11 Right Heart Anomalies
Basic Principles
Right Ventricle

Figure 11-1 Schematic of a tripartite right ventricle (RV): inlet (1), body or trabecular portion (2), and outlet (3). Oriented toward the right ventricular aspect of the interventricular septum with the free wall of the RV removed. The inlet portion borders the tricuspid valve (TV), whereas the outlet portion borders the pulmonary valve (pulmV). The body contains the muscular or trabecular portion of the RV.
Ebstein’s Anomaly
Background

Figure 11-2 Schematic of Ebstein’s anomaly in an apical four-chamber (A4C) view. Note the apically displaced TV septal leaflet, enlargement of the right atrium (RA) with the atrialized portion of the RV (aRV). There is usually tricuspid regurgitation (TR) as well as a right-to-left shunt across a patent foramen ovale (PFO).
(From Otto CM. Textbook of Clinical Echocardiography, 4th ed. Philadelphia: Saunders/Elsevier, 2009.)
Overview of Echocardiographic Approach
Anatomic Imaging
Acquisition
Analysis

Figure 11-4 A4C view. The measurement of the TV apical displacement is shown from the mitral valve hinge point to the septal leaflet of the TV hinge point. Normal displacement is considered less than 0.8 cm/m2. Ebstein’s anomaly is defined by displacement greater than 0.8 cm/m2. This patient’s displacement of 0.65 cm is 3.6 cm/m2 and diagnostic for Ebstein’s anomaly.
TABLE 11-2 ANATOMIC CLASSIFICATION
Type A | Septal and posterior leaflet adherance without functional RV restriction of volume |
Type B | Atrialized RV with normal anterior leaflet hinge point |
Type C | Anterior leaflet stenosis |
Type D | RV entirely atrialized except for a small infundibulum |
Data from Carpentier A, Chauvaud S, Mace L, et al. A new reconstructed operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 2006;132:1285–1290.

Figure 11-5 Parasternal long axis (PLAX) view angled toward the TV. The large and redundant anterior TV leaflet is seen well in this view in two different patients with Ebstein’s anomaly. The arrows depict the anterior TV leaflet.

Figure 11-6 A4C view without and with the Great Ormond Street (GOS) ratio measurements. The ratio is the area of the RA + aRV divided by the area of the RV + LA + left ventricle. Severity is graded from 1 to 4 with 4 being the most severe. Grade 1 is a ratio less than 0.5; grade 4 is a ratio more than 1.5. In this patient, the GOS ratio is 0.32, which is a grade 1 on the severity scale. Clinically, this patient has been followed serially and has not required any interventions to date.
Pitfalls
Physiologic Data
Acquisition
Analysis

Figure 11-7 Subcostal (SC) view of an atrial septal defect (ASD) in a patient with severe Ebstein’s anomaly. A, Two-dimensional (2D) image demonstrating a dilated RA and a large atrial communication. B, Color flow Doppler demonstrating right-to-left flow across the interatrial septum (IAS).
Alternate Approaches
Key Points
Pulmonary Atresia with Intact Ventricular Septum
Background
Overview of Echocardiographic Approach
TTE plays an integral role in defining the anatomy and clinical severity of patients with pulmonary atresia with IVS (Table 11-3).
Anatomic Imaging
Acquisition
Analysis

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