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Background: Atrioventricular valve regurgitation (AVVR) is a devastating complication in children and young adults with congenital heart disease (CHD), particularly in patients with single ventricle physiology. Transcatheter edge-to-edge repair (TEER) is a rapidly expanding, minimally invasive option for the treatment of AVVR in adults that avoids the morbidity and mortality associated with open heart surgery. However, application of TEER in in CHD and in children is quite novel. We describe the development of a peri-procedural protocol including image-derived pre-intervention simulation, with successful application to four patients., Aims: To describe the initial experience using the MitraClip system for TEER of dysfunctional systemic atrioventricular valves in patients with congential heart disease within a pediatric hospital., Methods: A standardized screening and planning process was developed using cardiac magnetic resonance imaging, three dimensional echocardiography and both virtual and physical simulation. Procedures were performed using the MitraClip G4 system and patients were clinically followed post-intervention., Results: A series of four CHD patients with at least severe AVVR were screened for suitability for TEER with the MitraClip system: three patients had single ventricle physiology and Fontan palliation, and one had repair of a common atrioventricular canal defect. Each patient had at least severe systemic AVVR and was considered at prohibitively high risk for surgical repair. Each patient underwent a standardized preprocedural screening protocol and image-derived modeling followed by the TEER procedure with successful clip placement at the intended location in all cases., Conclusions: The early results of our protocolized efforts to introduce TEER repair of severe AV valve regurgitation with MitraClip into the CHD population within our institution are encouraging. Further investigations of the use of TEER in this challenging population are warranted., (© 2023 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Objective: Atrial arrhythmias and atrioventricular valve regurgitation (AVVR) are common causes of morbidity among adults with congenital heart disease (ACHD). The impact of rhythm control on AVVR in this population is unknown. We sought to determine whether a rhythm control strategy is associated with greater freedom from AV valve surgery than a rate control strategy., Design: Patients evaluated by both ACHD and electrophysiology specialists at a single academic center were screened for atrial arrhythmias and at least moderate-severe AVVR. Clinical and electrographic data were abstracted. All echocardiograms were interpreted by a single echocardiographer blinded to treatment strategy. Patients were followed until AV valve surgery, heart transplantation, death, or last clinical follow-up., Results: Rhythm control was attempted in 9 of 24 identified patients. Among these nine patients, arrhythmias were eliminated in three and reduced from persistent to paroxysmal in another three. In the rhythm control group, mean left ventricular ejection fraction improved from 54.4 ± 12.4% to 60.0 ± 11.5% (P = .02) and mean right ventricular systolic function increased nearly one grade (P = .02). AVVR did not decrease significantly. No significant change in left or right ventricular systolic function, or AVVR was observed among the 15 patients treated with rate control. Four-year survival free of AV valve operation and heart transplant was 88% in the rhythm control group and 31% in the rate control group (P = .04)., Conclusions: In ACHD patients with atrial arrhythmias and at least moderate-severe AVVR, a rhythm control strategy was associated with improved biventricular systolic function. This improvement in ventricular function and symptoms may allow valve surgery to be deferred., (© 2019 Wiley Periodicals, Inc.)

Background: Ventricular tachycardia (VT) is frequently encountered in patients with repaired and unrepaired congenital heart disease (CHD), causing significant morbidity and sudden cardiac death. Data regarding underlying VT mechanisms and optimal ablation strategies in these patients remain limited., Objective: To describe the electrophysiologic mechanisms, ablation strategies, and long-term outcomes in patients with CHD undergoing VT ablation., Methods: Forty-eight patients (mean age 41.3 ± 13.3 years, 77.1% male) with CHD underwent a total of 57 VT ablation procedures at two centers from 2000 to 2017. Electrophysiologic and follow-up data were analyzed., Results: Of the 77 different VTs induced at initial or repeat ablation, the underlying mechanism in 62 (81.0%) was due to scar-related re-entry; the remaining included four His-Purkinje system-related macrore-entry VTs and focal VTs mainly originating from the outflow tract region (8 of 11, 72.7%). VT-free survival after a single procedure was 72.9% (35 of 48) at a median follow-up of 53 months. VT-free survival after multiple procedures was 85.4% (41 of 48) at a median follow-up of 52 months. There were no major complications. Three patients died during the follow-up period from nonarrhythmic causes, including heart failure and cardiac surgery complication., Conclusion: While scar-related re-entry is the most common VT mechanism in patients with CHD, importantly, nonscar-related VT may also be present. In experienced tertiary care centers, ablation of both scar-related and nonscar-related VT in patients with CHD is safe, feasible, and effective over long-term follow-up., (© 2019 Wiley Periodicals, Inc.)

Patients with systemic right ventricles are often not able to tolerate frequent, rapid, or incessant atrial arrhythmias without developing significant symptoms and ventricular dysfunction. Atrial arrhythmias are associated with an increased risk of ventricular arrhythmias and sudden cardiac death. Rhythm disturbances must be aggressively addressed in this population with frequent screening, follow-up, and treatment., (Copyright © 2016 Elsevier Inc. All rights reserved.)

In a patient with Fontan palliation and persistent atrial fibrillation two triggers were identified that initiated atrial fibrillation (AF) from the superior vena cava (SVC) and the right atrium. SVC triggers are more common in patients with a normal-sized left atrium. Eliminating these triggers prevented AF from being sustained in this patient and thus pulmonary vein isolation was not pursued. The patient has remained AF free for 3 years without medications or repeat ablation. Targeting of potential right-sided triggers for AF ablation, before pulmonary vein isolation, should be considered; such an approach may reduce risks in these complex patients., (Copyright © 2016 Elsevier Inc. All rights reserved.)

With modern surgical techniques, there is significantly increased life expectancy for those with congenital heart disease. Although congenital pulmonary valve stenosis is not as complex as tetralogy of Fallot, there are many similarities between the 2 lesions, such that patients with either of these conditions are at risk for ventricular arrhythmias and sudden cardiac death. Those patients who have undergone surgical palliation for congenital pulmonary stenosis are at an increased risk for development of ventricular arrhythmias and may benefit from a more aggressive evaluation for symptoms of palpitations or syncope., (Copyright © 2016 Elsevier Inc. All rights reserved.)

In this case, the patient's ventricular tachycardia (VT) was specifically induced by coughing, which has not previously been described. Decreasing the rotational speed of the left ventricular assist device (LVAD) and increasing preload by stopping the patient's nitrates and reducing diuretic dose allowed improved filling of the left ventricle (LV) and increased LV volumes. When coughing recurred, the effects on the LV cavity were less pronounced and thus VT was reduced. Although ventricular arrhythmias are common after LVAD placement, this is a unique case in which VT was caused by coughing, which is ordinarily not considered arrhythmogenic., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: The purpose of this study is to review a series of patients with complex congenital heart disease in whom preprocedural imaging was used to assist placement of cardiac resynchronization therapy (CRT) leads., Background: CRT may be beneficial in patients with a failing systemic ventricle and transposition of the great arteries (TGA). However, complex coronary venous anatomy can be challenging for placement of CRT leads., Methods: Between October 2006 and June 2012, seven patients with either dextro-TGA (d-TGA) or levo-TGA (l-TGA) underwent preprocedural imaging prior to placement of CRT leads (three, d-TGA and four, l-TGA). Three patients underwent cardiac computed tomography (CT) and four underwent coronary angiography, which included levophase imaging of the coronary sinus (CS) or direct contrast injection of the CS. Where CS anatomy was appropriate with drainage into the systemic venous circulation, a transvenous approach was planned. In all other cases, the patient was referred for surgical placement of epicardial leads., Results: Seven patients were identified with either d-TGA or l-TGA who had undergone preprocedural imaging prior to placement of CRT leads (three, d-TGA and four, l-TGA). Three patients underwent cardiac CT and four underwent coronary angiography, which included levophase imaging of the CS or direct contrast injection of the CS. All seven patients had successful CRT lead placement guided by preprocedure imaging. Three patients required surgical placement whereas four were able to undergo transvenous placement. There were no complications. The majority of patients (four of seven) had improvement in New York Heart Association class as well as subjective improvement in exercise tolerance and energy. The majority of patients also had subjective improvement in systemic right ventricular function by echocardiogram and objective improvement in fractional area change of the right ventricle. The follow-up period ranged from 13 months to 55 months with a mean follow-up of 39 months., Conclusions: Placement of biventricular leads for CRT in patients with l-TGA or d-TGA is feasible. Preprocedural imaging of the CS allows for better assessment of its anatomy and helps determine procedural approach for CRT placement, thereby limiting unnecessary procedures. In the majority of patients, there was subjective improvement in functional status and right ventricular function; in addition, there was objective improvement in echocardiographic parameters of right ventricular function after CRT placement., (©2013 Wiley Periodicals, Inc.)

E-Cadherin is a transmembrane glycoprotein that mediates Ca2+-dependent intercellular adhesion in normal epithelium. In tumors of epithelial origin, E-cadherin expression frequently is reduced, an event that contributes to tumor invasion and metastasis. The role of E-cadherin in hematopoietic tissues is less clear. In normal bone marrow, E-cadherin is expressed on erythroid progenitors, CD34+ stem cells, and stromal cells, where it likely contributes to intercellular interactions during hematopoiesis. In this study, we used a nested-PCR approach to examine the methylation status of the E-cadherin 5' CpG island in blood and bone marrow samples from normal donors and in bone marrow from patients with acute leukemia. In normal peripheral blood mononuclear cells and bone marrow, E-cadherin was completely unmethylated. In peripheral blood mononuclear cells, expression was evident by reverse transcription-PCR. Immunoblotting confirmed E-cadherin protein expression in two lymphoblastoid cell lines derived from normal donors. In contrast, E-cadherin was aberrantly methylated in 4 of 4 (100%) leukemia cell lines, 14 of 44 (32%) acute myelogenous leukemias, and 18 of 33 (53%) acute lymphoblastic leukemias. Genomic bisulfite sequencing of primary leukemias confirmed dense methylation across the CpG island. Methylation was associated with loss of E-cadherin RNA and protein in leukemia cell lines and primary leukemias. Following treatment with 5-aza-2'-deoxycytidine, a methylated leukemia cell line expressed both E-cadherin transcript and protein. Our results show that methylation of E-cadherin occurs commonly in acute leukemia and suggests a hypothesis for E-cadherin down-regulation in leukemogenesis.