Coronary Artery and Pulmonary Artery Fistula: Rare Congenital Coronary Artery Fistula.

Coronary artery fistula is a rare condition that can be congenital or acquired. Most patients with these anomalies remain asymptomatic, but symptoms and complications may develop with increasing age. We discuss the clinical presentation, diagnostic work-up, and management options for this condition. (Level of Difficulty: Intermediate.).

A Case of Overlooked Popliteal Artery Entrapment Syndrome.

Popliteal artery entrapment syndrome (PAES) is an uncommon cause of lower extremity claudication that is often overlooked. It most commonly occurs in young athletes without risk factors for peripheral vascular disease. We present a case of a 47-year-old man who went undiagnosed for over 10 years despite multiple orthopedic, chiropractic, and neurosurgery consults. A definitive diagnosis of PAES was confirmed in the catheterization lab by angiography. The patient underwent popliteal artery bypass surgery and his symptoms completely resolved. PAES must be considered in the differential diagnosis of lower extremity pain, especially in younger patients.

Aortic stenosis and angiodysplastic gastrointestinal bleeding: Heyde's disease.

Obscure gastrointestinal (GI) bleeding can be a perplexing and difficult problem in elderly patients, especially if they are hemodynamically unstable. If aortic stenosis is also present, the cause of the GI bleeding may be explained. We present a 66-year-old man with a medical history of coronary artery disease who presented with acute GI bleeding. During his hospital course, the patient had a colonoscopy showing diffuse angiodysplasia and an echocardiogram showing severe aortic stenosis. This combination of angiodysplasia and aortic stenosis is known as Heyde's syndrome. It has been hypothesized that the aortic stenosis causes an acquired von Willebrand factor deficiency that leads to GI bleeding. Aortic valve replacement, when possible, can prevent recurrent GI bleeding in these cases, but medical decisions in these cases are complex and difficult.

Conductive energy devices: a review of use and deaths in the United States.

BACKGROUND: Conductive energy devices (CEDs) have been temporally associated with morbidity and mortality in police work, but the frequency of use and of complications is not certain. METHODS: This is a literature review using PubMed and Google Scholar searches to identify population-based CED studies, studies reporting morbidity and mortality with CEDs, and studies in healthy volunteers. RESULTS: Recent studies indicate that CEDs are used approximately 83 to 338 times per million population per year in the United States. The subjects have a typical profile, including young men with belligerent or bizarre behavior who often have a psychiatric disorder or are intoxicated with drugs. The mortality estimates range from 0.0% to 1.4% of subjects controlled with CEDs. Limited information from autopsy studies indicates that death is frequently associated with confounding factors, especially intoxication with illicit drugs. CONCLUSIONS: Conductive energy devices are used frequently during police work and are associated with a low but definite mortality rate. The use of CEDs and the management of at-risk subjects need more study.

Rosuvastatin therapy does not affect serum MMP-13 or TIMP-1 levels in hypercholesterolemic patients.

Matrix metalloproteinases degrade the collagen content of atherosclerotic plaque and reduce plaque stability. In tissue sections of atherosclerotic plaque, the expression of matrix metalloproteinases is increased. 3-Hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins) decrease the tissue expression of matrix metalloproteinases-1, -2, -3, and -9 in atheromatous plaque by attenuating the inflammatory process that leads to increased expression. However, it is not known whether statins decrease levels of matrix metalloproteinase-13--an enzyme crucial to the initiation of collagen degradation-as part of their plaque-stabilizing effect.We prospectively examined the effect of statin therapy on serum levels of matrix metalloproteinase-13, tissue inhibitor of metalloproteinase-1, and low-density-lipoprotein cholesterol in 14 patients with hypercholesterolemia. All were at low risk for adverse cardiovascular events and were given 20 mg/d of rosuvastatin for 4 weeks. Post-therapy levels of matrix metalloproteinase-13 and tissue inhibitor of metalloproteinase-1 were compared with baseline levels. Although low-density-lipoprotein cholesterol levels were significantly decreased in the 14 patients (mean baseline level, 152 ± 21 mg/dL vs mean post-therapy level, 73 ± 45 mg/dL; P < 0.001), matrix metalloproteinase-13 and tissue inhibitor of metalloproteinase-1 levels were unchanged (matrix metalloproteinase-13, 0.295 ± 0.06 ng/mL vs 0.323 ± 0.11 ng/mL, P = 0.12; and tissue inhibitor of metalloproteinase-1, 400.8 ± 43.4 ng/mL vs 395.3 ± 47.5 ng/mL, P = 0.26). We conclude that even though there was a decrease in low-density-lipoprotein cholesterol, short-term, high-dose rosuvastatin therapy has no effect on matrix metalloproteinase-13 and tissue inhibitor of metalloproteinase-1 levels in hypercholesterolemic patients. However, further investigation is warranted.

A common electrocardiographic finding in an uncommon presentation.

Anterior ST-segment elevation is the hallmark electrocardiographic finding of acute anterior left ventricular infarction that is caused by occlusion in one of the branches of the left coronary artery. We report a case of marked ST-segment elevation in the precordial leads, with concomitant inferior ST-segment elevation that was caused by acute occlusion of the marginal branches of the right coronary artery (RCA) causing an isolated right ventricular myocardial infarction (RVMI) during coronary angioplasty. Isolated occlusion of the marginal branches of the RCA appears to be extremely rare. In the case presented, this was caused by an iatrogenic spiral dissection with subsequent stenting of the RCA. This case illustrates that diffuse ST-segment elevation in the precordial leads may occur due to the occlusion of the RCA or its branches. It is important to recognize this scenario, since the treatment of left ventricular myocardial infarction differs from that of RVMI, where maintaining adequate preload and avoiding vasodilators to preserve right ventricular stroke volume is crucial.

Excited delirium, restraints, and unexpected death: a review of pathogenesis.

Unexpected deaths periodically occur in individuals held in police custody. These decedents usually have had significant physical exertion associated with violent and/or bizarre behavior, have been restrained by the police, and often have drug intoxication. Autopsy material from these cases may not provide a satisfactory explanation for the cause of death, and these deaths are then attributed to the excited delirium syndrome. The pathogenesis of excited delirium deaths is likely multifactorial and includes positional asphyxia, hyperthermia, drug toxicity, and/or catecholamine-induced fatal arrhythmias. We suggest that these deaths are secondary to stress cardiomyopathy similar to the cardiomyopathy seen in older women following either mental or physical stress. This syndrome develops secondary to the toxic effects of high levels of catecholamines on either cardiac myocytes or on the coronary microvasculature. Patients with stress cardiomyopathy have unique ventricular morphology on echocardiograms and left ventricular angiography and have had normal coronary angiograms. People who die under unusual circumstances associated with high catecholamine levels have contraction bands in their myocardium. Consequently, the pathogenesis of the excited delirium syndrome could be evaluated by using echocardiograms in patients brought to the emergency centers, and by more careful assessment of the myocardium and coronary vessels at autopsy. Treatment should focus on prevention through the reduction of stress.

Gender differences in platelet aggregation in healthy individuals.

This study evaluated gender variability in platelet aggregation in response to common agonists. Platelet aggregation was measured in 36 healthy men and women free of any antiplatelet medication, aged 22-36 years, of Caucasian (White not of Hispanic origin), Hispanic, and African-American not of Hispanic origin. In this ex-vivo study, we investigated platelet aggregation in response to adenosine-5'-diphosphate (ADP), epinephrine (EPI), arachidonic acid (AA) and collagen (COL), using a platelet ionized calcium aggregometer (Chrono-Log Co.). Platelet aggregation response to all tested agonists was higher in females than in males regardless of ethnicity. The most significant differences were observed with collagen (P < 0.01). Among the ethnic groups, Caucasian women were most prone to platelet aggregation. Gender is a determinant of agonist effects on platelet aggregability in healthy subjects.

Left circumflex coronary artery to hepatic vein fistula: a case report and brief review of coronary vasculogenesis.

Coronary artery fistulas are rare coronary artery anomalies. Their clinical significance varies from a long asymptomatic course to overt heart failure and death. They are often detected incidentally with diagnostic coronary angiograms. Cardiologists increasingly encounter coronary artery fistulas secondary to recent improvements in cardiovascular imaging modalities. Management is still controversial, especially in asymptomatic cases with less significant shunts. Here, we describe a 62-year-old woman with a left circumflex artery to hepatic vein fistula found on coronary angiography. The patient is being managed conservatively using nuclear imaging studies and echocardiographic evaluation.

Papillary muscle dyssynchrony as a cause of functional mitral regurgitation in non-ischemic dilated cardiomyopathy patients with narrow QRS complexes.

OBJECTIVE: Mitral regurgitation (MR) increases mortality in dilated cardiomyopathy (DCM). We investigated the prevalence of functional MR in non-ischemic DCM patients with narrow QRS intervals and its association with papillary muscle dyssynchrony. METHODS: Ninety-three patients were enrolled consecutively in this cross-sectional study. Patients were evaluated for the presence of intraventricular (DYS Sep-Lat Sys) and papillary muscle (DYS Inter PAP Sys) systolic dyssynchrony using tissue Doppler echocardiographic imaging (TDI). Two-dimensional and Doppler echocardiography were used for quantification of MR. Statistical analyses were performed using unpaired t test, Mann-Whitney U test, correlation and logistic regression analyses. RESULTS: Thirty-seven patients (39%) had significant DYS Sep-Lat Sys and 25 patients (26%) had DYS Inter PAP Sys. Patients with DYS Inter PAP Sys had lower basal septum systolic (p=0.007) and late diastolic velocities (p=0.049), greater MR volume (p=0.01), effective regurgitant orifice (ERO) (p=0.01), and E/A ratios (p=0.03) than the patients without DYS Inter PAP Sys. Fifty-five patients with narrow QRS intervals were also evaluated for DYS Inter PAP Sys. Patients with DYS Inter PAP Sys and narrow QRS had lower basal septum TDI peak systolic velocities (p=0.038), higher MR volume (p=0.03) and ERO (p=0.03). Logistic regression analysis revealed that NYHA Class III-IV (OR=6.4, 95% CI: 1.1-37.1, p=0.038) and DYS Inter PAP Sys (OR=9.5, 95% CI: 1.17-75.78, p=0.034) were the independent predictors of functional MR >20 ml. CONCLUSION: Papillary muscle systolic dyssynchrony is common and correlated with functional MR in non-ischemic DCM patients with sinus rhythm and narrow QRS. Papillary muscle systolic dyssynchrony may help predict patients who will benefit from cardiac resynchronization therapy.

Mitral regurgitation reduces systemic coagulation activity in patients with rheumatic heart disease.

BACKGROUND AND AIM OF THE STUDY: Severe mitral regurgitation (MR) reduces left atrial thrombus formation in patients with mitral stenosis (MS) and atrial fibrillation (AF). Plasma D-dimer levels represent a biochemical marker for fibrinolytic activity in prothrombotic states. The prothrombotic burden in patients with mitral valve disease and/or AF was assessed using plasma D-dimer levels. METHODS: The study population included 89 patients with mitral valve disease, 21 with AF but normal valves, and 15 healthy controls. The mitral valve group was subdivided into patients with MS (n = 27), severe MR (n = 26), and MS with concomitant with severe MR (MS/MR; n = 36). These subgroups were further subdivided according to the atrial rhythm (sinus rhythm (SR)+AF). RESULTS: The mean left atrial size was increased in all groups with cardiac disease. D-dimer levels were highest in the MS+AF subgroup (527 +/- 134 microg/l). Patients with MS+AF, MS+SR, and nonvalvular AF had significantly higher D-dimer levels than controls (p < 0.01, by ANOVA). Patients with MR had normal levels of D-dimer. The atrial rhythm did not influence D-dimer levels in the MS/MR subgroup, or in the pure MR subgroup (p = NS). CONCLUSION: Plasma D-dimer levels correlated with the embolic risk in mitral valve disease and non-valvular AF. The highest levels were found in patients with MS+AF and non-valvular AF. Severe MR decreased the D-dimer levels in MS and/or AF to control levels.

Aortic coarctation complicated with multiple saccular aortic aneurysms.

Aortic coarctation is a congenital malformation of the aorta that should be diagnosed and corrected early in life. The natural history of unrepaired coarctation of the aorta includes the development of systemic hypertension and subsequent morbidity and death from cardiovascular disease. Here, we present a case with replacement of the aortic arch and its branches by tortuous multilobulated aneurysms as a result of untreated aortic coarctation.

Coronary artery aneurysms in Behçet's disease.

Behçet's disease is a rare chronic autoimmune vasculitis with characteristic skin findings. Vascular involvement generally affects veins more than arteries, and coronary arterial involvement is extremely uncommon. Here we report the significant coronary artery aneurysms detected in a 41-year-old man with Behçet's disease who had been in remission for 13 years. Our case illustrates the necessity of initial non-invasive cardiac assessment of patients with Behçet's disease.

Do comorbid psychiatric disorders contribute to the pathogenesis of tako-tsubo syndrome? A review of pathogenesis.

Patients with tako-tsubo cardiomyopathy have unusual characteristics, including a disproportionate occurrence in postmenopausal women and a strong association with mental and physical stress. These patients present with chest pain, abnormal electrocardiographic findings, and myocardial enzyme leaks. They appear to have acute coronary syndrome but have normal epicardial coronary vessels and reversible left ventricular dysfunction. High circulating levels of catecholamines likely cause this syndrome through direct injury of myocardial cells. The frequent occurrence in postmenopausal women has not been explained. This association might reflect changes in autonomic control of the cardiovascular system and in baroreceptor sensitivity in postmenopausal women. Women in this age group also have a high frequency of both depression and anxiety. These 2 psychiatric diagnoses are associated with episodic symptoms, including acute panic attacks, which can be associated with catecholamine release in the heart. Therefore, tako-tsubo cardiomyopathy may occur in postmenopausal women as a consequence of comorbid psychiatric diagnoses and normal age-related changes in autonomic control of the cardiovascular system. This hypothesis warrants prospective case-control studies.

Acute stress cardiomyopathy and deaths associated with electronic weapons.

Deaths associated with the use of electronic weapons almost always occur in young men involved in either civil disturbances or criminal activity. These situations are associated with high levels of circulating catecholamines and frequently associated with drug intoxication. The mechanism for these deaths is unclear. Clinical studies indicate that these high voltage electrical pulses do not cause cardiac arrhythmia. Acute stress cardiomyopathy provides an alternative explanation for deaths associated with electronic weapons and may provide a better explanation for the usual time course associated with taser deaths. Patients with acute stress cardiomyopathy usually have had an emotional or physical stress, have high circulating levels of catecholamines, present with an acute coronary syndrome but have normal coronary vessels without significant thrombus formation. They have unusual left ventricular dysfunction with so-called apical ballooning. This presentation has been attributed to the direct effects of catecholamines on myocardial cell function. Alternative explanations include vasospasm in the coronary microcirculation and/or acute thrombosis followed by rapid thrombolysis. Similar events could occur during the high stress situations associated with the use of electronic weapons. These events also likely explain restraint-related deaths which occur in independent of any use of electronic weapons. Forensic pathologists have the opportunity to provide important details about the pathogenesis of these deaths through histological studies and careful evaluation of coronary vessels.

Use of PTFE-covered stent in acute myocardial infarction of aneurysmatic coronary artery.

Coronary artery aneurysms are reported between 0.1-4.9% in coronary angiographies. Vessel wall weakening, inflammation and endothelial damage play a role in their development. They may compromise the coronary flow and play a source for recurrent embolization of thrombus fragments. Here we report a patient with coronary artery aneurysm in left anterior descending artery with a critical thrombosed proximal segment and resulting in acute myocardial infarction. Coronary artery aneurysm is managed with polytetrafluoroethylene (PTFE) covered stent and proximal infarct related segment is treated with a bare metal stent. Coronary patency is provided without any obvious complication at the end of the procedure.