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The incidence of congenital heart disease (CHD) is 0.5% to 1%, and common malformations are less frequent (0.15%).1 An increasing percentage of these infants survive to adulthood largely due to advances in cardiology, cardiac surgery, and perioperative anesthetic and critical care management.2 At present, adults with congenital heart disease constitute a significant and growing cardiac population of 5%.

In patients with CHD, transesophageal echocardiography (TEE) allows for the real-time acquisition of both anatomic and hemodynamic information, thereby helping in clinical decision making. During interventional cardiac catheterization procedures, TEE is instrumental in the monitoring and guidance of valvuloplasties, angioplasties, closure of intracardiac shunts, trans-septal atrial puncture, and electrophysiological ablation. During palliative and corrective surgical procedures, TEE is fundamental in confirming diagnosis; detection of unanticipated findings; modification of surgical procedures; assessment of the adequacy of the procedure; guidance of revision; monitoring of intracardiac air, ventricular volume, and myocardial performance; and formulation of anesthetic and postoperative management. The primary objectives of TEE in patients with CHD are to define important anatomic and hemodynamic information when data provided by other modalities are inadequate, establish a complete evaluation of complex congenital heart disease, and confirm or exclude a diagnosis of clinical relevance.

Congenital heart disease has been classified based on the level of complexity, presence or absence of cyanosis, and primary physiologic alterations. TEE image interpretation is therefore best performed using a segmental approach,3 where the heart is considered in terms of three segments (atria, ventricles, and arterial trunks), and these are connected via two junctions (atrioventricular and ventriculoarterial).4 The use of a segmental approach provides a systematic guide for verification that all significant chambers and valves and their relationships have been recorded. Important determinants in this segmental analysis include:

  • Visceral situs
  • Venoatrial connections (systemic and pulmonary veins)
  • Atrial situs (normal, inversus, right isomerism, or left isomerism)
  • Ventricular morphology (right versus left)
  • Atrioventricular septae
  • Atrioventricular valves
  • Semilunar valves
  • Ventricular outflows
  • Great arteries
  • Atrioventricular connections (concordant, discordant, double inlet, straddling, absent)
  • Ventriculoarterial connections (concordant, discordant, double outlet, single outlet)

The usual orientation of the organs with the liver on the right and the spleen on the left is referred to as situs solitus. Situs inversus refers to a situation where given organs, or even all bodily organs, are reversed (eg, liver on the left and spleen on the right). Asplenia is associated with bilateral right-sidedness (right isomerism—liver on both sides), while polysplenia is associated with bilateral left-sidedness (left isomerism—spleen on both sides).

Persistent Left Superior Vena Cava

A persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly and occurs in 0.4% of the general population and in 4% to 11% of patients with congenital heart disease.5 The etiology of this defect is thought to be the failure of regression of left anterior and common cardinal veins and left sinus horn. In 90% of cases, the PLSVC ...

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