with tubular ducts versus conical ducts (p = 0.003), use of vascular plugs compared to conventional duct occluders (p = 0.05), and in duct closure with undersized devices (p = 0.001). There was no in-hospital mortality. Three patients needed surgical retrieval while others were successfully managed in catheterisation laboratory., Conclusions: Device embolisation complicates 4% of transcatheter duct closures, with need for surgery in one-fifth of them. Larger ducts with high pulmonary artery pressures in younger and smaller infants are more often associated with device embolisation. Tubular ducts are more prone for embolisation compared to usual conical ducts. Softer vascular plugs are often associated with embolisations. Intentional device undersizing to avoid vascular obstruction in small patients is a frequent risk factor for embolisation. Precise echocardiographic measurements, correct occluder choice, proper technique and additional care in patients with high pulmonary artery pressures are mandatory to minimise embolisations.

Severe tricuspid regurgitation in corrected transposition of great arteries is treated with early bioprosthetic replacement to prevent right ventricular failure. When bioprosthesis degenerates, surgery on cardioplegic arrest further complicates right ventricular function. We report the first transcatheter valve-in-valve implantation of tricuspid valve in a systemic right ventricle in corrected transposition describing the modifications due to anatomical differences in these patients.

Acute stent thrombosis may complicate neonatal arterial duct stenting for reduced pulmonary blood flow. Thrombolytic agents recanalise the clot but may cause bleeding around the vascular sheaths and other sites. Since early thrombus is platelet mediated, intravenous platelet glycoprotein inhibitor like eptifibatide is likely to be effective, but rarely utilised in neonates. Ductal stent thrombosis treated with eptifibatide is reported.

Right pulmonary artery to left atrial fistula is classified based on the right pulmonary artery branching, individual right pulmonary venous drainage, and presence of an aneurysmal segment. A rare association with scimitar syndrome and right lung devoid of blood supply from right pulmonary artery is described in this report. The anatomical and management differences between the different types are highlighted.

Background: Shape-memory abnormalities are seen in some nitinol atrial septal occluders. Variably described as cobra-head, tulip, and others, their incidence, mechanisms, clinical impact, and outcome have not been systematically analysed., Methods: We retrospectively reviewed all consecutive device closures in the last 6 years for deformations. Type and size of the occluder, deployment technique, size, and angulation/kinking of the delivery sheath were analysed. Procedural success, duration, and other complications were studied., Results: A total of 112 devices (11.8%) among 950 occluders used in 936 patients showed deformities. Fourteen of 936 received 2 devices. Deformities were transient and self-correcting in 40%. Multivariate analysis showed significant associations with oversized sheaths (p = 0.004), kinked/angulated sheaths (p < 0.001), special deployment techniques (p < 0.001), and twist in the device waist (p = 0.011). Despite more frequent deformities with Figulla (15.6%) and Amplatzer (13.9%) occluders than Cera occluders (6.6%) and larger devices (>24 mm - 14.6%) than smaller devices (less than or equal to 24 mm - 9.7%), they were not significant on multivariate analysis. In vivo manipulations corrected most deformities; nineteen needed in vitro reformations and four needed a change of device. Despite prolongation of the procedure, repeated attempts (mean 2.76 ± 1.7 attempts, with a range from 1 to 9 attempts), and supraventricular tachycardia in two patients, there were no serious adverse effects., Conclusions: Deformations were frequent in 11.8% of atrial septal occluders on a targeted search. Oversized and angulated/kinked sheaths, special techniques like pulmonary vein deployment and twist in device waist during procedure predisposed to deformities. While most deformities were corrected with manipulations, removal of the device was infrequently needed and change of device was rarely required. Long procedural time and multiple attempts for deployment did not affect procedural success.

Objectives: Ductal stents, right ventricular outflow tract stents, and aortopulmonary shunts are used to palliate newborns and infants with reduced pulmonary blood flow. Current long-term outcomes of these palliations from resource-restricted countries are unknown., Methods: This single-centre, retrospective, observational study analysed the technical success, immediate and late mortality, re-interventions, and length of palliation in infants ≤5 kg who underwent aortopulmonary shunts, ductal, and pulmonary outflow stents. Patients were grouped by their anatomy., Results: There were 69 infants who underwent one of the palliations. Technical success was 90% for aortopulmonary shunts (n = 10), 91% for pulmonary outflow stents (n = 11) and 100% for ductal stents (n = 48). Early mortality within 30 days in 12/69 patients was observed in 20% after shunts, 9% after pulmonary outflow stents, and 19% after ductal stents. Late mortality in 11 patients was seen in 20% after shunts, 18% after outflow stents, and 15% after ductal stents. Seven patients needed re-interventions; two following shunts, one following outflow stent, and four following ductal stents for hypoxia. Among the anatomical groups, 10/12 patients with pulmonary atresia, intact ventricular septum survived after valvotomy and ductal stenting. Survival to Glenn shunt after ductal stent for pulmonary atresia, intact ventricular septum and diminutive right ventricle was very low in two out of eight patients, but very good (100%) for other univentricular hearts. Among 35 patients with biventricular lesions, 22 survived to the next stage., Conclusions: Cyanotic infants, despite undergoing technically successful palliation had a high inter-stage mortality irrespective of the type of palliation. Duct stenting in univentricular hearts and in pulmonary atresia with an intact ventricular septum and adequate sized right ventricle tended to have low mortality and better long-term outcome. Completion of biventricular repair after palliation was achieved only in 63% of patients, reflecting unique challenges in developing countries despite advances in intensive care and interventions.

Anomalous origin of the left coronary artery from the pulmonary artery causes heart failure and death in infancy. In rare adult survivors with well-developed collaterals, surgical left coronary ligation to arrest steal is often combined with bypass grafting. Transcatheter left coronary artery closure in a symptomatic adult as an alternative to surgical ligation resulted in complete resolution of inducible ischaemia on myocardial perfusion imaging.

Large coronary artery fistulas draining to the left heart structures causing heart failure are very rare. Interventional closure of such fistulas is limited to isolated reports, often in asymptomatic patients. Technical differences in these interventions include either deep arterial cannulation till their exit or transseptal arteriovenous circuit formation. Transcatheter closure of three large symptomatic fistulas in small children is reported.

Abernethy malformation connecting to a supradiaphragmatic vein may be missed due to their rarity or lack of awareness. Embryological reasons explain the involvement of the left superior caval vein in these connections.

Systemic right ventricular function in congenitally corrected transposition depends on septal geometry. Suprasystemic left ventricular systolic pressures and high end-diastolic pressures after Fontan surgery paradoxically preserve right ventricular function.

Isolated subclavian artery is a rare anomaly. A second steal due to a patent arterial duct further reduces arm perfusion. Surgical anastomosis of the isolated vessel to aorta normalises arm perfusion. Simple echocardiographic clues aid in the diagnosis. An associated moderate sized ventricular septal defect was non-surgically closed along with catheter closure of the duct resulting in improved arm perfusion.

Communications between the pulmonary artery and left atrium cause cyanosis. The images document serial haemodynamic changes in such a fistula from fetal life to the postnatal period with a successful transcatheter intervention.

Abernethy malformations manifest as hepatopulmonary syndrome, pulmonary vasculopathy, or encephalopathy. A novel intervention in a child with portosystemic shunt and inferior caval vein hypoplasia led to complete normalisation of hypoxia and relief of obstruction in the inferior caval vein. Embryological explanations of venous anomalies may indicate that inferior caval vein anomalies are frequent but under-recognised in patients with Abernethy malformation.

A neonatal ductal stent deployed in a straight short conical duct on second postnatal day migrated owing to inadequate ductal constriction. It was successfully retrieved using a larger balloon and redeployed in the duct again. Intravenous indomethacin prevented further stent migration. This is the first report of successful transcatheter retrieval and repositioning of a migrated expanded neonatal ductal stent.

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.

When the inferior caval vein is occluded or abnormal, jugular and hepatic veins provide alternative routes for interventions. For pulmonary artery stenting, transhepatic access may give a relatively straighter route than that from the jugular veins. We describe the challenges and strategies during transhepatic bilateral pulmonary artery stenting after arterial switch operation complicated by occluded inferior caval vein and congested hepatic veins.

Isolation of the subclavian artery is associated with "steal" of blood from the carotid circulation to the arm, through the circle of Willis and the vertebral artery. When associated with a patent arterial duct, there is an additional "steal" of blood from the arm to the lungs, through the arterial duct because of the lower pulmonary vascular resistance. When this combination manifests clinically with arm ischaemia on the side of the isolated subclavian artery, closure of the arterial duct will prevent the "steal" of blood from the subclavian artery to the pulmonary artery and may improve the blood flow to the arm. We report three patients with this unusual combination of the "steal" phenomenon that improved after interventional closure of the arterial duct. This report discusses the embryological basis of the defect, clinical and echocardiographic clues to diagnose this unusual anomaly, angiographic findings, and transcatheter management options.

Anatomically corrected malposition is characterized by concordant atrioventricular and ventriculoarterial connection and parallel arrangement of arterial trunks. Left juxtaposition of the atrial appendages, a frequent association of this anomaly, alters the atrial septal plane. In this report, I describe the insertion of a device to close an atrial septal defect, as guided by transoesophageal echocardiography.

Background: Arterial access is traditionally considered mandatory during coil occlusion of the patent arterial duct. Arterial access necessitates heparinization and carries the risk of femoral artery occlusion in small children., Methods and Results: Between July 1999 and May 2001, we attempted coil occlusion of patent arterial ducts in 104 patients without arterial access. The patients were aged from 3 months to 14 years. The median age was 2 years. They weighed 3-35 kg. The median weight was 9.8 kg. The duct diameter at pulmonary artery insertion was 1.8-3.5 mm. The patients were selected on basis of echocardiographic evaluation of duct diameter at pulmonary artery insertion and morphology of the ampulla. Doppler color flow imaging was used in the catheterization laboratory to confirm duct closure. Arterial access was required in 21 patients. The reasons included accidental puncture in 5 patients, failure to obtain venous access in 1 patient, aortic embolization in 3 patients, poor echo images in 2 patients, requirement for additional coils in 8 patients and, failure to cross the duct from pulmonary artery in 2 patients. The fluoroscopic time ranged from 2.2 to 20 min with a mean of 5.3+/-3.8 min. Immediate closure was achieved in 98 patients and this included 79 of the 83 patients in whom arterial access was avoided. Color Doppler 3-24 h later showed residual flow in 2 patients. Four patients had new-onset left pulmonary artery turbulence with peak gradients below 5 mm of mercury. Coil embolization occurred in 6 patients and all coils were retrieved. Three-month follow up information was available for 78 patients. Small residual ductal leaks were seen in 4 patients, 2 of whom had leaks at 24 h. Two patients had recanalized their ducts., Conclusion: It is feasible to occlude small patent arterial ducts with coils using venous access alone in carefully selected patients with excellent immediate and short-term results.