Asian Cardiovasc Thorac Ann 2007;15:e63-e65
© 2007 Asia Publishing EXchange Ltd
Two Surgical Cases of Acute Aortic Dissection in Pregnancy with Marfan Syndrome
Hidetaka Wakiyama, MD,
Michihiro Nasu, MD,
Hiroshi Fujiwara, MD,
Aki Kitamura, MD,
Yukikatsu Okada, MD
Department of Cardiovascular Surgery, Kobe General Hospital, Kobe, Japan
For reprint information contact: Hidetaka Wakiyama, MD, Tel: 81 78 3024321, Fax: 81 78 302 2487, email: hidewacky{at}aol.com, Department of Cardiovascular Surgery, Kobe General Hospital, Minatojima, Naka-Machi 4-6, Chuo-Ku, Kobe 650-0046, Japan.
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ABSTRACT
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We describe 2 surgical cases of acute aortic type A dissection during pregnancy in women with Marfan syndrome. Both of them underwent emergency aortic root replacement under deep hypothermia; one patient was in her 21st week of pregnancy and the other was treated 1 day after a normal delivery. The patients experienced fair postoperative courses, but intrauterine fetus death was confirmed in the first case.
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INTRODUCTION
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Acute aortic dissection in pregnancy is often a troublesome entity because rapid and appropriate surgical decision making is required to save the life of both mother and baby. Aortic root enlargement (> 4 cm) and Marfan syndrome significantly predispose to aortic dissection in pregnancy.1–3 However, the optimal surgical management of acute aortic dissection in pregnant women is still unresolved.4–6
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CASE 1
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A 36-year old primigravida with Marfan syndrome was transferred to our hospital with a diagnosis of acute type A aortic dissection. She was in the 21st week of pregnancy. Echocardiography showed aortic dissection with severe aortic regurgitation due to annuloaortic ectasia. Fetal echocardiographic findings were normal. An emergency operation was scheduled in spite of the high risk of fetal death. She had undergone vertebral fixation for severe scoliosis when she was 13-years old (Figure 1
). Pectus excavatum was also observed (Figure 2). Because of the severe chest deformity, the heart was markedly shifted to the left side (Figure 2). A transverse incision to the left of the sternum in addition to a median sternotomy was mandatory to approach the heart. The aortic root was strikingly dilated to 60 mm, with a strong thrill.
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Figure 1. Postoperative radiograph showing severe scoliosis that had been treated by rod fixation. The patient refused to undergo a radiographic study preoperatively because of potential adverse effects on the fetus.
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Figure 2. Computed tomography showing the heart markedly shifted into the left chest due to prominent pectus excavatum.
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Cardiopulmonary bypass was established between the right common femoral artery and bicaval cannulae. The ascending aorta was opened under deep hypothermia and total circulatory arrest, and aortic root replacement was performed with a composite graft consisting of a bioprosthetic valve and a woven polyester graft, because the patient desired to have a child in the future. Several small entries were confirmed just distal to the sinotubular junction. No other entry was found in the aortic arch. Cardiopulmonary bypass and circulatory arrest times were 288 min and 33 min, respectively. The lowest rectal temperature was 20°C. After the operation, fetal demise was confirmed, and a stillborn fetus was delivered 7 days later. The patient was successfully weaned from the ventilator in spite of the severe chest deformity. A year after the operation, she was found to have an uneventful recovery.
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CASE 2
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A 38-year-old woman in her 39th gestational week was hospitalized and delivered a healthy infant. She had suffered dyspnea in the first trimester, and an echocardiogram had shown moderate aortic regurgitation associated with annuloaortic ectasia. She was not aware of having Marfan syndrome and had already experienced two normal deliveries. Subsequent to the delivery, she suffered an acute type A aortic dissection. Urgent surgery was performed under deep hypothermia. The intimal flap and entry were seen in the ascending aorta. The ostium of the right coronary artery was involved, and the aortic dissection extended proximally to the aortic root. Aortic root replacement was undertaken with a composite graft consisting of a mechanical valve and woven polyester graft, and concomitant right coronary artery bypass grafting. Bypass and circulatory arrest times were 250 and 31 min, respectively. The lowest temperature was 19°C. The postoperative course was uneventful. At 6 years postoperatively, the patient was doing well.
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DISCUSSION
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Aortic dissection is often a catastrophic complication of pregnancy.1,3,4,7,8 It is reported that acute aortic dissection in young women occurs mostly in the 3rd trimester of pregnancy.1,2,7 Aortic root enlargement > 4 cm and/or Marfan syndrome significantly predisposes to aortic dissection in pregnancy.1,2 Although little is known about the prevalence of acute aortic dissection in pregnant women with Marfan syndrome, Rossiter and colleagues2 reported that 2 out of 21 pregnant women with Marfan syndrome were affected by aortic dissection. As for the results of cardiac surgery in pregnant women, Becker and colleagues6 found that pregnancy carries a mortality of 1.5%, whereas fetus mortality is up to 16.2%. This is mostly dependent on the maturity of the fetus, and some reports suggest that cardiopulmonary bypass may cause intrauterine fetal death.4,5,7 Weiss and colleagues8 observed that maternal mortality is significantly higher in operations for aortic dissection compared to valve surgery. Fetal/neonatal death is more frequent in surgery performed as an extreme emergency and/or under deep hypothermic circulatory arrest, as in our first case.8 Although various modes of cardiopulmonary bypass with different flow rates, temperatures, and sites of arterial perfusion have been advocated, the optimum modality is still unknown.4–7
In our first case, the patient was a primigravida and strongly wanted to have a child, even if the fetus were to die during the operation. Hence, a bioprosthetic valve was used because of the advantage of not requiring anticoagulants. Some reports suggest that heparin administration with strict monitoring is an alternative to warfarin with its possible teratogenesis when anticoagulation is mandatory for pregnancy.9 However, taking into account the higher risk of cardiovascular events during pregnancy in Marfan patients, these drugs are not suitable for mother or fetus.6,9 Because the fetus was too immature in case 1, maternal lifesaving was of the highest priority. Deep hypothermic circulatory arrest was utilized to make sure there were no more entries at the aortic arch and to avoid aortic clamping near the distal anastomosis, although the adverse effect of hypothermia in pregnant women is still controversial.4,5,7 In addition, the physical characteristics associated with Marfan syndrome should be thoroughly assessed preoperatively. In case 1, a left thoracotomy with sternal transection was required to obtain a good surgical view, as the heart was not exposed at all by a simple sternotomy. In cases of extreme chest deformity, additional thoracoplasty would be mandatory to avoid circulatory and respiratory deterioration. As rapid progression of the diameter of the false lumen of the descending aorta or arch has been reported previously, strict follow-up by computed tomography is crucial.2 When such a patient is pregnant, great attention should be paid to aortic enlargement distal to the arch, with a close liaison between cardiologists and obstetricians.
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REFERENCES
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- Immer FF, Bansi AG, Immer-Bansi AS, McDougall J, Zehr KJ, Schaff HV, et al. Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg 2003;76:309–14.[Abstract/Free Full Text]
- Rossiter JP, Repke JT, Morales AJ, Murphy EA, Pyeritz RE. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynecol 1995;173:1599–606.[Medline]
- Meijboom LJ, Vos FE, Timmermans J, Boers GH, Zwinderman AH, Mulder BJ. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 2005;26:914–20.[Abstract/Free Full Text]
- Pomini F, Mercogliano D, Cavalletti C, Caruso A, Pomini P. Cardiopulmonary bypass in pregnancy. Ann Thorac Surg 1996;61:259–68.[Abstract/Free Full Text]
- Buffolo E, Palma JH, Gomes WJ, Vega H, Born D, Moron AF, et al. Successful use of deep hypothermic circulatory arrest in pregnancy. Ann Thorac Surg 1994;58:1532–4.[Abstract]
- Becker RM. Intracardiac surgery in pregnant women. Ann Thorac Surg 1983;36:453–8.[Abstract]
- Sakaguchi M, Kitahara H, Seto T, Furusawa T, Fukui D, Yanagiya N, et al. Surgery for acute type A aortic dissection in pregnant patients with Marfan syndrome. Eur J Cardiothorac Surg 2005;28:280–5.[Abstract/Free Full Text]
- Weiss BM, von Segesser LK, Alon E, Seifert B, Turina MI. Outcome of cardiovascular surgery and pregnancy: a systematic review of the period 1984–1996. Am J Obstet Gynecol 1998;179:1643–53.[Medline]
- Oran B, Lee-Parritz A, Ansell J. Low molecular weight heparin for the prophylaxis of thromboembolism in women with prosthetic mechanical heart valves during pregnancy. Thromb Haemost 2004;92:747–51.[Medline]
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ABSTRACT
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