"Treat-to-close": Non-repairable ASD-PAH in the adult: Results from the North American ASD-PAH (NAAP) Multicenter Registry.

Background: Adults presenting with an unrepaired atrial septal defect and pulmonary arterial hypertension (ASD-PAH) are typically classified as "correctable" or "non-correctable". The use of directed PAH medical therapy in non-correctable ASD-PAH leading to favorable closure candidacy, repair status and long-term follow-up is not well studied. We therefore sought to characterize response to PAH targeted therapy in 'non-correctable' ASD-PAH.

3D Printing is a Transformative Technology in Congenital Heart Disease.

Survival in congenital heart disease has steadily improved since 1938, when Dr. Robert Gross successfully ligated for the first time a patent ductus arteriosus in a 7-year-old child. To continue the gains made over the past 80 years, transformative changes with broad impact are needed in management of congenital heart disease.

Insurance access in adults with congenital heart disease in the Affordable Care Act era.

Background: Adults with congenital heart disease (ACHD) have traditionally been viewed as an underinsured population. Whether this is true in the Affordable Care Act era is unknown. We determined insurance patterns in ACHD patients compared to the non-ACHD cardiology population in a contemporary cohort.

Impact of pregnancy on autograft dilatation and aortic valve function following the Ross procedure.

Objective: The effects of pregnancy on autograft dilatation and neoaortic valve function in patients with a Ross procedure have not been studied. We sought to evaluate the effect of pregnancy on autograft dilatation and valve function in these patients with the goal of determining whether pregnancy is safe after the Ross procedure.

Design: A retrospective chart review of female patients who underwent a Ross procedure was conducted.

Dynamic Evaluation of Coronary Anomalies Originating from the Opposite Sinus of Valsalva (ACAOS).

Coronary anomalies originating from the opposite sinus of Valsalva (ACAOS) are a rare anomaly associated with sudden cardiac death. Dynamic, invasive evaluation using coronary angiography, intravascular ultrasound, and fractional flow reserve can more clearly identify important pathophysiologic variants and guide treatment. This dynamic evaluation can assist the clinician in the appropriate surgical and percutaneous treatment options and aid in patient counseling.

Increased Body Mass Index Is Associated with Congestive Heart Failure and Mortality in Adult Fontan Patients.

Objective: Obesity affects adults with congenital heart disease (CHD). The effect of an increased body mass index (BMI) with respect to morbidity and mortality has not been evaluated in adults with complex CHD. Our objective was to evaluate the effects of increased BMI on heart failure and mortality in univentricular patients who had undergone Fontan palliation.

Contraceptive Use and Unintended Pregnancy in Women With Congenital Heart Disease.

Objective: To identify patterns of contraceptive use and pregnancy in an academic adult congenital cardiology practice.

Methods: In this cross-sectional study, from October 2013 through March 2014, 100 women with congenital heart disease aged 18-45 years were recruited from an academic congenital heart disease clinic and administered a survey regarding pregnancy history, contraception use, and understanding of pregnancy-related and contraceptive-related risk. The primary outcome was current use of long-acting reversible contraception, including intrauterine devices or subdermal implants.

Segmental analysis of congenital heart disease: putting the "puzzle" together with computed tomography.

Advances in surgical and medical treatment for congenital heart disease have resulted in greater life expectancy. As a result, there has been an increase in the utilization of cross-sectional imaging for diagnosis and management of complex congenital heart disease. This manuscript describes a morphological and sequential segmental approach to deciphering the code of complex congenital heart defects in cross-sectional imaging, mostly computed tomography.

Cardiovascular disease in late survivors of tetralogy of fallot: a tertiary care center experience.

Patients with tetralogy of Fallot can survive to late adulthood; however, there are few data on cardiovascular outcomes in this population. We conducted a single-center retrospective analysis of cardiovascular outcomes and risk factors in 208 patients with tetralogy of Fallot to better evaluate the burden of cardiovascular disease in this group. Descriptive statistics were used to determine the prevalence of relevant cardiovascular risk factors and outcomes, including a composite analysis of cardiovascular disease.

Usefulness of medical therapy for pulmonary hypertension and delayed atrial septal defect closure.

A subset of adult patients with an open atrial septal defect (ASD) have pulmonary arterial hypertension (PAH). We sought to identify predictors of response to PAH-specific medical therapy in this group. Invasive hemodynamic and clinical parameters from 12 patients with an open ASD and PAH (pulmonary vascular resistance [PVR], 8.

Massive global right ventricular hypertrophy with both fixed and dynamic obstruction and pulmonary valve dysplasia.

Congenital pulmonary valvular dysplasia is an unusual condition typically associated with Noonan syndrome. Among its other cardiac manifestations, occasional patients with Noonan syndrome have been demonstrated to have hypertrophic nonobstructive cardiomyopathy, which may be biventricular. We report a unique case of pulmonary valvular dysplasia, dynamic right ventricular outflow tract obstruction and profound right ventricular hypertrophy with only mild left ventricular hypertrophy, in a patient without Noonan syndrome.

Inferior type sinus venosus atrial septal defect: MR findings.

Atrial septal defects can occur at various levels of the interatrial septum. Sinus venosus atrial septal defect (SVASD) results from abnormal resorption of the embryologic sinus venosus, and may be of the superior or inferior type. In this paper, we describe a 46-year-old man with inferior-type SVASD who presented with arrhythmias and dyspnea.

Role of protein binding in renal elimination of leptin.

Objective: Leptin, a hormone produced by fat which signals to the brain the extent of fat stores, is known to be eliminated from circulation primarily by the kidney. The hormone circulates in both free and protein-bound forms, but there is little information concerning the inter-relationship of these forms of leptin, or which form is influenced by physiological processes such as renal elimination. We studied total, free and bound concentrations of leptin in ambulatory adults undergoing catheterization for diagnosis/management of congenital cardiac disease.

Identification and localization of a fatty acid response region in the human plasminogen activator inhibitor-1 gene.

The increased expression of plasminogen activator inhibitor type-1 (PAI-1) is associated with increased concentrations of fatty acids in blood and may accelerate atherogenesis in diabetes. The present study was designed to define mechanisms by which nonesterified (free) fatty acids (FFAs) augment the expression of PAI-1. FFAs increased PAI-1 protein and mRNA expression by HepG2 cells.

The human plasminogen activator inhibitor type I gene promoter targets to kidney.

The plasminogen activator inhibitor type 1 (PAI-1) gene encodes the physiological inhibitor of tissue-type and urokinase-type plasminogen activators and is induced by cytokines such as transforming growth factor-beta (TGF-beta). Studies have identified DNA sequence elements within the first 1.3 kb of the 5'-upstream DNA that mediate cytokine responsiveness in transfected cells in vitro.

Selective induction of the creatine kinase-B gene in chronic volume overload hypertrophy is not affected by ACE-inhibitor therapy.

Hypertrophied and failing myocardium has been shown to undergo creatine kinase (CK) isoform switching, resulting in increased MB and BB components. We tested the hypothesis that chronic volume overload hypertrophy due to mitral regurgitation in the dog causes CK isoenzyme switching and that this could be reversed by angiotensin converting enzyme inhibitor therapy. Thirteen adult mongrel dogs had mitral regurgitation induced by mitral valvular chordal rupture: six were treated with ramipril for 4 months and seven were untreated for 4 months.

Expression of the Gs protein alpha-subunit disrupts the normal program of differentiation in cultured murine myogenic cells.

The manner in which growth factors acting at the cell surface regulate activity of myogenic basic-helix-loop-helix proteins in the nucleus and thus control the fate of committed skeletal myoblasts remains poorly understood. In this study, we report that immunoreactive Gs protein alpha-subunits (Gs alpha) localize to nuclei of proliferating C2C12 myoblasts but not to nuclei of differentiated postmitotic C2C12 myotubes. To explore the biological significance of this observation, we placed a cDNA encoding Gs alpha in an expression vector under the control of a steroid-inducible promoter and isolated colonies of stably transfected C2C12 myoblasts.

Characterization of a nuclear protein that interacts with regulatory elements in the human B creatine kinase gene.

The B creatine kinase gene is regulated by an array of positive and negative cis-elements in the 5'-flanking DNA that function in both muscle and nonmuscle cells. In C2C12 myogenic cells M and B creatine kinase mRNAs are coordinately up-regulated in the early stages of myogenesis and then undergo distinct regulatory programs. The B creatine kinase gene is down-regulated in the late stages of myogenesis as M creatine kinase becomes the predominant species in mature myotubes.

A novel protein interacts with the major transforming growth factor-beta responsive element in the plasminogen activator inhibitor type-1 gene.

Multiple transforming growth factor-beta (TGF-beta) responsive elements have been identified within the 5'-flanking region of the plasminogen activator inhibitor type-1 (PAI-1) gene. This study was designed to characterize the major TGF-beta responsive element (-804 to -546). DNA footprint assays showed that the region of protein contact (-726 to -703) did not include consensus sequences for any known transacting factors.