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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.

Open access

Maria Respondek-Liberska, Łukasz Sokołowski, Maciej Słodki, Katarzyna Zych-Krekora, Iwona Strzelecka, Michał Krekora, Iwona Maroszyńska, Jadwiga Moll and Jacek Moll

Abstract

Total anomalous pulmonary venous connection (TAPVC) is a congenital heart defect (CHD), in which all pulmonary veins connect to the systemic veins or to the right atrium/coronary sinus instead of the left atrium. We present a case report of fetus with prenatally diagnosed isolated infracardiac type of TAPVC in 38th week of gestation. In fetal echocardiographic examination performed in the Department of Prenatal Cardiology, the fetus presented lack of visible pulmonary veins connection to left atrium, abnormal venous confluence behind left atrium, additional vein leading from abdominal cavity to mediastinum and abnormal smooth Doppler blood flow in pulmonary confluence. The accurate prenatal diagnosis allowed to deliver the neonate at term, in tertiary center one day after diagnosis, and to perform surgical reposition of pulmonary veins the following day. The neonate was referred home in a good condition after 28 days of hospitalization. This case is a good example of the value of the 3rd trimester echocardiography.

Open access

Katarzyna Więckowska, Katarzyna Zych-Krekora, Maciej Słodki and Maria Respondek-Liberska

Abstract

Objectives: Coarctation of the aorta (CoA) is an irreversible congenital heart defect. Its prenatal diagnosis is not rare a subject to false-positive conclusion. We present a novel hypothesis explaining the basis of this error.

Methods: Ten cases of prenatal suspicion of the coarctation of the aorta (based on disproportion at the level of 4 chamber view and mediastinum) coincidenced with the umbilical cord wrapped around the fetal body were found in the Filemaker datebase of the Fetal Cardiology Department. Only single pregnancies were taken into account. In all cases another cardiac and extracardiac malformations were excluded.

Results: The mean maternal age was 29,6 years. The mean gestational age was 33 7/8 weeks. All fetuses were in a good cardiovascular condition. The usual position of the umbilical cord was neck, but they were also location such as nucha, abdomen or lower limb. At birth, all newborns had normal anatomy of the heart. We conclude that the explanation of the false diagnosis was haemodynamic, resulting from the compression of the fetal neck by the umbilical cord that resulted in a disproportion of cardiac blood flow, “mimicking” CoA.

Conclusions:

1. Functional disturbances can mimic prenatal CoA.

2. Umbilical cord position (specially enlacing the fetus neck) should be taken into consideration in suspected cases of fetal CoA.

Open access

Katarzyna Więckowska, Katarzyna Piątek and Maria Respondek-Liberska

Abstract

INTRODUCTION: Cardiac tumors (CT) have been diagnosed since the eighties of the last century. The three most commonly diagnosed types of CT are rhabdomyomas, teratomas and fibromas. Severe fetal disturbances, such as arrythmias, fetal hydrops, ventricular obstructions, may be associated with the heart tumor.

AIM OF THE STUDY: To present the key role of the echocardiographic examination in the perinatal care and to present some demographic changes in this population of the youngest patients.

MATERIAL AND METHODS: From the Filemaker database of the Department for Diagnosis and Prevention of Congenital Malformations, Polish Mother’s Memorial Hospital & Medical University of Lodz, all cases with prenatal diagnoses of cardiac tumor in singleton pregnancy were compiled over a 22-year period (1993–2015). 33 cases of heart tumors in fetuses had 72 fetal echo examinations. The average number of cases per year was 1,5 (range 1 – 5). The retrospective analysis of the material was performed using the Statistica package. The analysis included the assessment of the cardiovascular condition defined by Cardiovascular Profile Score, location and number of tumors; age, past obstetric history, place of residence of the mother, type of delivery and neonatal follow-up.

RESULTS: The number of fetuses with cardiac tumor cases did not increase during this period. The maternal age was 28,1 years (± 5,2 years) and was decreasing over time. The primigravidae constituted 60% of examined population. The history of past miscarriages was observed in 26% of women. The majority of tumors were detected and diagnosed in the third trimester – at mean 29,6 hbd, but the diagnosis was made earlier in the past decades (regression analysis, p=0,042). The average duration of pregnancy was 36,6 hbd (range: 27-42 hbd). There was no statistical difference between decades in term on pregnancy duration.

The cardiac tumors were more frequent in the female fetuses (71,4% to 28,6%; X2 test; p=0,006). The proportions between the groups of single and multiple tumors changed in last five years – currently the multiple tumors are more frequent (87,5%). The mortality in the group of single tumors was twice as high as in the group of multiple tumors. The percentage of cesarean sections between 1993 and 2005 was 67% and between 2006 and 2015 was 75% (p=0,63). The mortality of neonates with cardiac tumors declined over the period taken into account.

CONCLUSIONS: The perinatal care of the fetuses with cardiac tumors improved over last 20 years. The characteristics of analyzed population had shown that fetal CTs affected healthy, young mothers, more often primigravidae, more often female fetuses. The prevalence of CTs did not increase over time. The gestational age at the time of the diagnosis decreased, thus the period of fetal echo monitoring increased. However, the rate of cesarean sections also increased, which requires further studies and explanations.

Open access

Alexis C. Gimovsky, Brianne Whitney, Dennis Wood and Stuart Weiner

Abstract

BACKGROUND: The Myocardial Performance Index (MPI) is a Doppler derived myocardial function tool and can be used to evaluate systolic and diastolic function in fetuses. The objectives of this study were to investigate the MPI during labor and compare it to values in non-laboring women.

METHODOLOGY: 40 women with uncomplicated, term, singleton pregnancies were recruited to this prospective observational study at Thomas Jefferson University Hospital. Controls were a retrospective cohort of women > 34 weeks who underwent third trimester fetal echocardiography. Fetal left and right sided isovolumic contraction time, isovolumic relaxation time and ejection time were recorded before, during and after contractions. Right and left sided MPI was then calculated.

RESULTS: Laboring patients and non-laboring patients were comparable for age, race, gravidity and parity. During labor the average left MPI was 0.63 ± 0.17 and the average right MPI was 0.62 ± 0.20. The coefficient of correlation between MPI and cervical dilation was 0.15 for left MPI Index and 0.14 for right MPI. When comparing non-laboring to laboring women, the average left MPI for non-laboring women was 0.34 ± 0.04, p = <0.001.

CONCLUSIONS: Myocardial Performance Index is a non-invasive, easily attainable measure of cardiac function that can be obtained during labor and does not change with cervical dilation. MPI is significantly different between laboring and non-laboring women. The fetal MPI may help define fetal status in labor.

Open access

Bettina F. Cuneo

Abstract

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..

Case Report:

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.