(II-1.A.4d) 30-year-old patient with arterial pulmonary hypertension and ascending aortic aneurysm Natasza Herman, Paweł Prochownik, Urszula Gancarczyk, Monika Komar, Bartosz Sobień, Piotr Podolec

  EXPERTISE 1 “Development of the European Network in Orphan Cardiovascular Diseases” “Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”

Posted in 1. Pulmonary hypertension, 4. PAH associated with, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.1) 51-year old woman with idiopatic pulmonary arterial hypertension, atrial flutter and thrombus in right atrium Barbara Widlińska, Marcin Waligóra, Anna Tyrka, Monika Komar, Agnieszka Sarnecka, Mateusz Brózda, Paweł Prochownik, Urszula Gancarczyk, Natasza Herman, Piotr Podolec

  EXPERTISE 1   “Development of the European Network in Orphan Cardiovascular Diseases” “Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”

Posted in 1. Idiopathic PAH, 1. Pulmonary hypertension, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.4d) 71-year-old patient with patent ductus arteriosus P. Prochownik, A. Sarnecka MD, G. Kopeć MD, PhD, M. Komar MD, PhD, Prof. P. Podolec MD, PhD

  BRIEF COMMENTS EXPERT: Krzysztof Szymoński, MD Affiliation: Department of Cardiovascular Surgery and Transplantology, John Paul II Hospital, Krakow, Poland COMMENT Due to current ESC guidelines the patient qualifies for PDA closure. Tricuspid valvuloplasty may also be considered. EXPERT: Grzegorz Kopeć, MD, PhD Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow COMMENT Because there is no possibility of pulmonary hypertension targeted treatment the patient qualifies for PDA closure. Coexisting tricuspid regurgitation seems to be secondary to …

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(II-1A.1) Idiopathic pulmonary artery hypertension

  BRIEF COMMENTS EXPERT: Prof. Henryk Siniawski MD, PhD, cardiac surgeon Affiliation: Deutsches Herzzentrum, Berlin, Germany COMMENT What was the initial function of the heart – in case of iPAH usually only lungs transplantation is performed, RV function often normalizes after lung-transplantation. Perhaps solitary lung transplantation would safe patient’s coronary arteries. EXPERT: Prof. Egle Ereminiene MD, PhD Affiliation: Hospital of Lithuanian University of Health Sciences, Kaunas COMMENT Poor RV function was the main reason of complex lungs and heart transplantation. …

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(II-1A.5) 41-year-old patient with chronic thromboembolic pulmonary hypertension Marcin Waligóra MD, Agnieszka Sarnecka MD, Grzegorz Kopeć MD, PhD, Prof. Piotr Podolec MD, PhD

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Background Acute pulmonary embolism is rarely complicated by development of pulmonary hypertension (PH) with estimated incidence rate of 0,5-2%. It is almost impossible to determine the overall prevalence of chronic thromboembolic pulmonary hypertension (CTEPH) since not all of these patients have a history of acute pulmonary embolism. For patients after acute pulmonary embolism, diagnosis can be made after 3 months of effective anticoagulation, and remaining signs of PH. Final diagnosis can be made after confirming pre-capillary PH during right heart …

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(II-1A.4b) Pulmonary arterial hypertension associated with HIV and HCV infection Michał Furdal, Ewa Mroczek, Jakub Stępniewski

Background Pulmonary arterial hypertension (PAH) is a rare, but one of the most frequent complications of HIV infection. 3- year survival of patients with HIV related PAH (HIV-PAH) remaining in functional class III or IV by WHO (FC WHO) reaches approximately 20-30%. It is believed that highly active antiretroviral therapy (cART) or dysfunction of immune system and CD4 lymphocytes has less likely influence on the occurrence or the course of PAH. There is still limited data on the utilization of …

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(II-1A.5) A 39-year-old woman with atypical variant of Klippel-Trenaunay syndrome and progressive thromboembolic pulmonary hypertension, successfully treated by pulmonary thromboendarterectomy Poręba M, Misztal M, Biederman A, Skrzypek A, Szatkowski P, Pirsztuk E, Czernecka-Mroczek D, Gackowski A , Nessler J

Background Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular malformation of veins, capillaries and lymphatic vessels which can be associated with thromboembolic events. We report on a female with severely complicated course of the disease. Case presentation We present a case of 39-year-old female with Klippel-Trenaunay syndrome (KTS) complicated by deep vein thrombosis and chronic thromboembolic pulmonary hypertension (CTEPH). Our patient was operated in adolescence and severe varicosities of her lower limb were excised. After second pregnancy she developed pulmonary …

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(II-1A.4d) 47 years old female with Patent Ductus Arteriosus and Pulmonary Hypertension. Jakub Stępniewski MD, Grzegorz Kopeć MD, PhD, Hanna Dziedzic-Oleksy MD, Anna Tyrka MD, Prof. Piotr Podolec MD, PhD

Background Ductus arteriosus (DA) is a vascular connection between the main pulmonary artery and the aorta that allows blood to bypass the fetus’s non-functioning lungs. After delivery, the DA undergoes active constriction and obliteration eventually. A patent ductus arteriosus (PDA) occurs when the DA fails to close postnatally. PDA is an uncommon clinical finding in adult population. Improvement of survival of premature infants at risk for PDA and an increase in cases discovered incidentally, the incidence of PDA is rising …

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(II-1A.4d) Patient with ventricular septal defect and Eisenmenger syndrome with gynecological complications Magdalena Kaźnica-Wiatr MD, Magdalena Nowacka MD, Grzegorz Kopeć MD, PhD, Maria Olszowska MD, PhD, Prof. Piotr Podolec MD, PhD

Background Eisenmenger syndrome is defined as pulmonary vascular obstructive disease which develops as a consequence of preexisting large left-to-right shunt such that pulmonary artery pressures approach systemic values and the direction of the flow becomes bidirectional or right-to-left. Congenital heart defects that can lead to Eisenmenger syndrome include “simple” defects like ASD, VSD, PDA or “complex” like AVSD, truncus arteriosus, aortopulmonary window and univentricular heart. Elevated pulmonary vascular resistance usually occurs in infancy, by the age of 2 years. Clinical …

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(II-1A.5) Chronic thromboembolic pulmonary hypertension Skaidrius MiliauskasMD, PhD, Deimante Hoppenot MD, PhD

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening condition in which organised thrombi obstruct the pulmonary vessels, causing increased pulmonary vascular resistance, progressive pulmonary hypertension (PH) and right heart failure. CTEPH is associated with considerable morbidity and mortalities. Even if more recent papers suggest that the prevalence of CTEPH is up to 3.8% in survivors of acute pulmonary embolism, most experts believe that the true incidence of CTEPH after acute pulmonary embolism is 0.5–2%. CTEPH can be found in …

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