Maciej Słodki

Measurement of the Great Vessels in the Mediastinum Could Help Distinguish True From False-Positive Coarctation of the Aorta in the Third Trimester

Objective. We investigated the utility of analyzing prenatal mediastinal measures of the great arteries in distinguishing true coarctation of the aorta (CoA) from false‐positive CoA. Methods. All fetuses in this study had suspicion of CoA based on the presence of right‐left heart disproportion. We defined 3 study groups: group 1, true fetal CoA; group 2, false‐positive fetal CoA with a narrow aortic arch; and group 3, false‐positive fetal CoA without a narrow aortic arch. Results. In group 1, the mean mediastinal pulmonary artery (PA) to ascending aorta (Ao) diameter ratio ± SD was 2.03 ± 0.48, and in group 2, the ratio was 1.60 ± 0.23. The difference was statistically significant (P = .0018, t test). In group 3, the mean PA:Ao ratio was 1.35 ± 0.14. The difference between groups 1 and 3 was statistically significant (P = .0002, t test). In our study group, for a PA:Ao ratio of 1.60, sensitivity was 83.0%; specificity, 85.0%; positive predictive value, 62.5%; and negative predictive value, 94.0%. Conclusions. In the third trimester, the main PA:Ao ratio as measured in the fetal mediastinum can be a helpful tool in distinguishing true CoA requiring neonatal cardiac surgery from false‐positive CoA and simple disproportion requiring medical attention but no surgery in the first month of postnatal life.

The Polish National Registry for Fetal Cardiac Pathology: organization, diagnoses, management, educational aspects and telemedicine endeavors†

We describe the National Registry for Fetal Cardiac Pathology, a program under the Polish Ministry of Health aimed at improving the prenatal diagnosis, care, and management of congenital heart disease (CHD).

The three-vessel view in the fetal mediastinum in the diagnosis of interrupted aortic arch.

Interruption of the aortic arch (IAA) is difficult to detect and diagnose in utero. However, prenatal diagnosis may be beneficial because IAA is rapidly fatal (median age, 10 d) if left uncorrected. Our objective was to review the direct and indirect echocardiographic markers associated with IAA, focusing on the importance of the three-vessel view (3VV), which is obtained during routine ultrasound examination to rule out malformations. We analyzed the fetal echocardiograms of nine fetuses and compared them with 56 normal controls. In each fetus, there was a large discrepancy between the diameter of the larger, dilated pulmonary artery (PA) and smaller, narrow aortic arch (Ao). The calculated ratio of PA/Ao in fetuses with IAA was 2.6 ± 0.4 compared with 1.1 ± 0.09 in normal controls (p < 0.0001). The calculated ratio of PA/Ao in fetuses with IAA type A was 2.1 ± 0.09 and IAA type B 2.9 ± 0.2 (p = 0.0007). Discrepancy between PA/Ao diameters should raise the suspicion of aortic arch anomalies and a large discrepancy is a nearly pathognomonic sign of IAA type B.

Ultrasound and echocardiographic findings obtained in the second and third trimesters of gestation in fetuses with normal karyotype and increased nuchal translucency.

Introduction Numerous papers have proven that an increased nuchal translucency is connected with a raised risk of chromosomal aberrations, but few analyses are related to the further state of fetuses with a normal karyotype. The aim of the study The aim of the study was to estimate the risk of cardiac defects and other developmental disorders in fetuses with increased nuchal translucency and normal findings of a standard cytogenetic examination. Methods The authors carried out a retrospective analysis of 5183 examinations of 3376 patients who reported to the Department of Diagnosis and Prophylaxis of Congenital Malformations in the Polish Mothers Memorial Hospital in Łódź in the period from January 2008 to March 2011 for prenatal ultrasound and echocardiographic examinations. The authors analyzed the results of the examinations performed in the second and third trimesters of gestation in fetuses with an increased nuchal translucency of ≥3 mm in the first trimester and with a normal karyotype. Results Fifty-seven patients (1.7% of the examined group) fulfilled the criteria necessary to be included in the study. In 31 pregnant women (54%) structural defects or anomalies of the fetus were found. Cardiac anomalies were detected in 17 fetuses (29.8%). The authors detected various types of cardiac defects such as tetralogy of Fallot, ventricular septal defect, atrioventricular septal defect, transposition of the great arteries and hypoplastic left heart syndrome. Conclusions In more than half of the fetuses with an increased nuchal translucency (NT ≥ 3 mm) and a normal karyotype, developmental defects of various organs appeared in the further course of pregnancy: mainly heart defects that were either isolated, or accompanied other anomalies.

Prenatal 3RD Trimester Expectation of Fetal or Neonatal Demise and Perinatal Team Approach

Abstract INTRODUCTION: The aim of this study was to present our current practice of counseling patients and families with the most severe congenital malformations in the 3rd trimester of pregnancy and to develop practical guidelines for our team and involved healthcare/ socialcare professionals. MATERIAL & METHODS: It was a retrospective evaluation of a series of fetal cases in 2017 from single tertiary center. Maternal obstetrical medical history, time of prenatal detection of the anomaly (1st, 2nd or 3rd trimester), time between last fetal echocardiography and delivery, type of delivery, neonatal birth weight and time of neonatal demise. The total study group was subdived into early demise (during the 1st day after delivery) or late demise > 1st day after delivery. RESULTS: Mean maternal age was 30,4 +/- 5,6 years, and varied between 26 and 38 years. No chronic maternal diseases were found in medical history and no congenital malformations were present in previous children. All women had 1st trimester ultrasound, in 9 cases, it was reported as normal (with NT measurement < 2 mm), in 2 cases extracardiac abnormalities were detected: diaphragmatic hernia and omphalocele ( in both fetal karyotype 46,XY). In nine cases, the abnormalities were detected in midgestation and with maternal wish to continue the pregnancies. There were 8 neonatal deaths within 60 minutes after delivery, including one intrapartum death and 3 “late” neonatal deaths in the intensive care unit (on 12th, 21st and 22nd day). We stress upon the prenatal team approach and counseling of future parents, in order to prepare them for poor neonatal outcome. CONCLUSIONS: 1. In the most severe cases when fetal or neonatal demise was suspected, the two different opinions of specialists might not be enough and a third opinion should be recommended before final decision. 2. A Fetal Team of specialists is necessary in cases of expected fetal/neonatal demise in order to prepare a written report of recommended perinatal management for all sides involved in this difficult problem.

Great Vessels Anomalies – Prenatal Echocardiography and Neonatal Angio-CT – A Pictorial Essay

Abstract 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.

Single Fetal Cardiac Tumors and Follow-Up Based on 13 Cases from the Fetal Cardiac Referral Center in 1993-2017

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.

Retrospective Analysis of Preterm Neonates with Congenital Heart Defects Delivered by Cesarean Section: Unfavourable Outcomes a Necessity for Fetal Cardiology Education During Obstetrical Training?

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.

Recommendations for Prenatal Echocardiography: A Report from International Prenatal Cardiology Collaboration Group.

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.

Emergency Cesarean Section in Case of Aneurysm of the Muscular Intraventricular Septum with Neonatal Follow-up - A Case Report and Review of the Literature

Abstract 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..