Agnieszka Żalińska, Sara Korabiewska, Michał Krekora, Krzystof Michalak, Marek Kopala, Ewa Cichos, Anna Romanowicz, Maciej Słodki and Maria Respondek-Liberska
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.
Paulina Kordjalik, Zdzisław Tobota and Maria Respondek-Liberska
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.
Oliver Graupner, Christian Enzensberger, Larissa Wieg, Aline Wolter, Can Yerebakan, Markus Khalil and Roland Axt-Fliedner
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.
Iwona Strzelecka, Maciej Słodki, Katarzyna Zych-Krekora, Michał Krekora, Mariusz Grzesiak, Iwona Maroszyńska and Maria Respondek-Liberska
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.
Julia Murlewska, Maciej Słodki, Roland Axt-Fliender, Giusseppe Rizzo, Mark Sklansky, Stuart Weiner, Isaac Blickstein and Maria Respondek-Liberska
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.
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.
Maria Respondek-Liberska, Maciej Łukaszewski, Adam Oleś, Michał Podgórski, Piotr Grzelak and Maciej Słodki
Introduction: Fetal echocardiography is a method of choice for diagnosing cardiovascular anomalies prenatally. However, in the majority of cases, the complexity of a defect creates a diagnostic challenge. Moreover, postnatal validation of sonographic findings rarely can be obtained. Nevertheless, the feedback is vital for improving diagnostic capabilities. Thus, the aim of this research was to compare results of prenatal echocardiography with postnatal angio-CT in patients with anomalies of great vessels. Material and methods: We retrospectively compared results of prenatal echocardiography and postnatal angio-CT in 10 patients with selected anomalies of the aortic arch. This was a qualitative analysis, thus discrepancies in recognized anomalies were compared between these two modalities. Results: In 8/10 patient diagnoses were fully consistent. Nevertheless, the tiny caliber of vessels created a diagnostic challenge (e.x. to differentiate the hypoplastic aortic arch from the aortic arch interruption). In the remaining case, the discrepancy was due to a problem with complete visualization of all branches of the aortic arch in prenatal ultrasound. Conclusions: Fetal echocardiography in tertiary center was a reliable method for assessment of great vessels anomalies. However, critically narrow vessels remain a diagnostic challenge and neonatal angio-CT seems to be the method of choice in cases of diagnostic doubts.
Iwona Strzelecka, Eliza Michalska, Katarzyna Zych-Krekora and Maria Respondek-Liberska
Introduction: Echocardiography of the fetal heart is an ultrasound examination that allows the evaluation of the anatomical structure and cardiovascular system usually performed in first half of pregnancy. Material and methods: This work presents the data of 107 fetuses with normal heart anatomy (NHA) and normal heart study (NHS) and their neonatal follow-up. In this group (in an addition to routine prenatal work-up) has been performed also a echocardiography examination in the third trimester of pregnancy, after 37th week of gestation: 61% of pregnant women were referred to the prenatal cardiology center due to the presence of high-risk pregnancies and 39% were low-risk pregnancies. Results: In two cases episodes of fetal arrhythmias were present during obstetrical examinations. After birth in the study group of 107 fetuses, 72% of newborns left the hospital during the 4 days and 28% newborns stayed in the hospital for longer time. In 16 cases their stay was extended due to maternal reasons and in 14 for newborns reasons. Conclusions: 1) In the neonate group, after prenatal cardiac evaluation > the 37th week of gestation such as “ normal fetal heart anatomy & normal heart study”, all newborns in our center were born in good general condition. 2) Late prenatal echocardiography in 3rd trimester of pregnancy maybe considered as additional tool to prove fetal well being, specially in high risk pregnancies.
Maria Respondek-Liberska, Jerzy Węgrzynowski, Przemysław Oszukowski, Ewa Gulczyńska, Elżbieta Nykiel, Lucjusz Jakubowski, Mariusz Grzesiak, Ewa Czichos and Hanna Romanowicz
This is a case report about very rare findings in 2nd half of pregnancy (after normal 1 trimester scan ) at 18th week of gestation fetal macrosomia was detected unrelated to maternal diabetes, and acceleration fetal growth later on with unusual cardiac abnormalities (fetal cardiomegaly, cardiomyopathy, partial abnormal venous connection ). Progressive features of congestive heart failure with polyhydramnios in a fetus with estimated 5500 g predicted a poor outcome and severe neonatal condition, which was presented and discussed with the parents to be. Casearean section was performed at 33rd weeks of gestation due to maternal dyscomfort, severe legs edema and her tachypnoe. Baby boy was delivered with birth weight of 5050g, Apgar 4 with mutiple tumors. Conservative care was introduced and neonated died on the 3rd day. Differential diagnosis was discussed with special attention to Costello syndrome however without proved by genetic make-up from neonatal blood.