A graphene-based hybrid material with quantum bits prepared by the double Langmuir-Schaefer method.

The scalability and stability of molecular qubits deposited on surfaces is a crucial step for incorporating them into upcoming electronic devices. Herein, we report on the preparation and characterisation of a molecular quantum bit, copper(ii)dibenzoylmethane [Cu(dbm)], deposited by a modified Langmuir-Schaefer (LS) technique onto a graphene-based substrate. A double LS deposition was used for the preparation of a few-layer-graphene (FLG) on a Si/SiO substrate with subsequent deposition of the molecules.

Preliminary experience with cardiac reconstruction using decellularized porcine extracellular matrix scaffold: human applications in congenital heart disease.

An ideal material for repair of congenitally malformed hearts would encourage tissue regeneration with growth potential. Decellularized porcine small intestinal submucosa extracellular matrix (SIS-ECM) promotes tissue regeneration in animal models and noncardiac human applications. This retrospective review evaluates SIS-ECM for reconstruction of congenital heart defects.

Era effect on post-transplant survival adjusted for baseline risk factors in pediatric heart transplant recipients.

Background: Post-transplant survival in pediatric heart transplant (HT) recipients has improved for recent era recipients. However, the era effect has not been assessed after adjusting for baseline risk factors in HT recipients.

Methods: We compared baseline characteristics and 5-year survival in pediatric HT recipients in three eras (early: July 1994 to June 1997, n = 1,153; middle: July 1997 to June 2000, n = 1,085; recent: July 2000 to June 2003, n = 1,138) for all recipients <18 years of age who were reported to the registry of the International Society for Heart and Lung Transplantation (n = 3,376).

Long-term outcome of palliation with internal pulmonary artery bands after primary heart transplantation for hypoplastic left heart syndrome.

The purpose of this study was to describe the long-term outcome of infants with hypoplastic left heart syndrome (HLHS) who underwent placement of internal pulmonary artery bands as part of a transcatheter palliation procedure followed by primary heart transplantation. Transcatheter palliation included stenting of the ductus arteriosus, decompression of the left atrium by atrial septostomy, and internal pulmonary artery band placement. Cardiac hemodynamics, pulmonary artery architecture, and pulmonary artery growth since transplantation are described.

Interventional cardiac catheterization procedures in pediatric cardiac transplant patients: transplant surgery is not the end of the road.

Objectives: The objectives of this study are to report the spectrum of cardiac lesions in pediatric patients post-orthotopic heart transplantation (OHT), the characteristics of patients who develop these lesions, and the feasibility of transcatheter intervention in treating these lesions.

Background: Indications for OHT in the pediatric population range from cardiomyopathy to complex congenital heart defects with and without prior palliation. These patients may have residual vascular access and great vessel abnormalities.

Pediatric heart transplantation after declaration of cardiocirculatory death.

In three infants awaiting orthotopic cardiac transplantation, transplantation was successfully performed with the use of organs from donors who had died from cardiocirculatory causes. The three recipients had blood group O and were in the highest-risk waiting-list category. The mean age of donors was 3.

Registry of the International Society for Heart and Lung Transplantation: tenth official pediatric lung and heart/lung transplantation report--2007.

This tenth official pediatric report of the International Society for Heart and Lung Transplantation (ISHLT) covers the international pediatric lung and heart-lung transplantation experience from 1982 to 2006. As of last year's report, pediatric lung and heart-lung transplant data are now reported separately from pediatric heart transplant data and adult lung transplant data. For the first time this year, Registry data are analyzed by geographic region in addition to the usual aggregate analyses.

Danon disease presenting with dilated cardiomyopathy and a complex phenotype.

X-linked dilated cardiomyopathy (XLCM) was first described in 1987 and associated with dystrophin gene (DMD) mutations a decade later in one of the original two families. Here we report long-term follow-up of the second family (XLCM-2), for which a DMD mutation was never found. Analysis of the lysosome-associated membrane protein-2 (LAMP-2) gene detected a novel mutation, confirming a diagnosis of Danon disease.

Carvedilol for children and adolescents with heart failure: a randomized controlled trial.

Context: Although beta-blockers improve symptoms and survival in adults with heart failure, little is known about these medications in children and adolescents.

Objective: To prospectively evaluate the effects of carvedilol in children and adolescents with symptomatic systemic ventricular systolic dysfunction.

Design, Setting, And Participants: A multicenter, randomized, double-blind, placebo-controlled study of 161 children and adolescents with symptomatic systolic heart failure from 26 US centers.

Percutaneous selective pulmonary artery bands (Joeys) in a pulmonary overcirculation model.

Background: Control of distal pulmonary artery (PA) pressure and flow is a critical step in palliating infants with complex congential heart disease. Surgical procedures to protect or isolate the pulmonary circulation carry significant risk and can be unpredictable. Interventional control of pulmonary pressure/flow with an intravascular device (band) could reduce risk and improve regulation of pulmonary flow.

Prevalence of desmin mutations in dilated cardiomyopathy.

Background: Desmin-related myofibrillar myopathy (DRM) is a cardiac and skeletal muscle disease caused by mutations in the desmin (DES) gene. Mutations in the central 2B domain of DES cause skeletal muscle disease that typically precedes cardiac involvement. However, the prevalence of DES mutations in dilated cardiomyopathy (DCM) without skeletal muscle disease is not known.

Indications for heart transplantation in pediatric heart disease: a scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young; the Councils on Clinical Cardiology, Cardiovascular Nursing, and Cardiovascular Surgery and Anesthesia; and the Quality of Care and Outcomes Research Interdisciplinary Working Group.

Background: Since the initial utilization of heart transplantation as therapy for end-stage pediatric heart disease, improvements have occurred in outcomes with heart transplantation and surgical therapies for congenital heart disease along with the application of medical therapies to pediatric heart failure that have improved outcomes in adults. These events justify a reevaluation of the indications for heart transplantation in congenital heart disease and other causes of pediatric heart failure.

Methods And Results: A working group was commissioned to review accumulated experience with pediatric heart transplantation and its use in patients with unrepaired and/or previously repaired or palliated congenital heart disease (children and adults), in patients with pediatric cardiomyopathies, and in pediatric patients with prior heart transplantation.

Initial transcatheter palliation of hypoplastic left heart syndrome.

Initial percutaneous transcatheter palliation of hypoplastic left heart syndrome is now feasible. The primary procedures for palliation include stenting of the ductus arteriosus with a self expanding nitinol stent to secure an adequate systemic blood flow, placement of an internal pulmonary arterial band to protect the pulmonary vascular bed and to prevent pulmonary overcirculation, and widening of the interatrial communication by blade and balloon septostomy or static balloon dilation to decompress the left atrium. Anatomic variations of the ductus arteriosus have important implications for technical success with ductal stenting.

Exercise capacity in pediatric heart transplant candidates: is there any role for the 14 ml/kg/min guideline?

A peak oxygen consumption (VO2) of < 14 ml/kg/min has been identified as a predictor of l-year mortality in adults with congestive heart failure (CHF) and is used as a criterion for listing for cardiac transplantation (OHT). The role of VO2 measurement in children awaiting OHT has not been thoroughly evaluated. We sought to assess the degree of exercise impairment and the clinical applicability of the 14 ml/kg/min rule in children awaiting OHT.

Altered ventricular mechanics in cardiac allografts: a tissue Doppler study in 30 children without prior rejection events.

Background: Tissue Doppler imaging (TDI), a non-invasive echocardiography technique, permits quantitative analysis of the regional distribution pattern of myocardial velocities. During normal childhood development, regional function changes markedly, including an increasing predominance of longitudinal velocities. This study analyzed the impact of heart transplantation on ventricular mechanics in growing children.

Thymopoietin (lamina-associated polypeptide 2) gene mutation associated with dilated cardiomyopathy.

Thymopoietin or TMPO (indicated by its alternative gene symbol, LAP2, in this work) has been proposed as a candidate disease gene for dilated cardiomyopathy (DCM), since a LAP2 product associates with nucleoplasmic lamins A/C, which are encoded by the DCM gene LMNA. We developed a study to screen for genetic mutations in LAP2 in a large collection of DCM patients and families. A total of 113 subjects from 88 families (56 with familial DCM (FDC) and 32 with sporadic DCM) were screened for LAP2 mutations using denaturing high-performance liquid chromatography and sequence analysis.