Congenital heart disease

OBJECTIVE: Hospital readmissions are increasingly becoming a metric for quality in the current landscape of changing and cost effective medicine. However, no 30-d readmission rates have been established for pediatric cardiac medical patients in the United States. Thus, the objective was to determine 30 d readmission rates and risk factors associated with readmission for pediatric cardiac patients, hypothesizing that pediatric cardiac patients would have significantly higher readmission rates than their general pediatric counterparts...

BACKGROUND: Long-term survival for persons born with congenital heart disease (CHD) is improved, but limited knowledge exists of this growing population's acquired cardiovascular risk profile. This study's purpose was to assess CHD survivors' risk for type 2 diabetes mellitus (T2DM) with attention to the impact of cyanotic CHD. METHODS AND RESULTS: This population-based cohort study included Danish subjects with CHD who were born between 1963 and 1980 and were alive at age 30 years...

BACKGROUND: High pulmonary vascular resistance (PVR) may be a risk factor for early and late mortality in both Glen shunt and Fontan operation patients. Furthermore, PVR may increase long after the Fontan operation. Whether pulmonary vasodilators such as phosphodiesterase 5 inhibitors can decrease PVR in patients with single ventricular physiology remains undetermined. METHODS AND RESULTS: This was a prospective, multicenter study. Patients with single ventricular physiology who have a PVR index higher than 2...

OBJECTIVES: We investigated the effect of balloon pulmonary valvuloplasty (BPV) as a palliative procedure for patients with tetralogy of Fallot (TOF). METHODS: This was a retrospective single-center study conducted between 2008 and 2014. We classified patients into three groups according to palliation: treatment with BPV (group I), with a Blalock-Taussig shunt (group II), and with infundibulectomy (group III). The growth of the pulmonary valve (PV) annulus and need for transannular patching (TAP) during total correction were compared between groups...

AIM: Medical imaging by using FDG-PET/CT (PET-CT) can detect, confirm or eliminate with high sensitivity areas of suspected infections in case of persistent fever of unknown origin in combination with other bacteriological examinations. The aim of this study was to assess the potential role of PET-CT in detecting or excluding infections or other inflammatory processes in patients with congenital heart defects (CHD). In addition we wanted to evaluate the practical impact of PET-CT on the subsequent clinical management...

OBJECTIVES: To evaluate the safety, feasibility, and efficacy of the MVP™ microvascular plug(Covidien) for closure of vascular anomalies in patients with congenital heart diseases (CHD). BACKGROUND: The MVP™ is a novel device with PTFE integrated. The device has been recently introduced in the radiological field and reported exclusively for neurological anomalies. METHODS: All CHD patients receiving the device from April 2015 until July 2015 were included in the study and followed up clinically as well as by transthoracic echocardiography...

PURPOSE: This study aimed to measure parents' readiness for discharge from a pediatric cardiology/cardiac surgical inpatient unit. DESIGN AND METHODS: An observational study was conducted at a single tertiary care pediatric cardiac surgical program; parents received teaching from a discharge coordinator, bedside nurse, and, if needed, dietician and pharmacist. We surveyed parents/guardians on the day of discharge and 2 weeks later. RESULTS: We enrolled 181 participants, 53% with children <12 months of age...

AIMS: To describe the outcomes of pregnancy in women with pulmonary hypertension. METHODS AND RESULTS: In 2007 the European Registry on Pregnancy and Heart Disease was initiated by the European Society of Cardiology. Consecutive patients with all forms of cardiovascular disease, presenting with pregnancy, were enrolled with the aim of investigating the pregnancy outcomes. This subgroup of the cohort included 151 women with pulmonary hypertension (PH) either diagnosed by right heart catheterization or diagnosed as possible PH by echocardiographic signs, with 26% having pulmonary arterial hypertension (PAH), in three subgroups: idiopathic (iPAH), associated with congenital heart disease (CHD-PAH), or associated with other disease (oPAH), and 74% having PH caused by left heart disease (LHD-PH, n = 112)...

Postoperative junctional ectopic tachycardia (JET) is a narrow complex tachycardia and most frequently occurs during and after surgical repair of certain types of congenital heart defects. Postoperative junctional ectopic tachycardia may produce unfavorable hemodynamics that prolongs stays in the cardiac intensive care unit and hospital, prolongs time on a ventilator, and occasionally requires the use of extracorporeal membrane oxygenation (ECMO) as rescue therapy. The present report describes a rare case of late-onset postoperative junctional ectopic tachycardia, which occurred 13 days after the deployment of a perimembranous ventricular septal defect (PmVSD) occluder in a 17-year-old female teenager...

Congenital cardiovascular malformations are the most common birth defects, with a complex multifactorial etiology. Genetic factors play an important role, illuminated by numerous cytogenetically visible abnormalities, as well as submicroscopic genomic imbalances affecting critical genomic regions in the affected individuals. Study of rare families with Mendelian forms, as well as emerging next-generation sequencing technologies have uncovered a multitude of genes relevant for human congenital cardiac diseases...

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive genetic disorder. It is characterized by heterogeneous clinical manifestations including primary features of the disease (rod-cone dystrophy, polydactyly, obesity, genital abnormalities, renal defects, and learning difficulties) and secondary BBS characteristics (developmental delay, speech deficit, brachydactyly or syndactyly, dental defects, ataxia or poor coordination, olfactory deficit, diabetes mellitus, congenital heart disease, etc.); most of these symptoms may not be present at birth but appear and progressively worsen during the first and second decades of life...

Common arterial trunk with associated double aortic arch is a very rare constellation of congenital heart disease. Prenatal diagnosis allows for surgical repair prior to development of respiratory morbidity, which is otherwise described in all cases with this association.

Prenatal diagnosis of tetralogy of Fallot remains less frequent compared to other major congenital heart defects. In this study, we examined how often the 3-vessel and trachea view was abnormal in a large series of prenatally diagnosed cases of tetralogy of Fallot. In addition, we compared its sensitivity to that of the traditional outflow tract views for detection of tetralogy of Fallot. We found that both views were abnormal in all fetuses with tetralogy of Fallot, showing reversed aortic-to-pulmonary valve and aortic arch isthmus-to-ductus arteriosus ratios in the outflow tract and 3-vessel and trachea views, respectively...

BACKGROUND: Echocardiographic parameters of ventricular deformation of the systemic right ventricle (sRV) predict adverse clinical outcome in patients after atrial repair of transposition of the great arteries (TGA). We assessed myocardial deformation on cardiac MRI (CMR) and correlated these with clinical and conventional CMR parameters in TGA patients. METHODS: Retrospective analysis of CMR studies in 91 TGA patients (66% male; mean age 30.1±5.1years) at two tertiary adult congenital heart centers was conducted...

BACKGROUND: Because of advancements in care, there has been a decline in mortality from congenital heart defects (CHDs) over the past several decades. However, there are no current empirical data documenting the number of people living with CHDs in the United States. Our aim was to estimate the CHD prevalence across all age groups in the United States in the year 2010. METHODS: The age-, sex-, and severity-specific observed prevalence of CHDs in Québec, Canada, in the year 2010 was assumed to equal the CHD prevalence in the non-Hispanic white population in the United States in 2010...

No abstract text is available yet for this article.

Over the past 20 years, the successes of neonatal and infant surgery have resulted in dramatically changed demographics in ambulatory cardiology. These school-aged children and young adults have complex and, in some cases, previously unexpected cardiac and non-cardiac consequences of their surgical and/or transcatheter procedures. There is a growing need for additional cardiac and non-cardiac subspecialists, and coordination of care may be quite challenging. In contrast to hospital-based care, where inpatient care protocols are common, and perioperative expectations are more or less predictable for most children, ambulatory cardiologists have evolved strategies of care more or less independently, based on their education, training, experience, and individual styles, resulting in highly variable follow-up strategies...

BACKGROUND: The National Quality Forum previously approved a quality indicator for mortality after congenital heart surgery developed by the Agency for Healthcare Research and Quality (AHRQ). Several parameters of the validated Risk Adjustment for Congenital Heart Surgery (RACHS-1) method were included, but others differed. As part of the National Quality Forum endorsement maintenance process, developers were asked to harmonize the 2 methodologies. METHODS AND RESULTS: Parameters that were identical between the 2 methods were retained...

No abstract text is available yet for this article.

OBJECTIVE: Biomarkers play a pivotal role in heart failure (HF) management. Reference values and insights from studies in adults cannot be extrapolated to the paediatric population due to important differences in pathophysiology and compensatory reserve. We assessed the diagnostic utility of four novel biomarkers in paediatric HF. METHODS: Midregional (MR) pro-atrial natriuretic peptide (proANP), soluble ST2 (sST2), growth differentiation factor-15 (GDF-15), MR-pro-adrenomedullin (proADM) and N-terminal pro-B natriuretic peptide (NT-proBNP) were measured in 114 patients and 89 controls...