Temporal change in cardiac function and clinical indices in adults with valvular pulmonic stenosis.

Background: Patients with palliated pulmonary valve stenosis (PVS) have less cardiac remodeling and symptoms as compared to patients with repaired tetralogy of Fallot (TOF) presenting with similar severity of right ventricular outflow tract (RVOT) disease. What is not known is whether patients with PVS versus TOF presenting with similar severity of RVOT disease at baseline, would have similar (or different) pace of cardiac remodeling and disease progression over time. The study objective was to compare temporal changes in clinical and cardiac function indices between adults with palliated PVS and repaired TOF presenting with moderate/severe RVOT disease.

Outcomes of Esophageal Varices in Adults With Fontan Palliation and Liver Cirrhosis.

Background: The purpose of this study was to define the risk and outcomes of esophageal varices in adults with Fontan palliation and liver cirrhosis undergoing esophagogastroduodenoscopy (EGD).

Method: The results of EGD, abdominal ultrasound, and liver biopsy, as well as clinic notes from the hepatologist, were reviewed to determine the diagnosis of cirrhosis and esophageal varices. The incidence of acute gastrointestinal bleeding complication was assessed among patients with esophageal varices using the time of EGD as the baseline.

Congenital Heart Disease in the Adult Cardiac Intensive Care Unit.

This article provides a broad overview of key concepts and more commonly encountered critical illness presentations in adult congenital heart disease (ACHD) patients. General principles are discussed, and the need for ACHD subspecialty consultation is emphasized. ACHD is categorized based on hemodynamic profile, and common clinical presentations are reviewed, including common pitfalls.

Evaluation of right ventricular strain in two separate cohorts with precapillary pulmonary hypertension.

Evaluation for right ventricular (RV) dysfunction is an important part of risk assessment in care of patients with pulmonary hypertension (PH) as it is associated with morbidity and mortality. Echocardiography provides a widely available and acceptable method to assess RV function. RV global longitudinal strain (RVGLS), a measure of longitudinal shortening of RV deep muscle fibers obtained by two-dimensional echocardiography, was previously shown to predict short-term mortality in patients with PH.

Transcatheter Mitral Valve Repair in a Tricuspid Atresia Patient With Potts and Glenn Shunts.

Our patient was a 50-year-old woman with tricuspid atresia who had undergone palliation with a Potts shunt to the left pulmonary artery as an infant and a classic Glenn shunt to the right pulmonary artery as a young child. Under general anesthesia, she underwent transcatheter edge-to-edge repair of the mitral valve for severe symptomatic mitral regurgitation. ().

Recurrent Coronary Artery Fistulae and a Novel Transforming Growth Factor Beta-3 Mutation.

Loeys-Dietz syndrome (LDS) is a rare connective tissue disease associated with mutations in transforming growth factor (TGF) signaling leading to an increased risk of arterial calcification, aneurysms, and/or dissections. We report a case in which genetics evaluation revealed a rare variant E244K in the TGFB3 gene. The variant leads to the substitution of glutamic acid for lysine, two amino acids with dissimilar properties.

Single-Barrel, Double-Barrel, and Fenestrated Endografts to Facilitate Transcatheter Pulmonary Valve Replacement in Large RVOT.

Objectives: The aim of this study was to test the hypothesis that narrowing the landing zone using commercially available endografts would enable transcatheter pulmonary valve replacement (TPVR) using commercially available transcatheter heart valves.

Background: TPVR is challenging in an outsized native or patch-repaired right ventricular outflow tract (RVOT). Downsizing the RVOT for TPVR is currently possible only using investigational devices.

PCSK9 inhibition in patients with heart transplantation: A case series.

PCSK9 inhibitors are potent low-density lipoprotein cholesterol-lowering medications. There is a lack of data regarding safety and efficacy of PCSK9 inhibitors in cardiac transplant patients. In this case series, we provide data supporting the low-density lipoprotein-lowering efficacy and short-term safety of PCSK9 inhibitors in three cardiac transplant patients.

Association of Antidepressant Medication Type With the Incidence of Cardiovascular Disease in the ARIC Study.

Background The association of antidepressant medication type with the risk of cardiovascular disease ( CVD ) is unclear. We hypothesized that selective serotonin reuptake inhibitors ( SSRI s) are associated with lower risks of CVD events relative to tricyclics and other non- SSRI antidepressants. Methods and Results We studied 2027 participants from the ARIC (Atherosclerosis Risk in Communities) study (mean age 63±10 years; 29% men; 78% white) treated with antidepressants at some time between 1987 and 2013.

Repeat Pulmonary Valve Replacement: Similar Intermediate-Term Outcomes With Surgical and Transcatheter Procedures.

Objectives: This study compares 30-day, 1-year, and 3-year echocardiographic findings and clinical outcomes of transcatheter pulmonary valve-in-valve replacement (TPVR) and repeat surgical pulmonary valve replacement (SPVR).

Background: In patients with adult congenital heart disease and previous pulmonary valve replacement (PVR) who require redo PVR, it is unclear whether TPVR or repeat SPVR is the preferred strategy.

Methods: We retrospectively identified 66 patients (TPVR, n = 36; SPVR, n = 30) with bioprosthetic pulmonary valves (PVs) who underwent either TPVR or repeat SPVR at Emory Healthcare from January 2007 to August 2017.

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary.

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.

CCR7 and CXCR4 Expression in Primary Head and Neck Squamous Cell Carcinomas and Nodal Metastases – a Clinical and Immunohistochemical Study.

Background: Squamous cell carcinomas (SCCs) are common head and neck malignancies demonstrating lymph node LN involvement. Recently chemokine receptor overxpression has been reported in many cancers. Of particular interest, CCR7 appears to be a strong mediator of LN metastases, while CXCR4 may mediate distant metastases.

De novo DQ donor-specific antibodies are associated with worse outcomes compared to non-DQ de novo donor-specific antibodies following heart transplantation.

Background: Antibody-mediated rejection (AMR) resulting from de novo donor-specific antibodies (dnDSA) leads to adverse outcomes following heart transplantation (HTx). It remains unclear what role dnDSA to specific HLA antigens play in adverse outcomes. This study compares outcomes in patients developing dnDSA to DQ antigens with those developing non-DQ dnDSA and those free from dnDSA.

Acute Embolic Myocardial Infarction and Heart Failure in a Fontan Patient: Recovery with Impella Device and Successful Transplantation.

Patients with Fontan palliation are at a higher risk of thromboembolic events, as well as sudden death. We report a case of acute thromboembolic myocardial infarction in a patient with Fontan circulation, presenting with acute heart failure. Coronary thrombolysis and reperfusion did not improve the myocardial dysfunction and warranted a unique application of mechanical support with Impella 2.

"Frontiers in Fontan failure: A summary of conference proceedings".

"Frontiers in Fontan Failure" was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical.

Catheter-measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End-organ Dysfunctions.

Background: In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood.

Methods: A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted.

Risk factors for major adverse events late after Fontan palliation.

Objective: Risk factors for major adverse events late after Fontan palliation are unknown. Prior studies have suggested ventricular function and morphology as important risk factors. The aim of this study is to (1) characterize the late major adverse event profile in adult Fontan patients and (2) identify additional risk factors that may contribute to adverse outcomes.

The effect of angiotensin-converting enzyme inhibitors on the rate of ascending aorta dilation in patients with bicuspid aortic valve.

Background: Bicuspid aortic valve (BAV), the most common congenital heart defect, is associated with progressive aortic dilation and increased risk of dissection and rupture. The use of an angiotensin-converting enzyme (ACE) inhibitor has recently proven effective in slowing the rate of aortic dilation in other aortopathies, suggesting it may also be useful in managing BAV. We sought to determine whether ACE inhibitors effectively reduce the rate of aortic dilation in adolescents and young adults with BAV.

Hemodynamic phenotype of the failing Fontan in an adult population.

Fontan failure can occur even with normal systolic ventricular function and often in the context of significant liver disease. We hypothesized that Fontan failure is hemodynamically distinct from traditional heart failure and characterized by low systemic vascular resistance (SVR) index and preserved cardiac index. Twenty-seven symptomatic adult Fontan (SAF) patients who underwent catheterization from 2001 to 2011 constituted our study group.