Potential for and timing of recovery in children with dilated cardiomyopathy.

Objective: Understanding the clinical course and time-frame for recovery is helpful to guide management and counselling following a diagnosis of Dilated Cardiomyopathy (DCM). We aimed to document outcomes and time to recovery for a cohort of patients with a dilated cardiomyopathy phenotype.

Methods: An observational cohort methodology was used to collect retrospective data from the departmental database for those identified with DCM.

Heart failure from heart muscle disease in childhood: a 5-10 year follow-up study in the UK and Ireland.

Aims: Our original study, the first national prospective study of new-onset heart failure from heart muscle disease in children, showed overall 1-year survival of 82%, and event (death or transplantation)-free survival of 66%. This study aimed to evaluate 5 + year outcomes of this important cohort.

Methods And Results: All centres in the UK and Ireland with 1-year event-free survivors participated ( = 14).

The management of conjoined twins: Cardiology assessment.

Structural cardiac defects occur in at least 1 twin in about 75% of conjoined twins with thoracic level fusion. Outcomes after surgical separation of thoracic level conjoined twins have been favorable when the hearts have been separate. However, even in this situation, the outlook is poor for an individual twin with an important cardiac defect.

Plasma membrane Ca2+-ATPase 4: interaction with constitutive nitric oxide synthases in human sperm and prostasomes which carry Ca2+/CaM-dependent serine kinase.

Deletion of the gene encoding the widely conserved plasma membrane calcium ATPase 4 (PMCA4), a major Ca(2+) efflux pump, leads to loss of sperm motility and male infertility in mice. PMCA4's partners in sperm and how its absence exerts its effect on fertility are unknown. We hypothesize that in sperm PMCA4 interacts with endothelial nitric oxide synthase (eNOS) and neuronal nitric oxide synthase (nNOS) which are rapidly activated by Ca(2+), and that these fertility-modulating proteins are present in prostasomes, which deliver them to sperm.

Correlation of maternal flecainide concentrations and therapeutic effect in fetal supraventricular tachycardia.

Background: Transplacental flecainide is an established therapy for fetal supraventricular tachycardia (SVT), but there is a paucity of data regarding the dose-response relationship.

Objective: The purpose of this study was to investigate the relationship between maternal flecainide concentrations, arrhythmia control, and adverse fetal effects in fetal SVT.

Methods: Fetuses with SVT treated with transplacental flecainide at our tertiary fetal cardiology unit between 1997 and 2012 were retrospectively studied.

Peak oxygen uptake correlates with survival without clinical deterioration in ambulatory children with dilated cardiomyopathy.

Background: Children stable at home with dilated cardiomyopathy remain at risk of death; there is evidence of survival benefit for transplantation out to 4 years postoperatively. The limited supply of donor organs makes risk stratification imperative, but although cardiopulmonary exercise test is well established as a powerful tool in adults with heart failure, no published studies have linked oxygen uptake to prognosis in children.

Methods And Results: Between 2001 and 2009, using cardiopulmonary exercise test and echocardiography, we studied 82 children (mean age, 13.

Prediction of outcome of tricuspid valve malformations diagnosed during fetal life.

There are conflicting reports on the prenatal validity of echocardiographic indexes used to assess tricuspid valve malformations (TVM) in postnatal life. The aim of this study was to determine which echocardiographic factors are of most prognostic significance in prenatally-diagnosed TVM and to develop a clinically-based prognostic scoring system to better inform prenatal counseling. From a prospective database, 44 fetuses with isolated TVM were identified from 1995 to 2004, inclusive.

New-onset heart failure due to heart muscle disease in childhood: a prospective study in the United kingdom and Ireland.

Background: We undertook the first prospective, national, multicenter study to describe the incidence and outcome of heart muscle disease-induced heart failure in children.

Methods And Results: Data were collected on patients admitted to a hospital through 2003 with a first episode of heart failure in the absence of congenital heart disease. All 17 pediatric cardiac centers in the United Kingdom and Ireland participated.