Research Article
Volume 7 Issue 6 - 2020
Complete Atrioventricular Septal Defect: Impact of Down Syndrome on Atrioventricular Valve Morphology and Haemodynamics and Clinical Outcomes
Meryem Haboub*, Abdenasser Drighil and Hanane Mechal
Cardiology Department, Hospital University Ibn Rochd, Casablanca, Morocco
*Corresponding Author: Meryem Haboub, Cardiology Department, Hospital University Ibn Rochd, Casablanca, Morocco.
Received: April 18, 2020; Published: May 14, 2020


Background: Other cardiac malformations might be associated with complete atrioventricular septal defect (CAVSD) such as abnormalities of the left atrioventricular valve. Down syndrome is known to affect the natural history of complete atrioventricular septal defect but associations between Down syndrome and outcomes remain to be established. The purpose of this study was to investigate the impact of Down syndrome on atrioventricular valve morphology and haemodynamics as well as on outcomes in children with CAVSD.

Methods: We performed a single-centre, retrospective and descriptive study with a prospective follow-up in the Congenital Heart Disease Unit of cardiology department over a 9-year period. We compared 2 groups of patients with CAVSD: Down syndrome patients (group 1) and non-Down syndrome patients (group 2). Demographic variables, echocardiographic features and outcomes were studied.

Results: 225 were diagnosed with CAVSD, 121 (54%) in group 1 and 104 (46%) in group 2. Atrioventricular valve annulus was hypoplastic in 6 patients (5.8%) of group 2 whereas all patients in group 1 had a normally developed atrioventricular valve annulus (p = 0.024). Papillary muscles were abnormal in 8 patients (6.6%) in group 1 versus 15 patients (14.4%) of group 2 (p = 0.048). Significant preoperative left atrioventricular valve regurgitation was lower in group 1 patients: 11 (9.1%) versus 23 (22.1%) (p = 0.02). Mortality rate was lower in group 1; 14 (11.5%) versus 23 (22.1%) (p = 0.03).

Conclusion. Down syndrome patients have a better atrioventricular valve anatomy, fewer preoperative left atrioventricular valve regurgitation and a lower long-term mortality rate after surgery.

Keywords: Complete Atrioventricular Septal Defect; Down Syndrome; Left Atrioventricular Valve Dysplasia; Left Atrioventricular Valve Regurgitation; Mortality


  1. Flyer DC. “Endocardial cushion defects”. In Nadas' Pediatric Cardiology Edited by: Fyler DC. Hanley and Belfus, Inc., Philadelphia (1992): 577-589.
  2. Ferencz C., et al. “Genetic and Environmental Risk Factors of Major Cardiovascular Malformations: The Baltimore-Washington Infant Study 1981-1989 Edited by: Anderson RH Armonk N”. Futura Publishing Co Inc (1997).
  3. Thankavel PP and Ramaciotti C. “Isolated mitral cleft in trisomy 21: an initially silent lesion”. Pediatric Cardiology2 (2006): 405-408.
  4. Munetaka M. “Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life?” European Journal of Cardio-Thoracic Surgery3 (2005): 405-409.
  5. Ajay R Desai. “Early Postoperative Outcomes Following Surgical Repair of Complete Atrioventricular Septal Defects: Is Down Syndrome a Risk Factor? (2013)”. Pediatric Critical Care and Medicine 1 (2014): 35-41.
  6. Formigari R., et al. “Better surgical prognosis for patients with complete atrioventricular septal defect and Down’s syndrome”. The Annals of Thoracic Surgery 2 (2004): 666-672.
  7. Andrew M Atz. “Surgical management of complete atrioventricular septal defect: Associations with surgical technique, age, and trisomy 21, 2011”. The Journal of Thoracic and Cardiovascular Surgery6 (2011): 1371-1379.
  8. Pierpont ME., et al. “Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital cardiac defects committee, council on cardiovascular disease in the young: endorsed by the American Academy of Pediatrics”. Circulation 115 (2007): 3015-3038.
  9. Shin M., et al. “Prevalence of Down syndrome among children and adolescents in 10 regions of the United States”. Pediatrics 6 (2009): 1565-1571.
  10. Margarita R Tumanyan. “Repair of Complete Atrioventricular Septal Defect in Infants with Down Syndrome: Outcomes and Long-Term Results”. Pediatric Cardiology 1 (2015): 71-75.
  11. Al-Hay AA., et al. “Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors”. The Annals of Thoracic Surgery 2 (2003): 412-421.
  12. Lange R., et al. “The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair”. The Journal of Thoracic and Cardiovascular Surgery 2 (2007): 304-310.
  13. Miller A., et al. “Long-term survival of infants with atrioventricular septal defects”. The Journal of Pediatrics 156 (2012): 994-1000.
  14. James D. “Contemporary outcomes of complete atrioventricular septal defect repair: Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database”. The Journal of Thoracic and Cardiovascular Surgery 6 (2014): 2526-2531.
  15. Meena Nathan. “Mid-term outcomes in unbalanced complete atrioventricular septal defect: role of biventricular conversion from single-ventricle palliation”. European Journal of Cardio-Thoracic Surgery (2017): 1-8.
  16. Naoki Wada. “Single ventricle repair in children with Down’s syndrome”. The General Thoracic and Cardiovascular Surgery 56 (2008): 104-108.
  17. Chi TPL and Krovetz LJ. “The pulmonary vascular bed in children with Down syndrome”. The Journal of Pediatrics 86 (1975): 533-538.
  18. Yamaki S., et al. “Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy”. The American Journal of Cardiology 51 (1983): 1503-1506.
  19. Fudge JC Jr., et al. “Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database”. Pediatrics 126 (2010): 315-322.
  20. Suzuki K Yamaki Sh MimoriSh., et al. “Pulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect”. The American Journal of Cardiology 86 (2000): 434-443.
  21. Margarita R Tumanyan. “Repair of Complete Atrioventricular Septal Defect in Infants with Down Syndrome: Outcomes and Long-Term Results”. Pediatric Cardiology 1 (2015): 71-75.
  22. Clapp SK., et al. “Surgical and medical results of complete atrioventricular canal: a ten year review”. The American Journal of Cardiology 59 (1987): 454-458.
Citation: Meryem Haboub., et al. “Complete Atrioventricular Septal Defect: Impact of Down Syndrome on Atrioventricular Valve Morphology and Haemodynamics and Clinical Outcomes”. EC Cardiology 7.6 (2020): 13-20.

PubMed Indexed Article

EC Pharmacology and Toxicology
LC-UV-MS and MS/MS Characterize Glutathione Reactivity with Different Isomers (2,2' and 2,4' vs. 4,4') of Methylene Diphenyl-Diisocyanate.

PMID: 31143884 [PubMed]

PMCID: PMC6536005

EC Pharmacology and Toxicology
Alzheimer's Pathogenesis, Metal-Mediated Redox Stress, and Potential Nanotheranostics.

PMID: 31565701 [PubMed]

PMCID: PMC6764777

EC Neurology
Differences in Rate of Cognitive Decline and Caregiver Burden between Alzheimer's Disease and Vascular Dementia: a Retrospective Study.

PMID: 27747317 [PubMed]

PMCID: PMC5065347

EC Pharmacology and Toxicology
Will Blockchain Technology Transform Healthcare and Biomedical Sciences?

PMID: 31460519 [PubMed]

PMCID: PMC6711478

EC Pharmacology and Toxicology
Is it a Prime Time for AI-powered Virtual Drug Screening?

PMID: 30215059 [PubMed]

PMCID: PMC6133253

EC Psychology and Psychiatry
Analysis of Evidence for the Combination of Pro-dopamine Regulator (KB220PAM) and Naltrexone to Prevent Opioid Use Disorder Relapse.

PMID: 30417173 [PubMed]

PMCID: PMC6226033

EC Anaesthesia
Arrest Under Anesthesia - What was the Culprit? A Case Report.

PMID: 30264037 [PubMed]

PMCID: PMC6155992

EC Orthopaedics
Distraction Implantation. A New Technique in Total Joint Arthroplasty and Direct Skeletal Attachment.

PMID: 30198026 [PubMed]

PMCID: PMC6124505

EC Pulmonology and Respiratory Medicine
Prevalence and factors associated with self-reported chronic obstructive pulmonary disease among adults aged 40-79: the National Health and Nutrition Examination Survey (NHANES) 2007-2012.

PMID: 30294723 [PubMed]

PMCID: PMC6169793

EC Dental Science
Important Dental Fiber-Reinforced Composite Molding Compound Breakthroughs

PMID: 29285526 [PubMed]

PMCID: PMC5743211

EC Microbiology
Prevalence of Intestinal Parasites Among HIV Infected and HIV Uninfected Patients Treated at the 1o De Maio Health Centre in Maputo, Mozambique

PMID: 29911204 [PubMed]

PMCID: PMC5999047

EC Microbiology
Macrophages and the Viral Dissemination Super Highway

PMID: 26949751 [PubMed]

PMCID: PMC4774560

EC Microbiology
The Microbiome, Antibiotics, and Health of the Pediatric Population.

PMID: 27390782 [PubMed]

PMCID: PMC4933318

EC Microbiology
Reactive Oxygen Species in HIV Infection

PMID: 28580453 [PubMed]

PMCID: PMC5450819

EC Microbiology
A Review of the CD4 T Cell Contribution to Lung Infection, Inflammation and Repair with a Focus on Wheeze and Asthma in the Pediatric Population

PMID: 26280024 [PubMed]

PMCID: PMC4533840

EC Neurology
Identifying Key Symptoms Differentiating Myalgic Encephalomyelitis and Chronic Fatigue Syndrome from Multiple Sclerosis

PMID: 28066845 [PubMed]

PMCID: PMC5214344

EC Pharmacology and Toxicology
Paradigm Shift is the Normal State of Pharmacology

PMID: 28936490 [PubMed]

PMCID: PMC5604476

EC Neurology
Examining those Meeting IOM Criteria Versus IOM Plus Fibromyalgia

PMID: 28713879 [PubMed]

PMCID: PMC5510658

EC Neurology
Unilateral Frontosphenoid Craniosynostosis: Case Report and a Review of the Literature

PMID: 28133641 [PubMed]

PMCID: PMC5267489

EC Ophthalmology
OCT-Angiography for Non-Invasive Monitoring of Neuronal and Vascular Structure in Mouse Retina: Implication for Characterization of Retinal Neurovascular Coupling

PMID: 29333536 [PubMed]

PMCID: PMC5766278

EC Neurology
Longer Duration of Downslope Treadmill Walking Induces Depression of H-Reflexes Measured during Standing and Walking.

PMID: 31032493 [PubMed]

PMCID: PMC6483108

EC Microbiology
Onchocerciasis in Mozambique: An Unknown Condition for Health Professionals.

PMID: 30957099 [PubMed]

PMCID: PMC6448571

EC Nutrition
Food Insecurity among Households with and without Podoconiosis in East and West Gojjam, Ethiopia.

PMID: 30101228 [PubMed]

PMCID: PMC6086333

EC Ophthalmology
REVIEW. +2 to +3 D. Reading Glasses to Prevent Myopia.

PMID: 31080964 [PubMed]

PMCID: PMC6508883

EC Gynaecology
Biomechanical Mapping of the Female Pelvic Floor: Uterine Prolapse Versus Normal Conditions.

PMID: 31093608 [PubMed]

PMCID: PMC6513001

EC Dental Science
Fiber-Reinforced Composites: A Breakthrough in Practical Clinical Applications with Advanced Wear Resistance for Dental Materials.

PMID: 31552397 [PubMed]

PMCID: PMC6758937

EC Microbiology
Neurocysticercosis in Child Bearing Women: An Overlooked Condition in Mozambique and a Potentially Missed Diagnosis in Women Presenting with Eclampsia.

PMID: 31681909 [PubMed]

PMCID: PMC6824723

EC Microbiology
Molecular Detection of Leptospira spp. in Rodents Trapped in the Mozambique Island City, Nampula Province, Mozambique.

PMID: 31681910 [PubMed]

PMCID: PMC6824726

EC Neurology
Endoplasmic Reticulum-Mitochondrial Cross-Talk in Neurodegenerative and Eye Diseases.

PMID: 31528859 [PubMed]

PMCID: PMC6746603

EC Psychology and Psychiatry
Can Chronic Consumption of Caffeine by Increasing D2/D3 Receptors Offer Benefit to Carriers of the DRD2 A1 Allele in Cocaine Abuse?

PMID: 31276119 [PubMed]

PMCID: PMC6604646

EC Anaesthesia
Real Time Locating Systems and sustainability of Perioperative Efficiency of Anesthesiologists.

PMID: 31406965 [PubMed]

PMCID: PMC6690616

EC Pharmacology and Toxicology
A Pilot STEM Curriculum Designed to Teach High School Students Concepts in Biochemical Engineering and Pharmacology.

PMID: 31517314 [PubMed]

PMCID: PMC6741290

EC Pharmacology and Toxicology
Toxic Mechanisms Underlying Motor Activity Changes Induced by a Mixture of Lead, Arsenic and Manganese.

PMID: 31633124 [PubMed]

PMCID: PMC6800226

EC Neurology
Research Volunteers' Attitudes Toward Chronic Fatigue Syndrome and Myalgic Encephalomyelitis.

PMID: 29662969 [PubMed]

PMCID: PMC5898812

EC Pharmacology and Toxicology
Hyperbaric Oxygen Therapy for Alzheimer's Disease.

PMID: 30215058 [PubMed]

PMCID: PMC6133268

News and Events

November Issue Release

We always feel pleasure to share our updates with you all. Here, notifying you that we have successfully released the November issue of respective journals and the latest articles can be viewed on the current issue pages.

Submission Deadline for Upcoming Issue

ECronicon delightfully welcomes all the authors around the globe for effective collaboration with an article submission for the upcoming issue of respective journals. Submissions are accepted on/before December 15, 2022.

Certificate of Publication

ECronicon honors with a "Publication Certificate" to the corresponding author by including the names of co-authors as a token of appreciation for publishing the work with our respective journals.

Best Article of the Issue

Editors of respective journals will always be very much interested in electing one Best Article after each issue release. The authors of the selected article will be honored with a "Best Article of the Issue" certificate.

Certifying for Review

ECronicon certifies the Editors for their first review done towards the assigned article of the respective journals.

Latest Articles

The latest articles will be updated immediately on the articles in press page of the respective journals.