Universität zu Köln

Recker, Florian ORCID: 0000-0001-9135-4338, Weber, Eva Christin, Strizek, Brigitte ORCID: 0000-0003-3625-7097, Herberg, Ulrike ORCID: 0000-0002-9386-0258, Brockmaier, Konrad, Gottschalk, Ingo, Geipel, Annegret, Gembruch, Ulrich ORCID: 0000-0001-8284-4669 and Berg, Christoph (2022). Prenatal Diagnosis and Outcome of Scimitar Syndrome: A Case Series of Six Patients. J. Clin. Med., 11 (6). BASEL: MDPI. ISSN 2077-0383

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Abstract

Scimitar syndrome is a rare disease characterized by hypoplasia of the right lung and partial anomalous pulmonary venous drainage to the inferior vena cava. All cases with a prenatal diagnosis of scimitar syndrome with or without associated malformations in an 18-year period (2000-2018) in two large tertiary referral centers (University of Bonn and University of Cologne, Germany) were retrospectively reviewed for the intrauterine course and postnatal outcome. Six cases were diagnosed in the study period. All presented with hypoplasia of the right lung, right-sided mediastinal shift, and abnormal pulmonary venous drainage to the inferior vena cava. Systemic arterial blood supply to the right lung, albeit postnatally present in all cases, could not be detected prenatally. Major associated anomalies were present in all cases and included atrial septal defect (n = 5), coarctation (n = 3), diaphragmatic hernia (n = 2), and VACTERL association (n = 1). Half of the cohort died within 6 months after birth and all three survivors suffer from long-term pulmonary sequelae. The primary hint to the prenatal diagnosis of scimitar syndrome is the abnormal position of the heart in the chest. If searched for, abnormal venous drainage can be identified prenatally and confirms the diagnosis. The prognosis depends on the presence of associated major anomalies and the need for neonatal intervention.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Recker, Florian UNSPECIFIED orcid.org/0000-0001-9135-4338 UNSPECIFIED Weber, Eva Christin UNSPECIFIED UNSPECIFIED UNSPECIFIED Strizek, Brigitte UNSPECIFIED orcid.org/0000-0003-3625-7097 UNSPECIFIED Herberg, Ulrike UNSPECIFIED orcid.org/0000-0002-9386-0258 UNSPECIFIED Brockmaier, Konrad UNSPECIFIED UNSPECIFIED UNSPECIFIED Gottschalk, Ingo 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:38-664880
DOI:	10.3390/jcm11061696
Journal or Publication Title:	J. Clin. Med.
Volume:	11
Number:	6
Date:	2022
Publisher:	MDPI
Place of Publication:	BASEL
ISSN:	2077-0383
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language PULMONARY VENOUS CONNECTION; LUNG; CLASSIFICATION; SEQUESTRATION Multiple languages Medicine, General & Internal Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/66488