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Congenital Defects Tutorial
Normal Cardiac Development Fetal Circulation Congenital Heart Defects Cardiac Transplantation

Introduction

Congenital Heart Defects (CHDs) are primarily structural alterations that arise due to errors in embryological development of the heart and great vessels. These resulting abnormalities range in severity from miniscule holes between chambers that may subsequently close to complex malformations that will require multiple surgical corrections to allow the affected patient to survive. Some defects are so severe that even today, despite major advances in treatment (e.g., fetal surgery), death occurs in utero or in early infancy.

CHD’s account for 28% of all major congenital anomalies and are estimated to occur in 8 of every 1,000 live births3. Of these, approximately 2 to 3 such patients will require expert cardiologic care2. Although CHD’s were previously the most common cause of death in infants associated with birth defects, breakthroughs in diagnostics and cardiothoracic surgery as well as an increased wealth of knowledge have has led to a continual decline in mortality rates. Currently, it is estimated that approximately 800,000 adults, or 1 in 150 people, in the United States are living with a corrected CHD1. Yet, these patients require the continued care of physicians and health care professionals: those that have an understanding of the natural progression of these defects and how to effectively care for them.

This CHD tutorial will cover the following sections in an effort to educate interested students, those in health professional programs, and/or patients.

  • Normal Cardiac Development: progression of events in utero resulting in a functional heart and corresponding vessels.
  • Fetal and Adult Circulatory Patterns: comparison of circulation during fetal and adult life.
  • Congenital Heart Defects: descriptions of the more common CHDs including relative anatomies, physiologies, clinical manifestations, and treatments.
  • Cardiac Transplantation: overview of transplantation as an ultimate procedure for the CHD patient with an otherwise untreatable or highly progressed disease state.
  • Prevalence: a relative comparison of the incidences of various CHD’s

References

  1. American Heart Association. (2013). Congenital cardiovascular defects [Statistical fact sheet]. Retrieved from: http://www.heart.org/idc/groups/heart-public/@wcm/@sop/@smd/documents/downloadable/ucm_319830.pdf
  2. Hoffman, J.I.E. & Kaplan, S. (2002). The incidence of congenital heart disease. Journal of the American College of Cardiology, 39, 1890-1900. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/12084585
  3. van der Linde, D., Konings, E.E.M, Slager, M.A., Witsenburg, M., Helbing, W.A., Takkenberg, J.J.M, & Roos-Hesselink, J.W. (2011). Birth prevalence of congenital heart disease worldwide. Journal of the American College of Cardiology, 58(21), 241-247. doi: 10.1016/j.jacc.2011.08.025