Eisenmenger syndrome: a silent killer in adults born with heart septal defect: experience from cardiac centre at Faisalabad

Eisenmenger syndrome is a term used to any large shunt between systemic and pulmonary circulation which results in high pulmonary arterial pressure and irreversible changes in pulmonary vascular bed with bidirectional shunt with physical limitation and shortness of breath. Eisenmenger syndrome particularly creates problems to fetus and mother in pregnancy and there is a particularly risk during aneasthesia while performing general surgery. We collected all consecutive patients above age 12 with atrial septal defect [ASD], ventricular septal defect [VSD] and patent ductus arteriosus [PDA] who attended echocardiography department between June 2008 to October 2010. We also analysed all pregnant females with Eisenmenger complex during this period. Out of 309 patients diagnosis of one of three shunts was confirmed either by transthorasic echocardiography with intravenous saline infusion or transesophageal echocardiography Eisenmenger syndrome was diagnosed in 39 patients [19 patients with ASD, 11 patients with VSD and 09 patients with PDA]. All 39 patients were followed till October 2010 and were alive. Among 03 pregnant females, 02 completed pregnancy without any hazard to child and mother. However tubal ligation was opted at time of delivery. One lady opted abortion and ligation to prevent further pregnancy. 05 patients underwent non cardiac surgery under general anesthesia without any complication. Eisenmenger syndrome a silent killer in a congenital treatable disease which is being neglected and diagnosis is being delayed. It seems Eisenmenger syndrome is on rise in Pakistan. We need to establish adult congenital heart disease department in each cardiac centre where trained persons should be appointed who had experience of congenital heart disease. Screening clinics need to be established at school and community level to diagnose this silent killer at a stage when pulmonary artery pressure is still reversible

Brugada syndrome: are we doing enough to prevent sudden death?

Brugada syndrome [BS] is an inherited arrythmogenic disease characterized by typical ECG changes in the form of an SR pattern in VI to V2, and ST segment elevation in VI to V3 and prolongation of the QT interval in right precordial leads. This syndrome carries an increased risk of sudden death due to arrhythmias. This disease was first described in 1992 by Joseph Brugada et al and was named Brugada syndrome by Yan and Antzelvitch in 1996. By 2003 more than 600 patients had been reported by Brugada et al and hundreds by others. A genetic aspect to BS is now recognized and been linked to the alpha subunit of the cardiac sodium channel gene SCN5A. Over five dozen mutations in SCN5A have been identified. Accentuation of the right ventricular notch under pathophysiological conditions leads to exaggeration of the J-wave or J-point elevation and a saddle-shaped configuration of the repolarisation waves. Diagnosis is essentially by electrocardiogram, either by spontaneous changes or by provocation by sodium channel blockers drugs, e.g., procainamide, flecainide. The role of electrophysiological studies in induction of arrhythmia in asymptomatic individuals by electron beam computed tomography and signal-averaged electrocardiogram is not settled. Unfortunately, an effective drug is not available at present, but quinidine has a place in treatment. New promising drugs are emerging like cilostazol and tedisamil. At present, implantation of an ICD is the only effective means of preventing sudden death