M. Elena Soto, Dirección de Investigación, Instituto Nacional de Cardiología Ignacio Chávez; Línea Cardiovascular, Hospital Observatorio ABC México; Ciudad de México, México
Miguel Santos-Aragón, Departamento de Cardiología, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México
Regina de la Mora-Cervantes, Departamento de Tomografía Computada, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México
Background: Marfan syndrome (MS) is associated with mutations in the FBN-1 gene, which encodes fibrillin-1, an essential protein in elastic fibers. Cardiovascular damage in MS has serious complications such as aneurysms and aortic dissection (AD), which increase during pregnancy. Objective: To evaluate the cardiovascular and clinical status of women with Marfan syndrome and pregnancy. Materials and methods: Retrospective study in women with MS or other connective tissue diseases (CTD) classified according to the 2010 Ghent criteria. Results: One hundred women were included, 35 were admitted as pediatric patients, and 65 as adults. Ten women without medical records were excluded. Forty-eight non-pregnant women and 46 pregnant women were analyzed. 20 births, 26 cesarean sections, 9 preeclampsia, and 14 miscarriages. Of the total group, 14 had congenital heart disease, 36 had AD, 47 had aortic insufficiency, 22 had mitral valve insufficiency, 15 had tricuspid valve insufficiency, 39 had valve prolapse, 67 had surgeries, and 15 had stents. 19 died, 5 due to aneurysm rupture, 4 had AD, 3 had cardiogenic shock, 5 had septic shock, and 2 had hypovolemic shock. Conclusions: Cardiovascular damage in pregnancies of women with MS or CTD is complex. The diagnosis of MS is late in some cases; it is at the time of delivery, and genetic counseling is not timely. These are areas of opportunity to impact on prevention, timely gynecological and obstetric status, and surgery.
Keywords: Marfan pregnancy. Cardiovascular impact. Aneurysm rupture. Aortic dissection.