Rare Marfan Syndrome Complication Threatens Pregnant Mother & Baby

Mẹ con thai phụ nguy kịch vì tình trạng lần đầu tiên ghi nhận ở Chợ Rẫy

This is the case of Ms. S.K. (36 years old, Cambodian nationality). In mid-October, the woman was transferred to Cho Ray Hospital with severe chest pain while 38 weeks pregnant.

Two Hospitals Race to Save Mother and Fetus

Dr. Nguyen Thai An, Head of the Department of Cardiovascular Surgery and Resuscitation at Cho Ray Hospital (HCMC), stated that examination and diagnosis confirmed the pregnant woman had Marfan syndrome with acute Type A aortic dissection and aortic root aneurysm, threatening the lives of both mother and fetus.

The treatment team determined that this complication was caused by the increased circulatory volume and physiological hemodynamic pressure during the late stage of pregnancy.

This is a cardiovascular emergency with a very high mortality rate, requiring intervention as soon as possible, especially within the first 48 hours. At this point, doctors faced a life-or-death dilemma: how to ensure the safety of both mother and fetus simultaneously?

The challenge in addressing the mother’s underlying condition was the risk of postpartum hemorrhage. A woman who undergoes a C-section can experience uterine bleeding, which is particularly dangerous and can be life-threatening for the mother.

The Cardiovascular Surgery and Resuscitation team at Cho Ray Hospital held an urgent consultation with obstetricians at Hung Vuong Hospital. After careful consideration, both parties agreed to proceed with an emergency C-section.

The surgery was successful, and a healthy baby boy was safely delivered. Simultaneously, the obstetrics team performed techniques to minimize the risk of postpartum hemorrhage for the mother.

24-36 hours after the C-section, the patient underwent further surgery by the Cardiovascular Surgery and Resuscitation team to address the aortic dissection. This was an exceptionally difficult operation because, in addition to the widespread lesions caused by Marfan syndrome, the patient’s aortic root was significantly dilated, accompanied by severe aortic valve regurgitation.

Facing this challenge, the doctors chose to replace the entire aortic root and ascending aorta with an artificial blood vessel while preserving the natural aortic valve.

The approximately 8-hour surgery using this technique helped the patient avoid mechanical valve replacement, thereby preventing the need for lifelong anticoagulant therapy.

Genetic Disease with High Mortality Risk

Dr. Nguyen Thai An explained that Marfan syndrome is a relatively common genetic disorder (affecting 1 in 3,000-5,000 people), but not all patients experience severe complications such as aortic aneurysm or dissection during pregnancy.

Over many years, Cho Ray Hospital has only treated about 5 cases of pregnant women with aortic lesions during pregnancy, all of whom were successfully saved. Notably, this is the first patient with Marfan syndrome-related aortic damage during pregnancy recorded at this hospital.

Experts recommend that individuals with Marfan syndrome undergo regular monitoring to detect aortic root aneurysm early and plan proactive surgery when necessary.

Women with this condition who intend to become pregnant should undergo cardiac examinations and aortic ultrasounds beforehand to assess risks. Additionally, family members of Marfan patients should consider genetic screening, as this is a hereditary genetic disease.

“Women should check their cardiovascular health before pregnancy, because during pregnancy, the circulatory system works much harder, which can exacerbate underlying conditions. If detected early, modern medicine can intervene to protect both mother and child,” emphasized the Head of the Department of Cardiovascular Surgery and Resuscitation at Cho Ray Hospital.

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