Publications by authors named "Joanna Dangel"

27 Publications

Fetal Cardiac Interventions-Polish Experience from "Zero" to the Third World Largest Program.

Authors:

Marzena Debska Adam Kolesnik Beata Rebizant Agnieszka Sekowska Agnieszka Grzyb Katarzyna Chaberek Jacek Witwicki Romuald Debski Joanna Dangel

J Clin Med 2020 Sep 7;9(9). Epub 2020 Sep 7.

Department of Perinatal Cardiology and Congenital Anomalies, Centre of Postgraduate Medical Education, US Clinic Agatowa, 03-680 Warsaw, Poland.

This article presents the technical aspects of the Polish fetal cardiac interventions (FCI) program, including preparation of the team and modifications in the technique of the procedure that aim to increase its safety for the mother and the fetus. Over 9 years, 128 FCI in 113 fetuses have been performed: 94 balloon aortic valvuloplasties (fBAV), 14 balloon atrioseptoplasties (fBAS) with stent (BAS+), 5 balloon atrioseptoplasties without stent placement (BAS-), and 15 fetal pulmonary valvuloplasties (fBPS). The technical success rate ranged from 80% (BAS-) to 89% (fBAV), while the procedure-related death rate (defined as death within 72 hours following the procedure) ranged from 7% (fBAV and fBPV) to 20% (BAS). There were 98 live births after all FCI (3 pregnancies continue). Median gestational age at delivery was 39 weeks in our center and 38 weeks in other centers.

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http://dx.doi.org/10.3390/jcm9092888	DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7576494	PMC

September 2020

Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry.

Fetal Diagn Ther 2020 Jul 7:1-9. Epub 2020 Jul 7.

University of California-San Francisco, San Francisco, California, USA.

Introduction: Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience.

Objectives: Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR).

Methods: We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics.

Results: Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9-31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgitation, and pulmonary valve patency. There were fetal complications in 55% of cases, including 7 deaths and 2 delayed fetal losses. Among those who underwent successful FCI, the absolute measurement of the TV increased by 0.32 (±0.17) mm/week from intervention to birth. Among 60 liveborn with known outcome, there was a higher percentage having a biventricular circulation following successful FCI (87 vs. 43%).

Conclusions: Our data suggest a possible benefit to fetal therapy for PAIVS/PS, though rates of technically unsuccessful procedures and procedure-related complications, including fetal loss were substantial. FCI criteria are extremely variable, making direct comparison to nonintervention patients challenging and potentially biased. More uniform FCI criteria for fetuses with PAIVS/PS are needed to avoid unnecessary procedures, expose only fetuses most likely to sustain a benefit, and to enable comparisons to be made with nonintervention patients.

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http://dx.doi.org/10.1159/000508045	DOI Listing

July 2020

Foetal goitrous hypothyroidism - easy to recognise, difficult to treat. Is combined intra-amniotic and intravenous L-thyroxine therapy an option?

Authors:

Marzena Dębska Małgorzata Gietka-Czernel Piotr Kretowicz Dagmara Filipecka-Tyczka Łukasz Lewczuk Joanna Dangel Romuald Dębski

Endokrynol Pol 2018 29;69(4):442-446. Epub 2018 Jun 29.

2nd Department of Obstetrics and Gynaecology, Centre of Medical Postgraduate Education, Warsaw, Department of Endocrinology, Centre of Medical Postgraduate Education, Warsaw.

Introduction: Foetal hypothyroidism negatively impacts somatic and neurological child development and can be the cause of serious obstetric and perinatal complications. We present a rare case of a large foetal dyshormonogenetic goitre, causing foetal neck hyperexten-sion, oesophageal compression, and cardiac high-output failure.

Material And Methods: A foetal goitre complicated by cardiomegaly and polyhydramnios was diagnosed at 23 weeks of gestation (WG) on a routine ultrasonographic (US) assessment in a healthy nullipara. Foetal blood sampling was performed and a severe foetal hypothyroid-ism was diagnosed. Treatment was undertaken with an intra-amniotic followed by combined intra-amniotic and intravenous injections of L-thyroxine (L-T4). A total of 11 doses of L-T4 were administered between 24-37 WG to the foetus.

Results: A complete regression of foetal goitre, cardiomegaly, and polyhydramnios was observed. At 38 WG the patient delivered vagi-nally a male infant with mild hypothyroidism and no signs of goitre or cardiomegaly on postnatal US. Neurological development of the one year old baby is normal.

Conclusions: The effective diminishing of serum TSH concentration and goitre size was reached after combined intra-amniotic and in-travenous L-T4 injections were given. L-T4 requirement in the foetus is equal to or above 15 μg/kg daily and should be given in weekly intervals due to its rapid metabolism by the foetus and by placental type 3 deiodinase. Intra-amniotic L-T4 administration may be inef-fective when a large goitre indisposes amniotic fluid swallowing by the foetus, so then the combined L-T4 injections into the umbilical vein and intra-amniotically in experienced hands seems to be a reasonable and effective option.

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http://dx.doi.org/10.5603/EP.a2018.0045	DOI Listing

December 2018

Early vesico-amniotic shunting - does it change the prognosis in fetal lower urinary tract obstruction diagnosed in the first trimester?

Authors:

Marzena Dębska Piotr Kretowicz Anna Olędzka Piotr Gastoł Joanna Dangel Małgorzata Świątkowska-Freund Romuald Dębski

Ginekol Pol 2017 ;88(9):486-491

Objectives: The aim of the study was to assess the outcome of vesico-amniotic shunting performed before 16 weeks of pregnancy in fetuses with severe megacystis diagnosed in the first trimester of pregnancy.

Material And Methods: Between January 2008 and October 2012 severe megacystis with the bladder length > 15 mm was diagnosed in 17 fetuses. The procedure of early vesico-amniotic shunting (VAS) was offered to 8 patients with presumably isolated LUTO. The procedure of VAS was performed in 6 fetuses. Before the intervention one or two procedures of vesicocentesis and urine analysis were performed.

Results: In all treated cases shunts provided urinary tract decompression. All babies were born prematurely, 2 of them died due to premaurity, 3 of them survived and have normal renal function at the age of 5-6 years. In 4/5 children accompanying malformations were later diagnosed, in 1 born prematurely neonate necropsy was not performed.

Conclusions: Our results suggest that early vesico-amniotic shunting in fetal LUTO is feasible and may potentially prevent not only pulmonary hypoplasia but also renal insufficiency. However, the rationale of the procedure needs further investigation due to a high risk of long-term morbidity and co-existing malformations in children Before offering the therapy detailed counseling of the parents about the possible pros and cons of the therapy is necessary.

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http://dx.doi.org/10.5603/GP.a2017.0089	DOI Listing

July 2018

Hypoplastic Left Heart Syndrome With Intact or Restrictive Atrial Septum: A Report From the International Fetal Cardiac Intervention Registry.

Circulation 2017 10 1;136(14):1346-1349. Epub 2017 Sep 1.

From C.S. Mott Children's Hospital, University of Michigan, Ann Arbor (D.W.J., C.G.F., S.K.G.); University of California-San Francisco (A.J.M.-G.); Baylor College of Medicine, Houston, TX (S.A.M.); Nationwide Children's Hospital, Columbus, OH (A.K.A.); University of Bonn, Germany (C.B., U.H.); Perinatal Cardiology Clinic, Medical University of Warsaw, Poland (J.D.); Children's Hospital of Wisconsin, Milwaukee (M.F.); University Hospital Bonn, Germany (U.G.); Hospital for Sick Children, Toronto, Ontario, Canada (E.J.); Florida International University, Miami (E.V.K., R.A.Q.); Boston Children's Hospital, MA (A.C.M.); Evelina London Children's Hospital, United Kingdom (O.M.); Technische Universität München, Germany (R.O.); Leiden University Medical Center, Netherlands (D.O.); Hospital do Coração, São Paolo, Brazil (C.A.P., S.R.P., F.P.); and Mount Sinai Hospital, Toronto, Ontario, Canada (G.R.).

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http://dx.doi.org/10.1161/CIRCULATIONAHA.116.025873	DOI Listing

October 2017

2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Data Standards.

J Am Coll Cardiol 2017 08 14;70(8):1029-1095. Epub 2017 Jul 14.

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http://dx.doi.org/10.1016/j.jacc.2017.06.027	DOI Listing

August 2017

2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Data Standards.

Circ Cardiovasc Qual Outcomes 2017 08;10(8)

Together with the National Association of Children's Hospitals, these organizations have merged and now comprise the Children's Hospital Association (www.childrenshospitals.org). International Society for Nomenclature of Paediatric and Congenital Heart Disease Representative. Child Health Corporation of America Representative. Association of European Pediatric Cardiologists Representative. The Society of Thoracic Surgeons Representative. ACC/AHA Task Force on Clinical Data Standards Liaison to the Writing Committee. Congenital Heart Surgeons' Society Representative. National Association of Children's Hospitals and Related Institutions Representative. American Academy of Pediatrics Representative. Congenital Cardiac Anesthesia Society Representative. Task Force Chair during the development of this document.

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http://dx.doi.org/10.1161/HCQ.0000000000000027	DOI Listing

August 2017

Postnatal Outcome of Fetal Left Ventricular Hypertrabeculation/Noncompaction.

Authors:

Claudia Stöllberger Christian Wegner Abraham Benatar Thomas K Chin Joanna Dangel Danielle Majoor-Krakauer Tapas K Mondal Shanthi Sivanandam Norman H Silverman Jaap van Waning Josef Finsterer

Pediatr Cardiol 2016 Jun 31;37(5):919-24. Epub 2016 Mar 31.

Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Vienna, Austria.

Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Prenatal diagnosis of LVHT can be established by fetal echocardiography. A review of 106 published cases showed that 46 cases with prenatally diagnosed LVHT were alive 0.5-120 months after birth. Since the course of cases with prenatally LVHT after publication is unknown, we aimed to collect follow-up-information. Information regarding vital status, cardiac and extracardiac morbidity was gathered by contacting the authors of the 46 cases. Fourteen of the 28 authors answered and gave information about 18 cases (six females, seven males, five gender-unknown, age 18 months to 10 years, mean follow-up 60 months). No differences were found between the 18 cases with follow-up and the 28 cases without follow-up regarding age, gender, cardiac or extracardiac comorbidities, and interventions. Three of the 18 cases had died subsequently from heart failure, osteosarcoma, and enterocolitis, respectively. Mutations or chromosomal abnormalities were found in six of the seven examined patients, extracardiac abnormalities in nine patients. Three patients received a pacemaker because of complete AV block, and two patients underwent heart transplantation. Cardiac surgical or interventional procedures were carried out in four patients. None suffered from malignant arrhythmias or had a cardioverter-defibrillator implanted. Based on the limited information, there are indications that cases with fetal diagnosis of LVHT have a continuing morbidity and mortality, even if they receive appropriate care. Since fetal LVHT is frequently associated with genetic abnormalities, further research about survival and underlying genetic causes is needed.

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http://dx.doi.org/10.1007/s00246-016-1369-8	DOI Listing

June 2016

International Fetal Cardiac Intervention Registry: A Worldwide Collaborative Description and Preliminary Outcomes.

J Am Coll Cardiol 2015 Jul;66(4):388-99

Leiden University Medical Center, Leiden, the Netherlands.

Background: Invasive fetal cardiac intervention (FCI) has been reported in single-institution series, promoting technical and physiologic success.

Objectives: This study describes the creation of an international registry of cases presenting for FCI, intended to compile technical and outcome data from a multicenter cohort.

Methods: For this initial analysis, the entire database of the International Fetal Cardiac Intervention Registry (IFCIR) was queried for details of diagnoses, procedures, and outcomes. Maternal-fetal dyads from January 2001 through June 2014 were included.

Results: Eighteen institutions submitted data by data harvest. Of 370 cases entered, 245 underwent FCI: 100 aortic valvuloplasties from a previous single-center report (excluded from additional reporting here), an additional 86 aortic and 16 pulmonary valvuloplasties, 37 atrial septal cases, and 6 unclassified cases. FCI did not appear to affect overall survival to hospital discharge. Among live-born infants with a fetal diagnosis of aortic stenosis/evolving hypoplastic left heart syndrome, more than twice as many were discharged with biventricular circulation after successful FCI versus those meeting institutional criteria but without any or successful FCI (42.8% vs. 19.4%, respectively). When fetal deaths were counted as treatment failures, the percentages were similar: biventricular circulation at discharge was 31.3% versus 18.5% for those discharged with univentricular palliation. Survival to discharge for live-born fetuses with atrial restriction was similar to that of those undergoing technically successful versus unsuccessful FCI (63.6% vs. 46.7%, respectively), although criteria for diagnosis were nonuniform.

Conclusions: We describe the contents of the IFCIR and present post-natal data to suggest potential benefit to fetal therapy among pregnancies considered for possible intervention and support proposals for additional work.

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http://dx.doi.org/10.1016/j.jacc.2015.05.037	DOI Listing

July 2015

[Familial congenital diaphragmatic hernia with anencephly-exencephaly and spina bifida].

Authors:

Maria Hussey Paweł Własienko Tomasz Adamczyk Joanna Dangel

Ginekol Pol 2015 Feb;86(2):155-9

Congenital Diaphragmatic Hernia (CDH) occurs with an estimated incidence of 1 to 2500 live births. Even though the exact etiology is still unknown, more and more often current research points out genetic factors as the possible cause of the defect. According to the latest data and the own experience 50-60% of CDH cases are isolated. The rest forms a group of CDH complicated by an additional anatomic defect or a genetic syndrome caused by a mutation of a single gene or the whole chromosome. We have presented a case study of a 32 years-old multigravida para 3, who has been referred to the Reference Centre of Prenatal Cardiology in 30 weeks of gestation due to the diagnosis of acrania with exencephaly, spina bifida and suspicion for CDH in a fetus. Although the patient's first child died due to CDH, the patient neither before nor during the pregnancy was getting a folic acid supplementation. Moreover, she has not agreed on a further cytogenetic testing or an advanced consultation with a clinical geneticist. The child died after delivery in a local hospital. The case was described to indicate the problem that the CDH diagnostic procedure is still missing a molecular genetic analysis especially in the cases of recurrent CDH. By saying that in the cases of CDH we should always strive to complete the molecular testing having in mind that by discovering pathogenesis and genes responsible for the formation of CDH we not only might improve the therapeutic methods but also find a way to prevent its development.

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http://dx.doi.org/10.17772/gp/2004	DOI Listing

February 2015

Changing physiology in the first- to third-trimester foetal circulation.

Authors:

Cardiol Young 2014 Oct 10;24 Suppl 2:13-8. Epub 2014 Oct 10.

2nd Department of Obstetrics and Gynecology,Perinatal Cardiology Clinic,Medical University of Warsaw,Warsaw,Poland.

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http://dx.doi.org/10.1017/S1047951114001395	DOI Listing

October 2014

Successful intrauterine treatment and good long-term outcome in an extremely severe case of fetal hemolytic disease.

Authors:

Marzena Dębska Piotr Kretowicz Anna Tarasiuk Joanna Dangel Romuald Dębski

J Ultrason 2014 Jun 30;14(57):217-22. Epub 2014 Jun 30.

II Klinika Położnictwa i Ginekologii, Centrum Medycznego Kształcenia Podyplomowego, Szpital Bielański, Warszawa, Polska.

A 34-year-old multiparous woman presented with anti-Rh-D antibodies (1: 512) and fetal hydrops at the 21(st) week of gestation. Ultrasound revealed massive fetal skin edema, ascites, hepatomegaly, placentomegaly, and anhydramnios. No fetal movements were observed. Fetal heart was enlarged, with reportedly decreased contractibility. The Doppler parameters were abnormal: the peak systolic velocity in median cerebral artery (MCA PSV) was increased (84 cm/s, 3 MoM), and absent end diastolic flow (AEDF) was reported in the umbilical artery. Ultrasound examination indicated severe fetal anemia and heart failure. Umbilical vein puncture was performed and the fetal blood count was determined (RBC 0.01 × 10(6)/µl, Ht 0.1%, PLT 67 × 10(3)/µl, WBC 2.1 × 10(3)/µl, indeterminable hemoglobin level). Packed red blood cells (0 Rh-, 30 ml) were immediately transfused to the fetus. Altogether, seven intrauterine transfusions were performed. Fetal hydrops disappeared gradually during the next few weeks. The male neonate (1860 g, 45 cm, Apgar score 3-4) was delivered after the last transfusion at 34(th) week of gestation due of intrauterine asphyxia. The infant was discharged after 21 days, in good condition, on breastfeeding. There was one 10 mm focus of periventricular leukomalacia in the brain, diagnosed based on trans-fontanel ultrasound, without any signs of damage to other organs. At the age of 5 years, the child is healthy, with no abnormalities in his neurodevelopmental parameters.

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http://dx.doi.org/10.15557/JoU.2014.0021	DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4579700	PMC

June 2014

[Increased nuchal translucency in chromosomally normal fetuses and pregnancy outcomes--a retrospective study].

Authors:

Julia Bijok Karolina Ziora-Jakutowicz Alicja Ilnicka Barbara Pawłowska Anna Jóźwiak Joanna Dangel Grzegorz Jakiel Tomasz Roszkowski

Ginekol Pol 2013 Mar;84(3):172-9

I Klinika Ginekologii i Połoznictwa--CMKP SPSK im. Prof. Ortowskiego w Warszawie, Polska.

Objectives: The objective was to study the outcomes of fetuses with increased nuchal translucency > or = 3.5 mm and normal karyotype.

Materials And Methods: We performed a retrospective study on pregnancy outcomes and children development in 87 women with increased fetal nuchal translucency and normal karyotype who underwent chorionic villus sampling at our department. Mean observation period was 12 months after birth. Adverse pregnancy outcome was defined as miscarriage and intrauterine fetal demise, termination of pregnancy structural defect, neonatal death, genetic syndrome and other major abnormalities.

Results: The total incidence of adverse pregnancy outcome was 39.1% (n = 34). The likelihood of an adverse pregnancy outcome, a major structural defect or major heart defect increased significantly with nuchal translucency (OR 3.77). 68 children (78.2%) were born alive. Nuchal translucency was significantly higher in newborns with adverse pregnancy outcome than in healthy children [4.1 mm vs. 5.7 mm; p < 0.01]. After a normal anomaly scan at 20 weeks gestation the risk of adverse outcome was 14.5% (n = 9, 9/62) and increased with nuchal translucency thickness from 10.2% for NT 3.5-4.4 mm to 100% for NT > or = 6.5 mm [p < 0. 0 1]. There was no significant relationship of fetal gender maternal age and persistence of the nuchal fold with adverse pregnancy outcome. The rate of neurodevelopmental delay was 3.4 % and was not higher than in the general population.

Conclusions: The overall risk of adverse pregnancy outcome was around 40% and was related to nuchal translucency thickness. After excluding structural defects, the chance of a favorable outcome was 85%. The rate of neurodevelopmental delay in fetuses with increased nuchal translucency normal karyotype and normal anatomy is not higher than in the general population.

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http://dx.doi.org/10.17772/gp/1559	DOI Listing

March 2013

Therapeutic challenges and management of heart failure during pregnancy (part 2).

Authors:

Karolina Kryczka Zofia Dzielińska Andrzej Przybylski Julia Szczudlik Joanna Dangel Marek Konka Krzysztof Czajkowski Jacek Różański Zofia T Bilińska Marcin Demkow

Med Sci Monit 2012 Jun;18(6):CQ9-13

Department of Coronary and Structural Heart Diseases, Institute of Cardiology, Warsaw, Poland.

A particularly dangerous condition in pregnant women is already dilated left ventricle with severe functional impairment. Taking as an example the case of woman with dilated cardiomyopathy (DCM) first diagnosed in 17th week of pregnancy, the paper discusses diagnostic, therapeutic challenges and management of heart failure during pregnancy. Repeat measurements of brain natiuretic peptide levels should be helpful in diagnosing heart failure. To distinguish DCM from peripartum cardiomyopathy the time of manifestation should be considered. The risk of serious events is associated with NYHA class and impairment of left ventricular ejection fraction. Angiotensin-converting enzyme inhibitors (ACE-I) and angiotensin-II receptor blockers are contraindicated in pregnancy because of fetal toxicity. The incidence of sight effects is associated with time of administration of ACE-I and duration of treatment. Possible sight effects of drugs in fetus should be monitored (mainly ultrasonographically). ICD can be implanted during pregnancy if indicated. To assess the time and mode of delivery, a multidisciplinary team of different specialists is required. Subsequent pregnancy is contraindicated in a patient with DCM and low ejection fraction of left ventricle.

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http://dx.doi.org/10.12659/msm.882859	DOI Listing

June 2012

Therapeutic challenges and management of heart failure during pregnancy (part I).

Authors:

Karolina Kryczka Zofia Dzielińska Andrzej Przybylski Julia Szczudlik Joanna Dangel Marek Konka Krzysztof Czajkowski Jacek Różański Zofia T Bilińska Marcin Demkow

Med Sci Monit 2012 May;18(5):CQ5-7

Department of Coronary and Structural Heart Diseases, Institute of Cardiology, Warsaw, Poland.

Therapeutic management in pregnant patients with heart failure still remains a challenge, even though in most pregnant women with cardiac diseases an outcome is good. A 32-year-old woman, 17 weeks pregnant, was admitted to hospital with heart failure (HF) NYHA class III/IV. Echocardiography revealed enlarged LV, LVEF 13%, significant mitral insufficiency and pulmonary hypertension. The patient wished to continue the pregnancy. In a life-threatening condition, metoprolol, enalapril, spironolactone (for 5 days), furosemide, and digitalis were administered. Enalapril was continued for 42 days. Then the patient was switched to a dihydralazine and isosorbide mononitrate regimen. The fetus was controlled ultrasonographically. In the 19th week of pregnancy, the patient's condition improved (NYHA class II, LVEF 23%). The patient experienced 2 more episodes of HF exacerbation. In the 26th week of pregnancy, in a primary prevention of sudden cardiac death and because of 2nd-degree AV block, an ICD was implanted. In the 32nd week of pregnancy a cesarean section was performed. A male infant was delivered. The patient made a good recovery and was discharged on the 7th postoperative day. The newborn was discharged after 4 weeks, in good general condition. At 1-year follow-up the patient presented NYHA class II.

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http://dx.doi.org/10.12659/msm.882716	DOI Listing

May 2012

Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients.

Authors:

Håkan Eliasson Sven-Erik Sonesson Gurleen Sharland Fredrik Granath John M Simpson Julene S Carvalho Hana Jicinska Viktor Tomek Joanna Dangel Paulo Zielinsky Maria Respondek-Liberska Matthias W Freund Mats Mellander Joaquim Bartrons Helena M Gardiner

Circulation 2011 Nov 10;124(18):1919-26. Epub 2011 Oct 10.

Pediatric Cardiology Q1:03, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.

Background: Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome.

Methods And Results: We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000-2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1-21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age < 20 weeks, ventricular rate ≤ 50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥ 1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4 ± 2.9 versus 24.9 ± 4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy.

Conclusions: Risk factors associated with a poor outcome were gestation < 20 weeks, ventricular rate ≤ 50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.

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http://dx.doi.org/10.1161/CIRCULATIONAHA.111.041970	DOI Listing

November 2011

[The first successful fetal aortic balloon valvuloplasty in Poland].

Authors:

Joanna Dangel Marzena Debska Adam Koleśnik Marek Dabrowski Piotr Kretowicz Romuald Debski Anna Brudkowska

Ginekol Pol 2011 Aug;82(8):632-6

Ośrodek Referencyjny Kardiologii Prenatalnej, Poradnia Perinatologii i Kardiologii Perinatalnej, II Klinika Połoznictwa i Ginekologii WUM, Warszawa, Polska.

Prenatal aortic valvuloplasty is performed only in few perinatal centers in the world. Critical aortic stenosis which can lead to hypoplastic left heart syndrome or severe fetal heart failure with nonimmune hydrops is an indication for this procedure. Prenatal intervention can change the natural course of the disease. Authors present the first successful fetal balloon aortic valvuloplasty in Poland. It was performed in a 29-week fetus with critical aortic stenosis, severe impairment of left ventricular function, heart failure and fetal hydrops. After successful intervention, without any complications, left ventricular function and fetal condition improved gradually Effective fetal intervention was possible after few months of preparation and building a team of specialists. This is the first successful fetal cardiac intervention in Poland, which opens the way to the new era of fetal cardiology and hopefully will lead to improve results in children with this critical heart defect.

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August 2011

[Comment to article Congenital heart defect-how precise is prenatal diagnosis?].

Authors:

Kardiol Pol 2011 ;69(2):152

Poradnia Perinatologii i Kardiologii Perinatalnej, II Klinika Położnictwa i Ginekologii, Warszawski Uniwersytet Medyczny, Warszawa.

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June 2011

Ablation of severe drug-resistant tachyarrhythmia during pregnancy.

Authors:

Lukasz Szumowski Ewa Szufladowicz Michał Orczykowski Robert Bodalski Paweł Derejko Andrzej Przybylski Piotr Urbanek Mariusz Kuśmierczyk Edward Koźluk Frederic Sacher Prashanthan Sanders Joanna Dangel Michel Haissaguerre Franciszek Walczak

J Cardiovasc Electrophysiol 2010 Aug 11;21(8):877-82. Epub 2010 Feb 11.

Institute of Cardiology, Alpejska 42, Warsaw, Poland.

Aims: The goal of this study was to describe mapping and ablation of severe arrhythmias during pregnancy, with minimum or no X-ray exposure. Treatment of tachyarrhythmia in pregnancy is a clinical problem. Pharmacotherapy entails a risk of adverse effects and is unsuccessful in some patients. Radiofrequency ablation has been performed rarely, because of fetal X-ray exposure and potential maternal and fetus complications. GROUP AND METHOD: Mapping and ablation was performed in 9 women (age 24-34 years) at 12-38th week of pregnancy. Three had permanent junctional reciprocating tachycardia, and 2 had incessant atrial tachycardia. Four of them had left ventricular ejection fraction < or =45%. One patient had atrioventricular nodal reciprocating tachycardia requiring cardioversion. Three patients had Wolff-Parkinson-White syndrome. Two of them had atrial fibrillation with ventricular rate 300 bpm and 1 had atrioventricular tachycardia 300 bpm. Fetal echocardiography was performed before and after the procedure.

Results: Three women had an electroanatomic map and ablation done without X-ray exposure. The mean fluoroscopy time in the whole group was 42 +/- 37 seconds. The mean procedure time was 56 +/- 18 minutes. After the procedure, all women and fetuses were in good condition. After a mean period of 43 +/- 23 months follow up (FU), all patients were free of arrhythmia without complications related to ablation either in the mothers or children.

Conclusion: Ablation can be performed safely with no or minimal radiation exposure during pregnancy. In the setting of malignant, drug-resistant arrhythmia, ablation may be considered a therapeutic option in selected cases.

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http://dx.doi.org/10.1111/j.1540-8167.2010.01727.x	DOI Listing

August 2010

[Successful outcome of a pregnancy with an extremely low fetal heart rate (34 bpm) due to isolated complete heart block--case report].

Authors:

Anita Hamela-Olkowska Joanna Dangel Maria Miszczak-Knecht

Ginekol Pol 2009 Sep;80(9):708-11

Poradnia Perinatologii i Kardiologii Perinatalnej, II Katedra i Klinika Połoznictwa i Ginekologii, Warszawski Uniwersytet Medyczny.

Isolated complete congenital heart block (CHB) in the majority of cases is associated with the presence of autoantibodies to SSA (Ro) and SSB (La) antigens in the maternal serum. The prognosis is less favorable in fetuses with a ventricular rate < 55bpm. We have reported a case of a fetus with an isolated non-autoimmune CHB with an extremely low ventricular rate (34bpm) in which the outcome was favorable. In the neonate the non-compaction of the myocardium was diagnosed.

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September 2009

[Estimation of the atrioventricular time interval by pulse Doppler in the normal fetal heart].

Authors:

Anita Hamela-Olkowska Joanna Dangel

Ginekol Pol 2009 Aug;80(8):584-9

Poradnia Perinatologii i Kardiologii Perinatalnej, II Katedra i Klinika Połoznictwa i Ginekologii, Warszawski Uniwersytet Medyczny.

Objectives: To assess normative values of the fetal atrioventricular (AV) time interval by pulse-wave Doppler methods on 5-chamber view.

Material And Methods: Fetal echocardiography exams were performed using Acuson Sequoia 512 in 140 singleton fetuses at 18 to 40 weeks of gestation with sinus rhythm and normal cardiac and extracardiac anatomy. Pulsed Doppler derived AV intervals were measured from left ventricular inflow/outflow view using transabdominal convex 3.5-6 MHz probe.

Results: The values of AV time interval ranged from 100 to 150 ms (mean 123 +/- 11.2). The AV interval was negatively correlated with the heart rhythm (p<0.001). Fetal heart rate decreased as gestation progressed (p<0.001). Thus, the AV intervals increased with the age of gestation (p=0.007). However, in the same subgroup of the fetal heart rate there was no relation between AV intervals and gestational age. Therefore, the AV intervals showed only the heart rate dependence. The 95th percentiles of AV intervals according to FHR ranged from 135 to 148 ms.

Conclusions: 1. The AV interval duration was negatively correlated with the heart rhythm. 2. Measurement of AV time interval is easy to perform and has a good reproducibility. It may be used for the fetal heart block screening in anti-Ro and anti-La positive pregnancies. 3. Normative values established in the study may help obstetricians in assessing fetal abnormalities of the AV conduction.

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August 2009

[The possibility of cardiovascular system evaluation in fetuses at 11.0 to 13.6 weeks of gestation in a reference perinatal cardiology centre].

Authors:

Paweł Własienko Anita Hamela-Olkowska Katarzyna Jalinik Joanna Dangel

Ginekol Pol 2009 Apr;80(4):268-73

Poradnia Perinatologii i Kardiologii Perinatalnej, II Katedra i Klinika Połoznictwa i Ginekologii, Warszawski Uniwersytet Medyczny.

Objective: To assess the possibility of foetal circulatory system evaluation between 11+0 to 13+6 weeks of gestation in a reference perinatal cardiology centre.

Material And Methods: A prospective study was conducted between January 2004 and June 2008 in patients admitted to the reference perinatal cardiology centre for a foetal echocardiographic examination. 1170 foetuses were examined: 31 in 2004, 167 in 2005, 310 in 2006, 406 in 2007 and 262 in the first half of 2008. All foetuses had early echocardiography performed during 11-13+6 weeks of gestation, following the Foetal Medicine Foundation guidelines. The examination included: position and size of the heart, 4-chamber view and outflow tracts evaluation. The flow through ductus venosus, atrio-ventricular and arterial valves were assessed with colour and pulsed Doppler. 982 patients with confirmed normal heart anatomy in the second trimester were further analysed.

Results: Mean maternal age was 30 years. 237 (20%) patients were >35 years old. Mean CRL (crown rump length) was 65 mm. 4-chamber view was obtained in 933 foetuses (95%) and outflow tracts in 813 (83%) cases. Diagnostic flow through the tricuspid valve was detected in 894 (91%) foetuses.

Conclusions: Early foetal echocardiography is available in a reference perinatal cardiology centre since 11th week of gestation, in most cases with transabdominal probe. Possibility of the foetal heart anatomy evaluation increases with gestational age. Only patients from the high risk group (i.e. previous child with a major cardiac defect, diabetes mellitus, monochorionic pregnancy) and foetuses with increased NT should be examined. In every case, a control echocardiographic examination in the second trimester must be performed.

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April 2009

[Evaluation of the embryonic and foetal heart rate at 6(+0) to 11(+6) weeks of gestation].

Authors:

Anita Hamela-Olkowska Katarzyna Wiech Katarzyna Jalinik Dariusz Zaryjewski Leszek Kornatowski Joanna Dangel

Ginekol Pol 2009 Mar;80(3):188-92

Il Katedra i Klinika Połoznictwa i Ginekologii AM w Warszawie.

Objective: To establish the normal range of embryonic heart rate (EHR) and foetal heart rate (FHR) at 6(+0) to 11(+6) weeks of gestation, to evaluate the risk of pregnancy loss depending on EHR and FHR values and to establish if checking of FHR with the use of M-mode method is possible in different levels obstetric departments.

Material And Methods: Transvaginal or transabdominal ultrasound using M-mode in 255 singleton pregnancies. Maternal age varied between 16 and 44 years (mean 29 +/- 5 years). There were 67% primigravidas. Gestational week was established on the basis of CRL measurements. Demographic factors, obstetrical history and follow-up were collected. Data was statistically reviewed.

Results: FHR varied between 47 and 192 bpm (mean 154 +/- 26 bpm). At 6 weeks, mean EHR was 116 +/- 21 bpm, then slowly increased, reaching mean 172 +/- 9 bpm at 10 weeks. At 11 weeks the mean FHR achieved the level of 165 +/- 7 bpm. The difference was statistically significant. The r-correlation ratio between FHR and the gestational week was 0.58. In case of 7 embryos (2.75%) at 6.1 to 8.1 weeks of gestation slow FHR was noted (< 100 bpm). The scan performed 7-10 days later revealed miscarriages in all cases.

Conclusions: EHR and FHR in the first trimester depends on gestational week. It increases since 6 to 9 weeks and decreases after 10 weeks. The highest values of FHR are observed between 9 and 10 weeks of gestation. The risk of early pregnancy loss increases significantly in case of detecting slow FHR. FHR can be checked by M-mode methods using any kind of ultrasound machine.

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March 2009

[Ablation of atypical, fast atrio-ventricular nodal tachycardia in a pregnant woman--a case report].

Authors:

Łukasz Szumowski Franciszek Walczak Joanna Dangel Ewa Szufladowicz Robert Bodalski Paweł Derejko Piotr Urbanek Ewa Michalak Jolanta Prokop Michał Orczykowski Włodzimierz J Musiał

Kardiol Pol 2006 Feb;64(2):221-4

Instytut Kardiologii, Warszawa.

We describe a case of a 24-year-old pregnant woman (35 hbd) who was admitted because of fast (240 bpm) supraventricular tachycardia which required electrical external cardioversion (transesophageal atrial pacing and drugs were ineffective). She underwent RF ablation during which a single RF application effectively cured atypical atrio-ventricular nodal tachycardia. The duration of fluoroscopy was 53 seconds. The child was delivered on time and with no complications.

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February 2006

[Conjoined twins--diagnostic problems, difficult decisions in choosing the most optimal management in one of the rarest pathologies of pregnancy].

Authors:

Renata Jaczyńska Krzysztof T Niemiec Monika Przybyłkowska Joanna Dangel Jerzy Zwoliński Ewa Helwich Jan Luczyc-Wyhowski Mariusz Furmanek

Ginekol Pol 2005 Aug;76(8):602-11

Klinika Połoznictwa i Ginekologii Instytutu Matki i Dziecka w Warszawie.

Conjoined twins are the rarest type of monozygotic, monochorionic, monoamniotic twins--incidence about 1:50000 - 1:100000. A conjoined twin pregnancy is a very special pathology in obstetrics and usually ends prematurely. 40% of twins are stillborn, while 35% die within 24 hours after delivery. The development of ultrasonography and in the last years also MRI, allows earlier detection and diagnosis of fetal malformation and enables precise evaluation of the anatomy, type of malformation and how the twins are conjoined. Chances of survival and way of delivery depends on the degree of fusion of the organs and possibilities of surgical separation. Obstetric care for a women with conjoined twins does not differ from that in a normal twin pregnancy, but the newborns require special medical care after delivery. We would like to stress the diagnostic problems and the difficult decisions that we encountered. The degree of conjoinment in this case excluded surgical separation of the twins. A multidisciplinary team of experts decided to end the pregnancy at 31 wks by cesarean section. Delivery at a later term could increase the risk of uterine rupture and surgical complications.

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August 2005

[RF ablation of a permanent atrial tachycardia in a pregnant woman without X-Ray use in the 24th week of pregnancy].

Authors:

Łukasz Szumowski Franciszek Walczak Hanna Siudalska Katarzyna Barańska Joanna Dangel Beata Kuśmierczyk Paweł Derejko Ewa Szufladowicz Krzysztof Karpiński Andrzej Przybylski Piotr Hoffman

Kardiol Pol 2005 Jan;62(1):81-6

Klinika Zaburzeń Rytmu Serca Instytutu Kardiologii w Warszawie.

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January 2005

Recommendations for the practice of fetal cardiology in Europe.

Authors:

Lindsey Allan Joanna Dangel Vlasta Fesslova Jan Marek Mats Mellander Ingrid Oberhänsli Renate Oberhoffer Gurleen Sharland John Simpson Sven-Erik Sonesson

Cardiol Young 2004 Feb;14(1):109-14

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http://dx.doi.org/10.1017/s1047951104001234	DOI Listing

February 2004