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3 results

  • Chapter 4 - Transitional Atrioventricular Septal Defect

  • from Section 1 - Left-to-Right Shunts
  • Edited by Laura K. Berenstain, James P. Spaeth
  • Book:
    Congenital Cardiac Anesthesia
    Published online:
    09 September 2021
    Print publication:
    16 September 2021, pp 20-24

  • Summary

    Atrioventricular septal defects are common congenital heart defects affecting the interatrial and interventricular septa as well as the atrioventricular valves. Atrioventricular septal defects occur due to the failure of endocardial cushions to properly develop and migrate to septate the heart during embryonic development; therefore they are also referred to as endocardial cushion defects. There are three common types or descriptions for atrioventricular septal defects: partial, transitional, and complete. An ostium primum type atrial septal defect exists in all types of atrioventricular septal defects. Partial and transitional atrioventricular septal defects have similar physiologic implications and are often diagnosed later in life as left-to-right shunting is not as significant; patients with complete atrioventricular septal defects have more significant left-to-right shunting with more severe pulmonary overcirculation, usually necessitating earlier intervention. This chapter discusses the pertinent findings and perioperative management of a patient with an unrepaired transitional atrioventricular septal defect.

  • Atrioventricular septal defects among infants in Europe: a population-based study of prevalence, associated anomalies, and survival

  • Nikolas Christensen, Helle Andersen, Ester Garne, Diana Wellesley, Marie-Claude Addor, Martin Haeusler, Babak Khoshnood, Carmel Mullaney, Judith Rankin, David Tucker
  • Journal:
    Cardiology in the Young / Volume 23 / Issue 4 / August 2013
    Published online by Cambridge University Press:
    27 November 2012, pp. 560-567
  • Objective

    To describe the epidemiology of chromosomal and non-chromosomal cases of atrioventricular septal defects in Europe.

    Methods

    Data were obtained from EUROCAT, a European network of population-based registries collecting data on congenital anomalies. Data from 13 registries for the period 2000–2008 were included.

    Results

    There was a total of 993 cases of atrioventricular septal defects, with a total prevalence of 5.3 per 10,000 births (95% confidence interval 4.1 to 6.5). Of the total cases, 250 were isolated cardiac lesions, 583 were chromosomal cases, 79 had multiple anomalies, 58 had heterotaxia sequence, and 23 had a monogenic syndrome. The total prevalence of chromosomal cases was 3.1 per 10,000 (95% confidence interval 1.9 to 4.3), with a large variation between registers. Of the 993 cases, 639 cases were live births, 45 were stillbirths, and 309 were terminations of pregnancy owing to foetal anomaly. Among the groups, additional associated cardiac anomalies were most frequent in heterotaxia cases (38%) and least frequent in chromosomal cases (8%). Coarctation of the aorta was the most common associated cardiac defect. The 1-week survival rate for live births was 94%.

    Conclusion

    Of all cases, three-quarters were associated with other anomalies, both chromosomal and non-chromosomal. For infants with atrioventricular septal defects and no chromosomal anomalies, cardiac defects were often more complex compared with infants with atrioventricular septal defects and a chromosomal anomaly. Clinical outcomes for atrioventricular septal defects varied between regions. The proportion of termination of pregnancy for foetal anomaly was higher for cases with multiple anomalies, chromosomal anomalies, and heterotaxia sequence.