Atrioventricular Canal and Dextrocardia: A Case Report with Implications for Community-Based Providers

2018 ◽  
Vol 37 (5) ◽  
pp. 281-291
Author(s):  
Lisa Clevenger ◽  
Amy Jnah ◽  
Desi Newberry ◽  
Andrew Heling
Keyword(s):  
Congenital Heart ◽  
Interprofessional Collaboration ◽  
Heart Defects ◽  
The United States ◽  
Atrioventricular Canal ◽  
Community Based ◽  
Physical Assessment ◽  
Diagnostic Strategies ◽  
Cardiac Assessment ◽  
Life Threatening

Congenital heart defects (CHD) are the most common congenital malformation reported in the literature, with a global incidence of eight per every 1,000 live births. In the United States approximately 40,000 infants are born each year with a CHD. Of the infants diagnosed with a CHD, one in every four heart defects are life threatening in origin. Early identification and treatment of congenital heart lesions, beginning with a comprehensive physical assessment after birth, are critical. For infants delivered at community-based hospitals, the importance of the physical assessment, timing of diagnostic strategies, anticipatory planning, and interprofessional collaboration among referring and accepting centers cannot be understated. This article presents a rare case of an infant with atrioventricular canal complicated by dextrocardia. Embryology, pathophysiology, epidemiology, symptomology, cardiac assessment, diagnostics, treatment, and nursing strategies for facilitating transfer of care from community-based hospitals to tertiary medical centers are discussed.

Cardiac Assessment in the Neonatal Population

2010 ◽  
Vol 29 (3) ◽  
pp. 135-142 ◽  
Author(s):  
Mary Clay Federspiel
Keyword(s):  
Blood Pressure ◽  
Congenital Heart ◽  
Heart Defects ◽  
Rapid Identification ◽  
Cardiac Defects ◽  
Physical Assessment ◽  
Cardiac Assessment ◽  
Positive Treatment ◽  
Life Threatening ◽  
Neonatal Population

Congenital heart defects are not uncommon among neonatal patients. Although most are benign, the prompt identification of a life-threatening anomaly is essential for rapid intervention and a positive treatment outcome. Cardiac defects may be identified in the newborn nursery with thorough and systematic physical assessment, including inspection, palpation, auscultation, and measurement of blood pressure and oxygen saturations. The ability of the nurse to identify irregular findings during physical assessment aids rapid identification and treatment.

scholarly journals Discontinuity of Cardiac Follow‐Up in Young People With Congenital Heart Disease Transitioning to Adulthood: A Systematic Review and Meta‐Analysis

2021 ◽  
Author(s):  
Philip Moons ◽  
Sandra Skogby ◽  
Ewa‐Lena Bratt ◽  
Liesl Zühlke ◽  
Ariane Marelli ◽  
...  
Keyword(s):  
United States ◽  
Systematic Review ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Defects ◽  
Meta Analysis ◽  
Transition Programs ◽  
The United States

Background The majority of people born with congenital heart disease require lifelong cardiac follow‐up. However, discontinuity of care is a recognized problem and appears to increase around the transition to adulthood. We performed a systematic review and meta‐analysis to estimate the proportion of adolescents and emerging adults with congenital heart disease discontinuing cardiac follow‐up. In pooled data, we investigated regional differences, disparities by disease complexity, and the impact of transition programs on the discontinuity of care. Methods and Results Searches were performed in PubMed, Embase, Cinahl, and Web of Science. We identified 17 studies, which enrolled 6847 patients. A random effects meta‐analysis of single proportions was performed according to the DerSimonian‐Laird method. Moderator effects were computed to explore sources for heterogeneity. Discontinuity proportions ranged from 3.6% to 62.7%, with a pooled estimated proportion of 26.1% (95% CI, 19.2%–34.6%). A trend toward more discontinuity was observed in simple heart defects (33.7%; 95% CI, 15.6%–58.3%), compared with moderate (25.7%; 95% CI, 15.2%–40.1%) or complex congenital heart disease (22.3%; 95% CI, 16.5%–29.4%) ( P =0.2372). Studies from the United States (34.0%; 95% CI, 24.3%–45.4%), Canada (25.7%; 95% CI, 17.0%–36.7%), and Europe (6.5%; 95% CI, 5.3%–7.9%) differed significantly ( P =0.0004). Transition programs were shown to have the potential to reduce discontinuity of care (12.7%; 95% CI, 2.8%–42.3%) compared with usual care (36.2%; 95% CI, 22.8%–52.2%) ( P =0.1119). Conclusions This meta‐analysis showed that there is a high proportion of discontinuity of care in young people with congenital heart disease. The highest discontinuity proportions were observed in studies from the United States and in patients with simple heart defects. It is suggested that transition programs have a protective effect. Registration URL: www.crd.york.ac.uk/prospero . Unique identifier: CRD42020182413.

Cardiovascular Disorders

2019 ◽  
pp. 309-326
Author(s):  
Lyla E. Hampton ◽  
Abigail C. Demianczyk ◽  
Casey Hoffman
Keyword(s):  
Quality Of Life ◽  
Birth Defects ◽  
Neurodevelopmental Disorders ◽  
Congenital Heart ◽  
Heart Defects ◽  
School Setting ◽  
The United States ◽  
Intervention Strategies ◽  
School Based

Congenital heart disease (CHD) is one of the most common birth defects in the United States, affecting approximately 1% of births per year, with most children surviving into adulthood. Despite improved survival, individuals with CHD remain at high risk for neurological, cognitive, and psychosocial challenges that affect quality of life across the lifespan and have specific implications for working with children with CHD in the school setting. This chapter describes several common complications of CHD, as well as acquired heart defects, that have implications for how children with the conditions present in the school setting. The chapter also provides information about common neurodevelopmental disorders associated with these medical conditions and the effects of the conditions on psychological adjustment and quality of life in children. Finally, the chapter concludes with a review of intervention strategies that school-based professionals may adopt when working with children with heart conditions.

scholarly journals Characteristics of Adults With Congenital Heart Defects in the United States

2020 ◽  
Vol 76 (2) ◽  
pp. 175-182 ◽  
Author(s):  
Michelle Gurvitz ◽  
Julie E. Dunn ◽  
Ami Bhatt ◽  
Wendy M. Book ◽  
Jill Glidewell ◽  
...  
Keyword(s):  
United States ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
The United States

scholarly journals Palivizumab for prophylaxis of respiratory syncytial virus infections in preterm neonates

2017 ◽  
Vol 229 (05) ◽  
pp. 259-260
Author(s):  
Ludwig Gortner
Keyword(s):  
Respiratory Syncytial Virus ◽  
Congenital Heart Defects ◽  
Congenital Anomalies ◽  
Congenital Heart ◽  
Heart Defects ◽  
The United States ◽  
Preterm Neonates ◽  
Virus Infections ◽  
Number Of Children ◽  
Syncytial Virus

Recent data indicate that the number of children cared for respiratory syncytial virus (RSV) infections in hospitals is in the range of 50,000 to 100,000 per year in the United States 3. Major risk factors include apart from prematurity specifically below 29 weeks, congenital heart defects, Down syndrome and further congenital anomalies. The American Academy of Pediatrics recommended in 2014 a restriction of Palivizumab prophylaxis to preterm infants of <29 weeks of gestation and those with a high-risk course postnatally and higher gestational ages apart from specific indications resulting from congenital anomalies including congenital heart defects 1.

scholarly journals Semaphorin3f as an intrinsic regulator of chamber-specific heart development

2021 ◽  
Author(s):  
Rami Halabi ◽  
Paula B. Cechmanek ◽  
Carrie L. Hehr ◽  
Sarah McFarlane
Keyword(s):  
Congenital Heart Defects ◽  
Heart Development ◽  
Congenital Heart ◽  
Molecular Mechanisms ◽  
Heart Defects ◽  
Border Region ◽  
Specific Gene ◽  
Atrioventricular Canal ◽  
Term Survival ◽  
Ventricular Cardiomyocytes

During development a pool of precursors form a heart with atrial and ventricular chambers that exhibit distinct transcriptional and electrophysiological properties. Normal development of these chambers is essential for full term survival of the fetus, and deviations result in congenital heart defects. The large number of genes that may cause congenital heart defects when mutated, and the genetic variability and penetrance of the ensuing phenotypes, reveals a need to understand the molecular mechanisms that allow for the formation of chamber-specific cardiomyocyte differentiation. We find that in the developing zebrafish heart, mRNA for a secreted Semaphorin (Sema), Sema3fb, is expressed by all cardiomyocytes, whereas mRNA for its receptor Plexina3 (Plxna3) is expressed by ventricular cardiomyocytes. In sema3fb CRISPR zebrafish mutants, ventricular chamber development is impaired; the ventricles of mutants are smaller in size than their wild type siblings, apparently because of differences in cell size and not cell numbers, with ventricular cardiomyocytes failing to undergo normal developmental hypertrophy. Analysis of chamber differentiation indicates defects in chamber specific gene expression at the border between the ventricular and atrial chambers, with spillage of ventricular chamber genes into the atrium, and vice versa, and a failure to restrict bmp4a mRNA to the atrioventricular canal. The disrupted atrioventricular border region in mutants is accompanied by hypoplastic heart chambers and impaired cardiac function. These data suggest a model whereby cardiomyocytes secrete a Sema cue that, through spatially restricted expression of the receptor, signals in a ventricular chamber-specific manner to establish a distinct border between atrial and ventricular chambers that is important for functional development of the heart.

scholarly journals THE CLINICAL FEATURES OF ATRIOVENTRICULAR CANAL DEFECT

2017 ◽  
Vol 2 ◽  
pp. 14-21
Author(s):  
Andriana Malska
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Features ◽  
Clinical Manifestation ◽  
Congenital Heart ◽  
Viral Infections ◽  
Heart Defects ◽  
Mitral Valve Insufficiency ◽  
Atrioventricular Canal ◽  
Atrioventricular Canal Defect ◽  
Sternal Border

Atrioventricular canal defect (AVCD) is a congenital heart defect, which occurs in 2.9 % of all congenital heart defects (CHD) and is characterized by a wide variety of anatomical forms and often don’t have clear cardiac manifestation. Untreated AVCD may lead to the development of pulmonary hypertension. Aim. To determine clinical features of AVCD in children, considering variable anatomical forms of the pathology and its association with genetic pathology. Materials and methods. Patients history and outpatient statistic records of children with AVCD, who were admitted to Lviv Regional Children’s Hospital from September 1999 till January 2016 have been analyzed (n=84). The aspects of clinical manifestation of AVCD without associated pathology have been identified (n=48). Clinical manifestation of complete (n=36) and incomplete (n=12) AVCD and clinical manifestation with and without Down syndrome have been discussed. Children with AVCD were divided into two groups: A – children with complete (n=36) and B – with incomplete (n=12) form of AVCD. Group A was divided into A1 – with trisomy 21 (n=14), A2 – without genetic pathology (n=22). Results. In group А2 – 36,36±10,26 % and in group В – 50±14,4 % children were asymptomatic. Dyspnea, increased sweating during feeds, growth retardation and frequent respiratory viral infections during early childhood period were leading symptoms. Most frequent auscultation findings were accent of II heart sound over the pulmonary artery and 2-3/6 systolic murmur over left sternal border. According to echocardiographic examination mitral valve insufficiency was predominantly of mild grade, tricuspid insufficiency and pulmonary hypertension was diagnosed in group А2 with the frequency of 9,09±6,13 %). Conclusions: The absence of clinical features in group A2 and B 36,36±10,26 and 50,00±14,40 respectively, saturation levels 92,36±0,49 % in patients without genetic pathology and 95,25±0,40 % with incomplete AVCD provide a need to adopt protocol of children examination with saturation level under 95 % and compulsory echocardiographic diagnosis within the first month of life

Maternal age and prevalence of isolated congenital heart defects in an urban area of the United States

2011 ◽  
Vol 155 (9) ◽  
pp. 2137-2145 ◽  
Author(s):  
Assia Miller ◽  
Tiffany Riehle-Colarusso ◽  
Csaba Siffel ◽  
Jaime L. Frías ◽  
Adolfo Correa
Keyword(s):  
United States ◽  
Urban Area ◽  
Congenital Heart Defects ◽  
Maternal Age ◽  
Congenital Heart ◽  
Heart Defects ◽  
The United States
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