A 38 y.o. male with a long history of syncopal episodes; often after he runs 5K a day, comes to the ED with chest pain.
the pain iis pleuritic and a CTA was done
normal coronary anatomy is shown below
All the coronaries come off the aorta.
Our patient had a coronary cameral fistula. This is a connection between a coronary artery and a cardiac chamber. It usually arises from the R coronary system and is asymptomatic. In the case of our patient there was a connection of the LAD to the pulmonary artery with resultant pulmonary hypertension. The LAD also had many tiny branches draining into the RV. Large fistulas can create a significant shunt and a “steal phenomenon” where blood that normally would go to the ventricle is shunted to the low pressure pulmonary artery system. This can lead to myocardial ischemia.
an example of an LAD to pulmonary artery fistula on cath
CAUSES OF CARDIAC CHEST PAIN IN YOUNG INDIVIDUALS
Anomalous coronary arteries-as in our patient can cause chest pain. This includes coronary arteries that traverse muscle which are known as bridging vessels.
Hypertrophic cardiomyopathy- is thought to be a genetic abnormality inherited in an autosomal dominant manner. It affects one in 500 people and can have a variable presentation from patients who are symptom free to those with arrhythmias and those with sudden death.
hypertrophic cardiomyopathy often presents with q waves in v5,v6
Vasculitis
-Kawasaki disease- is an acute self limited vasculitis that occurs in young children. It was first described in 1870 when an autopsy showed coronary artery aneurysms in a child who died at age 7. It often presents with a febrile illness and mucocutaneous changes. About 25% of children with untreated disease and 5% of those treated with immunoglobulin develop coronary artery aneursyms. This inflammation may lead to thrombus formation or plaque formation later in life.
The strawberry tongue which can be seen in Kawasaki’s can also be seen in scarlet fever and MIS -C (Multisystem inflammatory syndrome) in COVID
- Fibromuscular dysplasia- is an idiopathic vasculopathy affecting small to medium sized arteries. And can lead to coronary dissection.
fibromuscular dysplasia can cause a “beads on a string” appearance in coronary arteries. This is alternating strictures and aneursymal dilation.
Drugs- Drug related myocardial ischemia- may result in coronary vasospasm and ischemia. Cocaine is a leading offender.
Coronary artery dissection- occurs most often in young women in the peripartum period.
Coronary artery disease- The most common causes of myocardial ischemia in young adults are risk factors for inflammation like: raised triglycerides, low HDL, and hypertension
Our patient was referred for further studies to see if he would benefit from surgery. He is currently being followed at the Cleveland Clinic.
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Battisha A, Madoukh, Sheikh O, et al. Coronary fistula between left anterior descending artery and pulmonary artery leading to sudden cardiac death: case report with literature review. Curr Cardiol Rev. 2020;16(2):98-102.
Harle T, Kronberg K, Elsasser A. Coronary artery fistula with myocardial infarction due to steal syndrome. Case Reports Clin Res Cardiol. 2012Apr;10(4):313-15.
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Michelis K, Olin J, Dodov D, et al. coronary artery manifestations of fibromuscular dysplasia. J Am Coll Cardiol. 2014 Sep9;64(10):1033-46.