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Open access

Aneta Neskoromna-Jędrzejczak, Katarzyna Bogusiak, Krzysztof Chojnowski, Marta Robak and Jacek Treliński

Abstract

The preparation of patients with hemophilia before surgical operations and dental procedures constitutes a significant clinical challenge. This article presents the implantoprosthetic rehabilitation of a patient with severe hemophilia B (factor IX activity <1%). The patient was prepared for the surgical procedure with recombinant factor IX concentrate (Rixubis) during the clinical surgery study. Tooth extraction and the implantation of four dental implants in the mandible were planned: one dental implant of 3.7 mm diameter and 10 mm length in the place of tooth 35, and another of 3.2 mm diameter and 10 mm length in the place of tooth 37. The next two implants were implemented 1 month later: one implant 3.7 mm in diameter and 10 mm in length in the place of tooth 46, and another implant 3.2 mm in diameter and 10 mm in length in the area of tooth 44. Appropriate substitution of the missing coagulation factor, together with the use of local hemostatic therapy, allowed dental implantation to be performed without excessive blood loss in this patient with severe hemophilia B.

Open access

Takeshi Sugimoto, Kazuhide Morimoto, Hiromi Hashimoto, Yukie Kaneda, Shinya Ohata and Yoshiro Yasutomo

Abstract

An 88-year-old female was admitted with autoimmune hemolytic anemia (AIHA). Coagulation test revealed severe prolongation of activated partial thromboplastin time (APTT). APTT cross-mixing test with patient plasma and normal plasma demonstrated an inhibitory pattern. Several intrinsic coagulation factor activities, particularly factor IX, showed remarkable decreases, and the inhibitor titers for coagulation factors VIII and IX were elevated. Although AIHA with existing antiphospholipid (aPL) antibodies was diagnosed initially, purpura developed on extremities intermittently during the clinical course. Considering the possibility of coexisting acquired hemophilia, APTT cross-mixing test with patient’s plasma and equal amount of the recombinant factor VIII product instead of normal plasma was performed. The APTT value on equal mixing samples with patient plasma and recombinant factor VIII product was decreased to within the normal range, and coagulation factor IX activity was restored. These results indicate that the recombinant factor VIII product had a neutralizing effect on aPL antibodies. We concluded that recombinant factor VIII product may lead to the repair of incorrect results from the APTT-dependent diagnostic system in the presence of aPL antibodies.

Open access

Monika Adamska, Anna Komosa, Tatiana Mularek, Joanna Rupa-Matysek and Lidia Gil

Abstract

Cardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease. Delay in diagnosis and treatment may have a major impact on the prognosis and outcomes of patients. This review focuses on the presentation of the disorder and current novel approaches to the diagnosis of cardiac involvement in AL amyloidosis.

Open access

Agnieszka Żalińska, Sara Korabiewska, Michał Krekora, Krzystof Michalak, Marek Kopala, Ewa Cichos, Anna Romanowicz, Maciej Słodki and Maria Respondek-Liberska

Abstract

Introduction: Fetal cardiac tumors are anomalies, that occur rarely: from Nationwide Register of Fetal Cardiological Problems in Poland in years 2004-2016 amongst 8112 fetuses with cardiological problems, there were 85 fetuses with cardiac tumors, including 52 cases of multiple cardiac tumors (0,64%) and 33 of single anomalies (0,4%). Material: This analysis included 13 cases from single tertiary fetal cardiac center Lodz in years 1993-2017. Results: Ten out of 13 fetuses with single cardiac tumors (SFCT) had cardiomegaly: on average HA/CA was 0,49. The size of the tumor was different: the smallest one - 6 x 6 mm, the biggest 47 x 47 mm. The way of the delivery: in 10 cases there was CS and in 3 cases natural delivery. Birth weight was from 2000-3950 g (average 2989,2 g). Cardiosurgical resection of the tumor was performed on 4 newborns: at 2nd, 4th, 8th and 16th day of life (average 7,5 day). Four neonatal deaths were registered (31%): in 1st 2nd and 11th day (before surgery) and in the 28th day after the operation. Conclusions: Single fetal cardiac tumors (SFCT) can be diagnosed at 20 weeks of pregnancy, which allows to start echocardiographic monitoring, taking into consideration the potential risk of hemodynamic progression. SFCT can be the first sign of tuberous sclerosis complex in later prenatal or postnatal life. SFCT other than rhabdomyoma can be asymptomatic in newborn, but may require an early cardiosurgical resection.

Open access

Paulina Kordjalik, Zdzisław Tobota and Maria Respondek-Liberska

Abstract

Introduction: Analysis have been subjected to evaluate standard data reports from the Polish National Prenatal Cardiac Pathology Registry from the year 2016, compared to previous years. Material and methods: The methodology of this work was comparison of the data from previous years that have been published in the Prenatal Cardiology and records generated in www. orpkp.pl from the year 2016. The total number of records entered in the Registry in the year 2016 was n=774. Results: Top 10 most common prenatal CHD in 2016 were such as follow: HLHS, AVSD, VSD, d-TGA, TOF, AvS, muscular VSD, Aberrant origin R subclavian artery, RAA, CoA. In the centers Lodz and Krakow the most common prenatal congenital heart defects were severe CHD requiring surgery in 1 st month of life. In the contrast, in Warsaw the first place was taken by a “critical heart defects” regardless whether cardiac surgery was planned in first week (contemporary definition of prenatal critical heart defects) or first month - contemporary definition of severe planned heart defects of postnatal life. Conclusion: The fact that in 2016 most common cause of referrals to targeted the fetal chocardiography was abnormal, large vessels view and not the abnormal 4 chamber view of the heart, suggests more and better training of hysicians performing the screening or basic study of fetal heart in Poland.

Open access

Oliver Graupner, Christian Enzensberger, Larissa Wieg, Aline Wolter, Can Yerebakan, Markus Khalil and Roland Axt-Fliedner

Abstract

Introduction: The long-term outcome of patients with HLHS (hypoplastic left heart syndrome) is mainly determined by right ventricular function. Our study examines, whether there are differences in segmental right ventricular myocardial displacement and tissue velocities of fetuses with HLHS compared to healthy fetuses during gestation. Materials and methods: A prospective study was conducted including 20 fetuses with HLHS and 20 gestational age matched controls. c-TDI (colour tissue Doppler imaging) derived systolic and diastolic velocities as well as myocardial displacement were assessed in three different locations of the right ventricle (RV). A ROI (region of interest) was placed in the basal, middle and apical part of the myocardium. Possible changes of c-TDI indices in the course of pregnancy and between the three different segments were investigated in both groups. Results: HLHS fetuses showed significantly lower e' velocities measured in the basal and middle part of the RV compared to healthy controls (p < 0.05). Basal displacement showed significantly lower values in HLHS fetuses compared to controls. In control fetuses but not in HLHS fetuses there was a significant increase of basal diastolic velocities and displacement in the course of pregnancy. According to myocardial velocities and displacement values there was a significant decrease from the base of the fetal heart to the apex pointed in both groups. Conclusions: An altered right ventricular myocardial function in HLHS fetuses within different myocardial segments could be demonstrated. An apicobasal gradient with higher velocity and displacement values in the basal part of RV myocardium could be found in both groups. The technique may be of value in the prenatal assessment of myocardial function, however its role as a monitoring tool and outcome predictor needs to be defined.

Open access

Iwona Strzelecka, Maciej Słodki, Katarzyna Zych-Krekora, Michał Krekora, Mariusz Grzesiak, Iwona Maroszyńska and Maria Respondek-Liberska

Abstract

Introduction: Congenital heart defects are the most frequent reason for deaths during the neonatal and early infancy periods. The aim of this study was to retrospectively analyze singleton pregnancy outcomes of premature neonates with congenital cardiac defects delivered by Cesarean section. Materials and methods: A retrospective analysis was performed on 10,800 fetuses evaluated in our referral fetal cardiac center between 2010 and 2016. A group of 58 singleton pregnancies was selected with the following criteria: fetal heart defect, Cesarean section (C-section), and gestation of 37 weeks or less. Exclusion criteria included labor outside of our hospital and multiple pregnancy. Results: Isolated heart defects constituted 74,1% (43 cases) of the analysed data set. The majority of newborns were delivered at 36 weeks of gestation (43,1%), with an average of 33,6 weeks. In one case (1,7%), C-section took place at 22nd week. Birth weight of newborns < 2500g constituted 51,7% (30 cases). Neonatal deaths occurred in 60,3% (35 cases). Conclusions: Preterm neonates with congenital heart defect, delivered by C-section in our reference centre, during 2010 to 2016, had generally poor outcomes and high mortality rate. The average hospital stay of surviving neonates was approximately two months. An improvement of knowledge about prenatal cardiology is necessary in obstetrician management with fetuses with congenital heart diseases.

Open access

Julia Murlewska, Maciej Słodki, Roland Axt-Fliender, Giusseppe Rizzo, Mark Sklansky, Stuart Weiner, Isaac Blickstein and Maria Respondek-Liberska

Abstract

International Prenatal Cardiology Collaboration Group (IPCCG) links specialists from prenatal cardiology all over the world. In this recommendation we would like to focus on the fetal/prenatal echocardiography official report. So far many recommendations focused mainly on technical aspects of the fetal heart examination.

Open access

Agnieszka Nawara-Baran

Abstract

Vein of Galen Malformation (VGM) it is the most common cerebral arteriovenus malformation in fetuses and children. Usually VGM causes volume overload of the heart and can induce mass effect in the brain, causing progressive neurological impairment. Modern treatment allow on earlier therapy (before the 5th monthof life). This gives the newborn with isolated VGM and heart failure a chance of survival. This work presents case of isolated vein of Galen malformation with diagnosed cardiomegaly, monophasic flow through the tricuspid valve, large right heart, pericardiac effusion, dilatation of SVC and abnormal doppler exam. Postnatal management included aggressive medical treatment of cardiac failure and early transarterial embolization of the selected vessels feeding the aneurysm at 6 days of age. The newborn is 6 month old, growing normally without neurological deficits or developmental retardation and waiting of next stage therapy. New techniques of treatment give fetuses with VGM and prenatal signs of cardiac insufficiency a chance of survival and healthy life, in a group of selected cases. The analysis of the cardiovascular system in fetuses with VGM according to uniform criteria is essential in order to assess the influence of these malformations on the survivability of newborns.