Fetal Myocardial Performance (Tei) Index in Fetal Hemoglobin Bart’s Disease

Abstract

Purpose: To assess the effectiveness of Tei index in differentiating fetal Hb Bart’s disease in pregnancies at risk in the first half of pregnancy

Materials and Methods: Pregnancies meeting the inclusion criteria were consecutively recruited to the study. The inclusion criteria were as follows: 1) singleton pregnancies, 2) gestational age of 12 – 20 weeks, 3) at risk of Hb Bart’s disease, 4) confirmed fetal diagnosis of Hb Bart’s disease. Fetuses with frank hydrops fetalis or fetuses with chromosomal abnormalities or structural anomalies were excluded. Tei index was performed and immediately recorded before invasive prenatal diagnosis was performed.

Results: Of 152 fetuses, 50 were finally proven to be affected by Hb Bart disease, and the remainder was unaffected. Mean (± SD) Tei index were 0.48 ± 0.07 and 0.54 ± 0.08 in group of unaffected and affected fetuses, respectively. The mean difference was 0.05 which was statistically significant (p < 0.001). Likewise, mean (± SD) isovolumetric contraction time (ICT) in normal fetuses were also significantly different from that of affected fetuses.

Conclusion: In the first half of pregnancy, Tei index in the fetuses with Hb Bart's disease was significantly higher than that in normal fetuses implying that Tei index may be a novel useful and non-invasive tool for early detection of fetal Hb Bart’s disease among pregnancies at risk.

Zusammenfassung

Ziel: Beurteilung der Effektivität des Tei-Index, um das Hb-Bart’s-Hydrops-fetalis-Syndrom bei Risikoschwangerschaften in der ersten Hälfte der Schwangerschaft abzugrenzen.

Material und Methoden: Schwangerschaften, welche die Einschlusskriterien erfüllten, wurden in Folge in die Studie aufgenommen. Die Einschlusskriterien waren im Folgenden 1) Einlingsschwangerschaften, 2) 12 – 20 Gestationswoche, 3) Risiko eines Hb-Bart’s-Hydrops-fetalis-Syndroms, 4) bestätigte fetale Diagnose eines Hb-Bart’s-Hydrops-fetalis-Syndrom. Feten mit schwerem generalisiertem Hydrops fetalis oder Feten mit Chromosomen- und strukturellen Anomalien wurden ausgeschlossen. Die Tei-Index-Bestimmung wurde durchgeführt und sofort protokolliert, noch vor Durchführung einer invasiven Pränataldiagnostik.

Ergebnisse: Bei 50 der 152 Feten wurde letztlich ein Hb-Bart’s-Hydrops-fetalis-Syndrom diagnostiziert, die übrigen Feten waren nicht betroffen. Die mittleren (+SD) Tei-Indizes betrugen bei den unbeeinträchtigten Feten 0,48 + 0,07 und bei den betroffenen Feten 0,54 + 0,08. Der mittlere Unterschied war mit 0,05 statistisch signifikant (p < 0,001). Ebenso unterschied sich die mittlere (+SD) isovolumetrische Kontraktionszeit der normalen Feten signifikant von derjenigen der betroffenen Feten.

Schlussfolgerung: In der ersten Schwangerschaftshälfte war der Tei-Index bei Feten mit Hb-Bart’s-Hydrops-fetalis-Syndrom signifikant höher als bei normalen Feten. Dies lässt darauf schließen, dass der Tei-Index eine neue nützliche, nicht invasive Methode für die Frühdiagnose des Hb-Bart’s-Hydrops-fetalis-Syndrom bei Risikoschwangeren darstellen könnte.

Key words

heart - echocardiography - ultrasound-doppler - fetus

• References

• 1 Ko TM, Hsieh FJ, Hsu PM et al. Molecular characterization of severe alpha-thalassemias causing hydrops fetalis in Taiwan. Am J Med Genet 1991; 39: 317-320
• 2 Lemmens-Zygulska M, Eigel A, Helbig B et al. Prevalence of alpha-thalassemias in northern Thailand. Hum Genet 1996; 98: 345-347
• 3 Tongsong T, Piyamongkol W, Tongprasert F et al. Fetal splenic artery peak velocity (SPA-PSV) at mid-pregnancy as a predictor of Hb Bart's disease. Ultraschall in Med 2011; 32: S41-S45
• 4 Leung KY, Cheong KB, Lee CP et al. Ultrasonographic prediction of homozygous alpha(0)-thalassemia using placental thickness, fetal cardiothoracic ratio and middle cerebral artery Doppler: alone or in combination?. Ultrasound Obstet Gynecol 2010; 35: 149-154
• 5 Amann C, Geipel A, Muller A et al. Fetal anemia of unknown cause – a diagnostic challenge. Ultraschall in Med 2011; 32: E134-E140
• 6 Bahtiyar MO, Copel JA. Cardiac changes in the intrauterine growth-restricted fetus. Semin Perinatol 2008; 32: 190-193
• 7 Chao G, Zheng C, Meng D et al. Tei index: the earliest detectable cardiac structural and functional abnormality detectable in Hb Bart's foetal edema. Int J Cardiol 2009; 134: e150-e154
• 8 Falkensammer CB, Paul J, Huhta JC. Fetal congestive heart failure: correlation of Tei-index and Cardiovascular-score. J Perinat Med 2001; 29: 390-398
• 9 Ichizuka K, Matsuoka R, Hasegawa J et al. The Tei index for evaluation of fetal myocardial performance in sick fetuses. Early Hum Dev 2005; 81: 273-279
• 10 Letti Muller AL, Barrios PM, Kliemann LM et al. Tei index to assess fetal cardiac performance in fetuses at risk for fetal inflammatory response syndrome. Ultrasound Obstet Gynecol 2010; 36: 26-31
• 11 Tei C, Ling LH, Hodge DO et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function--a study in normals and dilated cardiomyopathy. J Cardiol 1995; 26: 357-366
• 12 Tei C, Dujardin KS, Hodge DO et al. Doppler echocardiographic index for assessment of global right ventricular function. J Am Soc Echocardiogr 1996; 9: 838-847
• 13 Tei C. New non-invasive index for combined systolic and diastolic ventricular function. J Cardiol 1995; 26: 135-136
• 14 Eidem BW, Tei C, O‘Leary PW et al. Nongeometric quantitative assessment of right and left ventricular function: myocardial performance index in normal children and patients with Ebstein anomaly. J Am Soc Echocardiogr 1998; 11: 849-856
• 15 Pellett AA, Tolar WG, Merwin DG et al. The Tei index: methodology and disease state values. Echocardiography 2004; 21: 669-672
• 16 Wolk MJ, Keefe JF, Bing OH et al. Estimation of Vmax in auxotonic systoles from the rate of relative increase of isovolumic pressure: (dP-dt)kP. J Clin Invest 1971; 50: 1276-1285
• 17 Grossman W, McLaurin LP, Rolett EL. Alterations in left ventricular relaxation and diastolic compliance in congestive cardiomyopathy. Cardiovasc Res 1979; 13: 514-522
• 18 Tongsong T, Tongprasert F, Srisupundit K et al. Venous Doppler studies in low-output and high-output hydrops fetalis. Am J Obstet Gynecol 2010; 203: 488-486
• 19 Tongsong T, Wanapirak C, Piyamongkol W et al. Fetal ventricular shortening fraction in hydrops fetalis. Obstet Gynecol 2011; 117: 84-91