Sudden death in hypertrophic cardiomyopathy: old risk factors re-assessed in a new model of maximalized follow-up.

Aims: in hypertrophic cardiomyopathy (HCM), the following five risk factors have a major role in the primary prevention of sudden death (SD): family history of SD (FHSD), syncope, massive wall thickness (MWTh) >30 mm, non-sustained ventricular tachycardia (nsVT) in Holter monitoring of electrocardiography, and abnormal blood pressure response to exercise (aBPRE). In HCM, as a genetic cardiac disease, the risk for SD may also exist from birth. The aim of the study was to compare the survival curves constructed for each of the five risk factors in a traditional follow-up model (started at the first presentation of a patient at the institution) and in a novel follow-up model (started at the date of birth).

[Left ventricular non-compaction in an infant with congenital heart defect].

Non-compaction of the left ventricle (NCLV) was categorised as unclassified cardiomyopathy by the World Health Organization in 1995. Over the last decade this condition has been identified as a distinct form of cardiomyopathy and a genetically heterogeneous disorder. Clinically, this may be coupled with the loss of contractility, arrhythmia, and thromboembolism.

[Isolated left ventricular non-compaction - various clinical presentations].

The isolated left ventricular non-compaction (LVNC) is an increasingly commonly diagnosed myocardial disorder resulting from a defective morphogenesis of the endomyocardium. This is associated with high rates of thromboembolism, cardiac failure, and cardiac arrhythmia. The report describes 3 cases of LVNC, presenting with different clinical pictures.

Genetic background of congenital conotruncal heart defects--a study of 45 families.

Introduction: The latest achievements in molecular diagnosis create new possibilities for evaluation of congenital abnormalities.

Aim: To present our preliminary experience with genetic diagnosis of congenital combined conotruncal heart defects.

Methods: The analysis comprised 35 families with more than one member suffering from conotruncal heart defects (Group I) and 10 families (Group II) having a child with the clinical features of CATCH 22 syndrome.

[Cardio-vascular disorders at the time of diagnosis of malignant solid tumours in children--own experiences].

Unlabelled: Cardio-vascular abnormalities in children with neoplastic disease are observed in some cases even during initial diagnostic examinations. The aim of the study was to evaluate the frequency and characteristics of cardiological and vascular disorders in the initial phase of malignant solid tumors in children.

Material And Methods: The study included 236 paediatric patients diagnosed with malignant solid tumours, treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdańk, between 1992 and 2004.

[Late side-effects of treatment in patients with nephroblastoma].

Unlabelled: The aim of the study was to analyse late side-effects of the antineoplastic treatment in patients with diagnosed nephroblastoma.

Material And Methods: In the years 1986-2002, 80 children were treated in the Gdańsk Centre, for nephroblastoma, 13 of them followed SIOP-6, 25 were treated according to SIOP-9 and the remaining 42, SIOP 93-01 programme.

Results: 70 patients had long-term clinical remission.

[Cardiovascular malformations in Turner syndrome].

Background: A literature review indicates that the prevalence of cardiovascular malformations in patients with Turner syndrome ranges from 17 to 47 percent.

Objectives: The aim of the study was to evaluate the cardiovascular system in patients with Turner syndrome, controlled by the Pediatric Endocrinology Clinic of the Medical University of Gdansk.

Material And Methods: The study included 55 females with Turner syndrome aged 3-20 years (mean age 9.

[Evaluation of systolic and diastolic function of the left ventricle in children with acute lymphoblastic leukaemia before treatment].

Between 1995 and 2001 echo-cardiography was performed in 244 children (128 boys, 116 girls) with acute lymphoblastic leukaemia (ALL) before the beginning of therapy with anthracyclines (medium 5.4 days after the diagnosis). The mean age at diagnosis was 5.

[Epidemiology of hypertensive retinopathy in young patients after coarctation of the aorta repair].

Purpose: Coarctation of the aorta is a cause of childhood hypertension. In some patients hypertension persists after coarctation repair. We evaluated hypertensive retinopathy in patients after coarctation repair, factors correlated with retinopathy, specificity of retinal findings.

[Aortic coarctation treated with bypass graft in adolescence and implantation of intravascular stent 12 years later--a case report].

A case of a 26-year-old female with aortic coarctation and Recklinghausen disease is presented. At the age of 14 years the patient underwent thoracic aortic bypass grafting. Twelve years later she developed arterial hypertension.

[Sudden cardiac death in children].

Natural, sudden deaths in children are the cause of about 5% of all deaths. The most frequent cause appears to be pathology of the cardiovascular system. Direct cause of death are arrhythmias or, rarely, haemodynamic disturbance coexisting with decrease in minute cardiac output.