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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 Piątek, Katarzyna Zych-Krekora, Joanna Płużańska, Ewa Gulczyńska and Maria Respondek-Liberska

Abstract

Complains about prenatal diagnoses usually touch late diagnosis, missed diagnosis or uncomplete diagnosis. Prenatal diagnose provides usually important information for parents, obstetrician and neonatologist. Successful perinatal care is based on a good cooperation of the perinatal team. This time we present a peculiar situation when improper reading of prenatal diagnosis had caused a lot of troubles for the patient, parents and hospital staff.

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

Hanna Romanowicz, Ewa Czichos, Katarzyna Zych-Krekora, Michał Krekora, Maciej Słodki and Maria Respondek-Liberska

Abstract

Introduction:

It was retrospective analysis of prenatal echocardiography findings in fetuses with congenital heart defects, who died in our institution and had an autopsy exams in years 2010 - 2015.

Material and methods:

Among total 115 deaths the pulmonary hypertension based on histopathology criteria was present in 83 cases (72%) as a leading cause of their deaths. Out of 83 neonates 40 underwent prenatal echo, 43 did not, however in both groups there were similar types of heart defects.

Results:

The prenatal echo findings from study group (n=40), from the last echo before the delivery were compared with control group and group of HLHS who did survive neonatal surgery and were discharged from hospital. There were statistical differences between pulmonary artery/aorta ratio in fetuses in control group and fetuses in study group („pulmonary hypertension” after birth) (p=0,044). There were statistical differences between pre-delivery pulmonary artery/aorta ratio in fetuses in study group (with „pulmonary hypertension” after birth) and in group of fetuses with HLHS, alive & well after first surgery (p=0,027). There were no differences between pulmonary artery/ aorta ratio fetuses in control group and fetuses with HLHS, alive & well after first surgery (p=0,38)

Conclusion:

1) Pulmonary hypertension was a frequent cause of neonatal deaths among our series of congenital heart defects

2) Dilatation of pulmonary artery (and increased pulmonary/artery ratio ) in fetal echo just before delivery may be an important risk factor for poor neonatal outcome in congenital heart defects.)

Open access

Iwona Strzelecka, Maciej Słodki, Katarzyna Zych-Krekora, Michał Krekora, Mariusz Grzesiak, Iwona Maroszyńska and Maria Respondek-Liberska

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.

Open access

Katarzyna Zych-Krekora, Anna Wójtowicz, Michał Krekora, Maciej Słodki, Hugues Gentillon and Maria Respondek-Liberska

Abstract

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.

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

Katarzyna Więckowska, Jerzy Węgrzynowski, Katarzyna Zych - Krekora, Joanna Kwiatkowska, Maciej Słodki and Maria Respondek-Liberska

Abstract

In this report a case of aortopulmonary window (APW) diagnosed at 26 hbd is presented. APW supported the pulmonary circulation in neonate afflicted with pulmonary stenosis. To our knowledge, this is the first report in the literature referring to observing their coincidence in fetal life.

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

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.