Congenital Heart Disease

Published on 06/02/2015 by admin

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Last modified 22/04/2025

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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.

Ventricular Septal Defect

When it comes to ventricular septal defects your diagnostic mileage may vary (making it appealing to test-writers and appalling to test-takers!).

The most common restrictive ventricular septal defect is the perimembranous defect.

So… always look for high-velocity systolic jets in the right ventricular cavity.

The left-to-right shunt most frequently enters the right ventricle “peri-where” the chordae of the septal tricuspid leaflets attach to the interventricular septum. The color flow jet is the Doppler equivalent of the typical physical finding of a loud systolic murmur with a palpable precordial thrill.

By the way, ventricular septal rupture is not congenital but it remains a highly lethal complication of acute myocardial infarction with similar clinical findings.

The Natural History May Fool You

Someone who had a loud systolic murmur during infancy (in the history that you obtained, ha!), and now only has a soft diastolic decrescendo murmur (on auscultation), may have closed a subaortic ventricular septal defect, but the aortic valve may not be adequately supported by the healed septum. Say hello to my little friend: aortic insufficiency (caused by a healed or healing ventricular septal defect).

But wait…there is more…

The lack of support in the area of the subaortic ventricular septal defect may enlarge (or as President Bush would say: “aneurysmificate”) the right sinus of valsalva.

Sinus of Valsalva aneurysms may rupture into the right ventricle.

This is where YOU come in:

Still More VSD Pitfalls

Down syndrome is associated with partial, or complete, av canal defects.

As a result, the mitral valve may have a cleft—don’t forget to look for it. It’s like a slice of pie was removed from the anterior mitral leaflet.

The tricuspid valve may send chordae (visible on TEE) to the crest of a ventricular septal defect.

When it comes to chordal attachments to the interventricular septum, the “septo-phobic” mitral says “no means no”; the “septo-phyllic” tricuspid may straddle the septum on both sides making surgical repair a problem.

Patients with Eisenmenger’s may have no Doppler flow across a ventricular septal defect; but there are clues… the pulmonary artery is way bigger than the aorta, and there may be severe pulmonic insufficiency.

There is a rare ventricular septal defect known as a Gerbode defect, with the shunt going from the left ventricle to the right atrium. The high-velocity systolic jet in the right atrium may be mistaken for tricuspid regurgitation, and may be mistakenly used to diagnose non-existent pulmonary hypertension (good thing you put the Swan in).

The mid-esophageal 75 to 90 degree TEE view can show three valves simultaneously. A ventricular septal defect jet in this view can appear to enter the right ventricle from the aortic valve. You are not going to fall for that! If it originated in the aorta it would be a ruptured sinus of Valsalva, and the color flow would be systolic AND diastolic.

The common perimembranous ventricular septal defect directs the SYSTOLIC jet to the tricuspid valve. The less common subpulmonic ventricular septal defect directs the jet (you guessed it) toward the pulmonic valve.

Atrial Septal Defects

Atrial septal defects may remain undiagnosed until adulthood. The murmur is subtle and can be missed. Yes, yes, fixed splitting of the second heart sound is a valuable clue.

You should know THREE different atrial septal defects.

The most common is the secundum defect. Surgeons love to fix these. From the right atrium it looks like the circular center of a crater is missing. On TEE examination the thin membrane of the fossa ovalis is indeed absent. To confirm the presence of a hole (as opposed to echo dropout), use color flow Doppler and also look for a negative contrast effect when you fill the right atrium with agitated saline. If it is not a hole but a flap, then it is a patent foramen ovale. If the right atrium and right ventricle are not dilated in an adult, it is not a hole. It’s a flap.

A defect of the “roof” of the right atrium, close to the sinus node, near the entry of the superior vena cava into the right atrium is called a sinus venosus atrial “septal” defect. It is associated with anomalous drainage of a right pulmonary vein.

Lastly, primum atrial septal defect is due to failure of the atrial septum to connect to the crux of the heart. This is part of the av canal defect and is easily recognizable. However, you can easily miss an associated small ventricular septal defect on a TEE.

Oh yes, we did say three, but… if you inject saline into a left arm vein and the contrast shows up first in the LEFT atrium, you have got yourself a persistent left superior vena cava with a fourth kind of “atrial septal” defect—an unroofed coronary sinus.

Pulmonary Valve Stenosis

Pulmonary valve stenosis may be isolated, or associated with other lesions. It is part of the tetralogy of Fallot. It is also associated with atrial septal defects. Tet + ASD = Pentalogy of Fallot (honest!). The normally thin and notoriously difficult-to-image pulmonic valve may be thick (dysplastic) and it may dome—indicating fused commissures.

The physical exam of severe pulmonic stenosis includes a harsh, loud, widely disseminated murmur. It sounds like you are clearing your throat. Doppler examination parallel to the stenotic jet (harder to do than it would seem from the high esophagus) gives the gradient. Look at the pulmonic valve carefully before they start cutting the patient for a Ross procedure. Right ventricular hypertrophy is also a clue to an abnormality causing pressure overload.

Questions

1. Which of these congenital abnormalities is most likely to result in a dilated right ventricle?

2. Which association is uncommon?

3. Sinus of Valsalva aneurysm ruptures into the:

4. All the following require TEE evaluation of cardiac veins EXCEPT:

5. The following can be present AFTER “definitive” tetralogy repair EXCEPT:

6. AV canal defects are associated with the following EXCEPT:

7. The following eponyms go with transposition EXCEPT:

8. Least likely to permanently improve symptoms of hypertrophic cardiomyopathy:

9. Atrio-ventricular discordance with ventriculo-arterial discordance indicates:

10. Single papillary muscle in the left ventricle is associated with:

Answers

1. Atrial septal defect results in dilatation of the right cardiac chambers because of the right-side volume overload. Pulmonic valve stenosis results in right ventricular hypertrophy because of the pressure overload. Ventricular septal defect results in left ventricular volume overload (oh, don’t ask why). Transposition of the great vessels (D-TGA) needs to be repaired for survival, and will result in dilatation of the systemic ventricle (anatomic right) with the now abandoned Mustard repair. If D-TGA is repaired with the arterial switch procedure, the right ventricle will be unaffected. Anatomically corrected transposition of the great vessels (L-TGA) results in dilatation of the systemic (anatomic right) ventricle.

    How do you confuse an echocardiographer? Show him two shovels and ask him to take his pick.

2. The longest one with the italics: E

3. Always pick C. You should Google E. D is like the Tooth Fairy, Santa, and a cardiac surgeon with a sense of humor (non-existent).

4. E. Snowman cardiac shadow: the head of snowman is the superior mediastinal shadow delineated by the SVC on the right, vertical vein on left side and left brachicephalic vein superiorly. Body of snowman is the heart. Snowstorm is brought to you courtesy of pulmonary congestion.

    Scimitar (shamelessly self-promoted): http://rwjms1.umdnj.edu/shindler/scimitar.html

5. C. They usually do a good job closing the VSD. Thank God for small favors.

6. C

7. Duh!

8. E.

9. D (fooled you)

10. A

Author’s note: This may seem like “the ultimate bail”, but let me emphasize—google any of these lesions/defects/operations and you will see videos/power points/animations. If you want to learn this material, use this book in conjunction with the wealth of material online.

Christopher J. Gallagher

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