Congenital heart disease

The Brugada syndrome is an inherited disorder associated with risk of ventricular fibrillation and sudden cardiac death in a structurally normal heart. Diagnosis is based on a characteristic electrocardiographic pattern (coved type ST-segment elevation ≥2 mm followed by a negative T-wave in ≥1 of the right precordial leads V1 to V2 ), observed either spontaneously or during a sodium-channel blocker test. The prevalence varies among regions and ethnicities, affecting mostly males. The risk stratification and management of patients, principally asymptomatic, still remains challenging...

BACKGROUND: This study sought to assess, using subjective (self-assessment) and objective (MCQ) methods, the efficacy of using heart models with ventricular septal defect lesions produced with three-dimensional printing technology in a congenital heart disease curriculum for medical students. METHODS: Three computed tomography datasets of three subtypes of ventricular septal defects (perimembranous, subarterial and muscular, one for each) were obtained and processed for building into and printing out 3D models...

AIMS: Religion and spirituality can be resources for internal strength and resilience, and may assist with managing life's challenges. Prior studies have been undertaken primarily in countries with high proportions of religious/spiritual people. We investigated (i) whether being religious/spiritual is an independent predictor of patient-reported outcomes (PROs) in a large international sample of adults with congenital heart disease, (ii) whether the individual level of importance of religion/spirituality is an independent predictor for PROs, and (iii) if these relationships are moderated by the degree to which the respective countries are religious or secular...

Adult congenital heart disease (ACHD) patients are at risk for end-stage heart failure; heart transplantation (Htx) represents the only definitive therapy available although not easily achievable for all patients. The study aims to assess the pathway difficulties and outcomes of ACHD patients with end-stage heart failure referred for Htx evaluation. This is a single center retrospective study on ACHD patients with end-stage heart failure referred to Htx evaluation from 2004 to 2015. Demographic data, medical history, failure modality, and follow-up were obtained from patient charts...

We assessed the value of existing guidelines for the evaluation of suspected congenital heart disease (CHD) in term neonates and propose a revised algorithm. Retrospective chart review of newborns referred for cardiac evaluation at a tertiary care center was performed. A total of 777 newborns qualified for the study. Among these, 3 critical and 8 major CHD were identified. The sensitivity of the combination of abnormal physical examination and pulse oximetry screening for major and critical CHD was 100%. The cost to detect a case of critical CHD, based on echocardiograms done for all abnormal electrocardiograms, was 3...

Down syndrome (DS) is the most common autosomal chromosome anomaly. DS is frequently associated with congenital heart disease (CHD). Patients with DS have 40-60% chance of having CHD. It means that CHD in DS is not only due to trisomy 21 and there are some other genetic factors underlying CHD in DS children. In this study, a total of 240 DNA samples from patients were analyzed including 100 patients with CHD only, 110 patients having CHD along with DS and 30 patients with isolated DS. A cardiovascular gene panel consisting of probes for 406 genes was used to screen DNA samples of all 240 patients for mutation identification...

Heart failure (HF) in the adult with congenital heart disease (ACHD) is associated with high morbidity and mortality and has been implicated as the leading cause of death in this patient population. The diagnosis of HF in ACHD involves a combination of clinical suspicion from subjective patient history, anatomic imaging, functional diagnostic studies, and rhythm evaluation. Once diagnosed, the approach to management of HF in this population varies widely and by lesion. Unfortunately, there is a paucity of literature available delineating the optimal management of these patients, making clinical decision-making extremely challenging...

BACKGROUND: Since the onset of pediatric catheter ablation, the pediatric electrophysiology community has reported outcomes via various registries (PAPCA [Prospective Assessment After Pediatric Cardiac Ablation], PCAR [Pediatric Catheter Ablation Registry]). Most recently, a modern era pediatric and congenital ablation registry (MAP-IT [Multicenter Pediatric and Congenital EP Quality Initiative]) was developed for eventual incorporation into the National Cardiovascular Data Registry (NCDR) IMPACT (Improving Pediatric and Adult Congenital Treatment) registry...

Patients with systemic morphological right ventricles (RVs), including congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries with a Mustard or Senning atrial baffle repair, have a high likelihood of developing systemic ventricular dysfunction. Unfortunately, there are a limited number of clinical studies on the efficacy of medical therapy for systemic RV dysfunction. We performed a systematic review and meta-analysis to assess the effect of angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), beta blockers, and aldosterone antagonists in adults with systemic RVs...

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No abstract text is available yet for this article.

Secundum atrial septal defect (ASD) is the most common adult congenital heart defect and can present with wide variation in clinical findings. With the intention of preventing morbidity and mortality associated with late presentation of ASD, consensus guidelines have recommended surgical or percutaneous ASD closure in adults with right heart enlargement, with or without symptoms. The aim of the present analysis was to determine if the protective effect of secundum ASD closure in adults could be qualified by pooling data from published studies...

The complex nature of congenital heart disease (CHD) has hindered the establishment of management standards for peri-catheterization anticoagulation. We sought to describe anticoagulation practice variability among providers performing cardiac catheterization in children and adults with CHD. A web-based survey ( http://www.surveymonkey.com ) was distributed to pediatric and congenital interventional cardiologists. Respondents were queried on their training, practice setting, years in practice, and case volume...

Quality indicators for adult congenital heart disease (ACHD) were recently published due to a lack of consensus regarding delivery of care to adults with congenital heart disease (CHD). The objective of this study was to examine adherence to quality indicators for the care of patients with tetralogy of Fallot. Adults with tetralogy of Fallot seen in outpatient cardiology clinics at a tertiary care facility between July 2014 and June 2015 were included, and electronic medical records for each visit were reviewed...

No abstract text is available yet for this article.

The worldwide population of adults with congenital heart disease (ACHD) has grown steadily over the last few decades, estimated at approximately 1.4 million people in 2010 (Gilboa et al., 2016). Innovations in surgical and medical treatment of children with congenital heart disease (CCHD) have dramatically improved survival, resulting in a growing population of ACHD patients with unique health needs (Marelli et al., 2014; Khairy et al., 2010). This growth has significant implications for health care providers and health systems, which must adapt to meet the demands of caring for this complex population...

PURPOSE OF REVIEW: The aim of this study is to review genetics of congenital heart disease (CHD) with a focus on clinical applications, genetic testing and clinical challenges. RECENT FINDINGS: With improved clinical care, there is a rapidly expanding population of adults, especially women, with CHD who have not undergone contemporary genetic assessment and do not understand their risk for having a child with CHD. Many patients have never undergone assessment or had genetic testing...

Congenital heart disease is the most common birth defect worldwide, and accounts for a high proportion of the world's infant mortality. About 9 of every 10 babies born each year are born in areas without adequate access to heart surgery; overcoming this problem will necessitate addressing the worldwide shortage of an estimated 3,700 pediatric cardiac surgeons. Establishing sustainable heart surgery programs requires more than an investment of money: political, social, and cultural issues unique to each environment need to be addressed...

Optimal outcomes are as much influenced by critical decision making pathways as by the technical skill of the operator. The complexity and potential cognitive traps underlying critical decision making has long been recognized in the aviation and business communities, however, remains a largely subconscious, unexamined discipline amongst congenital cardiac interventionalists. Challenges to making good decisions in the catheterization laboratory include heuristics, biases, and cognitive traps. In this paper we discuss some of the more common decision making challenges encountered and we address potential solutions to such decision making with particular focus towards standardization...

OBJECTIVE: The aim of this study was to compare inpatient outcomes and costs for children with respiratory syncytial virus and congenital heart disease before and after the change in management guidelines for respiratory syncytial virus prophylaxis. DESIGN: Hospital discharge data from the Vizient (formerly University HealthSystem Consortium) were queried from October 2012 to June 2014 (Era 1) and July 2014 to April 2016 (Era 2) for patients aged <24 months with an any International Classification of Disease (ICD)-9 or ICD-10 code for congenital heart disease (745-747...