Coronary artery ectasia is a rare disease that occurs in only 0.3-4.9% of people in North America. Coronary artery ectasia is characterized by the enlargement of a

coronary artery The coronary arteries are the arterial blood vessels of coronary circulation, which transport oxygenated blood to the heart muscle. The heart requires a continuous supply of oxygen to function and survive, much like any other tissue or organ of ...

to 1.5 times or more than its normal diameter.Lin, C.T., Chen, C.W., Lin, T.W., and Lin, C.L (2008) Coronary artery ectasia, ''Tzu Chi Med'', 20(14), 270-274 The disease is commonly

asymptomatic In medicine, any disease is classified asymptomatic if a patient tests as carrier for a disease or infection but experiences no symptoms. Whenever a medical condition fails to show noticeable symptoms after a diagnosis it might be considered asy ...

and is normally discovered when performing tests for other conditions such as

coronary artery disease Coronary artery disease (CAD), also called coronary heart disease (CHD), ischemic heart disease (IHD), myocardial ischemia, or simply heart disease, involves the reduction of blood flow to the heart muscle due to build-up of atherosclerotic pla ...

stable angina Angina, also known as angina pectoris, is chest pain or pressure, usually caused by insufficient blood flow to the heart muscle (myocardium). It is most commonly a symptom of coronary artery disease. Angina is typically the result of obstru ...

and other acute coronary syndromes.Hsu, P.C., Su, H.M., Lee, H.C., Juo, S.H., Lin, T.H., Voon, W.C., Lai, W.T., and Sheu, S.H.(2014)Coronary artery collateral circulation in patients of coronary ectasia with significant coronary artery disease.''PLOS ONE'',9(1), doi: 10.13.71Antoniadia, A.P., Chatzizisis, Y.S., and Giamoglou, G.P. (2008) Pathogenic mechanisms of coronary ectasia. ''Int J Cardio'', 130, 335-343 Coronary artery ectasia occurs 4 times more frequently in males than in females and in people who have risk factors for heart disease such as smokers.Li, J., Nie, S., Qian, X., Zeng, H., and Zhang, C. (2009) Chronic inflammatory status in patients with coronary artery ectasia. ''Cytokine'', 46, 61-64 While the disease is commonly found in patients with

atherosclerosis Atherosclerosis is a pattern of the disease arteriosclerosis in which the wall of the artery develops abnormalities, called lesions. These lesions may lead to narrowing due to the buildup of atheroma, atheromatous plaque. At onset there are usu ...

and coronary artery disease, it can occur by itself and in both cases, it can cause health problems. The disease can cause the heart tissue to be deprived of blood and die due to decreased blood flow, and blockages due to blood clots or spasms of the blood vessel. This blood flow disruption can cause permanent damage to the muscle if the deprivation is prolonged. Coronary artery ectasia also increases the chance of developing large weak spots in the affected coronary arteries, or

aneurysms An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall. Aneurysms may be a result of a hereditary condition or an acquired disease. Aneurysms can also be a nidus (s ...

that can rupture and result in death. The damage can result in angina which is pain in the chest and is a common complaint in these patients.

Signs and symptoms

Pathology

Coronary artery ectasia is commonly found in patients with diseases of the connective tissue and an increased inflammatory response such as

Marfan syndrome Marfan syndrome (MFS) is a multi-systemic genetic disorder that affects the connective tissue. Those with the condition tend to be tall and thin, with long arms, legs, fingers, and toes. They also typically have exceptionally flexible joints a ...

and

Kawasaki Disease Kawasaki disease is a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age. It is a form of vasculitis, where blood vessels become inflamed throughout the body. The fever typically lasts for more tha ...

. It can also be found transiently in patients that have undergone stent placement resulting in the stretching of the vessels 1. Coronary artery ectasia is characterized by an increased wall stress of the vessel, thinning of the arterial wall which causes progressive dilation and remodelling of the vessel. The permanent dilation of the artery is thought to be mainly caused by inflammation, triggered by disease, chemicals, or physical stress of the vessel. A meta-analysis study has shown that The pooled unadjusted OR of CAE in subjects with HTN in comparison by subjects without HTN was estimated 1.44 (95 % CI, 1.24 to 1.68
Bahremand, M., Zereshki, E., Matin, B.K. et al. Hypertension and coronary artery ectasia: a systematic review and meta-analysis study. Clin Hypertens 27, 14 (2021). https://doi.org/10.1186/s40885-021-00170-6
The inflammatory response results in an over expression of

matrix metalloproteinases Matrix metalloproteinases (MMPs), also known as matrix metallopeptidases or matrixins, are metalloproteinases that are calcium-dependent zinc-containing endopeptidases; other family members are adamalysins, serralysins, and astacins. The MMPs be ...

, cysteine proteinases, and serine proteinases that causes the partial breakdown of the vessel and weakens it. The inflammation response will also trigger

platelet Platelets, also called thrombocytes (from Greek θρόμβος, "clot" and κύτος, "cell"), are a component of blood whose function (along with the coagulation factors) is to react to bleeding from blood vessel injury by clumping, thereby ini ...

activation which increases the risk of blood clots. The risk of blood clots will increase due to the turbulent blood flow of the enlarged vessel which can activate platelets and form clots.Varol, E., Uysal, B, A., Dogan, A., Ozaydin, M. and Erodogan, D. (2012) Mean platelet volume as a prognostic value in patients with coronary artery ectasia. ''Clin Appl Thromb-Hema'',18(4), 387-392 Inflammation elevated oxidative stress is increased, and antioxidant activity is depressed in coronary artery ectasia. This imbalance can cause damage to the cells and cause them to die, weakening the vessels further.Sezen, Y., Bas, M., Polat, M., Yildiz, A., Buyukhatipoglu, H., Kucukdurmaz, Z., Kaya, Z. and Demirbag, R. (2010) The relationship between oxidative stress and coronary artery ectasia. ''Cardio J'', 17(5), 488-494 The activation of the inflammatory response causes a detectable increase in

C reactive protein C-reactive protein (CRP) is an annular (ring-shaped) pentameric protein found in blood plasma, whose circulating concentrations rise in response to inflammation. It is an acute-phase protein of hepatic origin that increases following interleukin-6 ...

interleukin-6 Interleukin 6 (IL-6) is an interleukin that acts as both a pro-inflammatory cytokine and an anti-inflammatory myokine. In humans, it is encoded by the ''IL6'' gene. In addition, osteoblasts secrete IL-6 to stimulate osteoclast formation. Smooth ...

tumor necrosis factor alpha Tumor necrosis factor (TNF, cachexin, or cachectin; formerly known as tumor necrosis factor alpha or TNF-α) is an adipokine and a cytokine. TNF is a member of the TNF superfamily, which consists of various transmembrane proteins with a homolog ...

and

cell adhesion molecules Cell adhesion molecules (CAMs) are a subset of cell surface proteins that are involved in the binding of cells with other cells or with the extracellular matrix (ECM), in a process called cell adhesion. In essence, CAMs help cells stick to each ...

, which can be used as a diagnostic marker,.

Diagnosis

To discover the extent and severity of coronary artery ectasia there are a variety of diagnostic tools used. The most common method for discovering the disease is through

angiography Angiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins, and the heart chambers. Modern angiography is performe ...

. Angiography is the procedure where a contrast dye is entered into the vessels and an x-ray is taken, which will allow the vessels to be seen on the x-ray.Aydn, M., Texn, I.O., Dogan, S.M., Yildirum, N., Arasli, M., Sayn, M. and Aktop, Z.(2009)The levels of tumor necrosis factor-alpha and interleukin-6 in patients with isolated coronary artery ectasia.''Mediat Inflamm''.Doi:10.1155/2009/106/145 Using angiography clinicians are able to display the size, location and number of vessels affected by the disease. Is can also be analyzed through other methods such as

intravascular ultrasound Intravascular ultrasound (IVUS) or intravascular echocardiography is a medical imaging methodology using a specially designed catheter with a miniaturized ultrasound probe attached to the distal end of the catheter. The proximal end of the cath ...

, and

magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wave ...

. Using these diagnostic methods, it has been discovered that the disease normally occurs most often in the

right coronary artery In the blood supply of the heart, the right coronary artery (RCA) is an artery originating above the right cusp of the aortic valve, at the right aortic sinus in the heart. It travels down the right coronary sulcus, towards the crux of the hear ...

, followed by the

left anterior descending artery The left anterior descending artery (also LAD, anterior interventricular branch of left coronary artery, or anterior descending branch) is a branch of the left coronary artery. Blockage of this artery is often called the ''widow-maker infarction' ...

, and finally the

left anterior circumflex artery Left may refer to: Music * ''Left'' (Hope of the States album), 2006 * ''Left'' (Monkey House album), 2016 * "Left", a song by Nickelback from the album ''Curb'', 1996 Direction * Left (direction), the relative direction opposite of right * L ...

.Lin, C.T., Chen, C.W., Lin, T.W., and Lin, C.L (2008) Coronary artery ectasia, ''Tzu Chi Med'' 20(14), 270-274 Using these methods Coronary artery ectasia can be divided into four different types: Type 1¬→diffuse ectasia in 2-3 different vessels, Type 2¬→ diffuse disease in 1 vessel and local disease in another, Type 3¬→ diffuse disease in one vessel and Type 4¬→ localized or segmental ectasia.

Treatment

There are currently no cardiovascular society guidelines or recommendations for the treatment of coronary artery ectasia. Experts in the field urge clinicians to consider anti-platelet therapy, such as Aspirin, to reduce thrombus formation in pocket vorticies associated with turbulent blood flow. Dual anti-platelet therapy and full anticoagulation are currently under investigation. The primary etiology of coronary ectasia in adults is atherosclerosis, thus treatment with statin therapy should be considered. Statin therapy may also reduce inflammation and matrix metalloproteinase activation which may reduce the progression of vessel ectasia. Some studies have also suggested the use of

angiotensin converting enzyme inhibitors Angiotensin-converting-enzyme inhibitors (ACE inhibitors) are a class of medication used primarily for the treatment of high blood pressure and heart failure. They work by causing relaxation of blood vessels as well as a decrease in blood volum ...

, as ACE gene polymorphisms have been implicated in disease progression. Risk factor modification is recommended; including tobacco cessation, blood pressure control and avoidance of illicit substance use, specifically cocaine.

References

{{Circulatory system pathology Heart diseases