Association of Body Mass Index With Clinical Features and Outcomes in Adults With Fontan Palliation.

Background With improving survival of patients with single ventricle physiology who underwent Fontan palliation, there is also an increase in the prevalence of overweight and obesity in these patients. This tertiary care single-center study aims to determine the association of body mass index (BMI) with the clinical characteristics and outcomes in adults with Fontan. Methods and Results Adult patients (aged ≥18 years) with Fontan who were managed at a single tertiary care center between January 1, 2000, and July 1, 2019, and had BMI data available were identified via retrospective review of medical records.

Journey Toward Improved Long-Term Outcomes After Norwood-Sano Procedure: Focus on the Aortic Arch Reconstruction.

The disadvantage of right ventricle-to-pulmonary artery (RV-PA) shunt is the need for more unplanned interventions to address stenosis in the shunt or branch pulmonary arteries, as compared to the modified Blalock-Taussig shunt group. Ring-enforced RV-PA PTFE conduit and dunk technique minimized these complications and right ventricle (RV) damage. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation; therefore, most surgeons prefer to use homograft, autologous pericardium, or bovine pericardium to reconstruct the neoaorta.

Breaking the Rules: Left Common Carotid Artery from the Main Pulmonary Artery in an Infant with a Vascular Ring.

Anomalous left common carotid artery is a unique and rare abnormality. Most aortic arch anomalies can be explained by the totipotential aortic arch. Rare anomalies may not fit traditional arch regression models.

Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle.

Objective: This tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.

Methods: This retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.

Association of Insurance Status With Emergent Versus Nonemergent Hospital Encounters Among Adults With Congenital Heart Disease.

Background Although the number of hospital visits has exponentially increased for adults with congenital heart disease (CHD) over the past few decades, the relationship between insurance status and hospital encounter type remains unknown. The purpose of this study was to evaluate the association between insurance status and emergent versus nonemergent encounters among adults with CHD ≥18 years old. Methods and Results We used California Office of Statewide Health Planning and Development Database from January 2005 to December 2015 to determine the trends of insurance status and encounters and the association of insurance status on encounter type among adults with CHD.

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

Background: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported.

360-Degree Cone Reconstruction for Ebstein's Anomaly.

Several modifications of the cone procedure as first described by da Silva have been reported. We have used a complete 360-degree leaflet detachment coupled with a 60-degree counterclockwise rotation in performing the cone procedure in children and adults. We believe that complete leaflet detachment coupled with counterclockwise rotation provides both for better alignment of the leaflets and subvalvular apparatus and for a more symmetric distribution of tension after leaflet reattachment, thus potentially resulting in better valve function and longevity of repair.

S-Nitrosohemoglobin Levels and Patient Outcome After Transfusion During Pediatric Bypass Surgery.

Banked blood exhibits impairments in nitric oxide (NO)-based oxygen delivery capability, reflected in rapid depletion of S-nitrosohemoglobin (SNO-Hb). We hypothesized that transfusion of even freshly-stored blood used in pediatric heart surgery would reduce SNO-Hb levels and worsen outcome. In a retrospective review (n = 29), the percent of estimated blood volume (% eBV) replaced by transfusion directly correlated with ventilator time and inversely correlated with kidney function; similar results were obtained in a prospective arm (n = 20).

Scimitar Syndrome-Complex Surgical Revision 3 Decades After Repair.

We present a case of a 39-year-old woman with scimitar syndrome who had a 2-patch repair 3 decades previously and presented with a right-to-left shunt of the inferior vena cava (IVC) to the left atrium resulting from baffle dehiscence. We discuss details of our reoperative repair.

Venous Myocardial Infarction in an Infant with Obstructed Totally Anomalous Pulmonary Venous Drainage and Coronary Sinus Ostial Atresia.

We report a rare causal association between obstructed supracardiac totally anomalous pulmonary venous drainage and coronary sinus ostial atresia. Our 12-week-old patient developed venous myocardial infarction secondary to coronary venous hypertension because her sole route of coronary venous drainage was obstructed. She recovered after the obstruction was relieved by balloon dilation.

Characteristics of Hospitalizations for the Glenn Procedure in Those With Isomerism Compared to Those Without.

Isomerism, also known as heterotaxy, is a unique clinical entity in which there are mirror imaged findings in the thoracic. In the abdomen, the arrangement of organs can be random. The anatomic findings also have functional consequences.

Pyloric exclusion for treatment of complicated duodenal atresia.

Duodenal atresia (DA) is a well-described congenital anomaly that usually responds well to surgical correction. Associated defects are common, and these confounding variables often influence outcome. The authors present a case of a newborn female with an unusual constellation of problems including DA with annular pancreas, trisomy 21, and coarctation of the aorta.

Fetal diagnosis of superior vena cava aneurysm.

Superior vena cava aneurysm is a rare intrathoracic vascular lesion with only 27 cases reported in the literature. The majority are fusiform and can be associated with cystic hygroma due to the close embryonic relationship between lymphatic vessels and systemic veins. This is the first report of superior vena cava aneurysm diagnosed with fetal echocardiography in a fetus with a cystic hygroma.

Staged cleft sternum repair using dermal allograft and synthetic mesh.

Cleft sternum is a rare congenital chest deformity that develops during the first trimester. Failure of the process of midline mesenchymal strip fusion leads to absence of the sternum, resulting in cleft formation. Multiple surgical approaches have been described in the closure of sternal clefts.

Long-term Outcome and Risk of Heart Block After Surgical Treatment of Subaortic Stenosis.

Although mortality following repair of subaortic obstruction is low, aggressive resection may increase morbidity. We sought to evaluate outcomes and risk of atrioventricular heart block (AVB) after subaortic resection in the current era. Simple obstruction was defined as a discrete subaortic membrane and complex as multilevel or diffuse narrowing.

Management of deep wound complications with vacuum-assisted therapy after Berlin Heart EXCOR ventricular assist device placement in the pediatric population.

Wound complications after ventricular assist device (VAD) placement remain a formidable challenge to surgeons. The Berlin Heart EXCOR VAD is a versatile pulsatile system that has been successful in pediatric patients of all ages and sizes. Prevention of device-related complications such as infection, particularly in pediatric patients, remains an essential issue in minimizing patient morbidity and mortality.

Experience with the Levitronix CentriMag in the pediatric population as a bridge to decision and recovery.

Short-term mechanical circulatory support in the pediatric population with acute cardiac failure has traditionally been limited to extracorporeal membrane oxygenation given the limited availability of pediatric-sized pumps. The Levitronix CentriMag system (Thoratec Corporation, Pleasanton, CA, USA) offers expanded options for short-term support for this population. We report our experience with the successful use of the CentriMag in the pediatric population as a bridge to decision after postcardiotomy ventricular failure and as a bridge to recovery after heart transplantation.

Effect of ABO-incompatible listing on infant heart transplant waitlist outcomes: analysis of the United Network for Organ Sharing (UNOS) database.

Background: Midterm heart transplant outcomes of ABO-incompatible (ABO-I) organ use in infants are favorable. ABO-I transplantation has resulted in reduced waitlist mortality in some countries. This study assessed the effect of an ABO-I listing strategy on pre-transplant outcomes in the United States.

Coarctation repair in neonates and young infants: is small size or low weight still a risk factor?

Objective: Previous reports of neonatal coarctation repair demonstrate a high rate of recurrent arch obstruction in small neonates. This study assesses the effect of patient size on reintervention and survival in neonates and infants undergoing repair of simple aortic coarctation.

Methods: From 1996 to 2006, 167 neonates and infants younger than 90 days with simple coarctation underwent repair.