(IV-1D.2c) 17-year-old male with asymptomatic aortic stenosis Joanna Łuszczak MD, Lidia Tomkiewicz- Pajak MD, PhD, Maria Olszowska MD, PhD, Prof. Piotr Podolec MD, PhD, Paweł Rubiś MD, PhD, Jakub Podolec MD.
Background Bicuspid aortic valve (BAV) is the most common heart defect that occurs in 1-2% live births. BAV is associated with abnormalities of elastic fibres in aortic media that often leads to aortic dilatation. Moreover, aortic stenosis (AS) may develop, even at a young age. Patients with AS can remain asymptomatic for many years. Among the asymptomatic patients with preserved LV systolic function the risk of death is about 1%, but increases significantly when symptoms occur. Deterioration of the left …
(IV-1C.3.o) Patient with Taussig-Bing syndrome Drabik L., MD, Tomkiewicz-Pająk L., MD, PhD, Prof. Olszowska M., MD, PhD, Prof. Podolec P., MD, PhD
Background Taussig-Bing syndrome is a rare congenital heart malformation consisting of transposition of the aorta to the right ventricle and malposition of the pulmonary artery with subpulmonary ventricular septal defect. Taussig-Bing syndrome is frequently associated with other cardiac abnormalities: right ventricular outflow tract obstruction, aortic arch obstruction and unusual coronary pattern [1].There are essential differences between Taussig-Bing syndrome and transposition of the great arteries with ventricular septal defect and overriding pulmonary artery. Taussig-Bing syndrome is characterized by true double-outlet right …
(IV-1D.1b) Patient with Ebstein anomaly, VSD and heart failure Prof. Andrzej Gackowski MD, PhD, Anton Chrustowicz MD, PhD
Background Ebstein anomaly is a rare congenital defect. Tricuspid valve in these patients is displaced towards the apex of the right ventricle. As a result part of the right ventricular muscle is located in functional atrium and the right ventricular volume is decreased comparing to normal. Depending on the degree of valve malformation and RV atrialisation different clinical presentation is possible – from mild symptoms to severe right heart failure. Case presentation We present a case of 35-years old lady …
(IV-1D.1b) 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 …
(IV-1C.3b) 21-year-old male with Truncus Arteriosus Type I Komar M. , Wilkołek P. , Kopeć G. , Sobień B. , Stępniewski J. , Tyrka A., Widlińska B., Podolec P.
Experts: Skalski J., Pająk J., Olszowska M., Tomkiewicz- Pająk L.
Background Truncus arteriosus communis (TAC) is a rare congenital heart disease appearing in 0.034 to 0.56 per 1,000 newborns and representing 1.4% to 2.8% of all cases of congenital heart diseases. In this anomaly, a single arterial trunk arises from the heart, overrides the interventricular septum, and supplies systemic, pulmonary, and coronary circulations. Without surgical treatment, 80% of patients die within the first year of life, usually in early infancy. Repair of TAC during the neonatal and early infant period …