Universität zu Köln

Gottschalk, Ingo, Strizek, Brigitte ORCID: 0000-0003-3625-7097, Menzel, Tina, Herberg, Ulrike ORCID: 0000-0002-9386-0258, Breuer, Johannes, Brockmeier, Konrad, Geipel, Annegret, Gembruch, Ulrich ORCID: 0000-0001-8284-4669 and Berg, Christoph (2020). Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History. Fetal Diagn. Ther., 47 (5). S. 420 - 429. BASEL: KARGER. ISSN 1421-9964

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Abstract

Purpose: To assess the intrauterine course, the outcome, and to establish a new prenatal echocardiographic scoring system to predict biventricular (BV) versus univentricular (UV) outcome of fetuses with severe pulmonary stenosis or atresia with intact ventricular septum (PSAIVS). Methods: All cases of PSAIVS diagnosed prenatally over a period of 14years were retrospectively collected in 2 tertiary referral centers. Results: Forty-nine fetuses with PSIVS (n = 11) or PAIVS (n = 38) were identified prenatally. Nineteen (38.8%) fetuses had additional ventriculocoronary connections (VCCs) and 21 (42.9%) fetuses had right ventricular hypoplasia. Four (8.2%) pregnancies were terminated, 2 (4.1%) ended in intrauterine fetal death, 4 (8.2%) in neonatal death, and 5 (10.2%) children died in infancy or childhood, including one case with compassionate care. Thirty-four of 44 (77.3%) fetuses with the intention-to-treat were alive at latest follow-up, 25 (73.5%) with BV, and 9 (26.5%) with UV circulation. Most significant predictive markers of UV circulation were Vmax of tricuspid regurgitation (TR) <2 m/s, right ventricle/left ventricle length ratio <= 0.6, and presence of VCC. A scoring system including these 3 markers had 100% sensitivity and 100% specificity predicting an UV outcome if more than one of these criteria was fulfilled. All 25 liveborn infants that were suitable for BV repair survived, whereas only 9 out of 14 candidates for UV repair survived. None of the 14 fetuses with predicted UV outcome would have met the inclusion criteria for fetal intervention, as 10 of them had VCC and the remaining 4 had absent TR or Vmax Conclusion: The prognosis of prenatally diagnosed PSAIVS is good if BV circulation can be achieved, while postnatal mortality in UV circulation is high within the first 4 months of life. Postnatal outcome can be predicted prenatally with high accuracy using a simple scoring system. This information is mandatory for parental counseling and may be useful in selecting fetuses for intrauterine valvuloplasty.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Gottschalk, Ingo UNSPECIFIED UNSPECIFIED UNSPECIFIED Strizek, Brigitte UNSPECIFIED orcid.org/0000-0003-3625-7097 UNSPECIFIED Menzel, Tina UNSPECIFIED UNSPECIFIED UNSPECIFIED Herberg, Ulrike UNSPECIFIED orcid.org/0000-0002-9386-0258 UNSPECIFIED Breuer, Johannes UNSPECIFIED UNSPECIFIED UNSPECIFIED Brockmeier, Konrad UNSPECIFIED UNSPECIFIED UNSPECIFIED Geipel, Annegret UNSPECIFIED UNSPECIFIED UNSPECIFIED Gembruch, Ulrich UNSPECIFIED orcid.org/0000-0001-8284-4669 UNSPECIFIED Berg, Christoph UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-335002
DOI:	10.1159/000502178
Journal or Publication Title:	Fetal Diagn. Ther.
Volume:	47
Number:	5
Page Range:	S. 420 - 429
Date:	2020
Publisher:	KARGER
Place of Publication:	BASEL
ISSN:	1421-9964
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language AORTOPULMONARY COLLATERAL ARTERIES; CONGENITAL HEART-DISEASE; TRICUSPID-VALVE SIZE; PRENATAL-DIAGNOSIS; PREDICTORS; VALVULOPLASTY; TETRALOGY; OUTCOMES; FALLOT; REPAIR Multiple languages Obstetrics & Gynecology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/33500