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• Marfan syndrome and pregnancy
  K Dhital
  Published on: 9 March 2001

• Published on: 9 March 2001

  Marfan syndrome and pregnancy

  • K Dhital

  Dear Editor:

  We read with interest Professor Treasure's recent paper on the cardiovascular surgical aspects of Marfan syndrome.[1] We wish to add to the discussion by raising the significant cardiovascular risks faced by Marfan patients during pregnancy, a problem that he touched on in a previous editorial.[2] This is an important and controversial issue in the management of adult cardiovascular manifestations of Marfa...

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  Dear Editor:

  We read with interest Professor Treasure's recent paper on the cardiovascular surgical aspects of Marfan syndrome.[1] We wish to add to the discussion by raising the significant cardiovascular risks faced by Marfan patients during pregnancy, a problem that he touched on in a previous editorial.[2] This is an important and controversial issue in the management of adult cardiovascular manifestations of Marfan syndrome, which is not mentioned in his recent and otherwise excellent review.

  It is well recognised that, apart from the 50% risk of passing on this autosomal dominant condition, pregnancy itself is associated with increased risk of cardiovascular, most particularly aortic, events.[3] [4] The majority of patients with no or only mild aortic dilatation have an uncomplicated and uneventful pregnancy.[5] However, the higher risk of catastrophic cardiovascular events in women with moderate to severe aortic or mitral valve pathology, and particularly in those with evidence of significant aortic dilatation, necessitates very close multidisciplinary monitoring during pregnancy, with timely intervention to safeguard the wellbeing of both mother and foetus.

  Before pregnancy, any woman with a diagnosis of Marfan syndrome should be evaluated clinically and echocardiographically to assess the degree of cardiovascular symptoms and pathology. If at most, the cardiovascular problems are mild, and the aortic root diameter is under 4 cm,[3] then female Marfan patients wanting children should be counselled to reproduce early if possible, with warning of a small but potential risk during pregnancy. Those with significant cardiovascular problems and/or a dilated aortic root should be counselled against pregnancy. Despite this, controversy remains with respect to those asymptomatic women with an aortic root diameter between 4-5 cm who, mindful of the potential risks, wish to become pregnant. The choice remains between offering pre- conception prophylactic surgery, and that of allowing pregnancy to take place with reassurance of a stringent and regular high-risk follow-up in a dedicated multidisciplinary unit. The former choice poses the dilemma of inflicting peri- and post-operative surgical morbidity in an otherwise young and healthy individual whose aorta may not necessarily ever dilate to a point requiring surgery. The latter risks accelerated aortic dilatation, possibly complicated by dissection, during pregnancy and the need for aortic surgery with maternal and foetal morbidity.

  During pregnancy, any patient with Marfan syndrome requires regular echocardiography to check valvular pathology and any significant increase in aortic dimensions. Cardiovascular complications can occur early on in pregnancy as significant cardiac output and plasma volume changes can be seen as early as the 6th gestational week.[3] Due to the ability of beta- blockers to slow the rate of aortic dilatation,[6] their continued use is recommended in Marfan women during pregnancy.[7]

  When possible, labour should be allowed to proceed naturally to a vaginal delivery, but there should be a low threshold for caesarean section in the event of foetal mal-position, distress or significant maternal hypertension. Epidural anaesthesia is recommended in order to minimise the rise in blood pressure accompanying pain and anxiety of labour. It may also be prudent to administer prophylactic antibiotics in women with evidence of mitral valve prolapse in the context of the low but potentially serious consequence of bacteraemia associated with instrumental delivery. Finally, whilst the majority of female Marfan patients go through pregnancy without significant problems, it is important to keep in mind that no Marfan patient can be reassured of an event free pregnancy. Major aortic events have been described in Marfan patients with normal aortic roots in and out of pregnancy.[8] [9]

  Kumud Dhital
  Eric Rosenthal
  Adult Congenital Heart Disease Clinic
  Guy's Hospital
  St Thomas Street
  London SE1 9RT, UK

  References
  (1) Treasure T. Cardiovascular surgery for Marfan syndrome. Heart 2000;84:674-8.

  (2) Treasure T. Elective replacement of the aortic root in Marfan's syndrome. Br Heart J 1993;69:101-3.

  (3) Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med 1981;71:784-90.

  (4) Lipscomb KJ, Smith JC, Clarke B, et al. Outcome of pregnancy in women with Marfan's syndrome. Br J Obstet Gynaecol 1997;104:201-6.

  (5) Rossiter JP, Repke JT, Morales AJ, et al. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynecol 1995;173:1599-606.

  (6) Shores J, Berger KR, Murphy EA, et al. Progression of aortic dilatation and the benefit of long-term b-adrenergic blockade in Marfan's syndrome. N Engl J Med 1994;330:1335-41.

  (7) Elkayam U, Ostrzega E, Shotan A, et al. Cardiovascular problems in pregnant women with the Marfan syndrome. Ann Intern Med 1995;123:117- 22.

  (8) Rosenblum NG, Grossman AR, Gabbe SG, et al. Failure of serial echocardiographic studies to predict aortic dissection in a pregnant patient with Marfan's syndrome. Am J Obstet Gynecol 1983;146:470-1.

  (9) Murgatroyd F, Child A, Poloniecki J, et al. Does routine echocardiographic assessment of the aortic root diameter help predict the risk of dissection in the Marfan syndrome? Eur Heart J 1991;12;410.

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  Conflict of Interest:
  None declared.

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