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Related Experiment Videos

Complete atrioventricular septal defect.

B. Marais, N. N. Kalis, S. Shipton

    Cardiovascular Journal of South Africa : Official Journal for Southern Africa Cardiac Society [And] South African Society of Cardiac Practitioners
    |July 12, 2001
    PubMed
    Summary

    Complete atrioventricular septal defect (CAVSD) in infants, especially those with Down syndrome (DS), requires early screening and intervention. Optimal management involves timely surgical repair within six months to improve outcomes and reduce mortality.

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    An explanation of the unusual electrocardiographic findings in a patient with pulmonary agenesis and dextrocardia.

    Cardiovascular journal of South Africa : official journal for Southern Africa Cardiac Society [and] South African Society of Cardiac Practitionersยท2001
    See all related articles

    Area of Science:

    • Pediatric Cardiology
    • Congenital Heart Defects
    • Genetics

    Background:

    • Complete atrioventricular septal defect (CAVSD) is a severe congenital heart defect frequently associated with Down syndrome (DS).
    • Early surgical repair before six months is reported to significantly improve outcomes in affected infants.
    • Many reviews lack detailed selection criteria and exclude early mortality or inoperable cases.

    Purpose of the Study:

    • To describe the profile of children with CAVSD in a specific population.
    • To evaluate the effectiveness of screening procedures and treatment protocols.
    • To assess the relationship between treatment received and patient outcomes.

    Main Methods:

    • Retrospective descriptive study of 33 children with CAVSD (25 with DS, 8 without) over 15 years (1980-1994).

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  • Evaluation of diagnostic tools: auscultation, chest radiograph, ECG, and echocardiography.
  • Analysis of treatment modalities including medical management (digitalis, furosemide, ACE inhibitors) and surgical repair, considering Rastelli classification.

    • Main Results:

    • Basic diagnostic skills (auscultation, chest X-ray, ECG) showed 100% sensitivity for alerting to CAVSD.
    • Echocardiography was the most informative diagnostic tool.
    • Rastelli C classification indicated a poor prognosis; digitalis and furosemide remained primary medical treatments.
    • Despite earlier repairs in recent years, resource limitations meant most patients did not receive surgery; over 20% died before surgical consideration.

    Conclusions:

    • Thorough screening, particularly for infants with Down syndrome, is crucial for early detection and intervention.
    • Early referral for complete surgical repair within the first six months of life is essential for optimal management of CAVSD.
    • Local practice evaluation highlights the need for timely surgical access to improve survival rates.