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Maria Respondek-Liberska, Maciej Łukaszewski, Adam Oleś, Michał Podgórski, Piotr Grzelak and Maciej Słodki

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.

Open access

Iwona Strzelecka, Eliza Michalska, Katarzyna Zych-Krekora and Maria Respondek-Liberska

Abstract

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.

Open access

Maria Respondek-Liberska, Jerzy Węgrzynowski, Przemysław Oszukowski, Ewa Gulczyńska, Elżbieta Nykiel, Lucjusz Jakubowski, Mariusz Grzesiak, Ewa Czichos and Hanna Romanowicz

Abstract

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.

Open access

Barbara Swięchowicz and Maria Respondek-Liberska

Abstract

Heart defects which includes narrowing of aortic isthmus - aortic coarctation (CoA) are one of the most prevalent birth defects. Making a correct prenatal diagnosis of CoA is very difficult and problematic. We are still observing many false (+) and false (-) diagnoses. In presenting 3 cases with prenatal suspicion of CoA only one patient confirmed this defect in the postnatal life. In the fetal echocardiography inappropriate dimensions of great vessels and PA/Ao ratio are very relevant in the CoA diagnostics. Based on such suspicion before delivery we can select a group in which birth in the tertiary center, prostin infusion, control ECHO examinations and planned cardiac surgery will be needed. But wide differential diagnosis including pulmonary dilatation (due to pulmonary hypertension or fetal blood redistribution due to possible infection) is required.

Open access

Joana O. Miranda and José Carlos Areias

Open access

Katarzyna Zych-Krekora, Michał Krekora, Marek Kopala and Maria Respondek-Libserska

Abstract

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.

Open access

Katarzyna Pośpiech-Gąsior, Maciej Słodki and Maria Respondek-Liberska

Abstract

Cantrell’s pentalogy is a congenital defect characterized by uncompleted fusion of the anterior chest wall, resulting in an extrathoracic location of the heart. Ultrasound diagnosis during the first trimester of prenatal life is possible, and termination of pregnancy is usually chosen by pregnant women. We analysed 57 fetuses: 56 from literature and one additional recent case from our institute (from 2016) to evaluate what was the survival rate reported after prenatal diagnosis, including the possibility to terminate the pregnancy, intrauterine deaths and neonatal deaths. We found 10 survivors - 18% since 1984.

Despite dismal prognosis of fetal ectopia cordis, there is a chance for postnatal survivorship probably due to evolving anatomical structures, not only in the first trimester of pregnancy but also during the following weeks of prenatal life.

Open access

Iwona Strzelecka, Maria Respondek-Liberska, Maciej Słodki, Katarzyna Zych-Krekora and Bettina Cuneo

Abstract

Based on fourteen case reports from various centres from 1992-2015 and three original studies in 2006-2011, 122 fetuses were subjected to analysis. In these reports, transplacental digoxin treatment was administered to different cardiac anomalies such as SVT , Ebstein’s anomaly, critical AS , absent pulmonary valve syndrome, complete heart block, in foetuses with aneurysm/diverticulum of LV, in tricuspid atresia or dysplasia, rhabdomyoma, pulmonary atresia, HLHS with fibroelastosis, in TTTS and in extracardiac anomalies such as atriovenous malformation or sacrococcygeal teratoma. There was no statistical difference to suggest (Chi-square test) that digoxin was more efficient to control fetal arrhythmias than fetal congestive heart failure in nonarrhythmic patients.

Conclusions: Foetal cardiac insufficiency may appear due to different reasons (in normal heart anatomy or in heart defects, in normal sinus rhythm or due to foetal arrhythmias: tachycardias or severe bradycardia) and may be a cause of intrauterine demise. So far, we do not have strong evidence that digoxin treatment may prevent foetal death or prematurity. More research is needed to ascertain if the prolonging of pregnancy resulted from digoxin treatment or if improvement in foetal circulatory insufficiency was influenced by spontaneous regression of foetal cardiac symptoms.

Open access

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 and Isaac Blickstein

Abstract

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.