OBJECTIVES Pregnancy-related aortic dissection (AoD) in Marfan syndrome is a lethal catastrophe. Due to its rarity and limited clinical experience, there is no consensus regarding the optimal management strategy. We seek to present our 21-year experience in such patients , focusing on management strategies and early and late outcomes. METHODS Between 1998 and 2019, we managed 30 pregnant women with Marfan syndrome (mean age 30.7 ± 4.3 years) who sustained AoD at a mean of 28.3 ± 8.8 weeks of gestation (GWs). AoD was acute in 21 (70%), type A (TAAD) in 24 (80%) and type B (TBAD) in 6 (20%). Fourteen TAADs (58.3%, 14/24) and 2 TBADs (33.3%, 2/6) occurred in the third trimester or postpartum. The maximal aortic size was < 45 mm in 26.7% (8/30; 3 TAADs, 5 TBADs). Management strategy was based on the types of dissection and GWs (i.e. surgical versus medical treatment, surgery or delivery first). RESULTS TAADs were treated medically in 1 and surgically in 23. The timing of delivery and surgery were caesarean first at 35.4 ± 6.1 GWs in 7 (29.2%), followed by surgery after mean 46 days; single-stage C-section and surgery at 32.0 ± 5.0 GWs in 10 (41.7%); and surgery first at 18.0 ± 5.8 GWs in 6 (25%), followed by C-section after 20 days. Maternal and foetal mortality were 28.6% (2/7) and 14.3% (1/7), 10.0% (1/10) and 20.0% (2/10) and 16.7% (1/6) and 83.3% (5/6), respectively. Five TBADs (83.3%) were managed with C-section followed by surgery in 2 and medical treatment in 3. The respective maternal and foetal mortality were 50% (1/2) and 100% (2/2) and 33.3% (1/3) and 33.3% (1/3), respectively. One TBAD was managed surgically first followed by C-section, resulting in maternal survival and foetal death. Follow-up was complete in 95.8% (23/24) at 3.7 ± 2.9 years. Four late deaths occurred and reoperation was performed in 1 patient. Maternal and foetal survival were 64.3% and 54.1% at 6 years, respectively. CONCLUSIONS Management of AoD in pregnant women with Marfan syndrome should be based on types of dissection (surgical versus medical) and gestational age (delivery or surgery first), which largely determine maternal and foetal survival. Aortic repair should be considered prior to conception in women with Marfan syndrome even at diameters smaller than recommended by current guidelines.

BACKGROUNDAortic dissection during pregnancy is a rare but life-threatening event for mothers and fetuses. It often occurs in the third trimester of pregnancy and the postpartum period. Most patients have connective tissue diseases such as Marfan syndrome. Thus, the successful repair of a sporadic aortic dissection with maternal and fetal survival in the early second trimester is extremely rare.CASE SUMMARYA 28-year-old woman without Marfan syndrome presented with chest pain at the 16th gestational week. Aortic computed tomographic angiography confirmed an acute type A aortic dissection (TAAD) with aortic arch and descending aorta involvement. Preoperative fetal ultrasound confirmed that the fetus was stable in the uterus. The patient underwent total arch replacement with a frozen elephant trunk using moderate hypothermic circulatory arrest with the fetus in situ. The patient recovered uneventfully and continued to be pregnant after discharge. At the 38th gestational week, she delivered a healthy female infant by cesarean section. After 2.5 years of follow-up, the patient is uneventful and the child’s development is normal.CONCLUSIONA fetus in the second trimester may have a high possibility of survival and healthy growth after aortic arch surgery.

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