Noncardiac congenital anomaly

BACKGROUND: After pediatric heart operations, we sought to determine the incidence of unplanned cardiac reinterventions during the same hospitalization, assess risk factors for these reinterventions, and explore associations between reinterventions and outcomes. We hypothesized that younger patients undergoing more complex operations would be at greater risk for unplanned cardiac reinterventions and that operative mortality and postoperative length of stay (PLOS) would be greater in patients who undergo reintervention than in those who do not...

INTRODUCTION: The aim of the current study was to determine the effect of general anesthesia on neonatal brain activity using amplitude-integrated EEG (aEEG). METHODS: A prospective cohort study of neonates (January 2013-December 2015), who underwent major neonatal surgery for non-cardiac congenital anomalies. Anesthesia was administered at the discretion of the anesthetist. aEEG monitoring was started six hours preoperatively until 24 hours after surgery. Analysis of classes of aEEG background patterns, ranging from continuous normal voltage to flat trace in six classes, and quantitative EEG-measures, using spontaneous activity transients (SATs) and interSATintervals (ISI), was performed...

Congenital coronary artery anomalies as a whole are uncommon. Abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) is probably the most common congenital coronary defect. An overwhelming majority of the patients with untreated ALCAPA do not survive to adulthood. As yet, there is no consensus on the management of adults with ALCAPA. We describe a patient with breast malignancy and incidentally detected ALCAPA; primacy was given to treatment of the oncologic condition as a first step...

OBJECTIVES: We sought to develop a risk-adjustment methodology for length of stay in congenital heart surgery, as none exist. DESIGN: Prospective cohort analysis combined with previously obtained retrospective cohort analysis of a Department of Cardiovascular Surgery clinical database. PATIENTS: Patients discharged from Boston Children's Hospital between October 1, 2006, and May 31, 2014, that underwent a congenital heart surgery procedure(s) linked to one of 103 surgical procedure types...

During the past decade, a hybrid procedure has emerged and dramatically evolved as an alternative stage I palliation to the conventional Norwood procedure in neonates with hypoplastic left heart syndrome (HLHS). The hybrid approach avoids the need for cardiopulmonary bypass (CPB) utilizing stenting of the arterial duct and bilateral pulmonary artery banding. Cerebral and coronary perfusion pressure is maintained, and the pulmonary vasculature is protected from higher systemic pressure. Elimination of risks associated with CPB gains vital time to stabilize the patient and correct coexisting noncardiac anomalies and allows growth in preparation for the later stages of the Fontan pathway...

OBJECTIVE: To evaluate the incidence of brain injury after neonatal surgery for noncardiac congenital anomalies using magnetic resonance imaging (MRI). STUDY DESIGN: An MRI was obtained in 101 infants at 7 days [range: 1-115] after neonatal surgery for major noncardiac congenital anomalies. Brain injury was assessed using T1, T2, diffusion weighted imaging, and susceptibility-weighted imaging. RESULTS: Thirty-two preterm infants (<37 weeks of gestation) and 69 full-term infants were included...

OBJECTIVE: To delineate risk factors for failure of extubation in the operating room among pediatric cardiac surgery patients. DESIGN: Prospective, observational study. SETTING: Single center, tertiary care, teaching hospital. PARTICIPANTS: The study comprised 448 congenital cardiac surgery patients who were enrolled for intended extubation in the pediatric cardiac operating room over 5 years. INTERVENTIONS: The airways of enrolled patients were extubated in the operating room if predetermined suitability criteria were met...

The detection of newborn babies with potentially life-threatening, critical congenital heart defects (CCHDs) before they collapse or expire remains an important clinical challenge. The absence of physical signs and the difficulty assessing mild cyanosis means that the newborn baby check misses up to a third of babies. Fetal anomaly ultrasound scanning identifies an increasing proportion, but this screen is operator-dependent and therefore highly variable; although some units report very high detection rates, overall most babies with CCHD are still missed...

OBJECTIVES: The objectives of this review are to discuss the pathophysiology, clinical impact and treatment of major noncardiac anomalies, and prematurity in infants with congenital heart disease. DATA SOURCE: MEDLINE and PubMed. CONCLUSION: Mortality risk is significantly higher in patients with congenital heart disease and associated anomalies compared with those in whom the heart defect occurs in isolation. Although most noncardiac structural anomalies do not require surgery in the neonatal period, several require surgery for survival...

Ebstein's anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. Minor anomalies of the tricuspid valve may not be recognized until adulthood whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life. Echocardiography is the imaging modality of choice for both diagnosis and management of patients with Ebstein's anomaly. Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery...

BACKGROUND: Among patients with congenital heart disease (CHD), the coexistence of noncardiac congenital anatomic abnormalities (NC), genetic abnormalities (GA), and syndromes (S) may influence therapeutic strategies and outcomes. The appreciated prevalence of these abnormalities has risen because increased screening and improved diagnostic precision enable identification of these comorbidities in a larger fraction of neonates with CHD. We examined the contemporary prevalence and distribution of NC/GA/S across diagnostic groups among neonates undergoing cardiac operations using a large nationally representative clinical registry...

CONTEXT: Increasing concerns have been raised about the incidence of neurodevelopmental delay in children with noncardiac congenital anomalies (NCCA) requiring neonatal surgery. OBJECTIVE: This study aimed to determine the incidence and potential risk factors for developmental delay after neonatal surgery for major NCCA. DATA SOURCES: A systematic search in PubMed, Embase and the Cochrane Library was performed through March 2015. STUDY SELECTION: Original research articles on standardized cognitive or motor skills tests...

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. RESULTS: The median age at diagnosis of was 5...

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with high mortality. Survival is influenced by the extent of pulmonary hypoplasia and additional congenital defects. The purpose of this study was to assess the association of congenital anomalies and admission capillary carbon dioxide levels (PcCO2), as a measure of extent of pulmonary hypoplasia, on survival in neonates with CDH. METHODS: This is a retrospective review of neonates with CDH admitted to a tertiary neonatal intensive care unit between 1990 and 2014...

The patients with complicated congenital heart diseases are reaching adulthood with advances in corrective surgeries and medical management. Impact of anesthetic agents on complex cardiac and extra cardiac anomalies and presence of previous palliative procedures can be a challenge for the anesthesiologist perioperatively, while these patients present for cardiac/noncardiac surgeries. We report the perioperative management of a patient with ventricular septal defect, dextrocardia, pulmonary hypertension, and situs inversus who underwent a successful hernioplasty and hydrocelectomy with a combined spinal epidural anesthesia...

Cases with congenital heart defects (CHD) often have other associated anomalies. The purpose of this investigation was to assess the prevalence and the types of associated anomalies in CHD in a defined population. The anomalies associated with CHD were collected in all live births, stillbirths and terminations of pregnancy during 26 years in 346,831 consecutive pregnancies of known outcome in the area covered by our population based registry of congenital anomalies. Of the 4005 cases with CHD born during this period (total prevalence of 115...

OBJECTIVE: We sought to examine the association between increased first-trimester fetal nuchal translucency (NT) measurement and major noncardiac structural birth defects in euploid infants. STUDY DESIGN: Included were 75,899 singleton infants without aneuploidy or critical congenital heart defects born in California in 2009 through 2010 with NT measured between 11-14 weeks of gestation. Logistic binomial regression was employed to estimate relative risks (RRs) and 95% confidence intervals (CIs) for occurrence of birth defects in infants with an increased NT measurement (by percentile at crown-rump length [CRL] and by ≥3...

BACKGROUND: The prevalence of Down syndrome (DS)-affected births has increased during the past 30 years; moreover, children with DS have a higher incidence of congenital heart disease compared with their peers. Whether children with DS have better or worse outcomes after repair of congenital heart disease is unclear. We sought to identify differences in in-hospital mortality after cardiac surgery in pediatric patients with and without DS using a large national database. METHODS AND RESULTS: Children aged <18 years who underwent surgical intervention for congenital heart disease were identified using the Kids' Inpatient Database (2000, 2003, 2006, and 2009)...

AIMS: Common atrium (CA) is a rare congenital heart defect. We reviewed our experience of surgical treatment of CA and summarize the clinical features of CA and the key techniques for surgical correction. METHODS: Between August 1984 and August 2010, 37 consecutive cases of CA underwent corrective surgery. There were no clinical findings of Down, asplenia-polysplenia or Ellis-van Creveld syndromes in all cases. Mitral valvuloplasty was performed in 34 cases, and tricuspid valvuloplasty in 20 cases...

BACKGROUND: Congenital heart defects (CHDs) are the most common type of congenital anomaly. The precise etiology is unknown and the development of successful primary prevention strategies is challenging. Folic acid may have a protective role; however published results have been inconsistent. This study examines the impact of mandatory folic acid fortification (FAF) on the prevalence of CHDs. METHODS: CHD cases were ascertained using the Alberta Congenital Anomalies Surveillance System, Pediatric Cardiology Clinics, Pathology, and hospital records...