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Background <p>Pregnancy with Marfan syndrome complicated by acute Stanford type A aortic dissection carries extremely high maternal and fetal mortality, and no standardized management protocol exists.</p> Case presentation <p>A 30-year-old primigravida at 37⁺³ weeks’ gestation presented with acute type A aortic dissection extending from the aortic root (sinus diameter 55&#xa0;mm; Z-score &gt; 2) and fetal distress. A one-stop hybrid procedure was performed under a single anesthetic: cesarean section followed by subtotal hysterectomy, then Bentall procedure, total arch replacement, and frozen elephant trunk implantation. The core innovation was maintaining cardiopulmonary bypass in “wet standby” with combined sterile draping before cesarean delivery, enabling immediate circulatory support if hemodynamic collapse occurred. Total surgery time was 11&#xa0;h 25&#xa0;min, with cardiopulmonary bypass 244&#xa0;min, aortic cross-clamp 184&#xa0;min, and deep hypothermic circulatory arrest 22&#xa0;min. Bilateral selective antegrade cerebral perfusion guided by near-infrared spectroscopy was employed. Postoperatively, the patient developed transient spinal cord ischemia (grade I), successfully reversed by emergency lumbar drainage (cerebrospinal fluid pressure maintained &lt; 15 mmHg for 5 days) and systolic blood pressure elevation to 130–150 mmHg. Genetic testing confirmed a pathogenic FBN1 mutation (c.6410G &gt; C; p.Cys2137Ser), fulfilling revised Ghent criteria. The patient was discharged on postoperative day 14. At 3-month follow-up, she remained asymptomatic (NYHA class I) with patent graft and full motor recovery.</p> Conclusions <p>A one-stop hybrid procedure integrating pre-prepared cardiopulmonary bypass, meticulous surgical planning, and proactive complication management can achieve favorable maternal and fetal outcomes in this life-threatening condition. The detailed technical specifications provide a replicable framework for other centers.</p>

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Extracorporeal circulation management for stanford type a aortic dissection complicating pregnancy in a patient with marfan syndrome: a case report

  • 期刊论文

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  • 发表日期：

    2026年4月19日

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  • Xiaoxi Yang,
  • Manish Kumar,
  • Lei Jiang,
  • Hang Shi,
  • Baichun Wang

摘要

Background

Pregnancy with Marfan syndrome complicated by acute Stanford type A aortic dissection carries extremely high maternal and fetal mortality, and no standardized management protocol exists.

Case presentation

A 30-year-old primigravida at 37⁺³ weeks’ gestation presented with acute type A aortic dissection extending from the aortic root (sinus diameter 55 mm; Z-score > 2) and fetal distress. A one-stop hybrid procedure was performed under a single anesthetic: cesarean section followed by subtotal hysterectomy, then Bentall procedure, total arch replacement, and frozen elephant trunk implantation. The core innovation was maintaining cardiopulmonary bypass in “wet standby” with combined sterile draping before cesarean delivery, enabling immediate circulatory support if hemodynamic collapse occurred. Total surgery time was 11 h 25 min, with cardiopulmonary bypass 244 min, aortic cross-clamp 184 min, and deep hypothermic circulatory arrest 22 min. Bilateral selective antegrade cerebral perfusion guided by near-infrared spectroscopy was employed. Postoperatively, the patient developed transient spinal cord ischemia (grade I), successfully reversed by emergency lumbar drainage (cerebrospinal fluid pressure maintained < 15 mmHg for 5 days) and systolic blood pressure elevation to 130–150 mmHg. Genetic testing confirmed a pathogenic FBN1 mutation (c.6410G > C; p.Cys2137Ser), fulfilling revised Ghent criteria. The patient was discharged on postoperative day 14. At 3-month follow-up, she remained asymptomatic (NYHA class I) with patent graft and full motor recovery.

Conclusions

A one-stop hybrid procedure integrating pre-prepared cardiopulmonary bypass, meticulous surgical planning, and proactive complication management can achieve favorable maternal and fetal outcomes in this life-threatening condition. The detailed technical specifications provide a replicable framework for other centers.