Transcatheter device closure of perimembranous ventricular septal defect in children treated with prophylactic oral steroids: acute and mid-term results of a single-centre, prospective, observational study.

UNLABELLED: Background and Objective Although transcatheter closure of perimembranous ventricular septal defect is emerging as an accepted, viable alternative, conduction disturbances still remain a major concern. Although steroid treatment has shown encouraging results with complete recovery, efficacy of prophylactic use of steroids is still speculative. We aim to study the mid-term outcome of perimembranous ventricular septal defect closure in children who received prophylactic oral steroids. Materials and methods A prospective study was designed and antegrade device closure was attempted in eligible children who met the following inclusion criteria: age 3-18 years and weight >10 kg, defect diameter ⩽12 mm, and symptomatic, haemodynamic changes or history of infective endocarditis. Prophylactic steroid protocol consisted of 2 weeks oral prednisolone (1 mg/kg/day) initiated immediately after the procedure, and in the event of bradyarrhythmia it was escalated to 2 mg/kg. Patients were regularly followed-up at 1, 6, and 12 months and then annually. Patients with post-procedure heart block underwent Holter monitoring after a minimum of 1 year interval. RESULTS: Between May, 2007 and August, 2012, successful device closure was accomplished in 290/297 patients. Mean age and weight were 9±3.12 years and 21±8.27 kg, respectively. The defect measured 5±1.38 mm on echocardiography. Mean fluoroscopy time was 12.98±8.64 minutes. Eight patients with major complications included one each with device embolisation, haemolysis, severe aortic regurgitation, and five with bradyarrhythmias, including complete atrioventricular block in three, Mobitz II in one, and bifascicular block in one. Patients with complete atrioventricular block responded to high-dose steroid and temporary pacemaker. Minor complications included post-procedure heart block (n=22) and blood loss (n=2). At 18.23±13.15 months follow-up, 8/27 (five major, 22 minor) with arrhythmia had persistent post-procedure heart block of no clinical consequences. CONCLUSION: In our patient population, transcatheter device closure of the perimembranous ventricular septal defect with prophylactic oral steroid resulted in excellent closure rate and acceptably low incidence of conduction disturbances at mid-term follow-up.

Clinical and angiographic follow-up of coronary artery fistula interventions in children: techniques and classification revisited.

BACKGROUND: Transcatheter closure of coronary artery fistula has emerged as a safe and effective alternative to surgery. However, follow-up angiographic data after closure of the coronary artery fistula is extremely limited. We report our clinical and angiographic follow-up of children who underwent either transcatheter or surgical closure. METHOD: Clinical profile, echocardiography parameters, and closure technique were retrospectively reviewed from the hospital charts. Since 2007, 15 children have been intervened and followed up with electrocardiography, echocardiography, and angiography. RESULTS: A total of 15 children (six girls), with mean age of 6.7±5.4 years and weighing 16.3±9.8 kg, underwent successful closure (transcatheter=13, surgical=2) without periprocedural complication. Coronary artery fistula arose from the right (n=7) and left coronary artery (n=8) and drained into the right atrium or the right ventricle. Transcatheter closure was carried out using a duct occluder. Of the patients, two underwent surgical closure of the fistula on a beating heart. At 31.8±18.7 months follow-up, all the children were asymptomatic and had no evidence of myocardial ischaemia or infarction. However, follow-up angiography revealed thrombotic occlusion of fistula with the patent parent coronary artery in those having branch coronary artery fistula, and five of seven patients with parent coronary artery fistula had near-complete occlusion of fistula extending into the native coronary artery. CONCLUSION: Follow-up angiography revealed a high incidence of parent artery occlusion when the fistula was arising from the native artery and not from one of its branches. Coronary artery fistula intervention of the parent coronary artery fistula always carries the potential risk of ischaemia, unless the distal-most exiting segment is the primary site of occlusion.

Efficacy and safety of tenecteplase in pulmonary embolism.

Pulmonary embolism (PE) is a relatively common life-threatening cardiovascular condition associated with significant morbidity and mortality. We present the efficacy and safety data of weight-adjusted tenecteplase in 30 consecutive patients of acute PE. 30 patients (22 male, 8 female) with acute PE were included in the study and divided into three groups: (1) Acute PE complicated by shock stage and/or persistent hypotension (12 patients). (2) RV dilatation and/or dysfunction without hypotension (14 patients). (3) Severe hypoxemia without hypotension and RV dysfunction (4 patients). Predominant symptoms were dyspnoea, cough, chest pain, syncope and haemoptysis, noted in 100% (30), 40% (12), 54% (16), 32% (9) and 10% (3) of patients respectively. RV dilatation and dyskinesia were present in 86%, septal paradoxical movement in 73% and inferior venacava collapse absent in 53% of patients respectively. 12 patients presented with acute PE and cardiogenic shock, 14 patients showed RV dilatation and dysfunction with systolic BP >90 mmHg and four patients were having RV dilation without dysfunction but severe hypoxemia. There was significant reduction in right ventricular systolic pressure and improvement in right ventricular dysfunction. Our study shows that tenecteplase is very effective and safe in the treatment of PE with minimal risk of bleeding in high risk group and intermediate risk and even in selective low risk category group of patients. However, in view of small number of patients in study group, a large multicentre randomized study would be required to draw a firm conclusion regarding the thrombolysis in low risk category patient.

A rare case report of corrected transposition of the great arteries in association with tuberous sclerosis and cardiac rhabdomyomas.

The neuro-cutaneous syndrome tuberous sclerosis is commonly associated with rhabdomyomas in various organs including the heart. We are reporting a rare case of a 7-month old male child with congenitally corrected transposition of the great arteries associated with tuberous sclerosis and cardiac rhabdomyomas. To our knowledge, this rare association has not been reported so far.

Successful transcatheter closure of coronary artery fistula in a child with single coronary artery: a heavy load and a long road.

Single coronary artery is an uncommon variation of the coronary circulation. After transposition of great arteries, coronary artery fistulas are the most common associated cardiac anomalies in these patients. Transcatheter closure of coronary artery fistula (CAF) involving single coronary artery is a challenging intervention. In the absence of contralateral coronary artery, a complex anatomy of the CAF and a large myocardial perfusion territory of the dominant circulation pose an additional risk during interventional procedure. We report our experience of a successful transcatheter closure of a coronary artery fistula in a patient with single coronary artery.

Perventricular device closure of isolated muscular ventricular septal defect in infants: a single centre experience.

OBJECTIVES: To evaluate prospective single centre experience of mid-term safety and efficacy of perventricular device closure of isolated large muscular ventricular septal defect (mVSD) in high-risk infants. BACKGROUND: Surgical closures of large mVSD in infants represent a challenge with significant morbidity. METHODS: Between August 2008-2010, perventricular closure was attempted in 24 infants of 6.01 ± 2.37 months age and 4.27 ± 0.56 kg weight under TEE guidance. RESULTS: The device was successfully deployed in 21/24 infants. Size of mVSD was 8.42 ± 1.46 mm (6.1-12 mm). Mean procedure time was 28.8 ± 11.7 min. The closure rate was 84% immediately and 100% at 6 months. Four patients suffered major complications: 2-died, 1-esophageal perforation, 1-persistent CHB. At 26.23 ± 6.63 months follow-up two patients were symptomatic: 1-required device retrieval, 1-died of severe gastroenteritis. CONCLUSION: Perventricular device closure of isolated mVSD appears feasible option at mid-term follow-up and may either substitute or complement the conventional surgical technique in selected cases depending on institutional paediatric cardiac surgery performance.

Right Posterior Thoracotomy for Open-Heart Surgery in a Rare Morphology.

The closure of atrial septal defects through right-sided limited posterior thoracotomy has been well established in selected subsets. We present a case of large ostium secundum atrial septal defect, pulmonary valvar stenosis, absent right superior vena cava, and isolated left superior vena cava draining to right atrium via coronary sinus. The child successfully underwent total correction through limited posterior thoracotomy with necessary modifications of intraoperative steps.

Intramural Anomalous Right Coronary Artery From the Main Pulmonary Artery.

Anomalous origin of the right coronary artery from the main pulmonary artery (anomalous right coronary artery from pulmonary artery; ARCAPA) is a rare congenital anomaly. Here, we present an unusual case of anomalous right coronary artery from the main pulmonary artery with proximal intramural course.

Diagnostic accuracy of serum cystatin C for early recognition of contrast induced nephropathy in Western Indians undergoing cardiac catheterization.

AIMS: We aimed to compare the diagnostic efficacy of serum cystatin C (sCyC) for contrast induced nephropathy (CIN) in Western Indians undergoing cardiac catheterization. We also aimed to propose a clinically applicable cut-off of sCyC for early identification of CIN in this ethnic group. METHODS: In this prospective study, 253 patients undergoing coronary angiography and/or percutaneous coronary intervention were enrolled. The demographic and risk factor details, levels of sCr at baseline, 24 and 48h after the procedure, whereas baseline and 24h levels of sCyC were noted. Increase of 0.5mg/dl or ≥25% from baseline sCr was used to define CIN. Optimum cut off of sCyC for CIN diagnosis was obtained using Receiver Operating Characteristic (ROC) curve analysis. RESULTS: After 48h of contrast media (CM) exposure, the incidence of CIN was 12.25% (31 patients) according to sCr definition, where only 3.9% (10 patients) had sCr rise in 24h. Overall significant (p<0.0001) rise in mean levels of sCr (48h) and sCyC (24h) was observed in CIN patients. However, the mean sCr rise at 24h was non-significant. The optimum cut off of sCyC for diagnosing CIN was found to be a rise of ≥10% from baseline (AUC - 0.901; sensitivity - 100%, specificity - 77.89%). According to sCyC, 94 (37.15%) patients had CIN. CONCLUSION: We may conclude that a rise of ≥10% in sCyC at 24h could be used as a reliable marker for identification of CIN in western Indians undergoing cardiac catheterization.

Transcatheter device closure of pseudoaneurysms of the left ventricular wall: An emerging therapeutic option.

Left ventricular pseudoaneurysm is a rare but serious complication of acute myocardial infarction and cardiac surgery. While surgical intervention is the conventional therapeutic option, transcatheter closure can be considered in selected patients with suitable morphology of the pseudoaneurysm. We report a case of successful transcatheter closure of a left ventricular pseudoaneurysm orifice and isolation of the sac using an Amplatzer septal occluder.

Ductus arteriosus stenting: A promising percutaneous palliation in patients with duct-dependent pulmonary circulation.

OBJECTIVES: We aimed to study the feasibility and outcomes of ductal stenting in patients with duct-dependent pulmonary blood flow (PBF). METHODS: Duct-dependent hypoxic patients with confluent pulmonary artery (PA) branches were enrolled for ductal stenting and followed regularly. RESULTS: Sixty patients, with a median age of 12 (1-1095) days and weight of 2.8 (2.2-8.9) kg, were enrolled. Median right PA (RPA) and left PA (LPA) Z-scores were -1.23 (-10.54 to 2.81) and -0.96 (-8.03 to 3.0), respectively. Mean narrowest ductal diameter was 1.73±0.57 mm and length was 12.78±3.32 mm. Sixty-four stents with mean diameter of 4.21±0.32 mm and length of 14.34±3.44 mm were deployed in 59 patients. The procedure was unsuccessful in one. Post-stenting mean oxygen saturation (SO2) increased significantly from baseline of 68.88±7.47% to 90.43±6.04% (p<0001). Complications included pulmonary edema in one patient and acute stent occlusion in another. At a median follow-up of eight (2-14) months, mean SO2 (80.04±7.54%) was significantly higher than baseline (p<0.0001). Median RPA and LPA Z-scores, 0.56 (-2.89 to 3.29) and -0.02 (-2.81 to 3.86), respectively, were significantly higher than baseline. Six patients required re-interventions (shunt in three and angioplasty in three). Six patients died, three due to sepsis and another three with worsened cyanosis due to impaired PBF, probably due to ductal occlusion. CONCLUSION: Ductal stenting is an effective palliation in patients with duct-dependent PBF. It maintains adequate SO2 and promotes balanced PA growth at mid-term follow-up.

Transcatheter device occlusion of a large pulmonary arteriovenous fistula by exit closure: the road less travelled.

Large pulmonary arteriovenous fistula (PAVF) manifests as cyanosis and predisposes to serious complications of right-to-left shunt, and therefore necessitates early treatment. The emergence of antegrade transcatheter closure of feeding arteries as treatment of choice is limited by inherent risk of either recanalization or reappearance of new feeders and potential risk of systemic embolization. Additional closure of the draining vessel by transcatheter device occlusion should overcome the limitations of conventional antegrade technique. We describe two cases of successful transcatheter closure of a large PAVF by antegrade device closure of feeders as well as transseptal retrograde closure of the exiting channel.

Transcatheter closure of left pulmonary artery to left atrium fistula in a neonate using amplatzer duct occluder I.

A direct-fistula type communication between branch pulmonary artery (PA) to left atrium (LA), particularly left PA to LA, is a very rare congenital cardiopulmonary disorder. Although surgical repair is the conventional treatment, transcatheter device or coil closure is feasible in selected cases of a relatively frequent variant - right PA to LA fistula. Ours is the first case of successful transcatheter closure of a large left PA to LA fistula with Amplatzer duct occluder in a cyanotic neonate by transseptal approach.

Clinical profile of pediatric patients with long QT syndrome masquerading as seizures.

OBJECTIVE: To study the clinical profile of patients with long QT syndrome presenting as seizures. METHODS: Retrospective analysis of six pediatric patients admitted at authors' institute between October 2008 and January 2013 with seizures and a presumptive diagnosis of long QT syndrome (LQTS) was done. The diagnosis was made on the basis of updated Schwartz diagnostic criteria. Clinical data, investigation profile and follow up of patients was recorded in a standard format and analysed. RESULTS: All the 6 patients in the study were boys with a mean age of 10.3 ± 2.8 y at the time of diagnosis. The lag period between symptom onset and diagnosis was 5.6 ± 3.14 y. All patients had history of seizures with a history of precipitating event in 4 patients. Average baseline QTc interval was 556 ± 41.31 ms. Mean Schwartz score was 6.66 ± 1.16. Polymorphic VT was documented in 4 patients. After initiating standard treatment with betablockers, nicorandil, spironolactone or pacemaker, all the six patients were asymptomatic at a mean follow up period of 17.5 mo, with no recurrence of seizures. CONCLUSIONS: LQTS can cause seizures due to prolonged ventricular arrhythmias in high risk subgroup. Children, who present with LQTS and seizures, generally have a precipitating event causing seizures, and they respond well to drug therapy.

Transcatheter right ventricular outflow tract stenting in children with postoperative infundibular stenosis and preserved pulmonary valve function.

Recurrent or residual right ventricular outflow tract obstruction after early surgical repair of congenital heart disease is one of the most frequent indications for either surgical or transcatheter reintervention. Transcatheter stent implantation across the stenotic right ventricular outflow tract or conduit is a safe and effective alternative to surgical reintervention. However, chronic deleterious effects of pulmonary regurgitation can potentially counterbalance the early improvement in clinical and hemodynamic parameters, sometimes necessitating further intervention. While there are several studies documenting safe and effective palliation by transcatheter right ventricular outflow tract stenting in infants with tetralogy of Fallot, literature on isolated infundibular stent implantation sparing the normal pulmonary valve in postoperative infundibular restenosis is very scant. We report our experience of safety and feasibility of transcatheter right ventricular outflow tract stent implantation while preserving the native pulmonary valve function in two children with infundibular stenosis after surgical repair of congenital heart disease.

Percutaneous closure of patent ductus arteriosus via internal jugular vein in patient with interrupted inferior vena cava.

Transcatheter closure of patent ductus arteriosus (PDA) using various occluders and coils via femoral vein is a well established therapeutic option. However, in patients with interrupted inferior vena cava (IVC) it is not feasible to close the PDA percutaneously using traditional methods. We present a nine-year-old girl with IVC interruption in whom percutaneous closure of PDA was successfully accomplished via the transjugular approach.

Submitral left ventricular pseudoaneurysm after mitral valve replacement: early diagnosis and successful repair.

Late left ventricle (LV) rupture with pseudoaneurysm after mitral valve replacement is rare. We report its early diagnosis by advanced technologies, e.g. MRI and successful repair of a type I AV rupture through left atrial approach.

Etiopathogenesis of arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterised by progressive fibro-fatty replacement of right ventricular myocardium. Earlier studies described ARVC as non-inflammatory, non-coronary disorder associated with arrhythmias, heart failure and sudden death due to functional exclusion of the right ventricle. Molecular genetic studies have identified nine different loci associated with ARVC; accordingly each locus is implicated for each type of ARVC (ARVC1-ARVC9). So far five genes have been identified as containing pathogenic mutations for ARVC. Though mutations in each of the gene/s indicate disruption of different pathways leading to the condition, the exact pathogenesis of the condition is still obscure. This review tries to understand the pathogenesis of the condition by examining the individual proteins implicated and relate them to the pathways that could play a role in the aetiology of the condition. Cardiac ryanodine receptor (RYR-2), which regulates intra-cellular Ca(2+) concentration by releasing Ca(2+) reserves from the sarcoplasmic reticulum (SR), was the first gene for ARVC. The mutation in this gene is believed to disrupt coupled gating of RYR-2, causing after-depolarisation, leading to arrhythmias followed by structural changes due to altered intra-cellular Ca(2+) levels. Three other genes implicated for ARVC, plakoglobin (Naxos disease), desmoplakin (ARVC8) and plakophilin (ARVC9) have prompted the speculation that ARVC is primarily a disease of desmosomes. But identification of TGFbeta-3 for ARVC1 and the role of all these three genes (plakoglobin, desmoplakin and plakophilin) in cardiac morphogenesis indicate some kind of signal-transducing pathway disruption in the condition. The finding that ARVC as a milder form of Uhl's anomaly indicates similar ontogeny for the condition. Further, discovery of apoptotic cells in the autopsy of the right ventricular myocardium of ARVC patients does indicate a common pathway for different types of ARVCs, which is more specific for the right ventricular myocardium involving desmosomal plaque proteins, growth factors and Ca(2+) receptors.