Left ventricular dysfunction and mortality in adult patients with Eisenmenger syndrome.

Objectives: To identify risk factors associated with mortality in adult patients with Eisenmenger syndrome and to assess the effect of left ventricular dysfunction on mortality in these patients.

Methods And Results: One hundred twenty-two adult patients with Eisenmenger syndrome were retrospectively evaluated for signs and symptoms of heart failure, and underwent electrocardiography and laboratory investigations. Available echocardiograms were analyzed and left ventricular function was assessed both qualitatively and quantitatively.

Left ventricular aneurysm in a patient with mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): clinical and pathological correlation.

A 22-year-old man was diagnosed with mucopolysaccharidosis type VI, also known as Maroteaux-Lamy syndrome, which is a known cause of cardiac valvular disease. He presented with exercise intolerance and was diagnosed with a large, apical, left ventricular aneurysm (LV aneurysm) and subsequently underwent left ventricular aneurysmectomy with improvement in clinical status. Previous echocardiograms revealed that the LV aneurysm was new and is, therefore, likely acquired rather than congenital.

A bicuspid pulmonary valve associated with tetralogy of fallot.

Congenitally bicuspid pulmonary valves are uncommon. When they occur, it is usually in association with other congenital cardiac lesions, most often a tetralogy of Fallot. We present a rare case of a patient with a congenitally bicuspid pulmonary valve who had pulmonary valve and RVOT reconstruction.

Electrophysiologic surgery in patients with congenital heart disease.

As patients with congenital heart disease increase in number and age, arrhythmia is becoming a more prevalent and important clinical problem. Although catheter-based therapy has revolutionized the management of arrhythmia, there remains an increasing patient population with congenital heart disease presenting for repair or reoperation with associated atrial or ventricular arrhythmias. Arrhythmia ablation may be safely and effectively included as an adjunct to repair of underlying structural cardiac lesions.