(IV-1D.2d) 21-year old patient with bicuspid aortic valve Monika Smaś-Suska, Lidia Tomkiewicz-Pająk, Natalia Dłużniewska, Maria Olszowska, Piotr Podolec
EXPERTISE 1 “Development of the European Network in Orphan Cardiovascular Diseases” “Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”
( I – 2A.0; IV – 1D.2d ) Patient with artificial aortic valve with aneurysm of ascending aorta Natalia Dłużniewska MD; Lidia Tomkiewicz – Pająk MD, PhD; Aleksandra Lenart MD, Monika Smaś – Suska MD, Prof. Maria Olszowska MD, PhD; Prof. Piotr Podolec MD, PhD
BRIEF COMMENTS EXPERT: Assoc. Prof. Jacek Kołcz, MD, PhD, pediatric cardiac surgeon Affiliation: Department of Pediatric Cardiac Surgery, Jagiellonian University Medical College, Krakow, Poland COMMENT Ascending aorta’s diameter has shown widening tendency. It is difficult to determine if the implanted AV is still suitable for the patient. I recommend consideration of AVR procedure (for example implantation of biological valve and plasty of the ascending aorta). Bioprothesis might improve the patient’s quality of life. Aortic homograft implantation would make possible …
Posted in 1. Abnormalities of the position and connection of the heart and vessels, 2. Connective tissue disorders causing aneurysmal disease, 2. Left heart valves, A. Aneurysmal disease of the aorta, Case presentations, D. Valves, I. Rare diseases of systemic circulation, IV. Rare congenital cardiovascular diseases, Others
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(IV-1D.1o) 47-year-old patient with severe tricuspid regurgitation Sarnecka A. MD, Kopeć G. MD, PhD, Leśniak-Sobelga A. MD, PhD, Prof. Podolec P. MD, PhD
Background Pathological tricuspid regurgitation is more often secondary than due to a primary valve lesion. Isolated primary tricuspid regurgitation progresses slowly and can be well-tolerated for years. However, the data on the natural history of this heart disease suggest that the prognosis is poor. The timing of surgical treatment of severe isolated tricuspid regurgitation can be difficult. The valve repair is preferable method of surgery. Case presentation Presentation A 47-year-old man with severe tricuspid regurgitation and suspicion of Ebstein anomaly …
(IV-1D.2c) 20-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 Aortic stenosis (AS) is the most common valvular heart disorder. The prevalence increases with age and is estimated on 2-7% in population over 65 years old. Congenital AS is often associated with bicuspid aortic valve. Deterioration of the left ventricle (LV) systolic function is a predictor of the worse outcome after aortic valve replacement (AVR). Among the asymptomatic patients with preserved LV systolic function the risk of death is relatively low- about 1%, but increases significantly when symptoms occur. …
(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-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 …