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OC161: Persistent left superior vena cava in fetuses at risk for congenital heart defects: associations and outcome

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OC161: Persistent left superior vena cava in fetuses at risk for congenital heart defects: associations and outcome
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  16th World Congress on Ultrasound in Obstetrics and Gynecology Oral communication abstracts anomalies, 12 of which were 22q11 deletions, occurring in 2/25 of the isolated group and 10/48 of the complex group, with a further 2complex cases probably 22q11 deletion. There were 29 pregnancyinterruptions, 4 intrauterine deaths, 31 live-births, 4 neonatal deathsand 3 patients lost to follow-up. The remaining 4 pregnancies arecontinuing. Conclusion:  The diagnosis of a right-sided aortic arch can bemade by fetal echocardiography, either as an isolated lesion orin association with other cardiac malformations, from as early as12 weeks gestation. It can be difficult to distinguish from a doublearch. Its increasing incidence in our series probably indicates thatthediagnosis waspreviously overlooked.Karyotyping intheabsenceof other abnormal findings may be unnecessary in every case, butestablishment of 22q11 micro-deletion status in those cases withother anomalies is important. OC159Right and double aortic arch detected in fetal life C. Berg 1 , F. Bender 1 , A. Geipel 1 , T. Kohl 1 , J. Breuer 2 ,U. Herberg 2 , R. Axt-Fliedner 3 , U. Gembruch 1 1 Department of Obstetrics and Prenatal Medicine, Universityof Bonn, Germany,  2 Department of Pediatric Cardiology,University of Bonn, Germany,  3 Division of Prenatal Medicine, University of L¨ ubeck, Germany Objective:  To evaluate the prenatal distribution, the associatedconditions and the outcome of right aortic arch (RAA) and doubleaortic arch (DAA) detected in fetal life. Methods:  Retrospective review of all cases detected prenatally inwhich the aortic arch (or one of the arches) was located to the rightof the trachea in the three-vessel-trachea view between 1998 and2005 in two tertiary referral centers in Germany. Results:  68 cases were detected in the study period. 19 (28%)had a RAA with mirror-image branching, 20 (30%) had a RAAof ‘unknown type’, 27 (40%) had a RAA with aberrant leftsubclavian or innominate artery and 2 (2%) had a DAA. All 18cases of RAA with mirror image branching were associated withcardiac defects, most frequently tetralogy of Fallot ( n = 11 ), 2 hadextracardiac malformations and 2 had a microdeletion 22q11. 19of the 20 cases with RAA of ‘unknown type’ were associated withheterotaxy syndromes (12 left isomerism; 7 right isomerism) andhad additional cardiac malformations (13 atrioventricular septaldefects; 13 discordant ventriculoarterial connection; 9 right outflowtract obstruction). 3 of the 26 cases of RAA with with aberrantleft subclavian or innominate artery had cardiac defects, 2 hadextracardiac malformations and 3 had a microdeletion 22q11. The2 cases with DAA were isolated findings. The outcome in ourseries solely depended on the associated cardiac and extracardiacmalformations. All cases with isolated anomalies of the aortic archsurvived and are currently doing well. Conclusions:  RAA detected in fetal life has to be followed bya meticulous inspection of the fetal anatomy as it is frequentlyassociated with other cardiac/non-cardiac malformations thatdetermine the outcome. Isolated RAA is a benign vascular anomalyand may not affect the outcome, however, karyotyping has tobe considered due to the sporadic association with microdeletion22q11. OC160Accuracy of prenatal echocardiographic diagnosis of conotruncal anomalies and fetal outcome A. Galindo, J. Arbues, J. M. Puente, D. Escribano,F. Guti ´errez-Larraya Hospital Universitario, Spain Background:  The objective of this study is to evaluate the accuracyof fetal echocardiography for the diagnosis of conotruncal defectsand to examine the outcome of those fetuses identified. Methods:  We retrospectively studied 179 cases of conotruncalanomalies prenatally diagnosed in a single tertiary referral centerfrom January 1990 to December 2005. Twenty-five out of the179 fetuses were lost in follow-up and were excluded from furtheranalysis. In the remaining cases postnatal information concerningthe cardiac anomaly was obtained either by echocardiography,cardiac catheterization, surgery, or autopsy. Conditions includedweretetralogyofFallot(TOF),doubleoutletrightventricle(DORV),transposition of the great arteries simple (D Tga S) and complex(D Tga C), corrected transposition (L Tga), truncus arteriosus (TA),interruptedaorticarch(Iaa),coarctationoftheaortawithventricularseptal defect (CoAo-Vsd) and pulmonary atresia with ventricularseptal defect (PA-VSD). We used the diagnosis made in the lastechocardiography before birth to analyze the accuracy rate. Results:  Mean gestational age at diagnosis was 24.3 weeks (range12 to 39). In 99/154 (64%) cases the defect was isolated. The mainresults are shown in the table. Conclusions:  Prenatal echocardiography constitutes an excellenttool evaluating conotruncal anomalies, with a high degree of agree-ment between prenatal and postnatal diagnosis. Fetal karyotype ismandatory because thehigh rate of associated chromosomal defects.Overallprognosisoffetuseswithconotruncalanomaliesispoorwiththe exception of d-transposition of the great arteries. OC161Persistent left superior vena cava in fetuses at risk forcongenital heart defects: associations and outcome L. Franceschetti, F. Taddei, M. Signorelli, C. Groli Spedali Civili, Brescia, Italy Objective:  To evaluate the incidence, associations and outcome of persistent left superior vena cava (PLSVC) in fetuses at risk forcongenital heart defects (CHD). Methods:  This is a retrospective study of 15 cases of PLSVCdetected prenatally between 2000 and 2005, on a population of 1155 consecutive fetuses at risk for CHD. PLSVC was diagnosed in OC160: Table Postnatal diagnosis Chromosomal defects OutcomePrenatal diagnosis (n) Same Other N (%) Most common TOP-IUDPostnatal death Alive TOF (39) 38 1 PA-Vsd 17 (43) 5 T21 18 6 15D Tga Simple (23) 17 6 D Tga C 0 3 1 19D Tga Complex (15) 13 2 Dorv 0 2 1 12L Tga (10) 10 0 4 1 5Dorv (36) 33 3 TOF 10 (28) 6 T18 23 4 9TA (15) 12 1 TOF, 2 PA-Vsd 5 (33) 3 microdeletion 22q11 5 5 5Iaa (6) 5 1 Vsd 3 (50) 2 microdeletion 22q11 1 1 4CoAo-Vsd (8) 4 3 Iaa, 1 Vsd 0 0 4 4PA-Vsd (2) 1 1 TA 2 (100) 2 microdeletion 22q11 2 0 0Total (154) 86% 14% 37 (24) 38% 15% 47% 406  Ultrasound in Obstetrics & Gynecology 2006 ;  28 :  359–411  3–7 September 2006, London, UK Oral communication abstracts the presence of a supernumerary vessel to the left of the pulmonarytrunkinthe3-vessel view,a‘cyst’atthelateralwalloftheleftatriumor a dilated coronary sinus in the 4-chamber view. A meticulousinspection of the fetal anatomy was performed for detection of associated anomalies. Postnatal follow-up was available for allsurviving patients. Prenatal diagnosis was confirmed by neonatalechocardiography or at autopsy. Results: Of1155 cardiac referrals, 231 had aCHD(20%).APLSVCwas detected in 15 fetuses (1.2% of population at risk for CHD;4.3% of fetuses with CHD). In 5 cases PLSVC had no associatedcondition.In10casesitwasassociatedwithCHD(6withanisolatedcardiac malformation, 3 with complex malformations syndromesand one with associated extracardiac anomalies). The most frequentCHD were ventricular septal defects and left ventricular outflowtract obstructions. The outcome of PLSVC was determined solelyby the associated conditions. All cases with isolated PLSVC orassociated correctable extracardiac malformations were alive andwell at birth or after surgery. Conclusions:  PLSVC is easily recognised on standard cardiacscreening.Itsdetectioninfetallifehastobefollowedbyameticulousinspection of fetal anatomy for its frequent association withcardiac/extracardiac malformations that determine the outcome.Isolated PLSVC is a benign vascular anomaly and may not affect theoutcome. OC162Fetal kinetocardiogram for early detection and treatment of first-degree AV block in fetuses exposed to maternalautoantibodies – a prospective study U. Elchalal, D. Mevorach, Z. Perles, S. Gavri, A. Nir,M. Nadjari, N. Yanai, A. J. J. T. Rein Hadassah Hebrew University Hospital, Israel  Aim: Toappraise thefeasibilityoffetalkinetocardiogram (FKCG)todetect 1st degree AV block in fetuses of SSA/SSB antibody-positivemothers. Methods:  FKCGs were performed in 60 SSA/SSB antibody-positivepregnant mothers enrolled in this prospective study (SLE [55],Sjogren disease [3], Mixed connective tissue disease [2]). FKCG wasobtained consecutively from 13 to 40 weeks of gestation and AVconduction time measured form raw-data scan-line cineloops andwas compared to AV conduction time measured in an age-matchedcontrol group of 120 normal fetuses. Results:  FKCG was obtained readily (acquisition time 1–4 minutes,mean 2 minutes) in all 180 studies. In 53 fetuses from diseasedmother, the AV conduction time (mean  90 + 13  msec) wasnormal with comparison to our laboratory control normal data( 84 + 11  msec). In 7 fetuses, 1st degree AV block was diagnosed(AV conduction time  > 3  Z-scores). All the mothers of fetuses with1st degree AV block received 2–4 mg/day Dexamethazone dailyuntil delivery and their newborn received dexamethazone support6 weeks after delivery. All fetuses treated had normalization of AVconductionwithin2 weeksoftreatmentandwerebornwithnosignsof AV block on EKG after delivery. Conclusions:  1) FKCG is useful in early detection of 1st degree AVblock in thefetus. 2)Dexamethazone may counteract the deleteriouseffectsof SSA/SSB antibodies on the fetal AV node if given very earlyupon detection of 1st degree AV block. OC163Sustained fetal bradycardia: mechanisms and pitfalls  J. S. Carvalho 1 , E. Jaeggi 2 1 Royal Brompton and St. George’s Hospitals, United Kingdom,  2 Hospital for Sick Children, Toronto, Canada Objective:  To assess accuracy in differentiating various mechanismsof bradycardia (brady) based on initial ultrasound exam. Preciserecognition of the arrhythmia mechanism is important as thisinfluences pregnancy management. Methods:  Retrospectiveanalysis ofall cases offetalbrady (heartrate ≤ 100  bpm) from 2 referral centres for fetal cardiology since 2000.Cases were identified from hospital databases. Echo and outcomedata were reviewed. Results:  Fifty six fetuses had sustained brady at 11–37 gestationalweeks (table). Suspected diagnosis CasesGestational age(weeks)Heart rate(bpm) Outcome Accuracy Sinus brady 2 30, 36 75–90 Resolved in 2 100%Atrial bigeminy 11 18–31 65–100 Resolvedin 11100%Complete AVB,isolated23 19–37 42–97 Resolved in 0 100%Complete AVB, heartdefect10 11–37 42–75 Resolved in 0 100%2:1 AVB, isolated (9),heart defect (1)10 18–29 65–75 Resolved in 7 30%AVB = atrioventricular block. Of 10 cases with suspected 2:1 AVB the arrhythmia progressedto 3:1 AVB (1 case), remained stable (1 case), alternated withventricular tachycardia (1 case) and resolved spontaneously onfollow-up exams in the remaining 7. Of these, 4 were transientlytreated with steroids and all 7 were subsequently shown to benegative for maternal antibodies. Careful review of the initialexam in these 7 cases shows the inter-atrial interval (A-A) tohave an alternating, repetitive pattern of 2 similar but distinctinter-atrial intervals (A-A1, longer and A1-A2, shorter). The A2atrial beat occurred slightly prematurely with A1-A2 time intervalsranging between 358–420 ms (atrial rates = 142–167  bpm). Non-conduction of this late-occurring atrial event ( > 350  ms) cannot beexplained by atrial, but junctional ectopy. Bigeminal prematurejunctional beats fail to depolarize the ventricles but conductretrograde to the atria, simulating 2:1 AVB. Conclusions:  Unlike other brady mechanisms, bigeminal junctionalblocked ectopics (‘pseudo 2:1 AVB’) was regularly overlooked as acause of sustained fetal brady. This arrhythmia, which is benign andnot indicative of primary impairment of AV conduction does notrequire any treatment and should be considered in the differentialdiagnosis of any suspected 2:1 AVB. OC164The influence of prenatal diagnosis on the outcome of children with isolated congenital heart disease I. B. Fuchs 1 , H. M ¨uller 1 , H. Abdul-Kaliq 2 , T. Harder 3 , J. W. Dudenhausen 1 , W. Henrich 1 1 Department of Obstetrics, Charit ´ e Campus Virchow,Germany,  2 Department of Pediatric Cardiology, GermanHeart Centre Berlin, Germany,  3 Department of Experimental Obstetrics, Charit´ e Campus Virchow, Germany Objective:  This study investigates if prenatal diagnosis improves theshort and longterm outcome of children with isolated congenitalcardiac disease. Methods:  Children with the prenatal diagnosis of an isolatedcongenital heart defect were compared with children, in whomthe diagnosis was achieved postnatally over a period of up to tenyears.Outcomecriteriawerepreoperativeclinicalcondition,surgicaloutcome and postoperative course, longterm morbidity and short-and longterm mortality. Results: Theprenatalgroup hadahigherpre-operativeo2saturation(  p < 0 . 07 ), less pre-operative cardiac insufficiency (  p < 0 . 05 ), lesspre-operative closure of the ductus (  p < 0 . 04 ), a shorter durationof post-operative mechanical ventilation (  p < 0 . 05 ), a lower need of re-surgery (  p < 0 . 02 ) and a shorter stay at the intensive care unit Ultrasound in Obstetrics & Gynecology 2006 ;  28 :  359–411  407
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