Universität zu Köln

Gottschalk, Ingo, Abel, Judith S., Menzel, Tina, Herberg, Ulrike, Breuer, Johannes, Gembruch, Ulrich ORCID: 0000-0001-8284-4669, Geipel, Annegret, Brockmeier, Konrad, Berg, Christoph and Strizek, Brigitte ORCID: 0000-0003-3625-7097 (2019). Prenatal diagnosis, associated findings and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center. J. Perinat. Med., 47 (3). S. 354 - 365. BERLIN: WALTER DE GRUYTER GMBH. ISSN 1619-3997

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Abstract

Objective: To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with double outlet right ventricle (DORV). Methods: All cases of DORV diagnosed prenatally over a period of 8 years were retrospectively collected in a single tertiary referral center. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. Results: Forty-six cases of DORV were diagnosed prenatally. The mean gestational age at first diagnosis was 21 + 4 weeks (range, 13-37). A correct prenatal diagnosis of DORV was made in 96.3% of the cases. If the relation of the great arteries, the position of the ventricular septal defect (VSD) and additional cardiac anomalies are taken into account, the prenatal diagnosis was correct in 92.6% of the cases. One case was postnatally classified as transposition of the great arteries with subpulmonary VSD and was excluded from further analysis. A total of 41 (91.1%) fetuses with DORV had major additional cardiac anomalies, 30 (66.7%) had extracardiac anomalies and 13 (28.9%) had chromosomal or syndromal anomalies. Due to their complex additional anomalies, five (11.1%) of our 45 fetuses had multiple malformations and were highly suspicious for non-chromosomal genetic syndromes, although molecular diagnosis could not be provided. Disorders of laterality occurred in 10 (22.2%) fetuses. There were 17 terminations of pregnancy (37.8%), two (4.4%) intrauterine and seven (15.6%) postnatal deaths. Nineteen of 22 (86.4%) live-born children with an intention to treat were alive at last follow-up. The mean follow-up among survivors was 32 months (range, 2-72). Of 21 children who had already undergone postnatal surgery, eight (38.1%) achieved biventricular repair and 13 (61.9%) received univentricular palliation. One recently born child is still waiting for surgery. All children predicted prenatally to need a single ventricle palliation, and all children predicted to achieve biventricular repair, ultimately received the predicted type of surgery. After surgery, 14 of 18 (77.8%) children were healthy without any impairment. Conclusion: DORV is a rare and often complex cardiac anomaly that can be diagnosed prenatally with high precision. DORV is frequently associated with major additional anomalies, leading to a high intrauterine and postnatal loss rate due to terminations or declined postnatal therapy. Without additional anomalies, the prognosis is good, although approximately 60% of children will have single ventricle palliation.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Gottschalk, Ingo UNSPECIFIED UNSPECIFIED UNSPECIFIED Abel, Judith S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Menzel, Tina UNSPECIFIED UNSPECIFIED UNSPECIFIED Herberg, Ulrike UNSPECIFIED UNSPECIFIED UNSPECIFIED Breuer, Johannes UNSPECIFIED UNSPECIFIED UNSPECIFIED Gembruch, Ulrich UNSPECIFIED orcid.org/0000-0001-8284-4669 UNSPECIFIED Geipel, Annegret UNSPECIFIED UNSPECIFIED UNSPECIFIED Brockmeier, Konrad UNSPECIFIED UNSPECIFIED UNSPECIFIED Berg, Christoph UNSPECIFIED UNSPECIFIED UNSPECIFIED Strizek, Brigitte UNSPECIFIED orcid.org/0000-0003-3625-7097 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-152503
DOI:	10.1515/jpm-2018-0316
Journal or Publication Title:	J. Perinat. Med.
Volume:	47
Number:	3
Page Range:	S. 354 - 365
Date:	2019
Publisher:	WALTER DE GRUYTER GMBH
Place of Publication:	BERLIN
ISSN:	1619-3997
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CONGENITAL HEART-DISEASE; PROGNOSIS; ECHOCARDIOGRAPHY; MALFORMATIONS; ABNORMALITIES; ACCURACY Multiple languages Obstetrics & Gynecology; Pediatrics Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/15250