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

Turk Gogus Kalp Damar Cerrahisi Derg

CICU, Heart Centre, National Clinical Research Center for Child Health, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Published: January 2024

AI Article Synopsis

  • * After an emergency cesarean section, the male newborn exhibited respiratory distress and low oxygen levels, leading to immediate transfer to the cardiac intensive care unit.
  • * The mother was diagnosed with Marfan syndrome based on the cardiovascular and genetic findings, but she unfortunately died from cardiac arrest after surgery; this case indicates that severe heart problems can arise from Marfan syndrome even in fetuses.

Article 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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10964307PMC
http://dx.doi.org/10.5606/tgkdc.dergisi.2024.24850DOI Listing

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