Electrical storm or naxos syndrome in an adult causing recurrent syncope

Among the rare and well-known causes of sudden cardiac death by malignant arrthymias is a condition called arrhythmogenic right ventricular cardiomyopathy. It commonly presents with right ventricular dilatation, dysfunction and ventricular tachycardia of left bundle branch morphology due to fibro-fatty infiltration of right ventricle in second to fifth decade of life, making it an unrecognized and important cause of sudden cardiac death. Two rare variants of arrhythmogenic right ventricular cardiomyopathy are Carvajal syndrome and Naxos syndrome. Both variants have systemic manifestations. Being a rare variant of arrhythmogenic right ventricular dysplasia, Naxos syndrome was initially described in the families of the Greek island of Naxos. It is a recessive disorder with cardio-cutaneous manifestations characterized by arrhythmogenic right ventricular cardiomyopathy, palmoplantar keratoderma and wooly hair. We report a rare case of Naxos syndrome in an adult patient presented with recurrent episodes of palpitation and syncope

Relationship of serum uric acid level and angiographic severity of coronary artery disease in male patients with acute coronary syndrome

Background and objective: The association between serum uric acid and ischemic heart disease remains controversial and it has not yet been established as cardiovascular risk factor. Our objective was to study the association of serum uric acid level with angiographic severity of coronary artery disease in men with acute coronary syndrome [ACS]

Methods: This cross-sectional study was conducted on 100 consecutive male patients presenting with ACS at Punjab Institute of Cardiology. Hyperuricemia was defined as serum uric acid level >/= 6.5 mg/dl. Severity of ischemic heart disease was assessed on the basis of Gensini score, number of diseased vessels, critical lesions and total occlusions on coronary angiogram

Results: Mean age of normouricemic group [n=59] was 52.62 +/- 9.46 years and mean age of hyperuricemic group [n=41] was 50.52 +/- 9.40 years [p=0.273]. Mean uric acid level; normouricemic group [4.75 +/- 1.05], hyperuricemic group [7.61 +/- 1.24], p < 0.001. Mean Gensini score; normouricemic group [22.15 +/- 21.52], hyperuricemic group [35.69 +/- 26.80]. Mann Whitney U test was applied to compare the Gensini score of two groups and it showed statistically significant difference [p value <0.006]. Critical lesions, total occlusions and multi-vessel disease were more frequent in hyperuricemic group but statistically significant difference was found only for total occlusions [p=0.013] and critical lesions [p=0.046]

Conclusions: Hyeruricemia is associated with higher Gensini score and more frequent total occlusions and critical lesions in men presenting with acute coronary syndrome

Usefulness of st segment depression limited to the recovery phase of exercise stress test

To determine the diagnostic value of ST segment depression limited to recovery phase of Exercise Tolerance Test [ETT]. An observational cross-sectional study. The study was carried out at Punjab Institute of Cardiology, Lahore, from March to August 2007 for a period of 6 months. In this study, 100 patients were selected with purposive non-probability sampling technique and were divided into two groups, group A having ST segment depression during exercise phase of ETT, and group B having ST segment depression only in recovery phase after undergoing ETT according to Bruce protocol. The patients were subjected to coronary angiography later on. The chi-square or Fischer's exact test of significance was applied at 0.05 level of significance to compare the coronary angiographic finding between two groups. Diagnostic value of ST segment depression was computed keeping angiographic finding as gold standard. There were no major differences between the two groups regarding angiographic finding. Significant coronary artery stenosis [>50% narrowing] was found in 68 [93%] patients of group A and 23 [85%] patients of group B [p=0.18]. Three vessel disease was found in 30 [41%] patients in group and 7 [26%] patients in group B [p=0.08]. Left main stem disease was found in 5 [6.8%] patients of group A and 1 [3.7%] patient of group B. Normal coronaries were found in 6% of patients among group A and 14% of patients among group B[p=0.12]. Specificity, sensitivity, Positive Predictive Value [PPV] and Negative Predictive Value [NPV] of ST segment depression in recovery phase was 55, 25, 85 and 6 percent respectively [95%Cl; 1.16-2.25]. In patients undergoing exercise stress test, ST segment depression occurring only in recovery phase of ETT has a diagnostic value largely comparable with that of ST segment depression induced during exercise phase of ETT. Thus careful evaluation of ST segment depression occurring only in recovery phase may add significantly to the clinical information derived from the results of ETT