(III-1B.1.o) 29-years old patient with heart and renal failure Anton Chrustowicz, Jolanta Rzucidło
Authors: Anton Chrustowicz 1 , Jolanta Rzucidło 1 1John Paul II Hospital in Krakow case presentations and videos are provided for private use only
(III-2A) 19-year-old patient with Down syndrome, with hypertrophic cardiomyopathy, after correction of congenital heart disease: ASD II, VSD, PDA Prof. Piotr Podolec MD PhD, Monika Smaś-Suska MD, Lidia Tomkiewicz-Pająk MD, Maria Olszowska MD PhD
Background Hypertrophic cardiomyopathy (HCM) is the most common monogenic cardiac disorder and has been estimated to occur in one of every 500 persons in the general population. The mortality due to HCM is estimated at 1% per year and it is the most common reason of sudden death (especially in young athletes). The natural history of HCM can be altered by a number of therapeutic interventions: ICDs for secondary or primary prevention of sudden death in patients with risk factors, …
(III-3B.2) Dynamic progression of aortic stenosis in the patient with Fabry disease despite enzyme replacement therapy during 4.5-year follow-up Petkow-Dimitrow P. MD, PhD, Dziedzic H. MD, Miszalski-Jamka T. MD, PhD
Keywords: aortic stenosis, Fabry disease Background Fabry disease is a rare X-linked lysosomal storage disorder leading to an accumulation of glycosphingolipids in all tissues and organs including the heart. Currently available data suggest that valve abnormalities are observed frequently in Fabry patients. Recently, a systematic evaluation of the severity of valve abnormalities was performed in a impressive series of Fabry patients in a reference German center. Accordingly, 111 patients with genetically proven Fabry disease were systematically monitored by echocardiography for …
(III-1A.4b) Dilated cardiomyopathy with atriovantricular conduction defects in patient Marchel M., Opolski G.
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case presentations and videos are provided for private use only
(III-5A.1.o) Left ventricular non-compaction with diaphragmatic hernia and heart displacement to the right side of the thorax Rubiś P., Miszalski-Jamka T., Podolec P.
Experts: John G. Cleland, Gąsior Z.
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case presentations and videos are provided for private use only
(III-5A.1.o) Isolated noncompaction of the left ventricle in a young athlete Karch I., Tomkiewicz-Pająk L., Podolec M., Nowacka M., Rubiś P.
Experts: Szydłowski L., Kordon Z., Mizia-Stec K., Podolec P., Kopeć G.
Background Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy due to abnormal morphogenesis of the endocardium and myocardium that occurs between 5-th and 8-th week of fetal life. It is characterized by prominent myocardial trabeculations accompanied by deep intertrabecular recesses which lie in continuity with the left ventricular cavity. Given the increased risk of sudden cardiac death associated with LVNC, athletes with this diagnosis should be excluded from most competitive sports. We report previously non diagnosed LVNC in …