Zusammenfassung
Ziel: Bewertung der Treffsicherheit der A.-lienalis-Maximalgeschwindigkeit zur Prädiktion
einer fetalen Hämoglobin(Hb-)Bart-Erkrankung bei gefährdeten Feten in der Mitte der
Schwangerschaft. Material und Methoden: Schwangere Frauen mit dem Risiko eines Hb-Bart-erkrankten Fetus wurden in der 18.–22.
Schwangerschaftswoche (SSW) in die Studie aufgenommen und erhielten vor Chordozentese
eine Messung der SPA-PSV. Die definitive Diagnose, die als Goldstandard herangezogen
wurde, basierte auf fetaler Hb-Typisierung mittels Hochleistungsflüssigkeitschromatografie
(HPLC). Ergebnisse: Insgesamt wurden 136 Feten aus 132 Einlingsschwangerschaften und 2 Zwillingsschwangerschaften
in die Studie eingeschlossen. Das mittlere maternale Alter war 28,7 ± 5,4 Jahre, das
mittlere Gestationsalter war 19,1 ± 1,02 Wochen, und die Inzidenz einer Hb-Bart-Erkrankung
lag bei 23,5 % (32 Feten). Unter Verwendung des 1,51-fachem Medianwerts (MoM) der
SPA-PSV als Diskriminationswert lag die Sensitivität, Spezifizität, der positiv prädiktive
Wert und der negativ prädiktive Wert der SPA-PSV zur Identifikation von betroffenen
Feten bei 84,4 % (32 von 36 Fällen), 98,1 %, 93,1 % bzw. 95,3 %. Fast alle gesunden
Feten hatten eine normale SPA-PSV. Schlussfolgerung: SPA-PSV-Messungen in der Mitte der Schwangerschaft können als ergänzende Methode
zur Identifizierung von Feten mit Hb-Bart-Erkrankung herangezogen werden und haben
eine hohe, wenngleich nicht perfekte Treffsicherheit. Die Methode könnten helfen,
bei manchen Feten das Risiko nicht erforderlicher Chordozentesen zu reduzieren.
Abstract
Purpose: To determine the accuracy of splenic artery peak systolic velocity (SPA-PSV) in predicting
fetal hemoglobin (Hb) Bart’s disease at mid-pregnancy among fetuses at risk. Materials and Methods: Pregnant women at risk of having a fetus with Hb Bart’s disease were recruited into
the study at 18 – 22 weeks of gestation and underwent SPA-PSV measurement before cordocentesis.
The final diagnosis used as a gold standard was based on fetal hemoglobin typing using
high performance liquid chromatography (HPLC). Results: A total of 136 fetuses from 132 singleton pregnancies and 2 twin pregnancies were
recruited into the study. The mean maternal age was 28.7 ± 5.4 years, the mean gestational
age was 19.1 ± 1.02 weeks, and the incidence of Hb Bart’s disease was 23.5 % (32 fetuses).
Using SPA-PSV above 1.51 Multiple of Median (MoM) as a cut-off point, the sensitivity,
specificity, positive predictive value and negative predictive value of SPA-PSV to
identify affected fetuses was 84.4 % (32 from 36 cases), 98.1 %, 93.1 % and 95.3 %
respectively. Nearly all normal fetuses had a normal SPA-PSV. Conclusion: SPA-PSV assessment at mid-pregnancy may be used as an adjunct method to identify
fetuses with Hb Bart’s disease with high, but not perfect, accuracy and may reduce
the risk from unnecessary cordocentesis in some fetuses.
Key words
pregnancy - fetus - ultrasound Doppler
References
1
Lemmens-Zygulska M, Eigel A, Helbig B et al.
Prevalence of alpha-thalassemias in northern Thailand.
Hum Genet.
1996;
98
345-347
2
Thumasathit B, Nondasuta A, Silpisornkosol S et al.
Hydrops fetalis associated with Bart’s hemoglobin in northern Thailand.
J Pediatr.
1968;
73
132-138
3
Tongsong T, Wanapirak C, Sirivatanapa P et al.
Prenatal eradication of Hb Bart’s hydrops fetalis.
J Reprod Med.
2001;
46
18-22
4
Tongsong T, Wanapirak C, Sirivatanapa P et al.
Amniocentesis-related fetal loss: a cohort study.
Obstet Gynecol.
1998;
92
64-67
5
Tabor A, Philip J, Madsen M et al.
Randomised controlled trial of genetic amniocentesis in 4606 low-risk women.
Lancet.
1986;
1
1287-1293
6
Ghidini A, Sepulveda W, Lockwood C J et al.
Complications of fetal blood sampling.
Am J Obstet Gynecol.
1993;
168
1339-1344
7
Tongsong T, Wanapirak C, Kunavikatikul C et al.
Fetal loss rate associated with cordocentesis at midgestation.
Am J Obstet Gynecol.
2001;
184
719-723
8
Alshimmiri M M, Hamoud M S, Al-Saleh E A et al.
Prediction of fetal anemia by middle cerebral artery peak systolic velocity in pregnancies
complicated by rhesus isoimmunization.
J Perinatol.
2003;
23
536-540
9
Bullock R, Martin W L, Coomarasamy A et al.
Prediction of fetal anemia in pregnancies with red-cell alloimmunization: comparison
of middle cerebral artery peak systolic velocity and amniotic fluid OD 450.
Ultrasound Obstet Gynecol.
2005;
25
331-334
10
Pereira L, Jenkins T M, Berghella V.
Conventional management of maternal red cell alloimmunization compared with management
by Doppler assessment of middle cerebral artery peak systolic velocity.
Am J Obstet Gynecol.
2003;
189
1002-1006
11
Rimon E, Peltz R, Gamzu R et al.
Management of Kell isoimmunization – evaluation of a Doppler-guided approach.
Ultrasound Obstet Gynecol.
2006;
28
814-820
12
Nicolaides K H.
Studies on fetal physiology and pathophysiology in rhesus disease.
Semin Perinatol.
1989;
13
328-337
13
Bahado-Singh R, Oz U, Mari G et al.
Fetal splenic size in anemia due to Rh-alloimmunization.
Obstet Gynecol.
1998;
92
828-832
14
Bahado-Singh R, Oz U, Deren O et al.
A new splenic artery Doppler velocimetric index for prediction of severe fetal anemia
associated with Rh alloimmunization.
Am J Obstet Gynecol.
1999;
180
49-54
15
Bahado-Singh R, Oz U, Deren O et al.
Splenic artery Doppler peak systolic velocity predicts severe fetal anemia in rhesus
disease.
Am J Obstet Gynecol.
2000;
182
1222-1226
16
Tongsong T, Tongprasert F, Srisupundit K et al.
High fetal splenic artery peak velocity in fetuses with hemoglobin Bart disease: a
preliminary study.
J Ultrasound Med.
2009;
28
13-18
17
Tongsong T, Wanapirak C, Sirivatanapa P et al.
Prenatal control of severe thalassaemia: Chiang Mai strategy.
Prenat Diagn.
2000;
20
229-34
18
Tongsong T, Tongprasert F, Srisupundit K et al.
Splenic artery: peak systolic velocity of normal fetuses.
Arch Gynecol Obstet.
2009;
[Epub ahead of print]
19
Tongsong T, Wanapirak C, Sirichotiyakul S et al.
Middle cerebral artery peak systolic velocity of healthy fetuses in the first half
of pregnancy.
J Ultrasound Med.
2007;
26
1013-1017
20
Srisupundit K, Piyamongkol W, Tongsong T.
Comparison of red blood cell hematology among normal, alpha-thalassemia-1 trait, and
hemoglobin Bart’s fetuses at mid-pregnancy.
Am J Hematol.
2008;
83
908-910
21
Srisupundit K, Piyamongkol W, Tongsong T.
Identification of fetuses with hemoglobin Bart’s disease using middle cerebral artery
peak systolic velocity.
Ultrasound Obstet Gynecol.
2009;
33
694-697
22
Tongsong T, Wanapirak C, Sirichotiyakul S et al.
Sonographic markers of hemoglobin Bart disease at midpregnancy.
J Ultrasound Med.
2004;
23
49-55
Prof. Theera Tongsong
Obstetrics and Gynecology, Chiang Mai University
110 Intavaroros Road
50200 Chiang Mai
Thailand
Phone: ++ 66/53/94 64 29
Fax: ++ 66/53/94 61 12
Email: ttongson@mail.med.cmu.ac.th