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DORV in fetuses: how to consult future parents at a prenatal cardiology center? Retrospective analysis of 39 cases

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Soroka M., Słodki M., Moczulska H., Respondek-Liberska M.
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2014
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vol. 4
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issue 3
13-17
EN
DORV [double outlet right ventricle] is defined as a defect in which the great vessels leave entirely or mostly from above the morphologically right ventricle. The proposed by us new prenatal classification of heart defects for the DORV defect including the division into isolated and coexisting with extracardiac defect, facilitates consultation and predicting prognosis for the fetus and newborn. Isolated DORV in fetuses is classified as a severe elective defect (expected cardiac intervention or surgery at 1 month of age) with a relatively good prognosis for newborns, regardless of the type of intracardiac anomalies (in our series of cases 100% survival). DORV in the fetus with coexisting extracardiac defects (ECM) regardless of type of anomaly had poor prognosis (in our study group 100% demise rate).
2
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Suspected ovarian cancer in a young patient with a congenital heart defect and a history of cardiac surgery and pacemaker implantation – difficult therapeutic decisions

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Kaźmierczak K., Stempniewski T., Kufel-Grabowska J., Nowakowski B.
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EN
After uterine cancer, ovarian cancer is the second most common female reproductive tract malignancy. In Poland, in 2015, there were 3735 new cases and 2738 deaths from ovarian cancer. Over 80% of all cases are diagnosed in women aged over 50, whereas only 6% – before 35 years of age. Most cases are diagnosed, when clinical symptoms have already appeared, and the disease is at least locally advanced. The case presented below describes a very young 28-year-old woman with a tumour in the pelvis, ascites and very high Ca 125 and HE 4 levels. The clinical symptoms as well as laboratory and imaging tests were suggestive of epithelial ovarian cancer that required urgent surgery due to progression of the disease. The surgery posed a significant risk of complications, but owing to the deterioration of the patient’s condition it was considered necessary. The patient was born with a severe, complex heart defect, had undergone two cardiac surgeries, and had a pacemaker (first VVIR, then DDD). She was also intensively treated for iatrogenic HCV infection and hypothyroidism. A multidisciplinary team (a gynecologist, cardiologist and anesthetist) were behind the decision to carry out a major gynecological operation which ended with success. For the past 6 years, the patient has been under constant oncological follow-up and enjoys a good health.
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Missed Down Syndrome and Congenital Heart Defect in Prenatal Ultrasound (Us) - Malpractice or Not? Opinions of 12 Experts

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Respondek-Liberska M.
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2015
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vol. 5
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issue 1
19-25
EN
An example of missed Down syndrome with congenital heart defect by prenatal ultrasound evaluation was presented. A jury of 12 physicians, experts in prenatal ultrasonography and echocardiography were asked in questionare was this malpractice or not. The answers were very different. The results of the questionaires were discussed with the background to the selected data from Eurocat, from Polish National Prenatal Cardiac Registry, from Polish Registry of Congenital Malformations by 2nd year of life, and financial data of the Polish Prenatal Program in Lodz Region. Should we increase the cost of screening or the cost of ultrasound and echo training ? Or just provide patients with better knowledge regarding the differences between expertise of primary care obstetricians and experts in referral centers
4
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Serum concentration of copeptin in newborns with congenital heart defect

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Tarko A., Suchojad A., Maruniak-Chudek I., Kordyś J., Simonova S.
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EN
INTRODUCTION: Neonates with congenital heart defects (CHD) require careful fluid management due to the risk of cardiovascular failure. Routine laboratory parameters are not the optimal tool to detect fluid overload; therefore the search for novel markers is justified. Copeptin (pre-pro-vasopressin – CTproAVP) may be one of them. The aim of the study was to analyze the influence of the current protocol of hydration in neonates with CHD on the physiological volume homeostasis measured with CTproAVP. MATERIAL AND METHODS: Ten term newborns with CHD hospitalized in neonatal intensive care before cardiac surgery were enrolled in the study. Four of them presented symptoms of respiratory insufficiency and all except two received alprostadil. Clinical management was routine, with the exception of CTproAVP measurement in the first five days of life with evaluation of serum and urine osmolality. Fluid intake was within the normal range for age. Term, healthy neonates (N = 200) served as the control. RESULTS: The current hydration protocol did not cause an increase in serum and urine osmolality compared to the controls. The effective osmolality of the analyzed body fluids was even lower in the neonates with CHD. The concentration of CTproAVP was also lower in the study group, but the difference was not statistically significant. There were no clinical signs of cardiovascular distress or overhydration. No factors explaining the variability in CTproAVP concentration were identified. CONCLUSIONS: These preliminary data suggest that the protocol of hydration does not cause dehydration or stimulation of CTproAVP release. It seems that even more restrictive protocols of fluid management can be applied in newborns with CHD at the risk of pulmonary congestion. A longer observation period is needed, including the postoperative period, to obtain more reliable information on optimal fluid management and the role of CTproAVP in monitoring volemia.
PL
WSTĘP: Noworodki z wrodzoną wadą serca (WWS) wymagają ostrożnej terapii płynami z uwagi na ryzyko wystąpienia niewydolności krążenia. Rutynowe badania laboratoryjne nie są optymalnym narzędziem w identyfikacji stanu przewodnienia i dlatego konieczne są poszukiwania nowych markerów. Kopeptyna (CTproAVP) może być jednym z nich. Celem pracy była analiza wpływu aktualnie obowiązującego protokołu nawadniania noworodków z WWS na homeostazę wolemii z wykorzystaniem CTproAVP. MATERIAŁ I METODY: Do badania włączono 10 noworodków z WWS hospitalizowanych na oddziale intensywnej terapii noworodka przed zabiegiem kardiochirurgicznym. Cztery z nich prezentowały objawy niewydolności oddechowej, a wszystkie z wyjątkiem dwóch otrzymywały alprostadil. Postępowanie kliniczne było rutynowe, z wyjątkiem pomiaru CTproAVP w pierwszych pięciu dniach życia wraz z oceną osmolalności surowicy i moczu. Podaż płynów mieściła się w zakresach normy dla wieku. Grupę kontrolną stanowiło 200 zdrowych donoszonych noworodków. WYNIKI: Aktualny protokół nawadniania nie powodował, w porównaniu z grupą kontrolną, wzrostu stężenia osmolalności surowicy i moczu. Efektywna osmolalność analizowanych płynów ustrojowych była nawet niższa u noworodków z WWS. Również stężenie CTproAVP było niższe w grupie badanej, lecz różnica nie była istotna statystycznie. Nie obserwowano klinicznych objawów niewydolności krążenia lub przewodnienia. Nie zidentyfikowano czynników, które wyjaśniałyby zmienność w stężeniu CTproAVP. WNIOSKI: Wstępne wyniki sugerują, że aktualny protokół nawadniania nie powoduje odwodnienia ani nie stymuluje uwalniania CTproAVP. Wydaje się, że u noworodków z WWS i ryzykiem przeciążenia krążenia płucnego można za-stosować nawet bardziej restrykcyjny protokół nawadniania. Konieczna jest dłuższa obserwacja, z włączeniem okresu pooperacyjnego, aby otrzymać bardziej miarodajne dane na temat optymalnego nawodnienia i roli kopeptyny w monitorowaniu wolemii.
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