It is well known the effect of cyclooxygenase inhibitor drugs on the ductus ... obstetricians should remain alert for fetal Hb Bart's disease, be it due to rare ...
23rd World Congress on Ultrasound in Obstetrics and Gynecology (NSAIDs), so their use is discouraged beyond 31 weeks of gestation. Also some meals and infusions rich in polyphenols are involved in the pathogenesis of this disturbance. We report a case of prenatal diagnosis of PCDA secondary to maternal ingestion of Diclofenac. A G1P0 pregnant woman was referred to our prenatal diagnosis Centre at 38 weeks of gestation for decreased fetal movements. Fetal echocardiography revealed cardiomegaly and pericardial effusion with hypocontractility more pronounced in the right ventricle, dilated pulmonary artery trunk with an absence of anterograde flow and lack of visualization of the ductus arteriosus. Amniotic fluid index (AFI) and umbilical - MCA Doppler assessment was normal. Immediate Cesarean section was performed. A male with Apgar scores 5/7/9 was born. The baby required only oxygen without mask for a mild respiratory distress. The postnatal echocardiogram reported dilated right cavities, tricuspid regurgitation and mild pulmonary hypertension. The infant evolved favorably and at discharge on day 2 the echocardiogram had normalized. It is well known the effect of cyclooxygenase inhibitor drugs on the ductus arteriosus in the third trimester of pregnancy, so their use is contraindicated. However, frequent self-medication can jeopardize fetal life. In this case the prenatal evaluation with Doppler was normal as well as AFI. The addition of fetal heart examination proved very helpful. Finally we would like to draw attention to the possibility of PCDA in cases of unexplained heart failure in the third trimester of gestation.
P02.13 Two unusual cases of haemoglobin Bart’s hydrops fetalis screened negative on universal prenatal screening for thalassemia K. Kou1 , H.H. Lee1 , B. Lau1 , W. Wong2 , A. Kan3 , M. Tang3 , C. Poon1 , K. Leung1
Poster abstracts
obstetricians should remain alert for fetal Hb Bart’s disease, be it due to rare occurrence of maternal UPD or non-paternity.
P02.14 Prenatal diagnosis of Hb H hydrops fetalis caused by haemoglobin Adana M. Alberry1 , D. Shyam2 , D. Thompson3 1 Fetal Medicine, Cambridge University Hospitals, Cambridge, United Kingdom; 2 Obstetrics and Gynaecology, Luton and Dunstable Hospital, Luton, United Kingdom; 3 Haematology Departments, Luton and Dunstable Hospital, Luton, United Kingdom
We present a rare case of fetal α thalassaemia with hydrops fetalis born to a Filipino couple. Maternal Hb suggested α-thalassaemia trait. Given the high risk ethnic background for α0-thalassaemia the couple were tested for α-globin gene cluster which showed highly unstable alpha-chain variant Hb Adana leading to an α+thalassaemia phenotype. The risk for hydrops fetalis was1 in 4. Amniocentesis showed normal karyotype. DNA analysis from amniocytes showed the fetus to be compound heterozygous for the α0-thalassaemia deletion and the Hb Adana mutation (genotype --FIL/ααAdana). The partners declined any interventions. Hydrops fetalis developed and intrauterine fetal death was diagnosed 28 weeks of gestation. National screening algorithm aims to identify couples at risk of Hb Barts hydrops fetalis due to homozygous α0-thalassaemia. Couples at risk for non deletional Hb H disease in England may not be identified by routine screening.
Supporting information can be found in the online version of this abstract
1 O&G, Queen Elizabeth Hospital, Hong Kong; 2 Pathology, Queen Elizabeth Hospital, Hong Kong; 3 O&G, Queen Mary Hospital, University of Hong Kong, Hong Kong
In universal prenatal screening for alpha thalassemia, if mean corpuscular volume (MCV) of a women is low (1.55MoM). Cordocentesis showed Hb of 4.7g/dL, and Hb Bart’s disease. As the couple kept the pregnancy, four in-utero transfusions were given in interval. The baby was delivered by emergency Caesarean section at 31 weeks who required regular blood transfusions after birth and waited for bone marrow transplantation. Investigations showed fetal Hb Bart’s disease from maternal uniparental disomy (UPD) 16. Case 2: A 21-year-old, G2P0, Chinese woman was referred to our hospital for management of fetal hydrops at 31 weeks. She had a low MCV (76 fL) related to Hb E while her husband’s MCV was normal. Ultrasound examination showed fetal ascites, cardiomegaly but normal MCAPSV (51.4cm/s). Cordocentesis showed Hb of 7.3g/dL. As fetal echocardiography showed features suggestive of near complete premature closure of foramen ovale, emergency Caesarean section was performed two days later. A baby girl of 1.48kg was delivered, but succumbed shortly after birth despite active resuscitation. Subsequent hematological and DNA studies showed Hb Bart’s disease, maternal alpha thalassaemia trait coinheritance with Hb E, and a possibility of non-paternity. If midtrimester anomaly scan shows fetal cardiomegaly or hydrops in a woman with low MCV but normal paternal MCV,
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P03: CARDIAC ANOMALIES AND HEART FUNCTION P03.01 Coarctatio aortae (CoA) diagnosed with B-flow and STIC P. von Brandis, C. Heien, T.M. Eggebø Gynecology and Obstetrics, Stavanger University Hospital, Stavanger, Norway Malformations related to the aortic arch are the most common undiagnosed severe heart defects. Children with CoA can be critically ill shortly after delivery and methods for prenatal detection of these abnormalities are highly desirable. Case 1: an unbalanced 4-chamber view was detected at the routine 19 week scan. Using colour Doppler a narrowing of the descending aortic arch was suspected. Using B-flow and STIC we observed that the flow signal persisted longer in the descending aortic arch than in ductus arteriosus and the descending aorta (Figure 1). C-section was performed in pregnancy week 31 because of breech presentation and premature contractions. Neonatal echocardiography did not confirm the suspected diagnosis. However, 4 days after delivery the blood pressure was higher in arms compared to legs. After one week the child developed heart failure and was transferred to a tertiary centre. CoA was diagnosed by CT-scan, and the baby was successfully operated. Case 2: an unbalanced 4-chamber view was found in the second trimester. Using B-flow and STIC a narrowing of the aortic arch was seen, and again persistent flow signal was observed in the descending aortic arch. Neonatal echocardiography could not confirm the
Ultrasound in Obstetrics & Gynecology 2013; 42 (Suppl. 1): 113–179.
