Giant coronary artery aneurysm secondary to Kawasaki disease: diagnosis in an adult by multi-detector row CT coronary angiography.

We present the case of an adult female patient with a giant coronary artery aneurysm secondary to Kawasaki disease diagnosed for the first time, as far as the authors are aware, on multi-detector row computed tomography (MDCT). The long-term complications relate to the persistence of these aneurysms with giant coronary aneurysms having the lowest regression rate, the highest risk of stenosis and strongest association with myocardial infarction. MDCT coronary angiography represents an ideal, alternative non-invasive imaging modality for the diagnosis and follow-up of the coronary arterial complications of Kawasaki disease, thereby avoiding invasive coronary imaging, and its use in the management algorithm should be considered. We also aim to contribute to the expanding clinical role of MDCT coronary imaging.

Increased plasma endothelin levels in angina patients with rapid coronary artery disease progression.

AIMS: To investigate the association between plasma endothelin levels and rapid coronary artery disease progression, as assessed by quantitative angiography. METHODS AND RESULTS: Changes in diameter were assessed in 224 coronary stenoses of 92 consecutive patients (62 men) with chronic stable angina pectoris who were on a waiting list for routine coronary angioplasty and underwent coronary angiography on two occasions: the first (diagnostic) angiogram was carried out at study entry and the second 5.5+/-3.0 months later, immediately prior to coronary angioplasty. A digital quantitative angiographic analysis system was used to assess differences in stenosis diameter between the first and second angiogram. Plasma immunoreactive endothelin levels were estimated by radioimmunoassay at study entry. Rapid coronary artery disease progression occurred in 29 (31.5%) patients according to pre-established criteria: 12 (41%) had a > or =10% diameter reduction of at least one pre-existing stenosis > or =50%, 10 (34%) had a > or =30% diameter reduction of a pre-existing stenosis <50%, 5 (17%) patients developed a new stenosis and 2 (7%) had progression of a lesion to total occlusion by the second angiogram. Baseline demographic, clinical and angiographic data were similar in patients with and without stenosis progression. Plasma endothelin levels were significantly higher in patients with rapid disease progression than in those without (5.7+/-2.0 pg. ml(-1)vs 3.9+/-1.6 pg. ml(-1), P<0.001). Multiple logistic regression analysis revealed that endothelin was an independent predictor of disease progression (P=0.001). Moreover, endothelin levels above 4.26 pg. ml(-1)(the median of the total endothelin concentrations) were associated with a sixfold increase in the risk of developing rapid stenosis progression. CONCLUSIONS: Plasma endothelin is raised in patients with coronary artery disease progression and may be a marker of risk of rapid stenosis progression. Endothelin may also play a pathogenic role in this process.

Low clinical utility of routine angiographic surveillance in the detection and management of cardiac allograft vasculopathy in transplant recipients.

BACKGROUND: Cardiac allograft vasculopathy (CAV), a form of accelerated atherosclerosis, is the major cause of late death in heart transplant recipients. Routine annual coronary angiography has been used as the standard surveillance technique for CAV in most transplant centers. HYPOTHESIS: The aim of this study was to investigate the clinical utility of routine angiographic surveillance in the detection and management of CAV in transplant recipients. METHODS: We reviewed the case notes and angiograms of 230 patients who underwent cardiac transplantation in our unit between January 1986 and January 1996 and survived beyond the first year post transplantation. RESULTS: Significant complications secondary to angiography arose in 19 patients (8.2%). Cardiac allograft vasculopathy was present on none of angiograms performed 3 weeks post transplantation, but was identified in 9 patients (4%) at the first annual angiogram and an additional 25 patients by the fifth annual angiogram. A target lesion suitable for angioplasty was only identified in two patients, and only limited procedural success was achieved in both cases. Twenty-five patients (11%) died during the study period, and the most common cause of late death was graft failure which occurred in 10 patients. All patients who died from graft failure had significant CAV at autopsy, but the most recent coronary angiogram had been normal in eight of these patients. CONCLUSIONS: These data clearly illustrate the limited clinical utility of routine angiographic surveillance for CAV in heart transplant recipients and prompted us to abandon this method of surveillance in our unit.

Campylobacter myocarditis; loose bowels and a baggy heart.

We report an unusual case of acute myocarditis associated with Campylobacter jejuni enterocolitis leading to severe impairment of left ventricular systolic function. Contrast-enhanced cardiac magnetic resonance imaging was used to confirm the presence of acute myocardial inflammation and its resolution.

Endothelial dysfunction, subangiographic atheroma, and unstable symptoms in patients with chest pain and normal coronary arteriograms.

BACKGROUND: Patients with chest pain and normal coronary arteriograms (CPNA) may present with unstable symptoms and other evidence of ischemia during clinical follow-up. Although repeat angiography usually proves negative, functional assessment of coronary vasomotor abnormalities may provide additional pathophysiologic information. HYPOTHESIS: The study was undertaken to evaluate the relationship between endothelial dysfunction and subangiographic atheroma in patients with CPNA undergoing repeat angiography because of unstable symptoms. METHODS: We investigated nine patients with CPNA (8 women, mean age 57 +/- 9 years) undergoing repeat angiography because of unstable anginal symptoms. After normal angiography, simultaneous coronary epicardial and microvascular vasomotor responses to intracoronary vasodilators [acetylcholine (10(-6) M), adenosine (18 micrograms) and nitroglycerin (300 micrograms)] were investigated in the left anterior descending artery using quantitative angiography and Doppler flow measurements. The presence of subangiographic atheroma was assessed by intravascular ultrasound. RESULTS: Three patients demonstrated proximal and distal epicardial vasoconstriction and a reduction in coronary flow in response to acetylcholine, indicating concordant epicardial and microvascular endothelial dysfunction. These changes were associated with chest pain and ischemic electrocardiographic changes in two patients. None of the remaining patients suffered chest pain in response to intracoronary acetylcholine. Six patients had significant subangiographic disease (intimal thickness > 0.3 mm) on intravascular ultrasound imaging, and multivariate analysis indicated a significant relationship (R2 = 0.89, overall p = 0.001) between the extent of subangiographic disease and both plasma cholesterol concentration and hypertensive history. No significant relationship was demonstrated between endothelial dysfunction and the extent of subangiographic disease. CONCLUSION: Concordant epicardial and microvascular endothelial dysfunction may be pathophysiologically and clinically significant in unstable patients with CPNA but does not appear to be directly related to the extent of subangiographic atheroma.

Negative stress echocardiographic responses in normotensive and hypertensive patients with angina pectoris, positive exercise stress testing, and normal coronary arteriograms.

OBJECTIVES: To systematically compare the results of dobutamine stress echocardiography in matched groups of hypertensive and normotensive patients with anginal chest pain and normal coronary arteriograms (CPNA). SETTING: University hospital. SUBJECTS: 33 patients with exertional anginal chest pain, a positive exercise stress ECG, and a completely normal coronary arteriogram; 17 had a history of systemic hypertension (14 women; mean (SD) age 57 (6) years), and 16 had no hypertensive history (12 women; age 54 (9) years). METHODS: Ambulatory ECG monitoring, dobutamine stress echocardiography, and thallium-201 single photon emission computed tomography (SPECT) were performed in all subjects. RESULTS: All patients had normal left ventricular systolic function at rest and none fulfilled the criteria for ventricular hypertrophy. Eight normotensive patients and 10 hypertensive patients had perfusion abnormalities on thallium SPECT (p = 0.61). Dobutamine infusion reproduced anginal pain in seven normotensive and seven hypertensive patients (p = 0.88). ST segment depression was also recorded in eight normotensive patients and seven hypertensive patients (p = 0. 61). No patient in either group developed regional wall motion abnormalities during dobutamine stress echocardiography. CONCLUSIONS: Neither hypertensive nor normotensive CPNA patients developed regional wall motion abnormalities during dobutamine stress echocardiography, despite the high prevalence of scintigraphic perfusion defects in both groups and the presence of chest pain and ST segment depression. Thus myocardial ischaemia was not present in either group, or else dobutamine stress echocardiography is insensitive to ischaemia caused by microvascular dysfunction.

Diagnostic value of exercise electrocardiography for predicting a positive scintigraphic test in patients with complete right bundle branch block.

BACKGROUND: The diagnostic value of exercise electrocardiography (ECG) in patients with complete right bundle block (cRBBB) remains controversial. The aim of this study was to investigate the diagnostic accuracy of exercise ECG for predicting ischemia in the presence of cRBBB. METHODS: From a series of 1300 consecutive patients attending for exercise ECG and 99mTc-tetrofosmin single photon emission computed tomography (SPECT), we identified 38 male patients with cRBBB and 38 matched controls with normal intraventricular conduction. Patients with left ventricular hypertrophy or previous revascularization were excluded. Exercise ECG (modified Bruce protocol) was considered positive at > or = 1 mm ST horizontal or downsloping depression in > or = 2 adjacent leads. SPECT imaging at rest and post-exercise was performed at least 48 hours apart. RESULTS: The odds ratio for ischemia comparing patients with positive and negative exercise ECGs was 11.0 (95% confidence interval 2.49-48.64, p = 0.002) in controls and 2.49 (95% confidence interval 0.64-9.08, p = 0.32) in cRBBB. The prior probability of ischemia was 0.37 in controls and 0.58 with cRBBB. The posterior probability after a positive test was 0.65 in controls and 0.68 in cRBBB. Thus, the utility of the test (posterior probability minus prior probability) was greater in controls (+0.28) than in cRBBB (+0.1). This difference was most pronounced in the anterior leads V1-V4 (controls +0.63 vs cRBBB +0.01) but less significant in the lateral leads V5-V6 (controls +0.26 vs cRBBB +0.21). Similar analysis also indicated reduced diagnostic value of negative exercise ECGs in cRBBB patients. CONCLUSIONS: The diagnostic value of exercise ECG is reduced in patients with cRBBB, although ST-segment changes in the lateral ECG territory provide superior predictive accuracy to those in the anterior leads. The use of SPECT as a first-line investigation may be justified in patients with cRBBB.

Elevated endothelin concentrations are associated with reduced coronary vasomotor responses in patients with chest pain and normal coronary arteriograms.

OBJECTIVES: The purpose of this study was to investigate the relationship between arterial and coronary sinus endothelin (ET) concentrations and coronary vasomotor responses during rapid atrial pacing in patients with chest pain and normal coronary arteriograms (CPNA). BACKGROUND: Plasma ET concentrations are significantly higher in CPNA patients than in healthy control subjects. METHODS: We investigated 19 carefully characterized CPNA patients (14 women; mean age 53 +/- 9 years) of whom 10 had positive electrocardiographic responses to exercise. The percentage fall in coronary vascular resistance (%d.CVR) after 10 min of rapid atrial pacing was determined using a thermodilution pacing catheter. Plasma ET concentrations were measured by radioimmunoassay on simultaneously drawn arterial and coronary sinus samples. RESULTS: No significant differences in ET concentrations were observed between men and women, but a strong statistical trend suggested that %d.CVR was lower in women than men (27[23 to 31]% vs. 34[29 to 45]%--median[interquartile range]; p = 0.07). Simple regression analysis including only the women (n = 14) suggested a significant relationship between baseline arterial ET concentrations and %d.CVR (R2 = 0.34; p = 0.06). Furthermore, stepwise multivariate regression analysis of the group as a whole indicated that both gender (p = 0.03) and baseline arterial ET concentration (p = 0.02) were independently predictive of %d.CVR (R2 = 0.44; overall p = 0.02); this relationship predicts that women with high ET levels would have the lowest %d.CVR during pacing. CONCLUSIONS: These data support the hypothesis that elevated ET activity may be associated with reduced coronary flow responses during rapid atrial pacing in CPNA patients.

Differential plasma endothelin levels in subgroups of patients with angina and angiographically normal coronary arteries. Coronary Artery Disease Research Group.

BACKGROUND: Raised plasma endothelin concentrations have previously been reported in patients with cardiac syndrome X, but it is not known whether these levels vary between clinically distinct subgroups in this heterogeneous condition. METHODS AND RESULTS: We compared plasma immunoreactive endothelin levels in 54 patients with angina pectoris and normal coronary angiograms and 21 healthy control subjects. The patient group was divided into 4 clinically distinct subgroups: 7 with left bundle branch block (group A); 7 with previous myocardial infarction (group B); 24 with positive exercise electrocardiography (group C); and 16 with negative exercise electrocardiography (group D). The plasma endothelin concentration was significantly higher in patients compared with control subjects (3.7 [2.9 to 4.3] vs 2.96 [2.4 to 3.4] pg/mL, respectively, median [interquartile range]; P=0.002). Endothelin concentrations were most significantly elevated in group A and group B (4.5 [3.6 to 5.2] pg/mL; P=0.005 and 4.1 [3.9 to 4.5] pg/mL; P=0.001, respectively). Plasma endothelin concentrations were also significantly elevated in group C (3.7 [2.8 to 4.1] pg/mL; P=0.02) but not in group D (3.0 [2.5 to 3.8] pg/mL; P=0.3). CONCLUSIONS: Plasma endothelin concentration is elevated in patients with angina pectoris and angiographically normal coronary arteries, particularly those with left bundle branch block or previous myocardial infarction.

Angiographic progression in patients with angina pectoris and normal or near normal coronary angiograms who are restudied due to unstable symptoms.

BACKGROUND: Syndrome X patients commonly remain symptomatic during follow-up and may be readmitted with unstable anginal symptoms. Angiographic disease progression must be considered as a possible mechanism for instability, particularly where multiple coronary risk factors are present and an interval of several years has elapsed since previous angiography. METHODS AND RESULTS: We reviewed data from 139 consecutive patients with chest pain and normal or near normal coronary angiograms (101 patients with completely normal angiograms and 38 patients with minimal lumenal irregularities). During a 5-year period, 24 patients (19 women, median age 56 years) underwent repeat angiography due to primary unstable angina (median interval between angiograms 58 months (range 8-130 months)). This group included three patients with minimal lumenal irregularities and four patients with left bundle branch block. Only two patients had progression to significant angiographic stenosis (> 30% diameter reduction); both were male patients with minimal irregularities at baseline angiography, left bundle branch block and multiple coronary risk factors. However, overall only two of 18 (11%) patients with one or more conventional coronary risk factors had angiographic progression. CONCLUSIONS: Unstable symptoms in patients with chest pain and previously normal or near normal coronary arteriograms are rarely due to angiographic disease progression. However, the presence of minimal lumenal irregularities at baseline angiography and LBBB may identify a sub-group at increased risk.

Elevated plasma lipoprotein(a) is associated with coronary artery disease in patients with chronic stable angina pectoris.

OBJECTIVES: We sought to assess the relation between plasma lipoprotein(a) [Lp(a)] levels, clinical variables and angiographic coronary artery disease (CAD) in patients with chronic stable angina. BACKGROUND: The relation between plasma Lp(a) levels and the severity and extent of angiographic CAD has not been studied in well characterized patients with stable angina pectoris. METHODS: We investigated clinical variables, lipid variables and angiographic scores in 129 consecutive white patients (43 women) undergoing coronary angiography for chronic stable angina. RESULTS: Plasma Lp(a) levels were significantly higher in patients with than in those without significant angiographic stenoses (> or =70%) (372 mg/liter [interquartile range 87 to 884] vs. 105 mg/liter [interquartile range 56 to 366], respectively, p=0.002). This difference remained significant when patients with mild or severe angiographic disease were compared with those with completely normal coronary arteries (312 mg/liter [interquartile range 64 to 864] vs. 116 mg/liter [interquartile range 63 to 366], respectively, p=0.02). However, subset analysis indicated that this difference achieved statistical significance only in women. Multiple logistic regression analysis indicated that Lp(a) concentration was independently predictive of significant angiographic stenoses (adjusted odds ratio [OR] 9.1, 95% confidence interval [CI] 2.0 to 42.1, p=0.006) and remained true even after exclusion of patients receiving lipid-lowering treatment (n=27) (OR 10.4, 95% CI 1.1 to 102.9, p=0.05). Lp(a) also had independent predictive value in a similar analysis using mild or severe angiographic disease as the outcome variable (OR 11.8, 95% CI 1.5 to 90.8, p=0.02). CONCLUSIONS: Our results indicate that elevated plasma Lp(a) is an independent risk factor for angiographic CAD in chronic stable angina and may have particular significance in women.

Low dose imipramine improves chest pain but not quality of life in patients with angina and normal coronary angiograms.

AIMS: We investigated patients with chest pain and normal coronary angiograms to determine whether low dose imipramine prescribed as add-on therapy to conventional anti-anginals reduced the incidence of chest pain and whether this led to an overall improvement in quality of life. METHODS AND RESULTS: We performed a randomized, double-blind, cross-over trial of imipramine 50 mg daily vs placebo in 18 women (median age 53 years; range 35-72) with chest pain and normal coronary angiograms who were suffering at least two anginal episodes per week despite conventional anti-anginal medication. Each treatment phase lasted 5 weeks and the incidences of chest pain and side effects were carefully recorded. Quality of life was monitored using a validated health profile questionnaire scoring perceived distress in six domains (pain, energy, mobility, sleep, emotional reactions and social isolation). The total number of chest pain episodes was significantly less during active treatment compared to placebo [11 (3-22) vs 21 (16-28)--median (interquartile range); P = 0.01]. However, a high incidence (83%) of side effects was reported during active treatment and three patients had to be withdrawn from the study as a consequence. No significant improvement was detected in any of the six quality of life domains when imipramine was compared to placebo. CONCLUSION: Imipramine reduces the incidence of chest pain in patients with chest pain and normal coronaries who remain symptomatic despite conventional anti-anginal therapy. The failure to demonstrate associated improvements in quality of life may have been due to the high incidence of side effects.

Serum endothelin levels and pain perception in patients with cardiac syndrome X and in healthy controls.

The possible algogenic effects of elevated serum endothelin levels in cardiac syndrome X were investigated in a case-control study that examined somatic pain perception in the forearm during submaximal effort tourniquet and cold immersion tests. Pain threshold to both ischemic and cold stimulation of the forearm was demonstrated to be significantly lower in patients with syndrome X than in matched healthy controls, and a negative correlation between ischemic pain threshold and endothelin levels was demonstrated.

Significance of tomographic signs in the diagnosis of bronchial carcinoma.

In a previous study the value of conventional tomography was assessed in the diagnosis of 100 potentially malignant opacities on the chest radiograph. To determine which of the radiological signs were most useful the radiologists reviewed 82 of the original 100 radiographs independently, searching for the presence or absence of 36 signs. The five commonest signs of bronchial carcinoma were a mass, coarse linear shadows contiguous to a mass, unilateral hilar enlargement, linear shadows from mass to periphery, and an irregular margin to a mass. The combination of either two or three of these signs was highly sensitive, 95% and 89% respectively, in detecting carcinoma. The most useful specific signs were lobulation of the mass and cavitation with thick or irregular walls.