Pregnancy-related cardiovascular events in marfan syndrome

Abstract

Abstract Background Some studies have suggested an increased risk of aortic dissection (AoDis) during pregnancy and higher rate of cardiovascular (CV) events on the long-term in Marfan syndrome (MFS) patients. Purpose To analyse the rate of CV events during pregnancy and follow up in our cohort of MFS patients. Methods We prospectively collected data of a cohort of women with MFS that attended our dedicated clinic between the years 2000-2021. This included demographics, clinical characteristics and pregnancy. CV events were defined as AoDis, urgent AoSurg or CV death. Patients with a previous event before the first pregnancy were excluded. Results We identified 148 women with a mean age at last follow up of 40±16 years and a mean follow up of 11 years (IQR 25-75: 4-17 years). Sixty-nine patients fell pregnant (130 pregnancies, median 2, range 1-4 pregnancies, age at delivery 29±5y) and 79 were never pregnant. Amongst the 69 ever pregnant patients, 2 patients had a type A AoDis during pregnancy (one was unaware of the MFS diagnosis; the other was under follow up with a pre-pregnancy aortic root of 34mm/Z-score 1.5 and at third trimester of 36 mm/Z-score 2.2). No maternal deaths were recorded. Pregnancy related rate of CV events was 2.9% and 1.5% in women with known MFS diagnosis before pregnancy. Ever-pregnant patients were older at diagnosis (32±17y vs 20±14y, p<0,001) and at the end of follow up (49±14y vs 33±13, p<0,001) compared to never-pregnant patients. Ectopia lentis was significantly higher in never-pregnant patients (45.3% vs 25%, p=0.03). There were no statistically significant differences in time of follow up or other clinical features of MFS. Only 36% of the ever-pregnant cohort were diagnosed before pregnancy. During follow up, 14 patients from the ever-pregnant group and 16 patients from the never-pregnant group underwent prophylactic AoSurg (p=0,72). Never-pregnant patients were younger at age of elective AoSurg (35±14y vs 45±13y, p=0.031). Eleven (7.4%) patients experienced a CV event (7 type A and 4 type B AoDis), 7 in the ever-pregnant group and 4 in the never pregnant group (p=0.09). Age at CV event was similar in the ever-pregnant and never-pregnant groups (48±11y vs 40±8y, p=0.26) In the ever-pregnant group, 3 patients experienced ³2 CV events during follow up compared to none in the never-pregnant group (p=0.1). Conclusion In our cohort of ever pregnant MFS patients, 2.9% of patients experienced a pregnancy related CV event. On the long term, never-pregnant patients were younger at age of elective AoSurg but there were no differences between the groups in prophylactic AoSurg or CV events.