Literature DB >> 14618656

Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience.

C Berg1, A Geipel, J Smrcek, M Krapp, U Germer, T Kohl, U Gembruch, A A Baschat.   

Abstract

OBJECTIVE: To assess the accuracy of fetal echocardiography in the prenatal diagnosis of cardiosplenic syndromes and the spectrum of associated anomalies.
METHODS: This was a retrospective survey of fetuses in our databases over a period of 10 years with postnatally confirmed prenatal diagnosis of cardiosplenic syndromes.
RESULTS: In 32 of 35 fetuses the prenatal diagnosis of cardiosplenic syndromes was confirmed postpartum. Twenty-two fetuses had left isomerism. Their main prenatal ultrasound features were interrupted inferior vena cava (n = 21), complete atrioventricular septal defect (n = 15), viscerocardiac heterotaxy (n = 15), persistent bradyarrhythmia (n = 12) and fetal hydrops or nuchal edema (n = 12). Twelve pregnancies were terminated, two fetuses were stillborn and eight infants survived. Ten fetuses had right isomerism. Their main sonographic features were juxtaposition of the descending aorta and inferior vena cava (n = 7), complete atrioventricular septal defect (n = 7), left persistent superior vena cava (n = 6) and viscerocardiac heterotaxy (n = 6). In this group there was one stillbirth, five infant deaths and four survivors. The overall survival rate and spectrum of other cardiac malformations were similar between the two groups. Prenatal diagnosis of other visceral features of cardiosplenic syndromes was inconsistent.
CONCLUSION: Cardiosplenic syndromes can be diagnosed with high accuracy by prenatal sonography. A diagnosis of left isomerism should be strongly suggested in the presence of a combination of at least two of the following: (1) complete atrioventricular septal defect or other structural heart disease; (2) interruption of inferior vena cava with azygos continuation; (3) early fetal heart block; (4) viscerocardiac heterotaxy. Right isomerism should be suspected in the presence of a combination of at least two of the following: (1) structural heart disease, namely complete atrioventricular septal defect; (2) juxtaposition of inferior vena cava and descending aorta; (3) viscerocardiac heterotaxy. Copyright 2003 ISUOG. Published by John Wiley & Sons, Ltd.

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Year:  2003        PMID: 14618656     DOI: 10.1002/uog.904

Source DB:  PubMed          Journal:  Ultrasound Obstet Gynecol        ISSN: 0960-7692            Impact factor:   7.299


  15 in total

1.  Prenatal diagnosis of left isomerism with normal heart: a case report.

Authors:  Nico De Paola; Santina Ermito; Antonella Nahom; Angela Dinatale; Elisa Maria Pappalardo; Sabina Carrara; Alessandro Cavaliere; Cristiana Brizzi
Journal:  J Prenat Med       Date:  2009-07

2.  3-dimensional mapping and radiofrequency ablation of atrial flutter in a patient with interrupted inferior vena cava.

Authors:  Siddharth Mukerji; Chotikorn Khunnawat; Sricharan Kantipudi; Atul Khasnis; Sundar Sankaran; Ranjan K Thakur; Krit Jongnarangsin
Journal:  J Interv Card Electrophysiol       Date:  2005-11       Impact factor: 1.900

3.  Perinatal outcome in fetuses with heterotaxy syndrome and atrioventricular block or bradycardia.

Authors:  Maria C Escobar-Diaz; Wayne Tworetzky; Kevin Friedman; Terra Lafranchi; Francis Fynn-Thompson; Mark E Alexander; Douglas Y Mah
Journal:  Pediatr Cardiol       Date:  2014-02-09       Impact factor: 1.655

4.  Global genetic analysis in mice unveils central role for cilia in congenital heart disease.

Authors:  You Li; Nikolai T Klena; George C Gabriel; Xiaoqin Liu; Andrew J Kim; Kristi Lemke; Yu Chen; Bishwanath Chatterjee; William Devine; Rama Rao Damerla; Chienfu Chang; Hisato Yagi; Jovenal T San Agustin; Mohamed Thahir; Shane Anderton; Caroline Lawhead; Anita Vescovi; Herbert Pratt; Judy Morgan; Leslie Haynes; Cynthia L Smith; Janan T Eppig; Laura Reinholdt; Richard Francis; Linda Leatherbury; Madhavi K Ganapathiraju; Kimimasa Tobita; Gregory J Pazour; Cecilia W Lo
Journal:  Nature       Date:  2015-03-25       Impact factor: 49.962

5.  Perinatal and infant outcomes of prenatal diagnosis of heterotaxy syndrome (asplenia and polysplenia).

Authors:  Maria C Escobar-Diaz; Kevin Friedman; Yishay Salem; Gerald R Marx; Brian T Kalish; Terra Lafranchi; Rahul H Rathod; Sitaram Emani; Tal Geva; Wayne Tworetzky
Journal:  Am J Cardiol       Date:  2014-06-06       Impact factor: 2.778

6.  Pre natal evaluation of heterotaxy syndrome by fetal echocardiography and correlating with autopsy.

Authors:  Madhavilatha Routhu; Imran Ali Mohammad
Journal:  Ultrasound       Date:  2019-03-19

Review 7.  The heterotaxy syndrome: associated congenital heart defects and management.

Authors:  Ravi Agarwal; Roy Varghese; Vimala Jesudian; Jeswin Moses
Journal:  Indian J Thorac Cardiovasc Surg       Date:  2020-03-27

8.  Congenital lobar emphysema associated with polysplenia syndrome.

Authors:  Naseer A Choh; Suhil A Choh; Majid Jehangir; Bashir A Naikoo
Journal:  Ann Saudi Med       Date:  2010 Nov-Dec       Impact factor: 1.526

Review 9.  Biliary atresia.

Authors:  Christophe Chardot
Journal:  Orphanet J Rare Dis       Date:  2006-07-26       Impact factor: 4.123

10.  Prenatal diagnosis of atrial isomerism in the Korean population.

Authors:  Mi-Young Lee; Hye-Sung Won; Jae-Yoon Shim; Pil-Ryang Lee; Byong Sop Lee; Ellen Ai-Rhan Kim; Young-Hwue Kim; Jeong-Jun Park; Tae-Jin Yun; Ahm Kim
Journal:  Obstet Gynecol Sci       Date:  2014-05-15
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