Congenital Heart Disease

Chapter 17


Congenital Heart Disease



Liliana Cohen and Daniel M. Shindler





Terminology and Associations


Since cardiac embryology can be summed up as 9 months of looping and unlooping, you can think of the congenitally malformed heart as having taken the wrong turn at Albuquerque, and not just one time….


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:



You say pairs of associations are boring?


We say TETRAlogy:



You should know the difference between overriding and straddling:




History


Before you start scanning, there is no shame in CHEATING. Talk to the patient and get the history.


Was there a surgical repair?


Who did the operation (surgeon and institution)?


At what age?


For example, an operation done right after birth may be palliative and just the first part of staged procedures. In patients born with hypoplastic left hearts, a Blalock Taussig Shunt, may be followed by a Glenn Shunt, followed by a Fontan. This is the Norwood heart. (Oh, don’t ask…).


Where is the scar—sternum or side of chest (ductus or coarctation repair)?


What year was the surgery?


There were no arterial switch operations for transposition until Jatene and the Lecompte maneuver (aka zee French Connection) in 1975. The surgeon maneuvers the pulmonary artery forward and then switches the great vessels back where they belong.


But wait, there is more: the coronary arteries need to be moved back too.


If this is Greek to you, please know that before that time, transposition of the great vessels was being repaired by the Mustard procedure (at the Mayo Clinic—heh! heh!) using an intra-atrial baffle to redirect blood.





More Names of Operations


If you got a little befuddled by the name of an operation, here are more:



The original, modified, imitated, emulated, glorified, immortalized on film (Alan Rickman and Mos Def), and still in use: Blalock-Taussig—subclavian artery to pulmonary artery shunt.


Anything “Fontan” means only three heart chambers. There are no operations named Kermit (frog heart….three chambers…. Get it???).


Anything “Rastelli”—look for a conduit (or at least a patch).


You don’t need to know this.


Do not read it.



Sometimes the name order is changed if Dr. Kaye’s or Dr. Stansel’s people are operating.


It is an anastomosis of the distal part of the proximal pulmonary artery to the side of the ascending aorta.


Blood flow to the pulmonary arteries is re-established by a graft from the subclavian artery or from the thoracic aorta.


It is used to treat single ventricles with transposition of the great arteries when there is obstruction of aortic flow due to subaortic stenosis.


We told you not to read this.




Using Descriptive Echocardiographic Lingo in Transposition


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.


The anatomic right ventricle may serve as the systemic ventricle but it always has an infundibulum. This means that the atrio-ventricular valve is separated from (not in fibrous continuity with) the semilunar valve.


Don’t say mitral valve—instead say: the atrio-ventricular valve that has only two leaflets, that is shaped like the mitre of a bishop, and receives blood from the (systemic or venous) atrium.


Don’t say pulmonary artery—say: the great vessel that bifurcates and has no coronary ostia.


Jun 4, 2016 | Posted by in CARDIOLOGY | Comments Off on Congenital Heart Disease

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