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

scholarly journals A surgical approach of an unusual variant of complete atrioventricular defect; A case report

2021 ◽  
Vol 29 (1) ◽  
Author(s):  
Hamad Qabha ◽  
Tariq Alanazi ◽  
Mohamad Khouqeer ◽  
Mohannad Dawary ◽  
Fareed Khouqeer
Keyword(s):  
Congenital Heart ◽  
Septal Defect ◽  
Heart Defects ◽  
Surgical Techniques ◽  
Long Term Results ◽  
Surgical Approaches ◽  
Atrioventricular Canal ◽  
Anatomical Variant ◽  
Atrioventricular Canal Defect ◽  
Complete Atrioventricular

Abstract Background Complete atrioventricular canal is a congenital heart defect that is characterized by an atrial septal defect, ventricular septal defect, and a common atrioventricular valve. Standard surgical techniques for repairing complete atrioventricular canal defect mainly includes repairing the defect with a single patch, a double patch, or with the modified single patch technique. Case presentation This paper presents a novel surgical repair technique of an unusual anatomical presentation for a complete atrioventricular canal defect in a patient with Down syndrome. Conclusions Unusual anatomical variant for congenital heart defects occurs frequently, which gives surgeons real opportunities to innovate surgical approaches. This patient was an example of an unusual anatomical presentation for complete atrioventricular canal, and the surgical technique used for this patient was novel. Follow up for these patients is mandatory for long term results.

scholarly journals Use of ATP-MgCl2 in the evaluation and treatment of children with pulmonary hypertension secondary to congenital heart defects.

Circulation ◽  
1994 ◽  
Vol 90 (3) ◽  
pp. 1287-1293 ◽  
Author(s):  
M M Brook ◽  
J R Fineman ◽  
A M Bolinger ◽  
A F Wong ◽  
M A Heymann ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

scholarly journals Case Report: A Rare Case of Right-Sided Papillary Fibroelastoma in a 1-Year-Old With Congenital Heart Disease

2021 ◽  
Vol 7 ◽  
Author(s):  
Ali Ahmad ◽  
Edward A. El-Am ◽  
Reto D. Kurmann ◽  
Donald J. Hagler ◽  
Melanie C. Bois ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Case Report ◽  
Mitral Regurgitation ◽  
Rare Case ◽  
Congenital Heart ◽  
Cardiac Tumors ◽  
Atrioventricular Canal ◽  
Case Summary ◽  
Atrioventricular Canal Defect

Introduction: Cardiac papillary fibroelastomas (PFEs) are the most common primary benign cardiac tumors, although they are somewhat unusual in children and typically seen on the left-sided cardiac valves.Case summary: A 10-week-old patient was found to have a partial atrioventricular canal defect, with associated tricuspid and mitral regurgitation. He was medically managed until 1 year of age, when surgical correction was done. During the procedure, a PFE was found incidentally on the TV.Conclusion: This is one of the youngest patients to be reported with PFE, thus adding to the literature of these unusual cases in children.

Ultrasound examination of pregnant women in diagnosing fetal cardiac pathology

2020 ◽  
Vol 69 (2) ◽  
pp. 43-50
Author(s):  
Viktoria A. Lim
Keyword(s):  
Pregnant Women ◽  
Fetal Heart ◽  
Fetal Echocardiography ◽  
Heart Defects ◽  
Care Service ◽  
Ultrasound Screening ◽  
Atrioventricular Canal ◽  
Cardiac Pathology ◽  
Organ Systems ◽  
Atrioventricular Canal Defect

Hypothesis/aims of study. Fetal heart defects are the most common malformations causing infant mortality. The task of the obstetric care service is to make a timely diagnosis, which includes high-quality ultrasound screening and, if necessary, fetal echocardiography. This study aimed to compare fetal echocardiography with postpartum echocardiography. Study design, materials and methods. 101 pregnant women with both isolated fetal heart defects and combined pathology were examined for the period 20172019. Results. The greatest number of heart defects was detected at 2331 weeks of gestation. The structure of the malformations is diverse, the most common one being a complete form of the atrioventricular canal defect. In multiple pregnancies, complex heart defects were often combined with abnormalities in other organ systems. Conclusion. It is recommended to describe the heart structure in detail from 2122 weeks of pregnancy. If cardiac pathology is detected in utero, it is mandatory to conduct an examination of other fetal organs.

Cardiac involvement in genetic disease

2010 ◽  
pp. 2834-2841
Author(s):  
Thomas A. Traill
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Family History ◽  
Genetic Disease ◽  
Congenital Heart ◽  
Cardiac Involvement ◽  
Atrioventricular Canal ◽  
Marfan's Syndrome ◽  
Atrioventricular Canal Defect ◽  
History Of

Many clinicians find themselves faced, from time to time, with a patient who has a family history of a known disorder, such as Marfan’s syndrome, or who has noncardiac features that suggest a syndrome. Down’s syndrome—25 to 50% have congenital heart disease, most characteristically atrioventricular canal defect....

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.

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 Challenges in the Patient With Pulmonary Hypertension and Atrial Septal Defect: Understanding When and How to Close the Defect

2019 ◽  
Vol 18 (1) ◽  
pp. 10-13 ◽  
Author(s):  
Mary P. Mullen
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Defects ◽  
Defect Closure ◽  
Long Term Outcomes ◽  
Septal Defects ◽  
Pulmonary Arterial

Atrial septal defects (ASDs) are common congenital heart defects in children and adults. Pulmonary arterial hypertension (PAH) is found in subsets of both pediatric and adult patients with atrial defects under varied clinical contexts. The pulmonary hypertension specialist is often faced with questions surrounding timing and method of defect closure, which may have significant impact on procedural and long-term morbidity and survival. This review highlights important differences in management between children and adults with ASDs associated with PAH, highlighting indications for closure, operability, types of closure, and long-term outcomes.

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