Efficacy of Ranolazine in a Patient with Idiopathic Dilated Cardiomyopathy and Electrical Storm

A case of idiopathic dilated cardiomyopathy with an arrhythmic storm refractory to the usual antiarrhythmic therapy will be reported. The idiopathic structural heart disease of the patient is a vulnerable anatomic substrate in itself, for electrical instability and reentry mechanism, because of heterogeneous areas of scarred myocardium and low left ventricle ejection fraction. In this case, the ranolazine administration was safe and effective for the prevention of further electrical storms. © 2013, International Research and Cooperation Association for Bio & Socio-Sciences Advancement. All rights reserved

Mid-term Outcome of 100 Consecutive Ross Procedures: Excellent Survival, But Yet to Be a Cure

The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (ContegraR) graft (31 patients). Additional procedures included Ross-Konno procedure (14%), resection of subaortic stenosis/myectomy (11%) and reduction plasty of the ascending aorta (25%). One patient died within the first 30 days (1%). Late deaths occurred in 4 patients (4%) 0.5-4.5 years postoperatively: causes included pulmonary hypertension due to endocardial fibroelastosis (2), subarachnoid haemorrhage (1) and sudden cardiac death (1). Five-year survival was 93.6 (95% CI 88.1-99.1)%. Moderate or severe aortic (autograft) regurgitation needing reoperation occurred in 8 patients with a 5-year freedom from autograft reoperation of 98.5 (95.6-100)%. Five-year freedom from reintervention (surgery or catheter based) on the right ventricular outflow tract conduit was 91.5 (85.5-96.5)%. Univariate predictors of this reinterventions were smaller graft size (p = 0.03) and use of a ContegraR graft (p = 0.04). Ross procedure can be performed with low mortality and good survival in the long term. Most of the reinterventions are related to the neo-right ventricular outflow tract and may be partly attributed to the lack of growth. While the Ross Procedure remains an invaluable option for aortic valve disease in children, new solutions for the neo-pulmonary valve as well as for the less often occurring problems on the autograft are needed