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CORONARY ARTERY ECTASIA
     

CURRENT KNOWLEDGE and SIGNICANCE of CORONARY ARTERY ECTASIA

Using multiple indirect observations and current understanding of endothelium-derived relaxation factor, a possible etiology that implicates overstimulation of endogenous nitric oxide is provided.

Current literature suggests that ectatic coronary arteries, even without the presence of coronary stenosis, are subject to thrombus formation, vasospasm, and spontaneous dissection.

Newer subgroups of ectasia are arising with the use of multiple interventional devices to dilate coronary artery stenosis. By design, these destroy the media of the coronary artery, and it is not clear whether these "iatrogenic" ectatic arteries are subject to the same complications as "idiopathic" coronary artery ectasia.

Further investigation is necessary to help define the benefit of the proposed treatment regimen, to clarify the prognosis of these newer groups of "iatrogenic" ectasia, and to confirm or disprove the hypothesis targeting nitric oxide as an etiologic factor.

Summary: Coronary artery ectasia is the abnormal enlargement of the coronary artery. The prognosis, treatment, and etiology of this disease remain an enigma. There is some evidence to suggest that the incidence of ectasia is increasing, and therefore understanding of this entity needs to improve.

Vincent L. Sorrell, M.D., Michael J. Davis, M.D., Alfred A. Bove, M.D., Ph.D.

January 20, 1996 issue of The Lancet.

Coronary Ectasia: Prevalence, Clinical and Angiographic Characteristics

S Lahiri, KK Sethi, R Jain, JPS Sawhney, VK Chopra,
S Dhawan, PK Khanna

Sir Ganga Ram Hospital, New Delhi

Coronary artery ectasia is a well-recognized pathological finding. The clinical significance of coronary ectasia is not clear. It has been suggested that the presence of ectasia alone is as important as coronary artery stenosis.

Over a study period of 3 years and 3 months (Jan 1999–Mar 2002), 3571 patients underwent coronary angiography in our institute.

Of these, 84 patients (2.3%), 70 males (83.8%) and 14 females (16.2%), met the criteria of coronary artery ectasia, diagnosed as a segment of the artery more than 1.5 times the diameter of the adjacent normal segment.

Fifty patients had ectasia without any significant narrowing of other coronary arteries (isolated ectasia) and 34 (40.4%) had associated significant coronary artery stenoses. The mean age of the group was 54.4+8.6 years.

Hypertension was the most common risk factor [46 (54.7%)], followed by diabetes [29 (34.5%)]; 17 (20.2%) were smokers, 15 (17.8%) were dyslipidemic and 3 (3.5%) had a family history of premature coronary artery disease.

While 46 patients (54.7%) presented with acute coronary syndrome [24 (28.5%) AMI and 22 (26.1%) unstable angina], 9 (10.7%) had stable angina, 26 (30.9%) had atypical chest pain and 3 (3.5%) had angina equivalent.

ECG revealed typical infarct pattern in 24 patients (28.5%), ST/T changes in 29 (34.5%), LBBB in 2 (2.3%), RBBB in 3 (3.5%) and normal pattern in 26 patients (30.9%).

The right coronary artery was the vessel most commonly affected [53 (63%)] followed by the left anterior descending [43 (51.1%)] and circumflex [24 (28.5%)] artery. Left main coronary artery ectasia was seen in 7 (8.3%) patients; 43 patients had single-vessel ectasia, 26 had two-vessel and 15 had three-vessel ectasia.

A thrombus in the ectatic segment was found in 13 patients, of whom 10 had acute coronary syndrome.

In patients presenting with acute coronary syndrome, the culprit lesion was found to be related to the ectatic segment in 67.4% of the patients based on ECG correlation.

Overall, 34% of the patients with isolated ectasia had definite acute coronary syndrome.

In the other 66% of patients, it was an incidental finding on coronary angiography.

Three patients underwent PTCA and 5 had CABG. In-hospital death was seen in 1 (1.1%) patient with coronary ectasia and acute anterior myocardial infarction with cardiogenic shock.

On follow-up of 22+13 months, of 70 patients there was 1 (1.4%) sudden cardiac death while 5 (7.1%) continued with atypical chest pain.

Thus, coronary ectasia occurs predominantly in males, and most often is an isolated finding. It may present as acute coronary syndrome. However, the long-term prognosis is good.

Summary:

Coronary artery ectasia is the abnormal enlargement of the coronary artery. The prognosis, treatment, and etiology of this disease remain an enigma. There is some evidence to suggest that the incidence of ectasia is increasing, and therefore understanding of this entity needs to improve.

EXCERPTS FROM ARTICLE BELOW

LIPOPROTEIN METABOLISM and ATHEROSCLEROSIS

FAMILIAL HYPERCHOLESTEROLEMIA

Among the 18 homozygotes 9 patients died.Eight of the 9 patients died of atherosclerotic cardiovascular disease, and one patient died of leukemia.

Table 2 @ Causes of death in heterozygous FH patients
Cause of death No Age (yrs) Cholesterol (mg/dl) Triglycer

 

@
M F M F M F M F
CHD 51 31 59}14 70}8 350}73 351}64 145}53 155}79
Stroke 4 8 64}5 71}4 365}143 345}66 130}72 151}66
Cancer 10 5 61}9 63}13 345}66 366}88 192}96 167}35
Others 6 8 64}18 72}10 315}89 361}84 132}53 148}78
Total 71 52 60}13 70}8 348}73 353}76 151}62 154}72

Table 2

 

Table 1. Causes of death in homozygous FH patients
No Case Sex Age Cause of Death Cholesterol Triglyceride
(yr) (mg/dl) (mg/dl)
1 KY F 27 Sudden death 609 126
2 KM F 42 Sudden death 610 180
3 SS F 29 Sudden death 1004 784
4 YE M 11 Heart failure 908 300
5 ST M 18 Heart failure 781 189
6 MI F 23 Sudden death 730 273
7 TT M 57 Sudden death 558 388
8 MK F 50 Leukemia 550 143
9 NY F 38 Sudden death 590 93
Mean @ @ @ @ 33 @ @ 704 275
SD 15 164 213

Table 1

Clinical Features in FH


caect-Figure 1

Cumulative number of fatal and non-fatal myocardial infarction in male and female FH patients


caect-Figure 2

Prevalence of clinical manifestations at different ages in the heterozygotes

Coronary angiographic studies have been done in homozygous and heterozygous FH patients.

A 23-year-old man with homozygous FH (M.T.) was found to have coronary ectasia by coronary angiography (caect-fig3.gif) , which was also frequently (18%) observed in heterozygous FH patients.


caect-Figure 3

One heterozygous FH patient (S.N.) with coronary ectasia died of myocardial infarction, whose autopsy findings showed severe coronary ectasia (2.7cm of coronary diameter) with narrowing of the lumen (caect-Figure 4 and caect-Figure 5).


caect-Figure 4

Progression of coronary ectasia in a patient with heterozygous FH .

caect-Figure 5

Coronary arteries obtained from autopsy sample. Diameter of coronary ectasia is 2.7mm.


Five homozygotes and 105 male heterozygotes and 56 female heterozygotes were studied by coronary angiography. Coronary stenosis index (CSI) was obtained by assigning score (0 to 5) to each of 15 coronary artery segments (caect-fig6.gif). According to the regression equations between age and CSI, coronary stenosis begins, on the average, at 13 years in the homozygotes, and then 17 and 25 years in the male and female heterozygotes, respectively. Thus, the cholesterol-lowering therapy by drugs or LDL-apheresis should be started as soon as possible in the homozygotes, at about 18 years-of age in the male heterozygotes, and later than 25 years of age in the female heterozygotes.


caect-Figure 6

Plots of correlation between age and coronary stenosis index (CSI) in homozygous(A) and male and female heterozygotes

Serum cholesterol levels in normal subjects, heterozygous and homozygous patients with FH showed distinct trimodal distribution of serum cholesterol levels (caect-fig7.gif see below) , and the high levels of serum cholesteol were due to high levels of LDL- and IDL-cholesterol (caect-fig8.gif,see below). Thus, FH is a disease of abnormal LDL metabolism.


caect-Figure7


caect-Figure 8


caect-Figure 9

Long-term treatment of FH with pravastatin was shown in caect-fig9


For the homozygous FH patients who are resistant to most of cholesterol-lowering drugs, liver transplantation, LDL receptor gene therapy or LDL-apheresis is indicated. A homozygous patient (Y.Y.) has been treated by LDL-apheresis using dextran-sulfate beads column . His xanthomas disappeared soon, and anginal attacks subsided after the twice a week treatment. LDL-apheresis using dextran-sulfate cellulose column is an intensive cholesterol-lowering therapy, and highly effective in removing apolipoprotein B-containing lipoproteins from plasma. Before and immediately after each LDL-apheresis, serum cholesterol decreased by 57%, and LDL-cholesterol by 66% and triglyceride by 74%. In contrast, HDL-cholesterol decreased by 22% . Forty-three heterozygotes had been treated by combination of LDL-apheresis and medication, while 87 heterozygotes had been treated by cholesterol lowering drugs alone for about 6 years15. Compared with drug therapy alone, LDL-apheresis produced greater reductions of total and LDL-cholesterol levels. On the analyses of Kaplan-Meier of the coronary events including non-fatal myocardial infarction, percutaneous transluminal coronary angioplasty, coronary artery bypass grafting and death of CHD, the rate of total coronary events was 72% lower in the LDL-apheresis (10%) than in drug therapy (36%)

Hiroshi Mabuchi, MD., PhD.

The Second Department of Internal Medicine
Kanazawa University School of Medicine
Takara-machi 13-1, Kanazawa 920-8641, Japan

TEL:(76)265-2250, FAX:(76)234-4271,
E-mail: mabuchi@med.kanazawa-u.ac.jpg