Anomalous Coronary Artery Origin in a Young Patient with Marfan Syndrome.

Marfan syndrome is an autosomal dominant genetic disorder that affects connective tissue and is caused by mutations in the fibrillin 1 gene present at chromosome 15. Aortic aneurysm is its main complication, and along the dilation of the aorta root and its descending portion (60-100%), with secondary aortic insufficiency, it increases risk of acute aortic dissection and death. Coronary artery anomalies affect between 0.3% and 1.6% of the general population and are the second leading cause of sudden death in young adults, especially if the anomalous coronary passes through aorta and pulmonary artery. The anomalous origin of the left main coronary artery in the right Valsalva sinus has a prevalence of 0.02%-0.05% and is commonly related to other congenital cardiac anomalies, such as transposition of great vessels, coronary fistulas, bicuspid aortic valve, and tetralogy of Fallot. Its association with Marfan syndrome is not known, and there is no previous report in the literature. We describe here a case of a female with Marfan syndrome diagnosed with symptomatic anomalous origin of the left coronary artery in the right Valsalva sinus.

Angiotensin-converting enzyme and apolipoprotein B polymorphisms in coronary artery disease.

The association between angiotensin-converting enzyme (ACE) as well as apolipoprotein B polymorphisms and dyslipidemia and coronary artery disease (CAD) is controversial. We assessed the distribution of ACE insertion and/or deletion, apolipoprotein B signal peptide insertion and/or deletion, and apolipoprotein B XbaI restriction fragment length polymorphisms in 388 nondiabetic patients. We studied 112 patients with angiographically defined asymptomatic CAD or with stable functional classes I and II angina and 139 patients with acute myocardial infarction who were age matched to 137 control subjects. Univariate analysis showed higher prevalence of Xba50% reduction of lumen diameter. Overall, multivariable regression disclosed traditional risk factors and elevated levels of apolipoprotein B for men and reduced levels of apolipoprotein AI for women as independent variables for CAD. After adjustment for the most important subset of risk factors (age, hypertension, hypercholesterolemia, and smoking), apolipoprotein B XbaI polymorphism was disclosed as an independent variable for CAD. Apolipoprotein B XbaI was also selected as an independent variable for acute myocardial infarction after adjusting for age, hypertension, hypercholesterolemia, and smoking. Thus, in addition to traditional coronary risk factors, apolipoproteins B and AI, and apolipoprotein B XbaI polymorphism could be considered predictors of CAD.

Effect of pravastatin on plasma removal of a chylomicron-like emulsion in men with coronary artery disease.

The speed of the plasma removal of chylomicrons, the lipoproteins that carry dietary lipids absorbed in the intestine, may influence atherogenesis. Thus, the effects of a 30-day pravastatin or placebo treatment on the plasma kinetics of chylomicron-like emulsions were evaluated in 25 patients with coronary artery disease who were not hypertriglyceridemic in a randomized, single-blinded study. Eleven patients (53 +/- 4 years, 10 men) received pravastatin 40 mg/day and 14 received placebo (52 +/- 3 years, 13 men). Emulsions labeled with triolein ((3)H-TO) and cholesteryl oleate ((14)C-CO) to assess lipolysis and clearance of chylomicron and remnants, respectively, were injected intravenously in a bolus after a 12-hour fast. Blood samples were collected during 60 minutes to determine radio isotope decaying curves and fractional catabolic rates. Subjects were studied at baseline and after the treatment period. Compared with placebo (data expressed as mean +/- SEM), pravastatin treatment increased the (14)C-CO fractional catabolic rates (70 +/- 45% vs 18 +/- 10%, p = 0.01), reduced total cholesterol (-21 +/- 3% vs -3 +/- 2% p = 0.0001), low-density lipoprotein (LDL) cholesterol (-25 +/- 5% vs 4 +/- 6%, p = 0.0001), and apolipoprotein B levels (-22 +/- 3% vs -7 +/- 3% p = 0.01). (3)H-TO fractional catabolic rates, plasma triglycerides, very-low-density lipoprotein (VLDL) cholesterol and high-density lipoprotein (HDL) cholesterol variations did not differ between the groups. The fractional catabolic rate of (14)C-CO was inversely correlated with plasma apolipoprotein B levels (r = -0.7, p = 0.04). This suggests that besides reducing LDL cholesterol, pravastatin also increases chylomicron remnant clearance, with possible antiatherogenic implications.

Cholesterol lowering with statins reduces exercise-induced myocardial ischemia in hypercholesterolemic patients with coronary artery disease.

Coronary flow reserve is mainly influenced by the combination of luminal stenosis and vascular dilation capacity. Thus, after statin treatment, the reduction of ischemic threshold in patients submitted to exercise testing could be intensely influenced by angiographic severity. In this study, we verify the effect of statin treatment on exercise-induced myocardial ischemia in hypercholesterolemic patients with a broad range of coronary angiographic severities. Patients with 2 consecutive positive exercise tests, coronary stenosis > or =70%, total cholesterol > or =300 mg/dl, and triglycerides < or =200 mg/dl were randomly assigned to a 16-week treatment period with either diet alone (n = 39) or diet plus statins (simavastatin, n = 31 and pravastatin, n = 10). Statin-treated patients had a significant variation in total cholesterol (-46% vs -2.7%; p <0.01), low-density lipoprotein cholesterol (-58% vs 0.8%; p <0.01), and high-density cholesterol (+28% vs -6%; p <0.05) in comparison with the diet-only group. After 16 weeks of treatment, 36 patients (92%) in the diet group still had positive exercise tests, whereas only 7 patients (15%) of the statin group had a positive test (p <0.01). The proportion of positive tests was significantly reduced in subgroups of patients with 1-, 2-, or 3-vessel disease. Regarding the severity of coronary stenosis, the proportion of positive tests was significantly reduced in patients with stenosis between 70% and 90% and in patients with stenosis > or =90%. Moreover, the proportion of positive tests tended to decrease to a greater extent in patients with mild coronary disease. In conclusion, cholesterol-lowering treatment with statins reduces exercise-induced myocardial ischemia in hypercholesterolemic patients with mild or severe epicardial coronary stenosis.

Mortality is not increased with inferior infarction associated with right ventricular infarction and atrioventricular block.

BACKGROUND: Little information is available about the relation between right ventricular infarction (RVI) and the presence of atrioventricular block. Thus, the objective of this study was to analyse a possible correlation between the conditions, which are frequently associated with inferior acute myocardial infarction (AMI). METHODS: We studied 107 patients with inferior AMI, of whom 49 had RVI, assessed by ST-segment elevation in right precordial leads (V3R-V4R) and by technetium-99m (99mTc) pyrophosphate scintigraphy. The diagnosis of atrioventricular block was made by continuous ECG monitoring during the first week of admission. RESULTS: The patients were divided into two groups: group A with RVI and group B with isolated inferior AMI. These groups were similar regarding sex, age, coronary risk factors, and time from the onset of precordial pain to hospital admission. Group A had a predominance of atrioventricular block (61.2 versus 15.5%, P < 0.0004), peak creatine kinase MB (82.5 +/- 22.4 versus 65.2 +/- 25.1 IU/l, P < 0.05), congestive heart failure or cardiogenic shock (57.1 versus 18.9%, P < 0.002), and proximal right coronary artery occlusion (80.4 versus 25.0%, P < 0.001). Non-Q-wave infarction was more frequent in group B patients (14.2 versus 34.4%, P < 0.01). The mortality rate was similar in the two groups (12.2 versus 13.7%). CONCLUSIONS: These data suggest that infarction extension from inferior wall to the right ventricle may be related to the development of atrioventricular block and does not increase mortality.

Sinus bradycardia as a predictor of right coronary artery occlusion in patients with inferior myocardial infarction.

Differentiation of right coronary artery (RCA) from left circumflex artery (LCxA) occlusion may be difficult since both can present an electrocardiographic pattern of inferior myocardial infarction (IMI). We studied 133 patients with IMI, 92 patients with RCA occlusion and 41 patients with LCxA occlusion. Risk factors such as previous MI, arterial hypertension, diabetes, smoking, and dislipemia, were similar for RCA and LCxA occlusions. Patients with RCA occlusion had a higher incidence of isolated IMI than patients with LCxA occlusion, 50% vs. 17%, respectively (P<0.001). Arterial hypotension was more prevalent (P<0.05) among patients with RCA (18%) rather than those with LCxA occlusion (2%). RCA occlusion presented an association with sinus bradycardia, an association not observed with LCxA occlusion (15% vs. 0%, respectively; P<0.01). Total atrioventricular block was only present among patients with RCA (18%). Proximal occlusions of the RCA presented lower heart rates (sinus bradycardia) than medial and distal occlusions (13% vs. 1% and 1%, respectively; P<0.0001 and P<0.001). Therefore, regarding patients with IMI: (1) sinus bradycardia is more frequent when the infarct-related artery is the RCA; (2) proximal occlusions of the right coronary predispose low heart rates; and (3) occlusion of the LCxA rarely induces sinus bradycardia.

Clustering of traditional risk factors and precocity of coronary disease in women.

BACKGROUND: Women usually develop coronary artery disease (CAD) 10 years later than men do. CAD in women is associated with menopausal status and the number and intensity of risk factors. But, when the age gap between men and women narrows, less is known about the influence of risk factors on CAD. METHODS: We assessed the prevalence of traditional risk factors in 850 men and 468 women with stable CAD who had mean age, 58.3+/-8.6 and 58.8+/-10.3 years (P=NS), respectively. RESULTS: Univariate analysis of risk factors showed that body mass index (BMI), hypertension (all three stages), diabetes, triglycerides (> or =2.8 mmol/l), cholesterol (> or =6.2 mmol/l) and family history were more prevalent in women. Smoking and previous myocardial infarction (MI) were more prevalent in men. Multivariable analysis disclosed hypertension, diabetes, dyslipidemia and family history as independent risk factors for women with stable CAD and smoking and previous MI as independent risk factors for men. CONCLUSION: Clustering of traditional risk factors may explain the precocity of CAD in women who are near in age to men.

Acute myocardial infarction after simultaneous thrombosis in normal right and left coronary arteries.

A 32-year-old male patient with clinical and electrocardiographic evidence of acute myocardial infarction underwent coronary angiographic study. We observed nonocclusive thrombosis simultaneously in right and left anterior descending coronary arteries, without confirmation of spasm or obstructive artery disease in other coronary branches. Documentation of coronary thrombosis in more than one artery is rare, and its pathophysiology is still unknown. With the advent of thrombolytic therapy and immediate coronary angiographic studies in patients with evolving myocardial infarction, it has been possible to confirm the presence of thrombus and the type of coronary disease. In this case, we observed total lysis of both thrombi and the final aspect of "normal" angiographically reperfused coronary arteries.

Prognostic significance of right ventricular dysfunction in patients with acute inferior myocardial infarction and right ventricular involvement.

Little is known about the influence of right ventricular (RV) dysfunction on prognosis of patients with acute inferior myocardial infarction (IMI) and RV involvement. Therefore, 99 consecutive patients (mean age 56.6 +/- 3.4 years) with RV involvement during acute IMI were followed for a 12-month period to clarify the influence of acute RV dysfunction on short- and long-term survivals. Forty-one patients with IMI evolved with severe arterial hypotension due to RV dysfunction, while 58 patients had no hemodynamic impairment due to RV involvement. Basal hemodynamic data (mean +/- SD) for patients with RV dysfunction were blood pressure (BP) 92/59 +/- 22/20 mmHg, systemic vascular resistance (SVR) 2314 +/- 252 dynes.s.cm-5, and cardiac index (CI) 1.3 +/- 0.3 l/min/m2. Patients without RV dysfunction demonstrated BP 113/74 +/- 20/16 mmHg (p < or = 0.05), SVR 1324 +/- 354 dynes.s.cm-5 (p < or = 0.01), and CI 2.6 +/- 0.5 l/min/m2 (p < or = 0.05). Angiographic differences noted were that hemodynamically compromised patients showed lower RV ejection fractions (0.27 +/- 0.08) than patients without hemodynamic disturbance [0.41 +/- 0.11 (p < or = 0.05)]; however, left ventricular ejection fractions were 0.48 +/- 0.10 and 0.52 +/- 0.12, respectively. Short- and long-term mortality rates were assessed during the follow-up period. Patients with hemodynamic impairment due to RV infarction had a higher mortality rate for the first month and for 11 subsequent months post MI than patients without hemodynamic impairment, that is 24.4 vs. 6.9 and 14.6 (p

Angiotensin-converting enzyme and apolipoproteins genes polymorphism in coronary artery disease.

BACKGROUND: Association between angiotensin-converting enzyme (ACE) as well as apolipoprotein (apo) AI, B, and E polymorphisms and dyslipidemia and coronary artery disease (CAD) is controversial. HYPOTHESIS: This study assessed the distribution of ACE insertion/deletion, apo AI A/G mutation, apo B signal peptide insertion/deletion, apo B XbaI restriction fragment length, and apo E polymorphisms in 388 nondiabetic patients. METHODS: The study population included 112 patients with stable CAD, 139 patients with acute myocardial infarction (AMI), and 137 age-matched control subjects. RESULTS: Univariate analysis showed higher prevalence of XbaI X+/X+ genotype in patients with CAD (p = 0.02). Angiotensin-converting enzyme and apo polymorphisms were not associated with lipid levels or severity of CAD. When all genotypes known to be related to CAD; such as ACE DD, apo AI GG, apo B del/del, and XbaI X+X+, and E4 allele of apo E, were pooled, again no significant differences among groups were seen. Multivariate regression analysis disclosed traditional risk factors and elevated levels of apo B for men and reduced levels of apo AI for women as independent variables for CAD. CONCLUSIONS: In addition to traditional coronary risk factors, apo B and AI could be considered predictors of CAD. No association between either form of CAD and polymorphisms was noted.

Temperature, air pollution, and mortality from myocardial infarction in São Paulo, Brazil.

An increase in daily mortality from myocardial infarction has been observed in association with meteorological factors and air pollution in several cities in the world, mainly in the northern hemisphere. The objective of the present study was to analyze the independent effects of environmental variables on daily counts of death from myocardial infarction in a subtropical region in South America. We used the robust Poisson regression to investigate associations between weather (temperature, humidity and barometric pressure), air pollution (sulfur dioxide, carbon monoxide, and inhalable particulate), and the daily death counts attributed to myocardial infarction in the city of São Paulo in Brazil, where 12,007 fatal events were observed from 1996 to 1998. The model was adjusted in a linear fashion for relative humidity and day-of-week, while nonparametric smoothing factors were used for seasonal trend and temperature. We found a significant association of daily temperature with deaths due to myocardial infarction (P < 0.001), with the lowest mortality being observed at temperatures between 21.6 and 22.6 degrees C. Relative humidity appeared to exert a protective effect. Sulfur dioxide concentrations correlated linearly with myocardial infarction deaths, increasing the number of fatal events by 3.4% (relative risk of 1.03; 95% confidence interval = 1.02-1.05) for each 10 microg/m(3) increase. In conclusion, this study provides evidence of important associations between daily temperature and air pollution and mortality from myocardial infarction in a subtropical region, even after a comprehensive control for confounding factors.

Beneficial effect of long-term use of a beta-blocker in patients before acute myocardial infarction.

The outcome of 38 beta-blocker users (group BB, 28 men and 10 women with a mean age of 56 +/- 4 years) was compared to that of 100 non-users (group NU, 69 men and 31 women with a mean age of 57 +/- 8 years) after acute myocardial infarction (AMI). The two groups were compared in terms of electrocardiographic (EKG) location of the AMI (anterior, inferior and lateral), EKG Q and non-Q wave infarction, clinical functional class of Forrester, serum creatine phosphokinase MB fraction (CKMB) peak release and intrahospital mortality. There were no differences between groups concerning sex or severity of coronary artery disease but arterial hypertension was 2-fold more prevalent in group BB. The EKG location of the AMI was similar in the two groups. Non-Q infarction was significantly more prevalent in group BB (37%) than in group NU (6%). The incidence of clinical functional class IV of Forrester and the serum CKMB peaks were significantly lower in group BB (2.6% vs 16.0% and 53 +/- 3 vs 68 +/- 9 IU/l, respectively). Intrahospital mortality was also significantly lower in group BB (2.6%) than in group NU (10%). These data suggest the beneficial effect of previous long-term use of beta-blockers as indicated by a lower incidence of cardiogenic shock and a significant decrease in intrahospital mortality after AMI.

Slow coronary run-off in patients with angina pectoris: clinical significance and thallium-201 scintigraphic study.

To determine whether or not slow coronary flow (SF) depends on hemodynamic variables, we studied 17 patients (15 men, mean age = 47.8 years) with SF at coronariography. Exercise thallium-201 myocardial scintigraphy revealed perfusion abnormalities in 13 (76.4%) patients. We then selected 89 individuals submitted to cinecoronariography for comparison: 15 were normal and 74 had heart disease. The coronary flow velocity was evaluated by the number of heart beats (HB) needed for coronary artery dye filling. The patients in the SF group had normal hemodynamic variables which were significantly different from those of patients with heart disease (P = 0.001). Patients with heart disease needed no more than 4 HB to fill their arteries, in contrast to 6.88 +/- 1.68 (5 to 11) in the SF group (P < 0.0001). Thus, in our patients with myocardial scintigraphy suggesting ischemia, SF was found to be an event which did not depend on hemodynamic factors.

[Multiple myocardial coronary bridges]. / Pontes miocárdicas múltiplas.

White woman, 31 years old, complaint pain in the left arm, at rest, and few months later she had pain in the precordial region with dyspnea, diaphoresis and dizziness without any inducing factor. One hour later, symptoms ceased spontaneously, remaining daily precordial pain. Subsidiary examinations as cineangiocoronariography revealed 100% of arterial lumen constriction in the middle portion of the descending coronary artery, during systole, as well as in the diagnoalis and marginal branches. Multiple myocardial coronary bridges has been described in necropsy, but during cinecoronarioangiography, this case appeared to be the first one.

[Fatal acute myocardial infarction in a young cocaine addict]. / Infarto agudo do miocárdio fatal em jovem viciado em cocaína.

A 28 year-old male cocaine abuser without coronary atherosclerosis suffered fatal myocardial infarction. Necropsy revealed several myocardial infarctions of different ages. Examination of the coronary arteries revealed fibrointimal thickening of the intimal layer of the coronary arteries.

[Rupture of the interventricular septum after acute myocardial infarction in the absence of typical symptoms and electrocardiographic changes. A case report]. / Rotura do septo interventricular pós-infarto agudo do miocárdio na ausência de sintomas típicos e de alterações electrocardiográficas. Relato de caso.

A 79 year old man with sudden dyspnea, syncope and third degree heart block underwent a pacemaker (PM) implantation. He persisted with these symptoms and on the third day after the procedure it was detected a precordial holosystolic murmur. A ventricular septal rupture consequence of PM implantation was suspected. The coronary-ventriculography revealed a 99% stenosis in right coronary artery, inferior myocardial infarction and an interventricular communication. Previously, there were no other clinical manifestations nor electrocardiographic alterations suggestive of myocardial ischemia or infarction in this patient. We discuss about the rare association of an unrecognized acute myocardial infarction complicated by ventricular septal rupture.

[Thrombosis in normal coronary arteries causing acute myocardial infarction in an alcoholic patient]. / Trombose em artérias coronárias normais causando infarto agudo do miocárdio em paciente alcoólatra.

A case of a young patient, heavy drinker, who suffered an acute myocardial infarction (AMI), and whose cinecoronariography, early post unsuccessful intravenous thrombolysis, showed the presence of thrombi in multiple arteries is reported. A second cinecoronariography performed on the 7th day post AMI revealed normal coronary arteries. There were no plasmatic lipid or coagulation abnormalities, nor other risk factors for coronary artery disease. We speculate about the role of the heavy alcohol ingestion in this particular case.

Does a circadian variation occur in myocardial ischemia over 48 hours in patients with unstable angina?

OBJECTIVE: To study the incidence of and variation in myocardial ischemia over 48 hours in patients with unstable angina. METHODS: Thirty-nine patients with unstable angina underwent long-term electrocardiography for 48 hours. The number of events and the period of time of ischemia (in minutes) were analyzed for the 48 hours, in two periods of 24 hours, and in periods of 4 hours. RESULTS: We analyzed 1755.8 hours of monitoring tapes, and ischemic episodes were detected in 18 (46.2%) patients, corresponding to 173 ischemic episodes, allowing the evaluation of 1304 minutes of ischemia.only 4 of which were (2.2%) symptomatic, Considering the entire period of time of recording and the predetermined time intervals, we observed a higher number of ischemic episodes (38) and a longer duration of ischemia (315.4 minutes) between 11:00 am and 3:00 pm. However, no significant differences occurred among the values in the different intervals. CONCLUSION: Long-term electrocardiography over 48 hours showed a high incidence (97.8%) of silent ischemic episodes in patients with unstable angina. No evidence of a circadian variation of myocardial ischemia in unstable angina was observed.

[Effect of treatment with chlorthalidone on reduction of left ventricular mass in patients with systemic arterial hypertension]. / Efeito do tratamento com clortalidona na redução da massa ventricular esquerda de pacientes com hipertensão arterial sistêmica.

PURPOSE: To evaluate the effects of the use of chlorthalidone on left ventricular mass of patients with mild and moderate systemic arterial hypertension (SAH). METHODS: Twenty-nine patients with mild and moderate SAH were studied with mean age of 48.4 years and 16 men. Clinical evaluation, systolic (SBP) and diastolic (DBP) blood pressure and heart rate (HR), in supine and standing positions, were obtained before and every 3 weeks, after 2 with placebo, during 12 weeks of treatment with 50mg of chlorthalidone each 48 hours. Laboratory data (hemogram, sodium, potassium, urea creatinine, glucose, hepatic aminotransferases and urinalysis) were done before and at end of study. Echocardiographic study was performed by M-mode before and after 6 and 12 weeks of treatment, and obtained the following data: diastolic diameter (DdLV), diastolic septum (DSTLV) and posterior wall thickness (PWTLV) of left ventricle. The left ventricular mass (LVM) was calculated by the formula: LVM = (DSTLV+PWTLV+DdLVE)3 - (DdLV)3 x 1.05/body surface, in g/m2. RESULTS: A significant reduction of SBP (p < 0.0001), DBP (p < 0.001) in supine and standing positions. HR did not show statistical difference. At echocardiographic study, M-mode, was observed a significant reduction of LVM after 12 weeks of treatment (181 +/- 67 to 156 +/- 34 g/m2, p < 0.01). CONCLUSION: Chlorthalidone was effective to control blood pressure and to reduce LVM of patients with SAH.

[Effect of ticlopidine and dipyridamole on platelet aggregation and count in patients with chronic stable angina pectoris]. / Efeito da ticlopidina e do dipiridamol sobre a agregação e contagem das plaquetas de pacientes com angina crônica estável.

PURPOSE: To compare the ticlopidine and dipyridamole effects on platelets count and aggregation in patients with stable coronary artery disease. PATIENTS AND METHODS: Eighty patients with stable coronary artery disease and mean of 58.3 +/- 5.8 years were studied. They were divided into two equal groups of 40 patients and each one treated with ticlopidine or dipyridamole. Platelets count and aggregation were examined before treatment and at first and fourth weeks of treatment. RESULTS: At the end of fourth week of treatment, spontaneous, induced by ADP or by adrenalin platelet aggregation inhibition was observed, respectively, in 82.5%, 72.5% e 67.5% of the patients in ticlopidine group. The spontaneous, induced by ADP or by adrenalin, platelet aggregation inhibition in the patients of dipyridamole group was, respectively, 40%, 30% e 27.5% (p less than 0.001). The platelets count did not change in both groups. CONCLUSION: The ticlopidine effect is much more evident in platelet aggregation inhibition than dipyridamole, and maybe a choice in the prevention of cardiovascular events.