Key result
Surgery fails to prevent fatal cardiac arrest in early-onset Marfan syndrome with giant PA aneurysm.
Case Report (n=1)
Main pulmonary artery dilatation and aneurysms during fetal life are likely signs of severe Marfan syndrome-related cardiac involvement.
Neonatal aortopulmonary aneurysms and stenoses may occur with maternal ankylosing spondylitis; leaves open causal link and need for larger cohorts.
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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Zhang et al. (2023) conducted a case report in Early-onset Marfan syndrome (n=1). Surgery was evaluated on Clinical outcome. A male neonate with early-onset Marfan syndrome presenting with fetal giant pulmonary artery aneurysm and aortic dilatation underwent surgery but died due to cardiac arrest.
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