Transcatheter Pulmonary Valve Replacement With the Sapien Prosthesis.

Background: There are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve.

Objectives: This study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve.

Methods: Data were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve.

Recent advances in transcatheter pulmonary valve delivery.

Purpose Of Review: Transcatheter pulmonary valve replacement has only been both approved and widely available for most congenital heart disease centers for a few years; its use and familiarity for interventionalists have greatly expanded our knowledge of its applicability to a multitude of clinical situations. Expanded worldwide use and longer time from implant have both served to better understand procedural limits and uncommon late adverse events.

Recent Findings: Although currently approved for implantation in the USA only in dysfunctional and circumferential right ventricle to pulmonary artery conduits, with expanded experience operators have been able to adapt the delivery of this valve in a large number of additional clinical scenarios.

Transcatheter pulmonary valve insertion, expanded use (beyond large conduits from the right ventricle to pulmonary artery), and future directions.

Transcatheter pulmonary valve insertion is the most important advance in congenital interventional cardiology since atrial septal defect devices became commonly available 15 years ago. It has changed the way we look at a number of diverse diagnoses and changes how we plan, diagnose, operate, and follow-up patients. It has changed how we counsel families expecting a child that may benefit from it.

Management of adults with Tetralogy of Fallot.

Tetralogy of Fallot is the most common form of cyanotic congenital cardiac disease. Patients with previously repaired tetralogy of Fallot are the most common patients seen in the Program for Adults with Congenital Heart Disease at The Johns Hopkins All Children's Heart Institute. Guidelines for the management of these patients are available from multiple sources including The American College of Cardiology (ACC) and The American Heart Association (AHA), The Canadian Cardiovascular Society, and The European Society of Cardiology (ESC).

Transcatheter pulmonary valve insertion, expanded use and future directions.

Transcatheter pulmonary valve replacement with the Melody® valve is an accepted alternative to surgical replacement of the pulmonary valve for some patients and therefore a complementary strategy in the long-term management of several groups of patients with congenital heart disease. It allows at least extending the time between sternotomies and possibly improving late outcomes. With a combined surgical and percutaneous approach, late morbidity for some of these patients will likely be diminished.

Transcatheter pulmonary valve insertion: when, how, and why.

Transcatheter pulmonary valve replacement is fast becoming an accepted alternative to repeat surgical pulmonary valve replacement for selected patients and therefore a complementary strategy in the long-term management of those requiring surgical pulmonary valve replacement. With a combined surgical and percutaneous approach, late morbidity for some of these patients may be diminished. This manuscript will review the current indications for this procedure, its limitations, and its benefits.

The hybrid approach--current knowns and unknowns: the perspective of cardiology.

The hybrid approach to the treatment of patients with hypoplastic left heart syndrome most commonly includes transcatheter placement of a stent in the arterial duct and surgical placement of bands on the branch pulmonary arteries via median sternotomy. This manuscript will review the concept of hybrid palliation and discuss topics related to several time intervals: peri-procedural, post-procedural, interstage, and comprehensive stage 2.

Pediatric catheterization laboratory anticoagulation with bivalirudin.

Background: Pediatric physicians regularly face the problem of uncertain procedural anticoagulation in children, especially in neonates. We sought to evaluate the safety, plasma concentration (pharmacokinetics, PK), pharmacodynamics (PD), and dosing guidelines of bivalirudin when used as a procedural anticoagulant in pediatric percutaneous intravascular procedures.

Methods And Results: Pediatric subjects undergoing percutaneous intravascular procedures for congenital heart disease were enrolled and received the current weight-based dose used in percutaneous coronary interventions (0.

Determinants of extracellular matrix remodelling are differentially expressed in paediatric and adult dilated cardiomyopathy.

Aims: The left ventricular phenotype of idiopathic dilated cardiomyopathy (DCM) can appear similar in paediatric and adult patients. However, the aetiology of paediatric DCM is usually idiopathic, and often leads an aggressive clinical course. A structural underpinning of DCM is extracellular matrix changes, which are determined by a balance between matrix metalloproteinases (MMPs) and tissue inhibitors of MMPs (TIMPs).

Plasma profiling determinants of matrix homeostasis in paediatric dilated cardiomyopathy.

Objective: Dilated cardiomyopathy is an important cause of cardiac failure in both children and adults, but is more progressive in children. In adult dilated cardiomyopathy, left ventricular remodelling is associated with changes in the plasma levels of matrix metalloproteinases and tissue inhibitor of metalloproteinases. Plasma matrix metalloproteinases and tissue inhibitors of metalloproteinase changes in paediatric dilated cardiomyopathy have not been examined.

Rejection with hemodynamic compromise in the current era of pediatric heart transplantation: a multi-institutional study.

Background: Survival after pediatric heart transplant has improved over time, as has the incidence of overall rejection. We studied the effect of era on the occurrence and outcome of rejection with hemodynamic compromise (HC).

Methods: Data from 2227 patients who received allografts between 1993 and 2006 at 36 centers in the Pediatric Heart Transplant Study were analyzed to determine incidence, outcome, and risk factors for rejection with HC in early (1993-1999) and recent (2000-2006) eras.

Usefulness of live three-dimensional transesophageal echocardiography in a congenital heart disease center.

Three-dimensional (3D) transesophageal echocardiography (TEE) has numerous potential applications in the care of patients with congenital heart disease (CHD). However, there were few data examining its utility in this setting. The aim was to describe the initial experience and feasibility of this modality at a tertiary CHD center.

Novel minimally invasive, intrapericardial implantable cardioverter defibrillator coil system: a useful approach to arrhythmia therapy in children.

Background: Current approaches to implantable cardioverter defibrillator (ICD) implantation in children remain challenging. Transvenous access may be limited due to patient size or anatomy, while epicardial patches require sternotomy or thoracotomy. We present an alternative approach; minimally invasive placement of a transvenous ICD coil within the pericardial space with active fixation.

Right ventricle-to-pulmonary artery shunt: alternative palliation in infants with inadequate pulmonary blood flow prior to two-ventricle repair.

Background: Traditional palliation of infants with biventricular hearts and inadequate pulmonary blood flow is a modified Blalock-Taussig shunt. The aim of this report is to assess the results of an alternative, right ventricle-to-pulmonary artery (RV-PA) shunt.

Methods: Between August 2004 and July 2007, 10 infants with biventricular hearts and inadequate pulmonary blood flow underwent palliation with an RV-PA shunt.

Transcatheter device occlusion of multiple large pulmonary arteriovenous malformations in two symptomatic pediatric patients.

Pulmonary arteriovenous malformations (AVMs) large enough to lead to clinically significant cyanosis are rare in the pediatric population. To date, there has been some experience with transcatheter embolization of pulmonary AVMs in children, primarily with coils or balloons. Herein, we report 2 cases of children who were progressively symptomatic and had physical manifestations of hypoxemia arising from large pulmonary AVMs.

Neoaortic root modification for late thrombosis after Norwood palliation.

A 5-year-old child with hypoplastic left heart syndrome presented with myocardial infarction 2 years after Fontan completion. Evaluation identified a thrombus in his native aortic root. Retrospective review of a prior catheterization revealed stasis in the native aortic root.

Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up.

Background: Ebstein's anomaly in the severely symptomatic neonate is usually fatal. Until recently, successful repair has not been reported and various palliative operations have been associated with prohibitive mortality. Recently, we published our initial results with biventricular repair in 3 severely symptomatic neonates.