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.
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.
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.
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.
Arkadiusz Michalak, Marta Witczak, Elżbieta Kukawczyńska, Marek Niwald and Maria Respondek-Liberska
Our clinical report describes a rare finding of a prenatally-detected congenital atrioventricular (AVB) block without associated maternal antibodies, which progressed from 1st/2nd degree AVB to complete heart block during second half of pregnancy. Obstetrical ultrasound at 12th week did not reveal any abnormalities and prenatal echocardiography (due to VSD in afamily member) at the 18th week of gestation detected 1st degree block, then bigeminy and bradycardia. Transplacental treatment with B-2-mimetics was introduced. The delivery was organized in a tertiary center and a pacemaker for the newborn baby was secured and implanted in 15th day of life. Currently the boy`s condition is good and stable. Before therapy with B-2-mimetics the mother underwent echocardiography and ECG which revealed clinically silent structural and conduction heart abnormalities. Literature findings suggest that parents of children with non-immune congenital or childhood AVB are more likely to carry clinically silent conduction abnormalities than general population. Given the corresponding findings in the mother and her son, they should be good candidates for genetic testing.
Katarzyna Zych-Krekora, Anna Wójtowicz, Michał Krekora, Maciej Słodki, Hugues Gentillon and Maria Respondek-Liberska
It was the second pregnancy of an otherwise healthy married couple. The fetus (male) had detailed echocardiography monitoring in the second half of the pregnancy due to progression of cardiomegaly, and echocardiographic features of congestive heart failure. Marfan syndrome was suspected based on cardiac anomalies. For the first time, the rupture of aneurysm of aortic sinus Valsalva was documented. Despite transplacental treatment with digoxin there was fetal demise at the 34th week of gestation and postmortem newborn phenotype confirmed prenatal diagnosis.
Marfan Syndrome is a rare genetic anomaly which can be diagnosed prenatally by detailed echocardiography, usually with bad prognosis (just opposite to “benign” case diagnosed later on in life span). The most common prenatal cardiac manifestations are cardiomegaly with signs of cardiac insufficiency. We present the case with new echocardiographic features.
Alexander N. Goel, Camila Reyes, Shauna Mclaughlin, Mark Wittry and Andrew C. Fiore
Retroesophageal course of the left brachiocephalic vein is a rare variant seen in patients with congenital heart disease. However, this anomaly without associated cardiac or aortic abnormalities is nearly unheard of, with only one prior case described in the literature. We present an infant with anomalous retroesophageal left brachiocephalic vein that was an incidental finding on computed tomography (CT). We also briefly discuss its embryologic and clinical significance.
Four cases of missed prenatal diagnosis by an experienced ultrasonographer and a fetal cardiologist from a tertiary teaching hospital are presented: 3-mm peri-membrane ventricular septal defect; hypoplastic aortic arch requiring prostin infusion after delivery; esophageal atresia with tracheal fistula; and right-sided diaphragmatic hernia. Freezed frames and cine loops of the prenatal ultrasound scans indicated that the missed anomalies were not visible in midgestation, suggesting that in the future, repeat ultrasound scans should be performed before delivery to reduce the occurrence of such diagnostic errors.
Joanna Płużańska, Jacek Więcek, Michał Krekora, Jolanta Kiełbasicz-Binikowska, Tomasz Talar, Maciej Słodki and Maria Respondek-Liberska
We present the case of aneurysm of the muscular intraventricular septum with accompanying cardiomegaly and abnormal venous flow patterns requiring emergency cesarean section and specialized neonatal treatment. At the age of 8 months our patient has no clinical symptoms with a 6 mm scar in intraventricular muscular septum..
The hydropic fetus with atrial flutter has high risk of fetal demise. In utero treatment is not as successful as SVT with 1:1 conduction. Even after conversion to sinus rhythm, close follow-up of the pregnancy is required for several reasons..
A 25 year old primigravida, at 26 wks of gestation had a fetal ultrasound which showed a tachycardic hydropic fetus with AFI 49. The rhythm was atrial flutter: AR 400 bpm and VR 200 bpm. The heart was structurally normal. Transplacental antiarrhythmic treatment with sotalol was started and 24 hours later, the fetus was still primarily in flutter with rare episodes of sinus rhythm and frequent PAC. Sotalol was increased but the mother’s QTc increased to > 500 ms, so the sotalol dose was reduced. On day 4th direct i.m. of digoxin was given and fetus was in sinus rhythm. Pharmacotherapy was continued. On the 16th day of sinus rhythm (at 33 wks), a marked change in FHR variability was seen. An US revealed the fetus was in sinus rhythm with a normal FHR. Because of the decreased FHR variability, the fetus was delivered by CS and the cord pH was 7.19, Apgars 1, 9 and 9. The neonate received no antiarrhythmic medications. On day 6 of postnatal life, an AV re-entrant tachycardia (AVRT) developed and sinus rhythm was successfully restored with sotalol and digoxin. The infant was treated for 18 months with no episodes of SVT or atrial flutter.