Chapter 17 Congenital Heart Disease Liliana Cohen and Daniel M. Shindler Where one congenital malformation can occur, others can occur. So do a complete stepwise exam on everyone!!! One congenital abnormality does not preclude another. As a matter of fact, there are associations: Atrial septal defect—pulmonic stenosis Bicuspid aortic valve—coarctation of the aorta Ebstein’s anomaly of the tricuspid valve—atrial septal defect. You say pairs of associations are boring? 1. Pulmonic stenosis—use transgastric and high esophageal views to image the pulmonic valve, determine thickness and look for doming (indicative of fused commissures). 2. Right ventricular outflow obstruction—mid esophagus 90 degree views and transgastric views (when you get good). 3. Ventricular septal defect—120 degree mid esophagus, rotate your wrist until you find it. You should know the difference between overriding and straddling: On the echo of tetralogy: the aorta “hovers over” the ventricular septum. That is called overriding. In atrio-ventricular canal defects an abnormal tricuspid valve “floats” over the ventricular septum AND may have chordal attachments to BOTH sides of the ventricular septum (like the legs of the rider on a horse – it straddles the septum). The presence of straddling needs to be sought during the TEE because it affects surgical repairability. Before you start scanning, there is no shame in CHEATING. Talk to the patient and get the history. Who did the operation (surgeon and institution)? Where is the scar—sternum or side of chest (ductus or coarctation repair)? But wait, there is more: the coronary arteries need to be moved back too. It’s gonna be bad if there is cyanosis, clubbing, or polycythemia! Are the lungs clear or congested on that chest X-ray (that you never look at)? If you got a little befuddled by the name of an operation, here are more: Anything “Rastelli”—look for a conduit (or at least a patch). Sometimes the name order is changed if Dr. Kaye’s or Dr. Stansel’s people are operating. Here are some suggestions for communicating with the grumpy person holding the scalpel on the other side of the plastic curtain. Transposition of the great vessels gets confusing real quick if you insist on using certain terms. Don’t say “right” ventricle—describe the ventricle instead. Don’t say pulmonary artery—say: the great vessel that bifurcates and has no coronary ostia.
Terminology and Associations
History
Physical Findings and Labs
More Names of Operations
Using Descriptive Echocardiographic Lingo in Transposition