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A Young Man With A Congenital Heart Defect

…in four parts, from Paul Levy’s blog.

It is, says Paul, “From a friend of a friend,” and it starts thus…

My son is sleeping right now…had a rough weekend – his blood pressure dropped, his blood count was decreasing, and he had chest and neck pain. The clinical team adjusted his meds, gave him a unit of blood, and are now trying to figure out what to do next. He is scared and worried and wants so desperately to be “normal” again. He is scheduled for leg surgery this afternoon and then we wait to see what the next steps will be.

While I have a few quiet moments, I thought I’d document the story of how he made it this far….it is a story of extraordinary luck and a fair amount of clinical heroism.

My son was born 17 years ago with transposition of the great arteries (his heart had over-rotated and was pumping in a way that didn’t allow oxygenated blood to move from the lungs to the body and back again) so he had a 9 hour operation at a week old to reconstruct his heart.

…read the rest of part one

…and parts two, three & four.

Here, in my opinion, is the best passage from the entire saga…

My son is receiving absolute top-notch care from the only place in the area that could have saved him, but was by luck, not by any “consumerism” on our part – we didn’t Google “teenage arterial switch survivor with heart attack” or pull up HealthGrades to find the best hospital or doctors to treat him….we have benefited from the kindness and skill of a community of health care providers affiliated with a hospital that was uniquely situated to help him, but the only choice we had in this was what hospital to drive him to.

In part 2, we learn the reason for the young man’s sudden collapse…

We learned much later that the problem that caused the heart attack was due to his reconstructive surgery when he was a baby…as he grew and became more active, one of the reimplanted coronary arteries became pinched between the rebuilt pulmonary artery and the aorta….this was an inevitable result of the surgery that saved his life 17 years ago and would have happened at some point – while swimming, riding his bike, walking in the neighborhood, playing lacrosse, or running by himself in the neighborhood as he trained for cross country….so the fourth link – he happened to have his attack while at a school with trainers equipped with an AED, with coaches and parents and teammates right there ready and able to help him. He wasn’t alone….and he was in the best possible place to have his attack (even though he complicated things a bit by having it in the woods and falling down a steep bank)

Congenital cardiovascular abnormalities, especially anomalous coronary arteries, are amongst the commonest of causes of sudden cardiac death in athletes.1 Ramona had posted about a young man who collapsed and died during the Little Rock Marathon in 2008. That unfortunate young athlete had a rare disease of the coronary arteries.

Coronary artery anomalies constitute 1–3% of all congenital malformations of the heart. In approximately 0.46–1% of the normal population, anomalies of the coronary arteries are found incidentally during catheter angiography or autopsy. The etiology of coronary artery anomalies is still uncertain. Maternal transmission of some types has been suggested, particularly when only a single coronary artery is involved. Familial clustering is also reported for one of the most common anomalies, in which the left circumflex coronary artery (CX) originates from the right sinus of Valsalva. Anomalies of the coronary arteries may also be associated with Klinefelter’s syndrome and trisomy 18 (i.e., Edwards syndrome). Cardiac causes for early and sudden infant death include anomalies of the coronary arteries; the Bland-White-Garland-Syndrome may be one relevant cause. Anomalies of the coronary arteries found in children may be associated with other congenital anomalies of the heart like Fallot’s syndrome, transposition of the great arteries, Taussig-Bing heart (double-outlet right ventricle), or common arterial trunk.2

Normal Coronary Arterial Anatomy

Normal Coronary Arterial Anatomy

Common variants are anomalies with origin from the contralateral side of the aortic bulb. These include an origin of the LMA or the LAD from the RSV or the proximal RCA and an origin of the RCA from the LSV or the LMA. There are four possible pathways for these aberrant vessels to cross over to their regular peripheral locations: (1) “anterior course” ventral to the pulmonary trunk or the right ventricular outflow tract, (2) “interarterial course” between the pulmonary artery and aorta, (3) “septal course” through the interventricular septum, and (4)”retro-aortic course”. Clinically, course anomalies of the coronary arteries are subdivided into “malignant” and “non-malignant” forms. Malignant forms are associated with an increased risk of myocardial ischemia or sudden death and mostly show a course between the pulmonary artery and aorta (i.e., “interarterial”). The most common case is an origin of the RCA from the LSV that courses between the aortic bulb and the pulmonary artery. Anomalies of the LMA or the LAD arising from the RSV with a similar course are associated with higher cardiac risk, too. It is suggested that myocardial ischemia and sudden death result from transient occlusion of the aberrant coronary artery, due to an increase of blood flow through the aorta and pulmonary artery during exercise or stress. The reason is either a kink at the sharp leftward or rightward bend at the vessel’s ostium or a pinch-cock mechanism between the aorta and pulmonary artery. Up to 30% of such patients are at risk for sudden death.2

The young man in this story probably had something like this after the surgical correction (Arterial Switch Operation) for TGA…

“Malignant” course of LAD

…a classical malignant course of the LAD between the Aorta and Pulmonary artery.


  1. Sudden Death in Young Athletes: Screening for the Needle in a Haystack – Free full text article in American Family Physician.
  2. Text about congenital coronary artery anomalies and the two figures are from this textbook – Multi-slice and Dual-source CT in Cardiac Imaging.

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*This blog post was originally published at scan man's notes*

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