[POL-PAVTI--Polish report on transcatheter pulmonary artery valve implantation of Melody-Medtronic prosthesis in the first 14 patients in Poland].

Aim: To assess the early results of the pulmonary artery valve transcatheter implantation (PAVTI) in pts included into POL-PAVTI registry. Detailed medical and economic analyses were performed.

Methods: Pulmonary artery valve implantation was performed in 14 pts (9 men), aged 16-31 (mean 24.

Isolated ventricular noncompaction mimicking arrhythmogenic right ventricular cardiomyopathy--a study of nine patients.

Background: Isolated ventricular noncompaction is considered to predominantly affect the left ventricle. It is characterized by increased left ventricular wall thickness and deep intertrabecular recesses with to-and-fro blood flow that remains in continuity with the ventricular flow. Aim of the study was to present a group of patients with isolated noncompaction of both ventricles mimicking arrhythmogenic right ventricular cardiomyopathy (ARVC).

[Non-surgical, transcatheter pulmonary valve implantation - first experience].

Transcatheter valve replacement has recently been introduced into clinical practice. We present our first experience with non-surgical, transcatheter pulmonary valve implantation in four patients (age 27-31 years, three females) with repaired congenital heart disease who required reintervention to the right ventricular outflow tract due to dysfunction of valve homograft. The Medtronic Melody Transcatheter Pulmonary Valve was successfully implanted in all four patients without complications, releasing the outflow obstruction, and normal function of the biological valve.

Economic evaluation of screening for familiar form of arrhythmogenic right ventricular cardiomyopathy in Poland.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease associated with fibrofatty tissue replacement in heart muscle leading to arrhythmia, heart failure or sudden death (SCD) often being the first manifestation in probands. At least 50% of cases of ARVC are inherited.

Aim: To evaluate costs and cost-effectiveness of diagnosis of the disease in asymptomatic relatives in Poland.

Ultrastructural evidence of intercalated disc remodelling in arrhythmogenic right ventricular cardiomyopathy: an electron microscopy investigation on endomyocardial biopsies.

Aims: The ultrastructural features of the myocardium in arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been systematically investigated so far. The recent discovery of gene mutations encoding intercalated disc proteins prompted us to perform a transmission electron microscopy study on endomyocardial biopsies.

Methods And Results: Twenty-one ARVC probands who fulfilled the international Task Force diagnostic criteria underwent right ventricular endomyocardial biopsy and screening of desmosome (D) protein encoding genes.

Thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Aims: Incidence and clinical presentation of thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) were analysed. In reports on ARVD/C, thromboembolism is rarely mentioned. The possible risk factors are: right ventricle (RV) dilatation, aneurysms, and wall motion abnormalities.

Familial form of arrhythmogenic right ventricular cardiomyopathy.

Background: Arrhythmogenic right ventricular dysplasia (ARVD) is characterised by fatty and fibrous infiltration of myocardial muscle. Clinical symptoms include dangerous cardiac arrhythmias and heart failure in the advanced form of the disease. ARVD is genetically determined in at least 50% of cases and is characterised by a marked variability of clinical presentation within one family.

[Arrhythmogenic right ventricular cardiomyopathy (dysplasia): etiology, clinical presentation, diagnosis and treatment].

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder characterised by progressive replacement of right ventricle myocardium by either segmental or diffuse fibro-fatty tissue, often with the involvement of the left ventricular wall and manifestations of arrhythmia. Familial occurrence with autosomal dominant inheritance, variable expression and incomplete penetrance is estimated as 1/5000. The recessive form called Naxos disease has also been reported.