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.
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.
Więckowska K, Węgrzynowski J, Zych - Krekora K, Kwiatkowska J, Słodki M, Respondek- Liberska M. Antenatal echocardiographic diagnosis of an aortopulmonary window combined with pulmonary stenosis in neonate. A case report. Prenat Cardio. 2015 Sep;5(3):33-37
In the article a case of a little APW coincidented with pulmonary stenosis (the first such description in Poland) in 26-week-old fetus of a thirty-two-year-old woman, gravida 3, was presented1.
Izabella Pietrzyk, Hanna Moczulska, Maciej Słodki, Krzysztof Szaflik and Maria Respondek-Liberska
The aim of this study was to check whether echocardiography is useful in patients with thoracic anomalies undergoing an invasive therapy in utero.
Material and Methods: Retrospective analysis of 42 pregnant women and their fetuses (2003 - 2012), which, due to the chest anomalies had genetic ultrasound and ECHO and then were subjected to an invasive intrauterine therapy.
Results: The mean maternal age was 30.2 years, there were 18 high risk pregnancies and 24 low-risk pregnancies, the average gestational age at diagnosis was 28.2 wks (17 - 38), the average week of delivery was 35 wks (24 - 41), the average birth weight was 2700g (700 - 4050g). The average number of fetuses with chest anomalies undergoing therapy in utero in our center was 4.2 per year. The most common anomaly was hydrothorax, then CALM and DH and one case of AS. Anomalies coexisted with generalized edema, ascites and/or polyhydramnios. Most often shunts and/or decompression of pleural fluid and / or abdominal cavity were performed. Structural heart defects occurred in 6 fetuses and functional anomalies in echocardiography were recorded in 29 fetuses (73%).
Selected group of 19 fetuses had echocardiography before and after surgery. In 14 fetuses hemodynamic improvement was observed and in 5 patients fetal functional changes have persisted. The time from the last treatment to the delivery averaged was 40,2 days (2 to 140).
The follow-up was analyzed in a group of 37 fetuses: there were 2 intrauterine deaths, 11 deaths after delivery and 24 infants were discharged home. Mean hospitalization duration of the live-born infants was 23.7 days (1 - 70). Hospitalization of 14 neonates with hemodynamic improvement after surgery was 25.5 days and in a group of five fetuses with no improvement after surgery, was mean 45.6 days.
Conclusions: The number of fetuses undergoing an invasive therapy due to anomalies of the chest during 2003 - 2012 remained at a similar level (an average of approximately 4 patients per year). Thoracic defects were often accompanied by functional anomalies in the circulatory system. Majority (73%) of fetuses had shown a significant improvement in cardiac efficiency after an invasive treatment. In the group of fetuses in which the interventional procedure has improved cardiovascular hemodynamics, average duration of hospitalization was shorter as compared to the group without haemodynamic improvement (25,5 days versus 45,6), however there was no statistically significant difference.
Julia Murlewska, Agnieszka Żalińska, Danuta Roik, Bożena Werner and Maria Respondek-Liberska
This case report presents a prenatal diagnosis with postnatal confirmation (by angio CT and computer reconstruction) of an isolated double aortic arch, with no blood disturbances and with no clinical symptoms after birth. Literature review was focusing on the possible symptoms in the future. Prenatal findings should be forwarded to neonatologist and pediatrician despite clinical silence.
Katarzyna Zych-Krekora, Michał Krekora, Marek Kopala and Maria Respondek-Libserska
Introduction: APVS (Absent Pulmonary Valve Syndrome) is a rare congenital heart disease. Its incidence according to The Polish National Registry of Fetal Cardiac Pathology in years 2004 - 2016 was 0.6%.This disease is caused by the absence or the residual pulmonary artery valve resulting in significant dilation of the pulmonary trunk and its branches. In utero deaths are reported. After birth the major problem is respiratory failure and high preoperative and postoperative mortality. Material and methods: In 1995 to 2016, 11 fetuses with APVS were diagnosed in our unit, at the average 27,5 weeks of gestation (min. 18.5- max 37.1 weeks of gestation). Two groups were analysed in this series of cases: “Old one” by 2011 (n = 6) and “New one” since 2011 (n = 5) and perinatal care as well as survival were compared. We analysed the fetal echo results, perinatal care including transplacental digoxin and steroids treatment in NEW group, the longevity of the pregnancy and neonatal/infants outcome. Results: In Old group the average day of cardiac surgery was day 91st after birth (max. 161) and the survival was 50% . In the New group the average surgery day was 41st day and the postoperative survival was 60%, however there was no statistical significance ( p > 0,05). Conclusions: There is no single parameter from prenatal life in foetuses with APVS which may allow to predict the positive outcome meaning neonatal survival. However optimal perinatal care (early detection of defect, transplacental digoxin at least 3-4 weeks, steroids, no preterm delivery, on-time delivery, postnatal care in tertiary center) and relatively early cardiac surgery may have combined impact on the improvement of survival after prenatal diagnosis of APVS, however more data are necessary to prove this hypothesis.
Hanna Moczulska, Małgorzata Piotrowicz, Katarzyna Janiak, Lucjusz Jakubowski and Maria Respondek-Liberska
Noonan syndrome is a frequent genetic disorder with autosomal dominant transmission. It is mainly characterized by congenital heart defects, short stature, and a variable degree of developmental delay. Its symptoms can be observed during fetal life, but most of them are not specific for Noonan syndrome. Cardiac symptoms such as pulmonary stenosis or hypertrophic cardiomyopathy seems to be the most specific. We present a case of Noonan syndrome monitored during fetal live using prenatal echocardiography with a brief review of the literature.
Katarzyna Leszczyńska, Krzysztof Preis, Maria Respondek-Liberska, Maciej Słodki, Dennis Wood, Stuart Weiner, Ulli Gembruch, Giusseppe Rizzo, Reuven Achiron, Jay D Pruetz, Mark Sklansky, Bettina Cuneo, Birgit Arabin, Isaac Blickstein and
Progress in the fields of fetal cardiology and fetal surgery have been seen not only in singleton pregnancies but also in multiple pregnancies. Proper interpretation of prenatal echocardiography is critical to clinical decision making, family counseling and perinatal management for obstetricians, maternal fetal medicine specialists, neonatologists and pediatric cardiologists. Fetal echocardiography is one of the most challenging and time-consuming prenatal examinations to perform, especially in multiple gestations. Performing just the basic fetal exam in twin gestations may take an hour or more. Thus, it is not practical to perform this exam in all cases of multiple gestations. Therefore our review and recommendations are related to fetal echocardiography in twin gestation.
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.