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Congenital Defects Tutorial
Normal Cardiac Development Fetal Circulation Congenital Heart Defects Cardiac Transplantation
Primary Heart Tube Systemic and Pulmonary Circulation Formation of Atrioventricular Valves Atrial Chambers Ventricular Septation Partitioning of Outflow Tract Conduction System Development of Blood Vessels Normal Anatomy and Relationships at Birth

Normal Anatomy and their Relationships at Birth

  • The relative orientations of right and left ventricles are determined by cardiac looping. Morphologically, the left ventricular endocardium is smoother with finer trabeculations than the right ventricle endocardium which is highly trabeculated.
    • Normally, the morphologic right ventricle lies to the right and anterior relative to the morphological left ventricle, referred to as "D-loop."
    • Reversal in direction of looping results in the morphological right ventricle lying posterior and to the left of the morphological left ventricle, which is then referred to as "L-loop."
  • The relationship of great vessels and their semilunar valves to the ventricles: normally, the aorta and its aortic semilunar valve are connected to the morphologic left ventricle and found posterior and rightward while the pulmonary trunk and its pulmonic semilunar valve are committed to the morphologic right ventricle and found more anterior and leftward.
  • Definitions of cardiac malpositions and heterotaxy syndromes:
    • "Cardiac malposition" refers to an abnormal location of the heart within the chest or relative to the abdominal organs. Abnormal sidedness is highly associated with other complex cardiac anomalies.
    • In the period between 1988 and 2002 at Children’s Hospital Boston, 724 patients were diagnosed with cardiac malposition (1%) and had a mortality rate of 19% (ch.39, Keane)
    • Three terms are commonly used to describe the location of the heart within the chest:
      • The term "dextrocardia" indicates that the heart is located in the right side of the chest rather than left.
      • "Mesocardia" refers to displacement of the heart toward the right but not completely in the right chest.
      • "Levocardia" refers to a heart located wholly in the left side of the chest.
    • These positions can be further classified based on the visceroatrial sinus which refers to the relative position of the right and left atria to abdominal organs.
      • Normal connection of visceral venous vessels (superior and inferior vena cava) is referred to as "situs solitus", which positions the stomach and spleen toward the left and appendix toward the right. The right lobe of the liver is normally larger than left and lies on the right side.
      • "Situs inversus" refers to the situation occurring when the positions of abdominal organs are reversed. In this case, the stomach and spleen lie on the right, the right (dominant) lobe of the liver lies to the left, and the appendix and inferior vena cava to the left. The left (rather than right) lung is typically tri-lobed.
      • "Situs ambiguous" refers to a group of anomalies characterized by a lack of sidedness. The abdominal organs may be positioned to either right or left side with the liver typically lying in the midline. In addition, these patients are often found to be either polysplenic or asplenic; the former typically having left-side dominance of atrial and pulmonary structures and the later right-side dominance.
    • Importantly, the position of the heart in the chest and with relation to the abdominal organs does not indicate anything about the specific segments or connections between organs. As such, an understanding of the physiology and clinical manifestation these defects requires segmentation and analyses of the heart, as well as non-cardiac abnormalities.

References

  • Abdulla, R., Blew, G.A., & Holterman, M.J. (2004). Cardiovascular embryology. Pediatric Cardiology, 25, 191-200. doi: 10.1007/s00246-003-0585-1
  • Anderson, R.H., Brown, N.A., Mohun, T.J., & Moorman, A.F.M. (2013). Insights from cardiac development relevant to congenital defects and adult clinical anatomy. Journal of Cardiovascular Translational Research, 6, 107-117. doi: 10.1007/s12265-012-940-3
  • Ashworth, M.T. (2013). Embryology of the heart (4th ed.). In Suvarna, S. (Ed.), Cardiac Pathology (109-115). Springer London. doi: 10.1007/978/1-4471-2407-8_6.
  • Schoenwolf, G.C, Bleyl, S.B., Brauer, P.R., & Francis-West, P.H. (2009). Development of the heart (4th ed.). In Larsen’s Human Embryology (337-384). Churchill Livingstone, Elsevier Inc.
  • Schoenwolf, G.C, Bleyl, S.B., Brauer, P.R., & Francis-West, P.H. (2009). Development of the vasculature (4th ed.). In Larsen’s Human Embryology (385-433). Churchill Livingstone, Elsevier Inc.