Although the etiology of CE is an open field where scientifics are still investigating ₇, several possible causes are recognized such as chronic exposure to herbicides, disease of Takayasu, polyarteritis nodosa, diseases of Kawasaki and Ehlers-Danlos, and other less important diseases like the syndrome of Marfan, rheumatic fever and syphilis ₃, ₇, ₁₃, ₁₇, ₁₈.

Atherosclerosis is, probably, the main cause of primary CE ₇, ₁₆, ₁₉, ₂₀. Percutaneous transluminal coronary angioplasty may favor secondary CE ₇, ₁₆, ₁₉ ; however, the alterations produced in the vascular wall by coronary dilations are rather recognized as producers of aneurysms, because the damage is, strictly, focal ₅,₇. Another cause recently accepted is the prolonged anabolic steroid use, because it is considered that it produces structural damage in blood vessels ₂₁. In addition, Sorrel et al ₁₉ consider that chronic exposure to vasodilator substances, such is nitric oxide may be included; in spite of being a fast local acting agent.

Androulakis et al ₂₂ suggest that CE would have some relation with diabetes mellitus and Gulec et al ₂₃stated that alterations of angiotensin converting enzyme gene could constitute a potent risk factor for CE formation. Androulakis et al ₂₄ say that CE is associated with the presence of aneurysms in other sites of the arterial tree, including the aorta, and they also say that although relation between arterial and venous ectasia is not established, they found in their study that the presence of varicose veins or phlebectasia in lower limbs was more frequent in patients with CE that in those who did not present it, which suggests the possible existence of a generalized defect of all vessel walls. Oomman et al ₂₅ and Triantafillidi and coworkers ₂₆ also agree this suggestion and reported cases with CE and aortic dissection, and CE with aneurysm of the basilar artery and phlebectasia. Altinbas et al ₂₇ describe the case of a woman who went to emergency room complaining of chest pain, and angiography revealed CE, enlargement of the ascending aorta and aortic coarctation.

Histological studies have demonstrated that there are certain atherosclerotic plaques that do not reduce the diameter of the lumen, presumably due to the phenomenon of arterial remodeling ₂₈, and it may play an important role on physiopathologic mechanism of CE. Diffuse hyalinization and lipid deposition, and the appearance of alterations in the intima and media layers, as well as calcifications, fibrosis and intramural hemorrhage are findings which appear on histopathologic exam of aneurysms. Some of these findings can also be observed in CE ₇. The presence of expanded segments in arteries with remarkable atherosclerosis and in those with structural changes as in the syndrome of Marfan supports the hypothesis that these are the result of a weakening of the media layer of the artery ₇.

We tried to find the etiology of this illness in our patient but venereal disease research was negative, as same as other specific laboratory tests that discard such diseases. We also performed an aortography, and looked for other ectatic arteries, however none positive result was found. That was why we inferred that his CE could be produce by atherosclerosis.

Markis et al ₂₉ were first in evaluating patients with CE prospectively. They described the clinical aspects of the evolution, during 24 months, of 30 selected patients out of 2457 consecutive coronary angiographies, and they observed that those patients, without obstructive coronary disease, had a greater prevalence of arterial hypertension and myocardial infarction than those from the control group; and presented a mortality rate of 15% in 2 years, equivalent to those with three-vessel disease. Palomo et al ₉ found that all their patients with CE presented antecedents of angina or infarction, and left anterior descending artery was the most frequently affected (48%) by severe stenosis.

Coronary disease of the transplanted heart is one of the presentation types of CE ₁₂. More than 70% of the transplanted patients had some expanded coronary segment, which was associated, in more than half of the cases, to thickening of intima layer ₁₂.

Endoh et al ₃₀ found that 65% of their patients with CE had myocardial infarction, 91% had coronary artery disease, and 66% had a single vessel affected with the ectatic illness. Major coronary events were seen in 37% of patients; and 72% of these events, that appeared during the follow up, happened in the first 4 years after the cardiac event that promoted the first angiography. Seventy eight percent of these events were acute coronary syndromes. There were no significant differences for age, nor for the number of arteries with CE. This authors ₃₀, as same as Guérios et al ₇, and Nyamu et al ₂ raise, that CE is not benign and must be carefully taken care.

Mrdovic et al ₃₁ showed a case, similar to ours, of a 47-year-old man with several admissions due to acute coronary syndromes where the electrocardiographic alterations appeared in different regions; coronary angiography showed the presence of diffuse ectasia of the coronary arteries without significant estenosis. The patient received thrombolytic therapy in the first admission and in the second he needed defibrillation because of cardiac arrest in ventricular fibrillation. In patients with CE and acute coronary syndrome there is an activation of the fibrinolytic system, probably induced by the formation of thrombus in the ectatic segments of coronary arteries ₁₄. Therefore it may stimulate the appearance of chest pain that occasionally appears in patients with CE, without concomitants obstructive injuries ₂,₃₂.

Nyamu et al ₂ reviewed 6938 coronary angiographies and they found that 134 (2%) had isolated CE; 118 were symptomatic, and of them, 34(25%), had a history of or presented with myocardial infarction, with correlation between the affected territory and the ectatic artery in 32 patients. Sixty two patients presented dyslipidemia, and 65% of this were hypertriglyceridemia. Left anterior descending was the most commonly affected artery. Diffuse ectasia most commonly involved the right coronary artery, and one patient had spontaneous coronary dissection. There was a greater prevalence of isolated CE that frequently affected the right coronary artery, whereas diffuse CE and involvement of left anterior descending artery were discrete.

Nishikawa et al ₃₃ showed the case of a 69-year-old woman who debuted with disnea on exertion, and echocardiogram revealed global hypokinesia of the left ventricle, which was dilated. Myocardial perfusion scintigraphy showed hypocaptation of the tracer which suggested the presence of a coronary artery disease rather than dilated cardiomyopathy, reason why coronary angiography was performed and no organic estenosis was observed; but diffuse CE was noted in three vessels. Intravascular ultrasound showed a severe CE with a maximal diameter of 8,2 mm (smaller than our patient). Coronary flow velocity was reduced, and spasm was not caused with the ergonovine test. These findings suggested the presence of alterations of microcirculation, probably by microthrombi, which could have produced myocardial ischemia and the later dilation. The patient was treated with nicorandil and ticlopidine and the left ventricle wall motion, as well as ischemia seen in the perfusion test, improved.

Coronary flow reserve is significantly reduced in patients with diffuse CE when comparing with control group ₃₄. Although volumetric coronary blood flow is higher in the patients with CE, the dysfunction of microcirculation, reflected by the reduction of the coronary flow reserve, may be the cause of myocardial ischemia induced by exercise ₃₄. The ectatic coronary segments have a media layer significantly thinner than the normal segments. Triantafyllidi et al ₃₅ found that the pulsating changes of the arterial wall were increased in the ectatic segments and the ectatic coronary segments due to atherosclerosis showed a diminution of the thickening of the media layer, and an increase of the pulsating changes of the coronary arterial wall, which may be indicative of mechanical and structural weakness of the artery that prone to aneurysms formation ₃₅. In fact, Senen et al ₅ suggest that the physiopathologic mechanism of CE is not focal and they are, probably, right; however, this mechanism has not been well established yet ₇. Sorrel et al ₁₉ suggest that there is an association between the chronic stimulus of endogenous nitric oxide and the relaxation of the vascular wall which favors the sprouting of CE.

In the study of Harikrishnan et al ₁₀144 patients with CE were diagnosed out of 3200 coronary angiograms (4.5%), and 122 presented coronary stenosis more than 50% (group A). Group B was formed by the 22 patients with CE who did not have obstructive coronary disease, and group C was formed by comparable patients who only had coronary disease without CE. All patients with CE presented similar epidemiologic features, however, the patients from group B showed better ventricular function and stress tests were negative. Nevertheless, in patients from group A, the incidence of myocardial infarction, coronary risk factors, and severity of coronary disease, as well as the stress test status were similar to those from group C.

On a mean follow up of 3±1.2 years, all patients (from 3 groups) had similar rates of cardiac events. In this study right coronary artery was the most commonly affected vessel, followed by circumflex, left anterior descending and, finally, the left main coronary artery. Diffuse CE was found more frequently in right coronary artery, and focal or localised CE was more common in left anterior descending artery.

It is considered that the ectatic areas, which were associated to coronary stenosis, are very prone to spasms, thrombosis and spontaneous dissection; being, therefore, potential causes of myocardial infarction ₇,₂₀. Spasms, microembolisms and the alterations of the blood flow, that appear in CE, are common causes of episodes of angina ₇.

Echocardiography may be useful to detect CE and may even detect the presence of intracoronary thrombus ₃₆. Tan et al ₃₇ raise that the echocardiographic coronary-aorta index (greater coronary diameter in diastole divided between the diameter of the aortic annulus) could be useful for detecting coronary dilation.

Magnetic resonance angiography, in spite of being a noninvasive method, has a demonstrated effectiveness for follow up of patients with CE ₄, and positron emission tomography is also useful for the diagnosis of coronary anomalies ₃₈. The suitable management and treatment of CE are not well established yet ₁₆. Nevertheless, it is known that cumarinic anticlotting therapy is a standard regime in this disease ₁, and trimetazidine may improve the exercise-induced angina, and also improve tolerance to physical activity in patients with CE ₁₅. In addition, antiplatelet therapy may be useful in the preservation of left ventricular function in patients with CE, as well as in diminishing symptoms ₃₉,₄₀. In cases of thrombotic occlusions, thrombolysis has shown effectiveness ₅,₄₁.