(IV-5A.2) (English) Patient after correction of Tetralogy of Fallot with pulmonary valve regurgitation and left pulmonary branch stenosis Tomkiewicz-Pająk L. MD, PhD, Podolec J. MD, Kopeć G. MD, PhD, Drabik L. MD, Assoc. Prof. Olszowska M. MD, PhD, Miszalski-Jamka T. MD, PhD, Assoc. Prof. Kostkiewicz M. MD, PhD, Tyrka A. MD
Keywords: correction of Tetralogy, pulmonary valve regurgitation, left pulmonary branch stenosis Background Tetralogy of Fallot (ToF) is a cyanotic congenital heart disease. Almost all unoperated patients used to die before 40 years of age. Prognosis after surgical correction is good, with a 35-year survival of 85%. Surgery is carried out to close ventricular septal defect and relieve right ventricular outflow tract obstruction. Patients who have reached adulthood after earlier repair of ToF frequently develop complications such as severe pulmonary regurgitation, …
(I-3A.2) Giant cell aortitis of the ascending aorta without signs and symptoms of systemic vasculitis Prof. Podolec P. MD, PhD, Stępniewski J, MD, Drabik L. MD, Prof. Ereminienė E. MD, PhD, Prof. Benetis R. MD, PhD, Dziedzic H. MD
Keywords: ascending aorta, giant cell aortitis Background Giant cell arteritis (GCA) is a chronic inflammatory disease of the medium and large elastic arteries, especially cranial vessels, that predominantly occurs in the elderly. The estimated incidence in Europe in individuals over 50 years of age varies between 32 and 290/million/year [1] Aortic aneurysm or dissection develops only in up to 18% of patients with GCA. Diagnosis is often delayed due to poor recognition of early nonspecific symptoms and the management of …
(IV-1D.1b) (English) Ebstein’s anomaly with bidirectional shunt through ASD Klima L., Dziedzic H., Rajzer M., Olszanecka A., Czarnecka D.
Keywords: Ebstein’s anomaly, bidirectional shunt, ASD Background Ebstein’s anomaly is a congenital heart defect that appears in 1 on 100 000 to 200 000 births. In this defect tricuspid valve is dislocated towards the apex of the right ventricle. The annulus of the valve is in the standard position and leaflets are attached to the walls and septum of the right ventricle. This leads to atrialization of a portion of the right ventricle. The right atrium is enlarged and the …
(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 …
(IV-2A.1) (English) 18-year-old patient with tetralogy of Fallot and coronary artery abnormality Smaś-Suska M.MD, Tomkiewicz-Pająk L. MD,PhD, Tyrka A. MD, Olszowska M. MD, PhD, Miszalski-Jamka T. MD, PhD, Podolec J. MD
Keywords: coronary artery abnormality, tetralogy of Fallot Background Tetralogy of Fallot is one of the most common cyanotic congenital cardiac diseases. This heart defect consists of ventricular septal defect, obstruction of the right ventricular outflow tract, overriding aorta and right ventricular hypertrophy. About 3% of patients have anomalous left anterior descending coronary artery which may necessitate a conduit type of repair. Conduits connect right ventricle and main pulmonary artery. There are two types of conduits: valved and non-valved. About 40% …
(I-2A.1) (English) 54-year-old female with Marfan’s Syndrome Prof. Podolec P. MD, PhD, Kopeć G.MD, PhD, Rubiś P. MD, PhD, Dziedzic-Oleksy H. MD, Hlawaty M. MD, PhD
Keywords: Marfan’s Syndrome, aortic dissection Background Marfan’s Syndrome is an inherited autosomal dominant disorder of connective tissue, that affects many organs including the cardiovascular, ocular, skeletal and pulmonary system, the skin and the dura. The classical cardiovascular manifestations of Marfan’s Syndrome are mitral valve prolapse and aortic root dilation, leading to aneurysm and dissection. Regular control studies such as echocardiography, magnetic resonance and computed tomography are required in patients with this disease. Case description A 54-year-old Caucasian female with Marfan’s …
(II-1C.1) 23-year-old man with Down syndrome, complete AV canal, Eisenmenger syndrome, and elevated Pulmonary Capillary Wedge Pressure (PCWP) Kopeć G., Stępniewski J., Tyrka A.,
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Keywords: Eisenmenger syndrome, Pulmonary Capillary Wedge Pressure, Down syndrome Background Pulmonary hypertension (PH) is a hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure (PAP)≥ 25 mmHg at rest as assessed by right heart catheterization. PH can be found in multiple clinical conditions. Complete atrioventricular canal (CAVC), an uncommon CHD often associated with Down syndrome, is characterised by an ostium primum atrial septal defect, a common atrioventricular valve and a variable deficiency of the ventricular septum …