Bibliography

Title: Cardiovascular Problems in Pregnant Women with Marfan Syndrome

Author: Sorel Goland, MD; Uri Elkayam, MD

Summary of findings

Our case scenario presents some of the challenges faced by the pregnant patient with MFS and
her physicians. Although diagnosed at a young age, she was not treated with β-blockers and did
not have a preconception evaluation and thus was exposed to a high risk of acute aortic
dissection during pregnancy. A finding of a dilated aorta before pregnancy would have
mandated surgery before conception. The case also demonstrates the dilemma faced by the
patient of having surgery during pregnancy, which is associated with high rate of fetal loss and
other fetal complications. Because of concern for her fetus, the patient preferred to assume the
risk herself and delayed the surgery to allow fetal maturity and delivery of the baby before the
operation. Because of an increased risk of aortic dissection due to the hemodynamic strain
involved with vaginal delivery, the delivery was done by cesarean section

Implication research findings/study to the following:

Future Nursing Research

With relation to the research in the article, the research stated that the most serious
complication in patients with MFS is aortic dissection, and pregnancy has been shown to be
associated with a substantial increase in the risk of this complication. This increased risk is
caused by the maternal cardiovascular changes of pregnancy such as an increase in blood
volume, heart rate, and stroke volume. A need for increased counseling and management for
pregnant women with the disease was emphasized by the researchers.
Nursing Education
Vaginal delivery is considered safe in patients with MFS who have an aortic diameter of
<40 mm. In these patients, C-section should be reserved for obstetrical indications. At the same
time, however, to reduce the stress of labor, epidural anesthesia to minimize pain and
hemodynamic fluctuation during labor and delivery and forceps or vacuum to shorten the
second stage of labor are recommended.
Nursing Practice
Patients with MFS when tended by the nurse should monitor daily the blood pressure of
the patient more importantly when it increases. A high blood pressure may increase the risk of
aortic complications. That is why strict blood pressure control is recommended for all pregnant
women with MFS.
Comparison of Findings to the Current Management of Clients in the Philippines
Based on the findings, the management of pregnant women with MFS should ideally be started
prior to conception. Women with MFS should be counseled about potential pregnancy-related
complications which include (i) the high risk of transmission of MFS (>50%) where severe
expression of the syndrome can occur even in children of mothers presenting with relatively mild
form of MFS; (ii) risk of the development of aortic dissection in the mother which carries a
substantial risk to the fetus, and (iii) high rate (∼40%) of obstetric complications and increased
mortality in the offspring. The patient should be also informed about the possibility of prenatal
diagnosis using both genetic testing and fetal echocardiography. The risk (1%) of fetal
miscarriage due to sampling for genetic testing should be discussed as well. Prenatal genetic
testing could be used in cases when the result will influence parent’s decisions regarding either
continuation or termination of pregnancy with an affected fetus.

References: Goland, S., & Elkayam, U. (2019). Marfan Syndrome and Pregnancy. Cardiac
Problems in Pregnancy, 4th Edition, 285–304. doi: 10.1002/9781119409861.ch19