(I-1B.3) Adult patient with vascular ring Prof. Piotr Podolec MD, PhD, Lidia Tomkiewicz-Pająk MD, PhD, Leszek Drabik MD, Henryk Olechnowicz MD PhD, Prof. Maria Olszowska MD, PhD

Background Vascular ring is rare congenital malformation, it accounts for less than 1% of all congenital heart problems. The condition occurs as often in males as females. Some infants with vascular ring also have another congenital heart problem. With vascular ring, some of the arches and vessels that should have changed into arteries or disappeared are still present when the baby is born. The primary symptomatology associated with vascular rings relates to the structures that are encircled by the ring, …

Posted in 1. Anatomical malformations of the arteries, 3. Aortic rings, B. Aorta and aortic arch main branches, Case presentations, I. Rare diseases of systemic circulation | Leave a comment

(I-1C.O) 28 -year – old man with anomalous origin of the right coronary artery from the ascending aorta. Sylwia Wiśniowka-Śmiałek MD, Agata Leśniak-Sobelga MD, Paweł Rubiś MD, Ph.D, Maciej Krupiński MD.

Background Coronary artery anomalies (CAAs) are a diverse group of congenital malformations, which clinical manifestations and pathology are highly variable . Being rare, they are recognized cause of myocardial ischemia and occasionally sudden cardiac death . The frequency of anomalous aortic origin of the coronary arteries is noted in 0,6 to 1,2 % of patients (based on coronary angiography), with significant higher incidence in young victims of sudden cardiac death in comparison to adults (4-15% versus 1% respectively). There is …

Posted in 1. Anatomical malformations of the arteries, C. Coronary arteries, Case presentations, I. Rare diseases of systemic circulation, Others | Leave a comment

(I-1B.6) Adult patient with coarctation of the aorta Lidia Tomkiewicz-Pająk MD, PhD, Justyna Błaut-Jurkowska MD , Maria Olszowska MD, PhD, Prof. Piotr Podolec MD, PhD, Prof. Tadeusz Przewłocki MD, PhD, Jakub Stpniewski MD, PhD

Background Coarctation of the aorta accounts for 5–8% of all congenital heart defects. The male-to-female ratio is 2:1. It is defined as a narrowing of the aorta, typically located in the area where the ductus arteriosus inserts.Coarctation of the aorta may occur as an isolated form or in association with various other cardiac and non-cardiac lesions. Signs and symptoms depend on the severity of CoA. Patients with serious CoA exhibit signs and symptoms early in life, while particularly mild cases …

Posted in 1. Anatomical malformations of the arteries, 6. Coarctation of the aorta, B. Aorta and aortic arch main branches, Case presentations, I. Rare diseases of systemic circulation | Leave a comment

(I-1C.4) Patient with coronaro-pulmonary fistula Justyna Błaut-Jurkowska MD, Leszek Drabik MD, Piotr Musiałek MD, PhD Lidia Tomkiewicz-Pająk MD, PhD, Bartosz Laskowicz MD, Prof. Maria Olszowska MD, PhD, Prof. Piotr Podolec MD, PhD, Prof. Tadeusz Przewłocki MD, PhD

Background Coronary artery anomalies affect about 1% of the general population. The definition of the abnormal versus the normal coronary anatomy presents a complex problem. Defect may affect number of ostia, proximal course or termination. Many coronary anomalies don’t cause symptoms and remain undetected. However, they can be associated with chest pain, sudden death, cardiomyopathy, arrythmias and myocardial infarction. Case presentation We report a case of a 61-year-old Caucasian man referred to our Clinic. He had a history of coronary …

Posted in 1. Anatomical malformations of the arteries, 4. Coronary fistula, C. Coronary arteries, Case presentations, I. Rare diseases of systemic circulation | 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-1B.6) 33 year-old patient with coarctation of the aorta and tetralogy of Fallot Lidia Tomkiewicz-Pająk, Tomasz Pawelec

Background Tetralogy of Fallot (TOF) is one of the most common congenital heart development disorders (CHDs). This condition is classified as cyanotic heart disorder. It consists of right ventricular (RV) outflow tract obstruction (RVOTO) (infundibular stenosis), ventricular septal defect (VSD), aorta dextroposition, and right ventricular hypertrophy. Patients with tetralogy of Fallot initially present with cyanosis shortly after birth, thereby attracting early medical attention. Surgical correction is a procedure of choice. It is usually performed in the infancy. Sometimes reoperations are …

Posted in 1. Anatomical malformations of the arteries, 6. Coarctation of the aorta, B. Aorta and aortic arch main branches, 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-1B.6) Adult patient with coarctation of the aorta (CoA) Danuta Sorysz MD. PhD, Barbara Zawiślak MD

Background Coarctation of aorta is a common congenital malformation that is usually treated in childhood. Adult patient with CoA have a important incidence of associated cardiac disorders include bicuspide aortic valve (up to 85%), parachute mitral valve and atrial fibrillation, ischemic heart disease. Clinical features include upper body systolic hypertension, lower body hypotension, a blood pressure gradient between upper and lower extremities (20 mmHg indicates significant CoA), radiofemoral pulse delay. Diagnostic methods are echocardiography, CT, NMR, angiography. In case of …

Posted in 1. Anatomical malformations of the arteries, 6. Coarctation of the aorta, B. Aorta and aortic arch main branches, 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-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 …

Posted in 2. Giant-cell arteritis, 3. Autoimmune vascular diseases, A. Primary systemic vasculitis: Predominantly large arteries, Case presentations, I. Rare diseases of systemic circulation | Leave a comment