Delivery in Marfan

There’s seemingly a dearth of information about pregnancy and delivery in Marfan patients and it’s a subject I get a lot of questions on. I was really lucky to happen to live in a city with an OB who specialized in Marfan and have access to one of the country’s top cardiologists during the latter half of my care (he’s now my regular cardiologist, since we moved to California). So, I’d like to share what I’ve learned from my experience, my doctors, and reading up online in a two part series. You can read the first entry, Pregnancy in Marfan, here.

Please keep in mind that I am not a doctor myself, and (like everything else in Marfan), what works for MOST patients may not be what’s right for YOU. Do your own research and talk with your doctors and experts! The National Marfan Foundation has resources and my OB has expressed a willingness to talk with any pregnant/soon-to-be-pregnant Marf woman. Email me at marfmom@gmail.com for his information or for copies of the papers I cite.

There are different points of view on what the best way for a woman with Marfan to deliver. I’m going to present the benefits and dangers of each, so that you’ll be able to work with your obstetrics team (OB, cardiologist, anesthesiologist) to plan the safest delivery for you and your baby.

Natural (unmedicated, vaginal) delivery: No one recommends this for Marfan patients. When your body is in pain, your blood pressure rises. A rise in blood pressure, particularly one that would be as prolonged as with labor, puts you at risk for dissection. You can dissect even if your aorta is not dilated prior to labor. Your descending aorta seems to be the most at risk for dissecting without prior dilation.

Medicated vaginal delivery (epidural or spinal catheter): My OB, Ohio cardiologist, and California cardiologist all agreed that this is the optimal form of delivery for many women with Marfan (those who do not have an aortic root close to 5 cm and those who have not experienced rapid aortic growth during the pregnancy). Prompt pain medication eliminates pain and therefore decreases the risk for aortic dissection. Recovery time for the mother and child is also greatly reduced.

Traditional c-section: This can post a similar risk to the natural delivery. Any time your body undergoes surgery, especially such a major surgery as a c-section, your blood pressure rises. In addition, Marfan skin does not always heal properly and there could be scarring complications. However, some OBs feel that a c-section is safest because it is in a more “controlled” environment than a vaginal delivery and is much shorter than labor would be. My c-section took 45 minutes; the average first labor is 12-18 hours (of active labor, usually defined as at least 4 cm dilated). And as I stated above, for women with significant aortic dilation or a rapidly growing aorta, c-section is the safest route. It is important to note, however, the several papers have documented the risk for uterine rupture after a c-section. In one paper, Dr. Reed Pyeritz (a member of the NMF’s Professional Advisory Board) wrote that he saw this in 4 of his 11 patients (Pyeritz, 1981). That said, that was also in 1981, long before Loeys-Dietz syndrome (LDS) was discovered. LDS carries with it a risk of uterine rupture and it is possible that some of these patients had LDS, not Marfan, and didn’t know it (my own posturing here).

C-section under general anesthesia: Dural ectasia, to a varying degree of severity, affects 60-70% of people with Marfan syndrome. Dural ectasia is like the equivalent of an aneurysm of the dura sac, the sac of fluid that protects the spinal cord. While it can occur anywhere along the spine, most often it is at the base of the spine, right where the epidural or spinal catheter would be inserted. In patients whose dural ectasia is severe enough, a c-section under general anesthesia might be performed. The reasoning for this is that the needle from the epidural or spinal cath would cause a tear in the dura sac, which would lead to a leak of the spinal fluid. This causes a TERRIBLE headache, which can last up to a month. “Marfriends” of mine who have had a spinal leak say the pain and nausea is only lessened by remaining flat on the back. You can imagine the blood pressure issues that could arise from such a headache, not to mention the postpartum problems, like trying to breastfeed.

Difference between an epidural and a spinal catheter: My understanding is that a spinal catheter has a slightly thinner needle and provides a stronger dose of medication. While a woman using an epidural might feel it begin to wear off towards the end stages of labor, my anesthesiologist told me that a spinal cath would leave a woman numb for quite some time after delivery. I am not sure of the additional risks of using a spinal catheter, although I would think it would make it slightly more difficult to push. I have heard some OBs do not allow their Marfan patients to push at the very end of labor and instead using their hands or a vacuum to assist the delivery.

Postpartum care: There is still the risk of aortic dissection after delivery. Women with Marfan must be closely monitored postpartum. An echo no later than a week after delivery is recommended, and again at one month postpartum. Many beta-blockers are safe for breastfeeding, but not all, so discuss your medication and dosage ahead of time with your OB and cardiologist if you intend to breastfeed. You should know that a prolonged high blood pressure postpartum could signal an aortic dissection, even if you have no pain.

My experience: Because of my dural ectasia, my obstetrics team, my orthopedist at Johns Hopkins and I decided at 37 weeks that it was safest for me to have a c-section under general anesthesia. My blood pressure spiked while I was under, to 170/107 (for comparison, I try to keep it at around 100/60 – 110/70). Even on a host of medications it took 5 days to get me to a systolic of 140 and 8-10 weeks to get down to a systolic of 120. No one knows why this happened. Luckily, I didn’t suffer any permanent damage (and no aortic growth). I wrote more about it here.

Moral of the story? Each method of delivery brings its own set of risks and benefits. Keep an open dialogue with your team (and if your OB isn’t meeting regularly with your cardiologist and the anesthesiologists, insist on it) during your pregnancy to determine what is safest for you.

And again: The opinions offered at Musings of a Marfan Mom are for informational purposes only and are not intended to be a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of a qualified healthcare provider with any questions you may have regarding Marfan syndrome and any medical condition. Never disregard professional medical advice or delay in seeking care because of something you have read here.

Citations:
Meijboom, L. J., Vos, F. E., Timermans, J., Boers, G. H., Zwinderman, A. H., Mulder, B. J. M. (2005). Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. European Heart Journal, 9, 914-920.

Pyeritz, R. E. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med. 1981;71:784-90.

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