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 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

Congenital Cardiovascular Defects in Infants With Down Syndrome

1989 ◽  
Vol 11 (4) ◽  
pp. 99-100
Author(s):  
Edward B. Clark
Keyword(s):  
Down Syndrome ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Atrioventricular Canal ◽  
Cardiac Evaluation ◽  
Cardiovascular Defects ◽  
Complete Atrioventricular ◽  
Patent Ductus

Editor's note: In response to a reader's question about cardiac evaluation of patients with Down syndrome, we asked Dr Clark for a review and recommendation for workup by the general pediatrician. RJH Infants with Down syndrome are at 50% risk for congenital cardiovascular malformations1 compared with a 0.3% (3 of 1000) risk for infants with normal chromosomes.2 These data confirm the original estimates of Rowe and Uchida3; other estimates have ranged from 20% to 70%. The types of heart defects found in persons with Down syndrome are severe: complete atrioventricular canal, ventricular septal defect, partial atrioventricular canal, atrial septal defect, tetralogy of Fallot, and patent ductus arteriosus.4

scholarly journals A case of a complete atrioventricular canal defect in a ferret

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Carlos F Agudelo ◽  
Vladimír Jekl ◽  
Karel Hauptman ◽  
Michal Crha ◽  
Meric Kocaturk
Keyword(s):  
Ventricular Septal Defect ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Lung Edema ◽  
Human Beings ◽  
Intact Male ◽  
Atrioventricular Canal ◽  
P Waves ◽  
Atrioventricular Canal Defect ◽  
Complete Atrioventricular

Abstract Background Atrioventricular canal defect is a rare congenital disorder of the heart and describes the presence of an atrial septal defect, a variable presentation of ventricular septal alterations including ventricular septal defect malformations in the mitral and tricuspid valves. The defect has been described in human beings, dogs, cats, pigs, and horses. Case presentation This paper describes the case of a complete atrioventricular canal defect in a four-year-old intact male pet ferret (Mustela putorius furo), which was presented due to posterior weakness, ataxia, and decreased appetite. A loud systolic murmur, dyspnea, and hind limb paraparesis were detected during the clinical examination. Thoracic radiographs showed generalized cardiomegaly and lung edema. ECG showed sinus rhythm with prolonged P waves and QRS complexes. Echocardiography showed a large atrial septal defect, atrioventricular dysplasia, and a ventricular septal defect. Palliative treatment with oxygen, furosemide, spironolactone, enalapril, diltiazem, and supportive care was chosen as the therapy of choice. The ferret recovered gradually during hospitalization. A follow-up examination at three and six months showed stabilization of cardiac function. Conclusions To the authors knowledge, this is the first time an atrioventricular canal defect has been described in a pet ferret.

scholarly journals The transcription factor Sox7 modulates endocardiac cushion formation contributed to atrioventricular septal defect through Wnt4/Bmp2 signaling

2021 ◽  
Vol 12 (4) ◽  
Author(s):  
Nanchao Hong ◽  
Erge Zhang ◽  
Huilin Xie ◽  
Lihui Jin ◽  
Qi Zhang ◽  
...  
Keyword(s):  
Transcription Factor ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Septal Defect ◽  
De Novo ◽  
Heart Defects ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Canal ◽  
Mesenchymal Transition ◽  
Atrioventricular Cushions

AbstractCardiac septum malformations account for the largest proportion in congenital heart defects. The transcription factor Sox7 has critical functions in the vascular development and angiogenesis. It is unclear whether Sox7 also contributes to cardiac septation development. We identified a de novo 8p23.1 deletion with Sox7 haploinsufficiency in an atrioventricular septal defect (AVSD) patient using whole exome sequencing in 100 AVSD patients. Then, multiple Sox7 conditional loss-of-function mice models were generated to explore the role of Sox7 in atrioventricular cushion development. Sox7 deficiency mice embryos exhibited partial AVSD and impaired endothelial to mesenchymal transition (EndMT). Transcriptome analysis revealed BMP signaling pathway was significantly downregulated in Sox7 deficiency atrioventricular cushions. Mechanistically, Sox7 deficiency reduced the expressions of Bmp2 in atrioventricular canal myocardium and Wnt4 in endocardium, and Sox7 binds to Wnt4 and Bmp2 directly. Furthermore, WNT4 or BMP2 protein could partially rescue the impaired EndMT process caused by Sox7 deficiency, and inhibition of BMP2 by Noggin could attenuate the effect of WNT4 protein. In summary, our findings identify Sox7 as a novel AVSD pathogenic candidate gene, and it can regulate the EndMT involved in atrioventricular cushion morphogenesis through Wnt4–Bmp2 signaling. This study contributes new strategies to the diagnosis and treatment of congenital 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.

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