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Early-onset Marfan syndrome with aortic dilatation and giant pulmonary artery aneurysm: A case report.

Authors :
Zhang QN
Xu FL
Shi SS
Source :
Turk gogus kalp damar cerrahisi dergisi [Turk Gogus Kalp Damar Cerrahisi Derg] 2023 Aug 01; Vol. 32 (1), pp. 93-96. Date of Electronic Publication: 2023 Aug 01 (Print Publication: 2024).
Publication Year :
2023

Abstract

A 30-year-old woman with ankylosing spondylitis was referred to our clinic with abnormal fetal echocardiography findings, including ascending aortic dilatation, giant main pulmonary artery aneurysm, and aortic and pulmonary valve stenosis at 22 weeks of gestation. The full-term male neonate was born by cesarean section and was transferred to the cardiac intensive care unit soon after delivery for respiratory distress with low percutaneous oxygen saturation. Based on cardiovascular and genetic analysis findings, the patient was diagnosed with Marfan syndrome. Surgery was performed; however, the patient died due to cardiac arrest. In conclusion, main pulmonary artery dilatation and aneurysms are uncommon in Marfan syndrome; therefore, presentation with these findings during the fetal life, as in the present case, is likely a sign of severe Marfan syndrome-related cardiac involvement.<br />Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.<br /> (Copyright © 2024, Turkish Society of Cardiovascular Surgery.)

Details

Language :
English
ISSN :
1301-5680
Volume :
32
Issue :
1
Database :
MEDLINE
Journal :
Turk gogus kalp damar cerrahisi dergisi
Publication Type :
Report
Accession number :
38545352
Full Text :
https://doi.org/10.5606/tgkdc.dergisi.2024.24850