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ECHO in Context
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 LEARN THE BASICS: Echocardiography | Doppler


The Changing Left Ventricle

Aortic Valve Disease: New Dimensions in Evaluation and Management

Heart Failure: Echo's Role in and Emerging Health Crisis

Chest Pain in Children & Adults: The Role of Echo

Mitral Regurgitation: New Concept

The Falling Left Ventricle: Diastolic & Systolic Function

Changing the Outcome of Coronary Artery Disease
Digital Integration
Doppler Echo

Chest Pain in Children and Adults

Mitral Regurgitation: New Concepts

Diastolic and Systolic Function

Changing the Outcome of CAD

2000 MV
2001 Chest Pain
2002 Heart Failure

Classification of Congenital Heart Disease

Many physicians dealing with adults find congenital heart disease extremely complex. The terms and classifications used over the years by pediatric cardiologists may be largely to blame. Most previous nomenclature systems are largely based upon embryology. Thus, such terms as L-loop and D-loop were common and generally confused most individuals. In this unit, a simple descriptive nomenclature, known as the sequential segmental approach, introduced during the 1980's is employed. It avoids terms derived from embryology and, where possible, uses simple and uncomplicated descriptions. Its major goal is to convey information simply and accurately without regard to how the lesions came to be.

The Sequential Segmental Approach

This newer nomenclature approach has remarkably simplified the classification of congenital heart disease. It is based on following the blood flow into the heart (systemic venous and pulmonary venous), through the heart (the atrioventricular valves and ventricles) and then out the great vessels (semilunar valves and great vessels). This nomenclature system is extraordinarily helpful to those conducting echocardiographic examinations as it forms a systematic guide for verification that all the pertinent chambers and valves and their relationships have been documented. The system is dependent on a few words that are very important in describing the various lesions:

Connection refers to the sequence of anatomic structures. Normally, the right atrium is connected to the right ventricle by means of the tricuspid valve. The right ventricle is then connected to the pulmonary artery by means of the pulmonic valve. Therefore, there are atrioventricular connections and ventriculo-great arterial connections.

Concordance describes the relationship between the various chambers, valves, and great vessels. In the normal heart all the connections and relationships in the anatomic sequence are concordant.

Fig. 2

Discordance describes abnormal relationships between the various chambers and great vessels. For example, when the right atrium leads into the morphologic left ventricle and the left atrium into the morphologic right ventricle, the atrioventricular relationships are discordant, as seen in Fig. 2. Likewise, the atrioventricular relationships may be concordant (normal) but the ventriculo-great arterial relationships may be discordant where the aorta rises from the right ventricle and the pulmonary artery from the left ventricle. Known formerly as transposition of the great vessels, these abnormal relationships would now be termed ventriculo-great arterial discordance as seen in the right panel of Fig. 2.

Fig. 3

Absent or imperforate connections - Valves normally form the connections between chambers. There are atrioventricular connections that lead from the right atrium to right ventricle or left atrium to vessels. When connections are not present, the term absent connection is used. Thus, when the tricuspid valve is absent, an absent right atrioventricular connection may not be totally absent, only severely malformed and does not allow blood to pass antegradely. In this setting the term imperforate connection may be used, also seen in Fig. 3.

Commitment further describes possible abnormalities of flow through valves into ventricles and great vessels. For example, in a patient with tetralogy of Fallot, the atria, atrioventricular valves, and ventricles are positioned normally, and concordant. Since the aorta overrides a ventricular septal defect the aorta is doubly committed to both ventricles. Likewise, in cases where there is only one ventricle (univentricular heart), both atrioventricular valves are usually doubly committed to the single ventricle.

Ambiguous is used when precise identification of a ventricle or other structure cannot be made. For example, in a univentricular heart with a doubly committed atrioventricular connection it may not be possible to always identify clearly whether it is the right or left ventricle. Thus, the single ventricle would be ambiguous.

Inlet refers to anomalies of the structures and flow into the ventricle.

Outlet refers to anomalies of the structures and flow out of the ventricles into the great vessels.

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