( 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 | Leave a comment

(I-2A.1) 29 y.o. female with suspiction of Marfan Syndrome, after ischemic stroke, with patent foramen ovale and dissection of the descending aorta Hanna Dziedzic-Oleksy MD, Jakub Stępniewski MD , Monika Komar MD, PhD, Grzegorz Kopeć MD, PhD, Jakub Podolec MD.

Background Marfan’s Syndrome is an autosomal dominant disorder of a connective tissue [1]. It affects many organs causing abnormalities of the cardiovascular, musculoskeletal and central nervous systems, eyes, lung, skin and the dura [2]. The classical cardiovascular manifestations of Marfan’s Syndrome are mitral valve prolapse and aortic root dilation, leading to aneurysm and dissection. A PFO (patent foramen ovale) is a flap-like opening between the left and right atria of the heart. It usually fuses shut after birth, but in …

Posted in 1. Marfan syndrome, 2. Connective tissue disorders causing aneurysmal disease, A. Aneurysmal disease of the aorta, Case presentations, I. Rare diseases of systemic circulation | Leave a comment

(I-2A.1) Patient with Marfan syndrome, aortic aneurysm and chest deformation Małgorzata Konieczynska MD, PhD, Ewa Wicher-Muniak MD, PhD, Magdalena Urbanczyk-Zawadzka MD, Agnieszka Pająk MD

Background Marfan syndrome is a disorder of connective tissue with wide variability in clinical symptoms. It affects the skeletal and cardiovascular systems, eyes, and skin. The disease is inherited mostly in autosomal dominant way, but about 25% cases are sporadic. It is caused by mutations in the fibrillin-1 gene. The most common cardiovascular complications are: aortic dilation, aortic valve insufficiency and mitral valve prolapse. Case presentation A 18-year-old male with Marfan syndrome was referred to hospital due to class II …

Posted in 1. Marfan syndrome, 2. Connective tissue disorders causing aneurysmal disease, A. Aneurysmal disease of the aorta, Case presentations, I. Rare diseases of systemic circulation | Leave a comment

(I-2A.1) 23 years old female with Marfan Syndrome and deformity of the spine Hanna Dziedzic-Oleksy MD, Lidia Tomkiewicz-Pająk MD, Prof. Piotr Podolec MD, PhD

Keywords: Marfan’s Syndrome, spine deformity, aortic aneurysm, pregnancy Background Marfan’s Syndrome is an autosomal dominant disorder of a connective tissue. It affects many organs causing abnormalities of the cardiovascular, musculoskeletal and central nervous systems, eyes, lung, 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. Case presentation A 23 y.o. Caucasian female (hight-169cm, arachnodactyly with wrist and thumb sign, severe chest deformity due to thoracic …

Posted in 1. Marfan syndrome, 2. Connective tissue disorders causing aneurysmal disease, A. Aneurysmal disease of the aorta, Case presentations, I. Rare diseases of systemic circulation | Leave a comment

(I-2A.1) 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 …

Posted in 1. Marfan syndrome, 2. Connective tissue disorders causing aneurysmal disease, A. Aneurysmal disease of the aorta, Case presentations, I. Rare diseases of systemic circulation | Leave a comment