Sydenham's chorea-clinical characteristics of nine patients.

Sydenham's chorea is still the most frequently seen form of acquired chorea in childhood in developing countries. Nine patients presented with Sydenham's chorea as one of the major criteria (one or more attacks) were evaluated their clinical characteristics and treatment. All patient were given sodium valproate. Clinical improvement began 11+/-4.0 days in sodium valproate treatment, and no adverse effect was seen due to the drugs. Echocardiography revealed rheumatic carditis sequela in all patient. The nine Sydenham's chorea patients presented 13 attacks of chorea in follow-up. We observed isolated chorea in 44% of patients, and 22% presented one or more recurrences. The interval between the attacks ranged from months to 2 years. The 90% of the patients were not on prophylaxis with benzathine penicillin. One patient had diagnosis of obsessive-compulsive disorder, and one had diagnosis of attention deficit hyperactivity disorder in the follow-up period. This report emphasizes the significance of Sydenham's chorea and sequela of rheumatic carditis association and a high risk of developing neuropsychiatric disorders in the follow-up period. Valproic acid is an effective and safe drug in the treatment of Sydenham's chorea.

An unusual case of postoperative giant left ventricular pseudoaneurysm.

Pseudoaneurysms of the left ventricle are rare complications of acute myocardial infarction or cardiac surgery. Three years after aneurysmectomy of a true left ventricular aneurysm, a 66 years old man presented with clinical features of congestive heart failure. The echocardiography showed an extra large chamber next to the posterolateral region of the left ventricle with massive thrombus and severe mitral regurgitation. The diagnosis of pseudoaneurysm was made and was subsequently confirmed by radionuclid angiocardiography and surgical findings. Left ventricular pseudoaneurysm formation is a fairly unusual and serious complication of left ventricular aneurysmectomy with potential catastrophic results. Within the long period of time the pseudoaneurysm insidiously may become giant and may result in heart failure. Recognition of this rare complication of aneurysmectomy has therapeutic importance because surgical correction is necessary. Such pseudoaneurysm formation is easily recognized by two-dimentional echocardiography and radionuclic angiocardiogram. Careful echocardiographic examination is warranted for detecting such a complication in patients after cardiac surgery.

Interrupted aortic arch in an adolescent male.

Interrupted aortic arch (IAA) is an uncommon and usually lethal congenital malformation. The present report describes an unusual case of IAA, an aneurysmal ascending aorta and a bicuspid aorta in a 15-year-old boy. He presented with general malaise, weakness of his legs, headache and hypertension that began six months earlier. He had suffered from effort intolerance since childhood. A three-dimensional gadolinium contrast-enhanced magnetic resonance angiogram demonstrated IAA and a markedly developed collateral circulation. IAA is an uncommon disease that is rarely encountered in an adolescent patient with nonspecific symptoms or hypertension. The present case also shows the clinical value of three-dimensional gadolinium contrast-enhanced magnetic resonance angiography for noninvasive assessment of the aortic arch, and magnetic resonance angiography can replace angiography in the assessment of aortic arch anomalies and visualization of well-developed collaterals.

Does exercise-induced severe ischaemia result in elevation of plasma troponin-T level in patients with chronic coronary artery disease?

OBJECTIVE: It has been reported that the loss of cell membrane integrity during severe reversible ischaemia results in elevation of plasma troponin T (TnT) in unstable angina. We investigated whether TnT is released into circulation during severe ischaemia (e.g. on treadmill exercise testing) in patients with chronic coronary artery disease (CAD). METHODS AND RESULTS: The study comprised 54 patients who had angiographically documented CAD (22 of them had a prior history of myocardial infarction) and 18 normal subjects. All cases underwent exercise thallium (Tl)-201 SPECT myocardial perfusion imaging. Blood samples for TnT analysis were obtained for each patient at pre-exercise, immediately after and 12 hours after exercise. SPECT images were divided into 20 segments. Patients with redistribution defects > or = 5 were considered to have severe ischaemia. Sixteen patients had severe ischaemia on SPECT images. The mean TnT levels of the patients with severe ischaemia at pre-exercise, immediately after and 12 hours after exercise were 0.009 +/- 0.008, 0.012 +/- 0.009 and 0.010 +/- 0.010 ng/ml, respectively. In normal subjects the mean TnT levels were 0.012 +/- 0.009, 0.010 +/- 0.009, 0.010 +/- 0.010 ng/ml, respectively. There was no significant difference neither between the two groups nor within each group with respect to pre-exercise and post-exercise TnT results. All TnT levels were within normal limits (< 0.1 ng/dl). Additionally, when the patients were grouped according to other SPECT variables (patients with reversible defects vs. fixed defects; patients with increased lung uptake of Tl-201 vs. without lung uptake; patients with transient left ventricle dilatation vs. those without) and angiographic findings (patients with multivessel disease vs. normal subjects), there were no significant differences with respect to pre-exercise and post-exercise TnT results. CONCLUSION: Severe ischaemia does not result in the elevation of the plasma TnT level in patients with chronic CAD.

Association between angiotensin converting enzyme gene polymorphism and coronary artery disease in individuals of the South-Eastern Anatolian population.

OBJECTIVE: The deletion (D) allele of the angiotensin-converting enzyme (ACE) gene has been proposed as a genetic marker of the risk of coronary artery disease (CAD). In this study we aimed to determine the relevance of ACE gene polymorphism for coronary artery disease in the South-Eastern Anatolian population. METHODS: Angiotensin converting enzyme genotypes were determined in 133 CAD patients who underwent coronary angiography. Severity of CAD was subgrouped according to the number of stenotic vessels on coronary angiography. The control group was selected from 154 healthy volunteers. Angiotensin converting enzyme genotypes were determined by agarose gel sizing after polymerase chain reaction (PCR) amplification. RESULTS: Frequency of ACE DD genotype did not differ between patients with CAD and control subjects. However the ACE II genotype in CAD group was significantly less frequent than in control group (p=0.02). The relative risks were 0.9 (95% CI=0.56-1.43) for the DD genotypes, and 2.2 (95% CI=1.09 - 4.11) for the II genotype. In the 2-vessel CAD subgroups, the II genotypes were significantly different from control group. CONCLUSION: Our study did not confirm the possibility that the ACE DD genotypes may be associated with predisposition to CAD in this certain population but there is a relationship between the least frequencies of the II genotype and CAD. The II genotype seems to be an independent protective factor for CAD in the South-Eastern Anatolian population.

Serum omentin 1 level is associated with coronary artery disease and its severity in postmenopausal women.

We evaluated whether serum omentin levels are associated with coronary artery disease (CAD) and its severity among postmenopausal women. We enrolled 193 consecutive postmenopausal women who had undergone coronary angiography for suspected stable CAD. The study population was divided into 2 groups based on the results of coronary angiography (CAD group, n=110 and control group, n=83). Omentin 1 levels were measured and disease severity was assessed using the SYNTAX score (SS) in the CAD group. Those patients with angiographic CAD had significantly decreased omentin 1 levels, compared to those without CAD (247.5+127.4 vs 506+246 ng/mL, P<.001). After adjusting for cardiovascular risk factors, a decreased omentin 1 level was found to be an independent predictor of both angiographic CAD and a high SS. Our data indicate that a decreased omentin 1 level is associated with CAD and its severity among postmenopausal women.

Symptomatic coronary-subclavian steal syndrome due to total occlusion of proximal left subclavian artery.

Symptomatic coronary-subclavian steal occurs infrequently. We report a case involving angina pectoris in a patient with a patent left internal thoracic artery graft on the left anterior descending coronary artery and total occlusion of the proximal left subclavian artery.