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10.5606/tgkdc.dergisi.2024.24850
Early-onset Marfan syndrome with aortic dilatation and giant pulmonary artery aneurysm: A case report
Qian-Nan Zhang1, Feng-Li Xu1, Shan-Shan Shi1
1CICU, Heart Centre, National Clinical Research Center for Child Health, Children?s Hospital, Zhejiang University School of Medicine, Hangzhou, China
DOI : 10.5606/tgkdc.dergisi.2024.24850
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.
Keywords : Marfan syndrome, prenatal diagnosis, pulmonary artery aneurysm, surgical intervention
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