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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.

Open access

Joanna Płużańska, Jacek Więcek, Michał Krekora, Jolanta Kiełbasicz-Binikowska, Tomasz Talar, Maciej Słodki and Maria Respondek-Liberska

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..

Open access

Maria Respondek-Liberska

Abstract

Four cases of missed prenatal diagnosis by an experienced ultrasonographer and a fetal cardiologist from a tertiary teaching hospital are presented: 3-mm peri-membrane ventricular septal defect; hypoplastic aortic arch requiring prostin infusion after delivery; esophageal atresia with tracheal fistula; and right-sided diaphragmatic hernia. Freezed frames and cine loops of the prenatal ultrasound scans indicated that the missed anomalies were not visible in midgestation, suggesting that in the future, repeat ultrasound scans should be performed before delivery to reduce the occurrence of such diagnostic errors.

Open access

Alexander N. Goel, Camila Reyes, Shauna Mclaughlin, Mark Wittry and Andrew C. Fiore

Abstract

Retroesophageal course of the left brachiocephalic vein is a rare variant seen in patients with congenital heart disease. However, this anomaly without associated cardiac or aortic abnormalities is nearly unheard of, with only one prior case described in the literature. We present an infant with anomalous retroesophageal left brachiocephalic vein that was an incidental finding on computed tomography (CT). We also briefly discuss its embryologic and clinical significance.

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

Arkadiusz Michalak, Marta Witczak, Elżbieta Kukawczyńska, Marek Niwald and Maria Respondek-Liberska

Abstract

Our clinical report describes a rare finding of a prenatally-detected congenital atrioventricular (AVB) block without associated maternal antibodies, which progressed from 1st/2nd degree AVB to complete heart block during second half of pregnancy. Obstetrical ultrasound at 12th week did not reveal any abnormalities and prenatal echocardiography (due to VSD in afamily member) at the 18th week of gestation detected 1st degree block, then bigeminy and bradycardia. Transplacental treatment with B-2-mimetics was introduced. The delivery was organized in a tertiary center and a pacemaker for the newborn baby was secured and implanted in 15th day of life. Currently the boy`s condition is good and stable. Before therapy with B-2-mimetics the mother underwent echocardiography and ECG which revealed clinically silent structural and conduction heart abnormalities. Literature findings suggest that parents of children with non-immune congenital or childhood AVB are more likely to carry clinically silent conduction abnormalities than general population. Given the corresponding findings in the mother and her son, they should be good candidates for genetic testing.