Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol versus losartan in young patients with MFS. Data were analyzed from the echocar

OBJECTIVE: Determine prevalence and associations with pulmonary hypertension (PH) in preterm infants.STUDY DESIGN: Prospective institutional echocardiographic PH screening at 36 to 38 weeks' corrected gestational age (GA) for infants born

OBJECTIVE: Trans-catheter (TC) pulmonary valve replacement (PVR) has become common practice for patients with right ventricular outflow tract obstruction (RVOTO) and/or pulmonic insufficiency (PI). Our aim was to compare PVR and right ventricular (RV) function of patients who received TC vs surgical PVR.DESIGN: Retrospective review of echocardiograms obtained at three time points: before, immediat

BACKGROUND: Pulmonary hypertension (PH) in infants with bronchopulmonary dysplasia (BPD) is associated with increased morbidity and mortality. Elevated levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) and decreased levels of amino acid precursors of nitric oxide (NO) have been associated with PH, but have not been studied in infants with PH secondary to BPD.OBJECTIVE: The aim of thi

Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality, occurring in 1% to 2% of the general population. Adults with degenerative aortic valve (AV) disease have been shown to have an elliptical shaped AV annulus. The goal of this study was to investigate the shape of the aortic annulus in children with BAV, coarctation of the aorta (CoA) with or without BAV, and normal contr

AIMS: Aortic stiffness and diastolic function are abnormal in adults with bicuspid aortic valves (BAVs). The goal of this study was to determine the relationship between aortic stiffness and left ventricular (LV) diastolic impairment in children with well-functioning BAV and no associated congenital heart disease.METHODS AND RESULTS: This is a retrospective review of echocardiograms in children wi

BACKGROUND: Pulmonary hypertension (PH) is a common problem in the neonatal intensive care unit and is associated with significant morbidity and mortality. The aim of this study was to identify a quantitative echocardiographic marker of septal curvature that can be used to accurately identify PH in NICU infants with concern for PH.METHODS: Echocardiograms of infants who were prematurely born and i

BACKGROUND: As the survival of children with cardiac disease increases, chronic complications of deep venous thrombosis from cardiac catheterization, particularly post-thrombotic syndrome, may be important to monitor for and treat, if needed. We aimed to determine the prevalence of this syndrome in children who underwent cardiac catheterization.PROCEDURE: We conducted a cross-sectional study of ch

BACKGROUND: Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers.METHODS: We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome w

BACKGROUND: Restrictive lung disease is a complication in childhood cancer survivors who received lung-toxic chemotherapy and/or thoracic radiation. Left ventricular dysfunction is documented in these survivors, but less is known about right ventricular (RV) function. Quantitative echocardiography may help detect subclinical RV dysfunction. The aim of this study was to assess RV function quantitat

BACKGROUND: Quantitative assessment of right ventricular (RV) function is important for the management of patients with congenital heart disease. Tissue Doppler (TDI)-derived myocardial acceleration during isovolumic contraction (IVA) is an echocardiographic measure of contractility that is independent of loading conditions. The aim of this study was to establish normative data for IVA at the late

Based on their review of abstracts submitted to the German Cardiac Society, Boehm et al. (2014) report better success of female vs male cardiologists publishing in journals with an impact factor ≥5. However, only 25% of conference abstracts were submitted by women, perhaps suggesting a paucity of women in academic cardiology. In this 'letter to the editor' we review gender statistics in the medica

BACKGROUND: The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects.METHODS AND RESULTS: Between 2007 and 2011, 21 clinical sites randomized 608 subj

OBJECTIVES: Early extubation in adults undergoing surgery for congenital heart disease has not been described. The authors report their experience with extubation in the operating room (OR), including factors associated with the decision to defer extubation to a later time.DESIGN: A retrospective chart review.SETTING: A tertiary-care teaching hospital.PARTICIPANTS: This study included adults under

BACKGROUND: Fast-tracking and early endotracheal extubation have been described in patients undergoing surgery for congenital heart disease (CHD); however, criteria for patient selection have not been validated in a prospective manner. Our goal in this study was to prospectively identify factors associated with the decision to defer endotracheal extubation in the operating room (OR).METHODS: We pe

PURPOSE OF REVIEW: As our understanding of the molecular regulation of cardiac development has progressed, an increasing number of genes that cause congenital heart disease when mutated are being identified. This review focuses on the progress made during the past year.RECENT FINDINGS: After PTPN11 was identified as a Noonan syndrome disease gene, additional discoveries have made clear that mutati

PTPN11 missense mutations cause approximately 50% of Noonan syndrome, an autosomal dominant disorder presenting with various congenital heart defects, most commonly valvar pulmonary stenosis, and hypertrophic cardiomyopathy. Atrioventricular septal defects and coarctation of the aorta occur in 15% and 9%, respectively. The aim of this study was to determine if PTPN11 mutations exist in non-syndrom