Introduction: Left ventricular pseudoaneurysm is a rare condition because in most instances ventricular free-wall rupture leads to fatal pericardial tamponade. Rupture of the free wall of the left ventricle is a catastrophic complication of myocardial infarction, occurring in approximately 4% of patients with infarcts, resulting in immediate collapse of the patient and electromechanical dissociation. In rare cases the rupture is contained by pericardial and fibrous tissue, and the result is a pseudoaneurysm. The left ventricular pseudoaneurysm contains only pericardial and fibrous elements in its wall-no myocardial tissue. Because such aneurysms have a strong tendency to rupture, this disorder may lead to death if it is left surgically untreated. Case report: In this case report, we present a patient who underwent successful repair of a left ventricular pseudoaneurysm, which followed a myocardial infarction that was caused by occlusion of the left circumflex coronary artery. Although repair of left ventricular pseudoaneurysm is still a surgical challenge, it can be performed with acceptable results in most patients.

Aims: Valproate is an antiepileptic drug with a wide spectrum efficacy in the management of various types of seizures and epileptic syndromes. It still makes the first choice drug in many forms of paediatric epilepsy syndromes. Although many side effects of valproate are well known, there is still difficulty in recognising of nocturnal enuresis as seldom but possible side effect of this drug. Methods: We describe six well documented cases of secondary nocturnal enuresis as a sole side effect owing to valproate therapy. All children (5 boys and 1 girl) had known epilepsy; idiopathic generalised epilepsy (4) and cryptogenic focal epilepsy (2) respectively. All but two of the children took valproate as monotherapy when manifesting this problem. The average dosage of valproate amounted 650mg/d. Enuresis embraced in three cases a comprehensive psychological and urological examination and treatment. Results: After sodium valproate was discontinued in the clinical setting, the symptoms disappeared in all patients. Conclusion: The nocturnal enuresis can lead to substantial functional impairment in everyday life and can be very irksome for the patient and his family. The purpose of this work is to highlight the possible side effects of this drug that all clinicians should be aware of when using it as an antiepileptic agent, particularly as there are still very few literature on this problem.

Objectives: To determine the prevalence of isolated left ventricular noncompaction (IVNC) as a cause of heart failure and heart transplantation. Methods: There were 960 patients seen in the heart failure clinic from 1987 to 2005, with a complete evaluation including echocardiography at our center (study population, 82% men, mean age 52 years). The following data were collected: type of heart disease, age at echocardiography and at heart transplantation, and frequency of heart transplantation. Echocardiographic diagnosis of IVNC was based on our published criteria. Results: The etiologies of heart failure were coronary artery disease (CAD; 37%), idiopathic dilated cardiomyopathy (33%), valvular heart disease (11%), congenital heart disease (5%), IVNC (3%), hypertensive heart disease (3%), hypertrophic cardiomyopathy (2%), myocarditis (1%), and <1% other diagnoses. Heart transplantation was performed in 253 patients (26%) due to idiopathic dilated cardiomyopathy (42%), CAD (39%), valvular heart disease (5%), congenital heart disease (5%), IVNC (2%), or other etiologies (≤1% each). Conclusions: The most common causes for heart failure remain idiopathic dilated cardiomyopathy, CAD and valvular heart disease. Strictly using the criteria for the definition of IVNC, IVNC is a rare underlying cardiomyopathy for both, heart failure (2.7%) and heart transplantation (2%) in our center.

AIMS Anderson-Fabry disease affects various organ systems due to glycosphingolipid accumulation. Enzyme replacement therapy (ERT) has been reported to decrease left ventricular wall thickening (LVWT) and to improve diastolic dysfunction. METHODS AND RESULTS This prospective study included 29 patients (patients; mean age 37 +/- 13 years) with genetically, enzymatically and/or biopsy-proven Anderson-Fabry disease and long-time ERT. Data on symptoms, cardiac medications and history of hypertension were collected and all patients had comprehensive echocardiographic examination prior to ERT and at follow-up. Disease was at an early stage with a total mean Mainz severity score index of only 18.6 +/- 13.0. Prior to ERT, 79% of patients reported acroparesthesia. The median creatinine level was 121 +/- 108 mcmol/L and LVWT was present in nine patients (31%). Binary appearance of the interventricular septum was found in 20% and posterobasal fibrosis in 83%. At median follow-up of 37 months, acroparesthesia decreased to 55% (P = 0.016). There was no change in creatinine levels. The incidence of LVWT was unchanged, only an increase in interventricular septal wall thickness from 11.7 +/- 0.4 to 12.5 +/- 0.5 was observed (P = 0.009). Left atrial size and the percentage of patients with binary appearance and posterobasal fibrosis were unchanged. There was a small improvement in diastolic function (29% decrease of E/Ea; P < 0.002). CONCLUSION Our Anderson-Fabry cohort had successful long-time ERT with impressive amelioration of subjective symptoms. Although there was not much improvement in cardiac changes apart from a slight improvement of diastolic function, at least, there was no progression of cardiac disease. For complete reversibility of cardiac changes in Anderson-Fabry disease, ERT might have to be started earlier in life and/or prescribed for a longer time.

The occurrence of generalised tonic-clonic seizures (GTCS) was investigated in patients with absence epilepsy (AE), evaluating the opinion that ethosuximide does not protect against GTCS. Our retrospective study included 238 patients with absences and generalised 3-Hz spike waves (SW). We analysed the efficacy of antiepileptic drugs (AED) and the occurrence of GTCS before, during and after treatment. We surveyed family history, treatment delay and EEG findings. Family history of epilepsy was positive in 28%. Children with 3-Hz SW lasting >10 s suffered less frequently from GTCS (p=0.002). Photosensitivity (3-Hz SW during photic stimulation) recorded in 47 children was more frequent in juvenile AE (p=0.0001), but not associated with higher rates of GTCS. GTCS occurred in 27 children (11%) before treatment, in 14 (5.8%) during treatment and in 8 (4.8%) after tapering AED. Valproate and ethosuximide monotherapy were equally effective on absences, carrying the same low risk of GTCS during treatment (2 valproate, 1 ethosuximide). Most GTCS occurred on drug combinations considered effective against GTCS. Risk factors for relapses after tapering AED were photosensitivity (p=0.002) and GTCS during treatment (p=0.02). GTCS are rare in patients with typical AE. Our data do not support the current opinion that ethosuximide is inefficacious on GTCS in AE.