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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.

Objectives We present a first-in-man clinical use of a new hybrid design stent in stenosed large vessels. Its unique C and S polylinks prevent foreshortening without compromising its strength. Its thin profile permits use of smaller introducer sheaths. Background Stent angioplasty is widely employed in large vessel and conduit stenosis. These procedures are associated with difficulties due to large stent profiles, stent fractures, foreshortening and recoil. Cobalt chromium stents have high tensile strengths compared to stainless steel stents. Methods A retrospective analysis of feasibility and safety of a new Cobalt chromium stent in large vessels namely aorta, pulmonary arteries and outflow conduits was done from two institutions. Demographic patient details, procedural results, complications and medium term follow-up were analyzed. Stent recoil, foreshortening, fractures were assessed. Results Twenty patients including three with aortic coarctations, seven with stenosed conduits and 10 with pulmonary artery stenosis underwent stent angioplasty using 23 stents. Three stents were deployed to expand further a previously implanted stent. Procedure was successful in all patients, lumen increased by 150-300%, gradients reduced in all patients. There was no stent recoil, foreshortening or fractures. There were no complications. At a follow up of 3-27 months, there were no stent related complications and the gradients remained stable. Conclusions The new Zephyr stent was useful in a wide variety of stenotic lesions involving large vessels including those that were previously stented. Lack of stent recoil and foreshortening seems to be an advantage for this new stent that needs validation in larger multicenter studies.

Objectives To assess the initial procedural and short to medium-term experience with the straight design of the Venus P-valve™ (Venus MedTech, Hangzhou, China) in dysfunctional right ventricular outflow tracts (RVOT). Background The Melody™ valve (Medtronic, Minneapolis, Minnesota) has been the only percutaneous valve option for smaller RVOT conduits. The straight Venus P-valve™ may provide an alternative to the Melody™ valve. Methods Retrospective data collection of patient characteristics, procedural data, clinical and imaging follow-up of the straight Venus P-valve™. Results Nine patients (four female) with a mean age of 23.1 ± 7.5 years and a mean weight of 72.7 ± 29.4 kg underwent straight Venus P-valve™ implantation between 03/2014 and 06/2016. All patients had right ventricle-to-pulmonary artery conduits which were pre-stented before the valve implantation. All valves were deployed successfully without any significant procedural complications. During the mean follow-up of 24 ± 9.1 months, there were no valve related re-interventions or deterioration in valve performance. There was one case of insignificant, single wire frame fracture and no cases of endocarditis. The cohort demonstrated a reduction in pulmonary regurgitation and tricuspid regurgitation, which was sustained throughout the follow-up. Similarly the gradient across the RVOT tract did not significantly increase. Conclusions Implantation of the straight Venus P-valve™ has provided satisfactory short to mid-term results with high success rates and no complications and may be considered as an alternative option in patients with RVOT dysfunction.

Congenital heart defects occurring together with upper limb malformations as a group of dominantly inherited disorders constitute the Heart-Hand syndrome (HHS) with varied phenotypic manifestations...

Complex cyanotic congenital heart diseases with left isomerism are sometimes associated with atrioventricular nodal conduction disturbances that may need permanent pacing. Surgical palliation in such anatomy connecting the superior vena cava to the pulmonary artery precludes a transvenous access for an endocardial pacing lead to the ventricles. Epicardial leads in these patients fail if the pacing thresholds are very high. We report transhepatic permanent ventricular lead implantation for a young boy with heterotaxy complicated by complete heart block.

Coronary sinus defects refer to interatrial communications that lie out of the confines of the atrial septum and leads from left to right shunt through the ostium of the coronary sinus. When associated with persistent left superior vena cava (PLSVC), mild systemic desaturation may occur depending on the extent of unroofing of the coronary sinus. Isolated defects without PLSVC are rare. Three-dimensional echocardiographic and surgical images are presented.

Background : Balloon-expandable pulmonary valves are usually not suitable for dilated native outflow tracts. Methods : Indian Venus P-valve registry was retrospectively analyzed for efficacy, complications, and midterm outcomes. Straight valve was used in prestented conduits in patients with right ventricular pressure above two-thirds systemic pressure and/or right ventricular dysfunction. Flared valve 1–4 mm larger than balloon waist was used in native outflow in symptomatic patients, large ventricular volumes, and ventricular dysfunction. Objectives : A self-expanding porcine pericardial Venus P-valve is available in straight and flared designs.. Results : Twenty-nine patients were included. Straight valve was successful in all seven conduits, reducing gradients significantly, including one patient with left pulmonary artery (LPA) stent. Flared valve was successfully implanted in 20 out of 22 native outflow tracts. Sharp edges of the older design contributed to two failures. Complications included two migrations with one needing surgery, endocarditis in one, insignificant wire-frame fractures in three, and groin vascular complication in one patient. There were no deaths or valve-related reinterventions at a mean follow-up of 47.8 ± 24.5 months (1–85 months). Modifications of technique succeeded in three patients with narrow LPA. There was significant improvement in symptoms, right ventricular volume, and pulmonary regurgitant fraction. Conclusion : Straight and flared Venus P-valves are safe and effective in appropriate outflow tracts. Straight valve is an alternative to balloon-expandable valves in stenosed conduits. Flared valve is suitable for large outflows up to 34 mm, including patients with LPA stenosis. Recent design modifications may correct previous technical failures. Studies should focus on durability and late complications.

Duct dependent aortic arch obstruction with borderline left ventricular hypoplasia presents a diagnostic dilemma. If the left ventricle (LV) is adequate to sustain systemic cardiac output without a patent duct, arch obstruction is relieved surgically and duct is divided. Inadequate LVs do not tolerate duct division, and these patients need more complex Norwood type surgeries. However, catheter-based interventions for arch obstruction can retain ductal patency. The progressive changes in anatomy and physiology of a neonate who presented with hypoplastic left heart structures and patent duct with advancing age and modified by serial catheter-based interventions are presented to highlight the use of interventions in this therapeutic dilemma.

Tuberous sclerosis (TSC) is an autosomal dominant multisystem disease associated with multiple cardiac Rhabdomyomas; however, congenital cardiac malformations are very rare in TSC. A 5-year-old girl with classical features of TSC had an associated large secundum atrial septal defect (ASD). The left to right shunt through the ASD was augmented by the multiple left ventricular masses. Nonsurgical closure of the ASD avoided the neurological and cardiac complications that may occur due to cardiopulmonary bypass during open-heart surgery. This first novel interventional report in TSC stresses on the value of such catheter interventions to mitigate risks in complex cardiac associations.

Fontan surgery and its modifications have improved survival in various forms of univentricular hearts. A regular atrial rhythm with atrioventricular synchrony is one of the most important prerequisite for the long-term effective functioning of this preload dependent circulation. A significant proportion of these survivors need various forms of pacing for bradyarrhythmias, often due to sinus nodal dysfunction and sometimes due to atrioventricular nodal block. The diversion of the venous flows away from the cardiac chambers following this surgery takes away the simpler endocardial pacing options through the superior vena cava. The added risks of thromboembolism associated with endocardial leads in systemic ventricles have made epicardial pacing as the procedure of choice. However challenges in epicardial pacing include surgical adhesions, increased pacing thresholds leading to early battery depletion and frequent lead fractures. When epicardial pacing fails, endocardial lead placement is equally challenging due to lack of access to the cardiac chambers in Fontan circulation. This review discusses the univentricular heart morphologies that may warrant pacing, issues about epicardial pacing, different techniques for endocardial pacing in patients with disconnected superior vena cava, pacing in different modifications of Fontan surgeries, issues of systemic thromboembolism with endocardial leads, atrioventricular valve regurgitation attributed to pacing leads and device infections. In a vast majority of patients following Glenn shunt and Senning surgery, an epicardial pacing and lead replacement is always feasible though technically very difficult. This article highlights the different options of transatrial and transventricular endocardial pacing., Highlights • Fontan surgery prolongs the mean survival of patients with univentricular hearts beyond 30 years. • 9-20% of survivors need permanent pacing for bradyarrhythmias. • Epicardial pacing is preferred as the first choice for permanent pacemaker. • There is 20-40% failure of permanent pacing at 10 years due to high threshold, lead fractures. • Repeated epicardial pacing with surgical revisions are difficult. • Endocardial pacing involves special techniques. • Manoeuvring through fenestrations,venous collaterals, hepatic veins, pulmonary valve or hybrid peratrial routes is needed. • Systemic thromboembolism, endocarditis and systemic AV valve regurgitation should be prevented by meticulously measures.

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.

Background Fatal mechanical complications of acute myocardial infarctions include free wall rupture and ventricular septal rupture. If pericardial adhesions wall off a free wall rupture, it may lead to formation of pseudoaneurysms that are characterized by a narrow mouth. Even though pseudoaneurysms are common after myocardial infarctions, they may also occur following surgery, trauma, and infections rarely. Case summary We present a case of a 62-year-old man who developed a left ventricular pseudoaneurysm 2 weeks after thrombolysis for an acute inferolateral myocardial infarction. Multiple non-invasive imaging modalities demonstrated the anatomy, regional and global ventricular function, distortion of mitral annulus by the eccentric large aneurysm. Pericardial scars after a previous coronary bypass surgery contained this left ventricular free wall rupture and helped in providing a safe window period for corrective surgery. Discussion While left ventricular pseudoaneurysms that develop following myocardial infarctions warrant emergency surgery due to the high impending chances of rupture and tamponade, previous surgical pericardial adhesions guarded against an imminent collapse. Multimodality imaging of the aneurysm helped in planning the surgical strategy.

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.

OBJECTIVES To study the feasibility of closure of large atrial septal defects (ASDs) using occluder devices >38 mm and assess the midterm complications. BACKGROUND Feasibility and safety of large occluders >38 mm and their follow-up are largely unknown. METHODS All patients with ASDs closed using devices >38 mm were retrospectively analyzed. Since outcome of patients receiving 40 mm devices were known before, patients receiving 40 mm devices were compared with those receiving 42-46 mm devices on demographic, hemodynamic parameters and procedural characteristics. RESULTS A total of 17 patients with 40-mm device and 31 patients with >40-mm device formed the cohort. The mean echocardiographic defect size was 36.3 ± 3.7 mm. In total 19 patients had deficient retroaortic margin; inferior margin was deficient in one. Larger defects needed special deployment techniques more often. There was acute technical success in all patients. Two procedures failed; one device embolization next day in a patient with deficient inferior margin needed surgery. Another elderly patient with restrictive left ventricular physiology died of multiorgan dysfunction. Two late deaths on follow-up were unrelated to the procedure. All other patients had symptom improvement. There were no erosions, thromboembolism or valvar regurgitation at a median follow-up of 39 (1-60) months. Apart from late-onset atrial flutter in one, there were no arrhythmias on follow-up. CONCLUSIONS Occluders larger than 38 mm in appropriately selected patients are feasible, successful and safe in majority and often require special deployment techniques. Complications during and after the procedure were infrequent. Very large defects with any deficient margin except retroaortic rim should be excluded from device closure.

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.

An antenatal referral for fetal echocardiography at 20 weeks of gestation with the suspicion of a cyst in the right atrium led to the diagnosis of an aorta to right atrial communication in a fetus. It was considered to be right coronary to right atrial fistula by fetal echocardiography. There was no other fetal anomaly and the cardiac hemodynamics remained stable throughout pregnancy. Postnatally, there was a progressive enlargement of the tunnel that led to a decision for intervention. Aortography showed an anterior aorta to right atrial tunnel, which was occluded in the catheterization laboratory. We describe in this report, the antenatal findings noted in ultrasound imaging along with the postnatal presentation and management of this rare congenital anomaly.

Background: Tetralogy of Fallot (TOF) with subarterial ventricular septal defect (VSD) is more common among Asians than Caucasians. Compared with the regular subaortic VSD postoperative right ventricular outflow obstruction is more common because of the sub-pulmonary extension of the defect. The objective of this study is to analyze the surgical implications and outcomes of patients with TOF - subarterial VSD in the current era. Patients and Methods: In all, 539 consecutive operated patients with TOF from May 2005 to September 2012 were retrospectively reviewed. Eighty-five patients had subarterial VSD. Seventy-nine of these underwent intracardiac repair. Preoperative clinical, echocardiographic features, operative and postoperative variables were assessed. Results: The median age at surgery was 6 years and the median weight was 14 kilograms. The male to female ratio was 1.7:1. TOF with subarterial VSD was associated with frequent use of transannular patch (74.6%). The early mortality was 2.5%. Follow up was 92% complete with a mean duration of 20 months with actuarial survival of 97.3% at 5 years. Two patients required reoperation for significant right ventricular outflow tract obstruction (RVOTO) at one year and three years, respectively. Conclusions: Intra cardiac repair for TOF with subarterial VSD has low perioperative mortality and morbidity. Transannular patch augmentation of the right ventricular outflow tract (RVOT) is required in a significant proportion of these patients. Precise suturing of the VSD patch, adequate infundibular resection and lower threshold for a transannular patch placement ensures a smooth early postoperative recovery.

Objectives: Narrowed right ventricular (RV) outflow conduits and pulmonary arteries (PA) increase RV pressures and warrant interventions. Stent angioplasty is an alternative to more morbid redo-surgery in developing countries. We evaluate the efficacy and safety of stenting and assess need for redo-surgical reinterventions on midterm follow-up after stent angioplasty. Methods: Patients who underwent conduit, main PA and bilateral branch PA stenting for elevated RV pressures were analyzed retrospectively. Success was defined as 20% reduction in RV pressures or RV-aortic pressure ratio; 50% reduction in gradients or 50% increase of luminal diameter. Procedural results, complications and need for redo surgeries on follow-up were assessed. Results: Among 60 patients aged 1–46 years, 57 were post-operative patients, who needed stenting at a median period of 48 months after surgery. Stenting succeeded in 98% and reduced RV pressures from 105.42 ± 28.39 mmHg to 54.46 ± 16.89 mmHg. Direct major procedural complications in five (8%) patients included procedural failure in one, stent migration in three and lung hemorrhage in one. None of the stented conduits needed a surgical change on a follow-up ranging 3–120 months. Following bilateral PA stenting in twenty-four patients, only two needed a repeat open-heart surgery during follow-up ranging 3–108 months. Catheter reinterventions on follow-up included elective percutaneous pulmonary valve implantation in nine patients and stent redilation in seven patients. Conclusions: Stent angioplasty was safe and effective. Surgery was postponed in all stenosed conduits. Elective redilation of stents after bilateral PA stenting may be needed for somatic growth; but open-heart repeat surgeries can be avoided in a majority. Keywords: Stent angioplasty, Conduit stenting, Pulmonary artery stents, Bifurcation stenting

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.

Objectives A novel Occlutech atrial flow regulator (AFR) implantation gives an atrial septal predefined predictable fenestration. Background Atrial septostomy relieves syncope in pulmonary arterial hypertension (PAH) by improving left heart filling, cardiac output and systemic oxygen transport despite hypoxia. Symptoms recur when small fenestrations close spontaneously. Methods AFR was implanted after informed consent in patients with severe PAH presenting with syncope and right heart failure. Symptoms, hemodynamics, echocardiographic parameters, brain natriuretic peptide (BNP) levels and device patency were serially documented. Results Twelve patients aged 28.3 ± 8.5 years with severe PAH underwent AFR implantation. All procedures were successful without any major complications. All patients had relief of syncope and 6-min walk distance improved significantly from 377.3 ± 33.2 to 423 ± 31.32 m. The cardiac index (2.36 ± 0.52 to 2.89 ± 0.56 L/min/m2) and systemic oxygen transport (367.5 ± 75.5 to 428.0 ± 67.1 ml/min/m2) also showed a significant improvement. Inferior caval vein congestion and pericardial effusion reduced due to improvement in heart failure, but other echocardiographic parameters of right ventricular function did not show significant change. The reduction in BNP levels too did not reach statistical significance. The device was patent in all patients at a median follow-up of 189 days (range 10–296 days) resulting in a significant reduction of oxygen saturations from 98 ± 0.18 to 85.26 ± 2.86% after exercise. Conclusions AFR implantation was feasible and safe in all patients with PAH. There was a significant improvement of symptoms, six-minute walk distance, cardiac index and systemic oxygen transport. The device maintained patency in short-term follow-up and the resultant hypoxia was tolerated well.

Fontan surgery streamlines the systemic venous return through the pulmonary circulation before filling the systemic ventricle in univentricular hearts. The venous congestion leads to effusions, lowers cardiac output, and affects organ perfusion. Fenestrations in the Fontan circuit improve forward flow through the ventricles, lower venous pressures, and reduce perioperative morbidity. When large fenestrations cause profound hypoxia and effort intolerance, there are no current techniques to reduce their size. Atrial flow regulators with a predetermined orifice were used off-label in three borderline patients with large undesirable fenestrations following extracardiac conduit Fontan surgeries. This resulted in improved oxygenation without marked elevation of venous pressures, while retaining the patency of the decompressive fenestration.

Promoting a fish as a flagship species (i.e. a charismatic animal that promotes awareness) is a management tool to improve the effectiveness of conservation measures. However, to be successful this approach requires strong stakeholder support. To investigate the feasibility of the flagship species approach and degree of stakeholder support in India, semi-structured interviews were conducted with forest managers, anglers and village members in Uttarakhand. Stakeholders were supportive of the flagship approach and the golden mahseer was favoured because of economic benefits associated with recreational angling.

Paravalvular prosthetic leak (PVL) leads to hemolysis, heart failure, and increased risk of infective endocarditis. Transcatheter closure (TCC) of PVL is an alternative to surgery; however, it is challenging due to varied anatomy. Retrospective analysis of patients undergoing TCC for PVL over a 6

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.

Tetralogy of Fallot with absent pulmonary valve syndrome is associated with absence of the left pulmonary artery in one-seventh of cases. Infants with this disorder commonly present very early in life with respiratory distress and heart failure. Because the pulmonary blood flow is primarily to the right lung only, severe respiratory distress occurs. Survival to late childhood without surgery is very rare due to clinical worsening in infancy. This report describes two adult natural survivors with this rare association who presented with extremely unusual unreported clinical features. The aneurysmally dilated main pulmonary artery was compressed on the left coronary ostium, causing angina in the first patient. The second patient had recurrent ventricular tachycardia originating from the dilated thinned out right ventricular outflow tract. Both patients had successful surgical correction with valved xenograft conduits.

Background: Older patients with combination of aortic coarctation and large patent ductus arteriosus can be managed with transcatheter interventions. The strategies depend on anatomy of coarctation and size of ductus arteriosus. Methods: We present three different patients with this combination. The anatomic factors like isthmic hypoplasia, dilatation of post coarctation descending aorta and size of ductus arteriosus were noted. Results: Patients with isthmic hypoplasia needed stent angioplasty of the coarctation. If there is no dilatation of post coarctation aorta, a single covered stent excluded the ductus arteriosus and relieved the coarctation gradients. Dilated post coarctation aorta precluded a covered stent and warranted closure of duct with occluder device and stent angioplasty of coarctation. When there is a good sized aortic isthmus in a discrete membranous coarctation, device closure of the duct and balloon aortoplasty was successful. Conclusions: In coarctation with patent ductus arteriosus associated with good sized aortic isthmus, closure of duct with duct occluder device and balloon aortoplasty would correct the lesions. If there is isthmic hypoplasia, device closure of the duct and stenting of the coarctation is needed. Covered stent is a reasonable alternative only in presence of non dilated descending aorta.

Introduction. Ductal stent (DS) in duct-dependent pulmonary circulation is less morbid than neonatal Blalock–Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair. Methods. This is a retrospective review of clinical follow-up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion. Results. Among 22 infants, four Group A patients followed for 26–54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short-term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5–14 months at a body weight of 5–7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6–8.5 kg after 8–15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow-up for hypoxia. Four patients had sudden death. Conclusions. The short-term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%.

Duplication of atrioventricular valves involves the mitral valve more often than the tricuspid valve and is often associated with other cardiac defects. Double-orifice tricuspid valve (DOTV) is often identified in surgery or autopsy and missed on echocardiography, as the two orifices are orthogonal to the imaging plane. If suspected on echocardiography, it masquerades as mild tricuspid hypoplasia. Three-dimensional echocardiography and magnetic resonance imaging of a DOTV are presented.

Conventional repair of the double-outlet left ventricle involves a complex intraventricular tunnel, extracardiac conduit, or the pulmonary artery translocation operation. We report an arterial switch operation and closure of ventricular septal defect for anatomic correction of this anomaly.

Pulmonary hypertension (PH) is a chronic progressive disease with mean pulmonary arterial pressure (MPAP) >25 mmHg, progressing to right ventricular dysfunction, heart failure and death. Medical management remains challenging with unpredictable outcomes. The efficacy of an interatrial shunt in

While aortico-right atrial tunnels with left to right shunt from aorta to right atrium are reported widely, pulmonary artery to right atrial tunnels have not been described so far. Such a tunnel will lead to a circular shunt with a recirculation of blood in the right sided cardiac chambers repeatedly bypassing the pulmonary capillary bed. This newly described pulmonary artery to right atrial tunnel was closed nonsurgically with a duct occluder after angiographic delineation.

All giant Kawasaki aneurysms may not regress fully; some may eventually calcify, undergo thrombosis, and get detected in asymptomatic adults at later age. Tomisaku Kawasaki initially described this illness as mucocutaneous lymph node syndrome in childhood in 1967 and coronary arteritis was recognized later. We present a 58-year-old male, possibly one of the oldest surviving patients with giant coronary aneurysms who presented with large secundum atrial septal defect (ASD) with heart failure. This indicates that the disease was perhaps prevalent outside Japan even before the first Kawasaki's description.

Epicardial pacing lead fixation is employed in patients with cavopulmonary anastamosis (Glenn shunts) when they need permanent pacing. Epicardial pacing in these patients may malfunction due to high pacing thresholds or diaphragmatic pacing. A novel technique of transatrial insertion of two endocardial screw-in pacing leads through right anterolateral minithoracotomy could achieve synchronous atrioventricular pacing in a patient with Ebsteins anomaly with symptomatic sinoatrial and atrioventricular nodal disease.

Between June 1999 and September 2002, 45 patients (age, 34 +/- 13 years; mean shunt ratio, 2.6 +/- 0.6) underwent transcatheter atrial septal defect (ASD) closure at our institution with the Amplatzer septal occluder (mean device size, 31.4 +/- 3 mm). Patients were selected by transesophageal echocardiography. The mean ASD dimension was 25.3 +/- 3.7 mm and 33 (73%) patients had deficient anterior rim. Specific procedural details included the use of 13 or 14 Fr introducer sheaths and the right upper pulmonary vein approach if the conventional approach failed. There were two procedural failures, with device embolization in both (surgical retrieval in one, catheter retrieval in one). During follow-up (3-30 months; median, 16 months), one patient (59 years) with previous atrial flutter had pulmonary embolism and was managed with anticoagulation. Two patients developed symptomatic atrial flutter. Fluoroscopy time was 31.6 +/- 19.5 min for the first 22 cases and 19.6 +/- 11.4 min for the rest (P = 0.04). Transcatheter closure of large ASDs is technically feasible but careful long-term follow-up is needed to document its safety.

Giant coronary artery aneurysms that occur in 0.5 to 1% of patients with Kawasaki disease can be fatal if associated with thrombosis. Some patients may show persistent inflammation and fever despite treatment with repeated doses of intravenous immunoglobulin (IVIG), steroids, and aspirin. This report describes an infant boy with resistant Kawasaki disease who presented with extensive coronary artery involvement and coronary thrombosis. His inflammation was not controlled with multiple doses of IVIG, parenteral and oral steroids, or high-dose aspirin, and he finally needed infliximab, a monoclonal antibody against tumor necrosis factor alpha. The progression of coronary thrombosis was arrested by the platelet glycoprotein 2b/3a receptor blocker, abciximab, during the acute phase of the disease.

Surgical aortopulmonary shunts palliate neonates with duct-dependent pulmonary circulation. The surgical problems included prolonged mechanical ventilation and intensive care stays, bleeding and transfusions, frequent use of multiple inotropes, pulmonary complications, sepsis, and injury to surrounding structures like phrenic nerve, recurrent laryngeal nerve, and thoracic duct. Ductal stenting (DS) provides a nonsurgical attractive alternative option to surgical aortopulmonary shunts. On follow-up after both procedures, there is a progressive fall in oxygen levels due to intimal ingrowth within the ductal stents and fibrointimal peel formation and thrombus within surgical shunts. 5–20 % of patients suddenly die on follow-up due to shunt or stent thrombosis. While the surgical shunts offer a longer palliation of few years, DS gives longevity of only 6–12 months. This difference in duration of palliation will influence patient selection for DS.

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.