Percutaneous Balloon-Expandable Stent Implantation to Treat Transverse Aortic Arch Obstruction: Medium- to Long-Term Outcomes of a Retrospective Multicenter Study.

Background: Transverse aortic arch obstruction is a challenging lesion for which stent implantation provides a potentially important alternate therapy. The objectives were to evaluate the technical, procedural, and medium-to-long-term clinical outcomes of percutaneous stent implantation of transverse aortic arch obstruction.

Methods: This is a retrospective, multicenter study of transverse aortic arch stent implantation.

A first-in-human phase I/IIa gene transfer clinical trial for Duchenne muscular dystrophy using rAAVrh74.MCK..

In a phase 1/2, open-label dose escalation trial, we delivered rAAVrh74.MCK. (also ) bilaterally to the legs of two boys with Duchenne muscular dystrophy using intravascular limb infusion.

Percutaneous Implantation of Adult Sized Stents for Coarctation of the Aorta in Children ≤20 kg: A 12-Year Experience.

Background: Stent implantation (SI) is more effective than balloon angioplasty for the treatment of coarctation of the aorta (CoA). Due to technical factors, balloon angioplasty is more commonly performed in small patients. We sought to evaluate outcomes of percutaneous adult sized SI for the treatment of CoA in small patients.

Three-Year Outcomes From the Harmony Native Outflow Tract Early Feasibility Study.

Background: The Harmony transcatheter pulmonary valve (TPV) was designed for treatment of postoperative pulmonary valve regurgitation in patients with repaired right ventricular outflow tracts.

Methods: The Native TPV EFS (Early Feasibility Study) is a prospective, multicenter, nonrandomized feasibility study. Three-year outcomes are reported.

Spontaneous reversal of stenosis in tissue-engineered vascular grafts.

We developed a tissue-engineered vascular graft (TEVG) for use in children and present results of a U.S. Food and Drug Administration (FDA)-approved clinical trial evaluating this graft in patients with single-ventricle cardiac anomalies.

Gene Delivery for Limb-Girdle Muscular Dystrophy Type 2D by Isolated Limb Infusion.

In a previous limb-girdle muscular dystrophy type 2D (LGMD2D) clinical trial, robust alpha-sarcoglycan gene expression was confirmed following intramuscular gene () transfer. This paved the way for first-in-human isolated limb infusion (ILI) gene transfer trial to the lower limbs. Delivery of scAAVrh74.

Leaflet morphology classification of the Melody Transcatheter Pulmonary Valve.

Objective: We sought to describe the leaflet morphology variation in the Melody Transcatheter Pulmonary Valve (TPV) and evaluate associated outcomes. The Melody TPV is constructed from harvested bovine jugular venous valves which have been rigorously tested. Natural anatomic leaflet variations are seen in the Melody TPV but have not been evaluated.

An Isolated Limb Infusion Method Allows for Broad Distribution of rAAVrh74.MCK. to Leg Skeletal Muscles in the Rhesus Macaque.

Recombinant adeno-associated virus (rAAV)rh74.MCK. is a muscle-specific gene therapy that is being developed to treat forms of muscular dystrophy.

Cerebral Blood Flow Following Hybrid Stage I Palliation in Infants with Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome (HLHS) palliation may result in altered cerebral blood flow with subsequent neurodevelopmental implications. The purpose of the study was to assess blood flow in the middle cerebral artery (MCA) and investigate the relationship with early neurodevelopmental outcomes in infants with HLHS after hybrid stage I. Transcranial Doppler (TCD) was performed to obtain peak systolic, end-diastolic, and mean velocities, as well as pulsatility index of the MCA in infants with HLHS (n = 18) at baseline and at 2, 4, and 6 months of age.

Harmony Feasibility Trial: Acute and Short-Term Outcomes With a Self-Expanding Transcatheter Pulmonary Valve.

Objectives: This study sought to obtain in vivo data to confirm assumptions on device loading conditions and assess procedural feasibility, safety, and valve performance.

Background: The Harmony transcatheter pulmonary valve (Medtronic, Minneapolis, Minnesota) was designed for patients with severe pulmonary regurgitation who require pulmonary valve replacement.

Methods: Three sites participated in this first Food and Drug Administration-approved early feasibility study using an innovative device design to accommodate the complex anatomy of the right ventricular outflow tract.

Patient Selection Process for the Harmony Transcatheter Pulmonary Valve Early Feasibility Study.

This early feasibility study was designed to obtain in vivo data to confirm assumptions on device loading conditions of the Medtronic Harmony transcatheter pulmonary valve (TPV). Secondary objectives included procedural feasibility, safety, and valve performance. The Harmony TPV was developed for nonsurgical pulmonary valve replacement in non-right ventricle-pulmonary artery conduit patients.

Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results.

Introduction: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring.

Methods: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues.

Intravascular Ultrasound Characterization of a Tissue-Engineered Vascular Graft in an Ovine Model.

Patients who undergo implantation of a tissue-engineered vascular graft (TEVG) for congenital cardiac anomalies are monitored with echocardiography, followed by magnetic resonance imaging or angiography when indicated. While these methods provide data regarding the lumen, minimal information regarding neotissue formation is obtained. Intravascular ultrasound (IVUS) has previously been used in a variety of conditions to evaluate the vessel wall.

Understanding the Hybrid Stage I Approach for Hypoplastic Left Heart Syndrome.

Hybrid stage I palliation combines cardiothoracic surgery and interventional transcatheter procedures for treatment of hypoplastic left heart syndrome. The approach is an alternative to the Norwood procedure, the traditional first stage of surgical palliation. Hybrid stage I palliation involves placing bilateral branch pulmonary artery bands and a patent ductus arteriosus stent through a median sternotomy, performed without cardiopulmonary bypass.

Percutaneous Patent Ductus Arteriosus (PDA) Closure in Very Preterm Infants: Feasibility and Complications.

Background: Percutaneous closure of patent ductus arteriosus (PDA) in term neonates is established, but data regarding outcomes in infants born very preterm (<32 weeks of gestation) are minimal, and no published criteria exist establishing a minimal weight of 4 kg as a suitable cutoff. We sought to analyze outcomes of percutaneous PDA occlusion in infants born very preterm and referred for PDA closure at weights <4 kg.

Methods And Results: Retrospective analysis (January 2005-January 2014) was done at a single pediatric center.

Mustard baffle obstruction and leak - How successful are percutaneous interventions in adults?

Atrial switch operations for D-Transposition of the great arteries (D-TGA) were performed until the late 20th century. These patients have substantial rates of re-operation, particularly for baffle related complications. This study sought to analyze the efficacy of percutaneous transcatheter intervention (PTI) for baffle leak and/or stenosis in adult atrial switch patients.

Iatrogenic embolization and transcatheter retrieval of a ventricular septal defect occluder in a dog.

A 7-month-old Irish Setter underwent transcatheter therapy of a muscular ventricular septal defect (VSD) and pulmonary valve stenosis. Standard devices for muscular VSD closure could not span the interventricular septum due to right ventricular hypertrophy, and an Amplatzer post-infarction muscular VSD occluder with a wider waist was successfully implanted. Following VSD closure, inflation of the balloon dilation catheter during balloon pulmonary valvuloplasty resulted in iatrogenic embolization of the VSD occluder to the left ventricular outflow tract.

An Overview of Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare and complex congenital cardiac lesion that has historically carried a poor prognosis. With advancements in surgical management, we have seen an improvement in the outcomes for children affected by this disease. However, this population continues to present challenges due to the complex anatomy and physiology associated with PA/VSD/MAPCA.

Early results of neurodevelopment following hybrid stage I for hypoplastic left heart syndrome.

Motor skills and neurodevelopment in infants with hypoplastic left heart syndrome (HLHS) who have undergone Hybrid Stage I palliation is unknown. The purpose of this study is to assess early neurodevelopment in infants with HLHS after Hybrid Stage I palliation. Developmental assessment was performed in HLHS infants who underwent Hybrid Stage I palliation at 2 and 4 months of age using the Test of Infant Motor Performance, and at 6 months of age, prior to undergoing the second staged surgery, using the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-III).

Stenting of the right ventricular outflow tract in 2 dogs for palliation of dysplastic pulmonary valve stenosis and right-to-left intracardiac shunting defects.

Two dogs with severe dysplastic pulmonary valve stenosis and right-to-left shunting defects (patent foramen ovale, perimembranous ventricular septal defect) underwent palliative stenting of the right ventricular outflow tract and pulmonary valve annulus using balloon expandable stents. One dog received 2 over-lapping bare metal stents placed 7 months apart; the other received a single covered stent. Both procedures were considered technically successful with a reduction in the transpulmonary valve pressure gradient from 202 to 90 mmHg in 1 dog and from 168 to 95 mmHg in the other.

Use and performance of the Melody Transcatheter Pulmonary Valve in native and postsurgical, nonconduit right ventricular outflow tracts.

Background: Melody Transcatheter Pulmonary Valve (TPV) replacement therapy represents an important advance in congenital cardiovascular interventions. The off-label extension of the Melody TPV to patients with nonconduit outflow tracts (right ventricular outflow tract [RVOT]) has the potential to vastly expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. However, knowledge on the performance of the Melody TPV in this setting is limited.

The Medtronic Melody® transcatheter pulmonary valve implanted at 24-mm diameter--it works.

Objectives: We report the Melody valve implanted and/or expanded to 24-mm diameter.

Background: The Medtronic Melody valve has been implanted up to 22 mm in the pulmonary position for over a decade.

Methods: A retrospective chart review was performed on 82 patients who underwent Melody valve implant.