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The incidence of congenital heart disease (CHD) is 0.5% to 1%.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 anatomical 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; detecting unanticipated findings; modifying surgical procedures; assessing the adequacy of the procedure; guiding revision; monitoring intracardiac air, ventricular volume, and myocardial performance; and formulating anesthetic and postoperative management. The primary objectives of TEE in patients with CHD are to define important anatomical 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.


Echocardiography of the congenital heart is 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 [A-V] and ventriculoarterial [V-A]) (Fig. 19-1).4

FIGURE 19–1.

Hearts are made up of three segments: atria, ventricles, and arterial trunks. These are connected via the atrioventricular valves and the ventriculoarterial (or conotruncal) junctions.

Transthoracic echocardiography allows for multiple planes of interrogation, so-called sweeps, of a congenital heart. Transesophageal echocardiography is more limited due to the confinement of the probe in the esophagus. Modified TEE views that allow for a “sweeping” approach to delineate congenital heart lesions have recently been described.5 A method of interrogating the congenital heart systematically with echocardiography is to follow the flow of blood into the heart from the systemic venous and pulmonary venous connections, through the heart (atria, atrioventricular valves, and ventricles), and then out of the heart through outflow tracts, semilunar valves, and great vessels. The midesophageal four-chamber (ME 4-CH) view forms the starting point and is the “go-to” view when “lost” during interrogation of the heart.

  • Step 1: Determine the cardiac position within the thorax by identifying the orientation of the base to apex in the ME 4-CH view. (Left - Levocardia, Right - Dextrocardia, Middle - Mesocardia.)

  • Step 2: Determine the atrial arrangement. Four different arrangements are possible (Fig. 19-2). Identify the atria by their atrial appendages and in case of the right atrium (RA) by the inferior vena cava (IVC) and coronary sinus (CS) draining into it. Useful views for this are the ME 4-CH, ...

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