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Eur J Cardiothorac Surg 2005;28:280-283
© 2005 Elsevier Science NL
Original articles |
a Department of Cardiovascular Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
b Department of Cardiovascular Surgery, Nagano Red Cross Hospital, Nagano, Japan
Received 6 December 2004; received in revised form 2 February 2005; accepted 14 February 2005.
* Corresponding author. Tel.: +81 263 37 2657; fax: +81 263 37 2721. (Email: masasaka{at}hsp.md.shinshu-u.ac.jp).
Abstract
Objective: Acute type A aortic dissection during pregnancy can be fatal to both the mother and the fetus. The goal of the present study was to characterize the prevalence, treatment and outcomes of this dangerous condition in an effort to determine optimal management. Methods: A retrospective study was conducted using data from four Marfan patients with acute type A aortic dissection during pregnancy at our institution between 1991 and 2003. Results: The mean gestational period at the time of operative repair was 31 weeks, with a range of 26–34 weeks, and the aortic root diameter ranged from 35 to 85mm. Two of the four patients underwent a combined operation with cesarean section followed by aortic repair. One patient underwent operative aortic repair following spontaneous delivery. The final patient underwent aortic repair with the fetus remaining in situ. Median sternotomy and cardiopulmonary bypass were established via the femoral artery with direct right atria drainage and left atrial venting in all patients. Composite graft replacement combined with re-implantation of the coronary artery and aortic valve replacement were performed in three patients, and aortic valve replacement with coronary artery bypass grafting of the right coronary artery was performed in one patient. Three of four patients underwent aortic arch repair utilizing antegrade cerebral perfusion and deep hypothermia with total circulatory arrest. The patient that underwent operative correction with the fetus remaining in situ experienced fetal demise with miscarriage just after cardiac surgery, and the patient died 4 days later secondary to disseminated intravascular coagulation and multi-organ failure. The remaining three cases recovered uneventfully, and the mothers and babies were discharged in good condition. Conclusions: Based on these data, we advocate cesarean section with concomitant aortic repair for patients with Marfan syndrome and acute type A aortic dissection during pregnancy. Minimization of deep hypothermic circulatory arrest time is also recommended for cases in which the fetus remains in situ.
Key Words: Aortic dissection • Pregnancy • Cardiopulmonary bypass
1. Introduction
Acute aortic dissection in pregnant woman is often associated with catastrophic outcomes for the mother and fetus unless prompt surgical management is undertaken. Marfan syndrome is a particularly important predisposing factor for aortic dissection in pregnancy. Because previous studies suggest that type A dissection during pregnancy is associated with aortic root enlargement (>4cm) or an increase in aortic root size during pregnancy in patients with Marfan syndrome [1,2], echocardiographic monitoring of pregnant patients with Marfan syndrome is critical. The present study reviews four cases of type A acute aortic dissection during late pregnancy in patients with Marfan syndrome. None of the patients had received preconceptional counseling regarding the risks of pregnancy at their institutions nor underwent echocardiographic monitoring of aortic root size. Ultimately, all four patients were referred to our institution for emergent aortic repair. The present study characterizes the treatment and outcomes of these patients in an attempt to further out understanding of this phenomenon and improve management decisions.
2. Materials and methods
2.1. Patient data
Four cases of acute type A aortic dissection during pregnancy in patients with Marfan syndrome at our institution between 1991 and 2003 were retrospectively reviewed. All four patients had a diagnosis of Marfan syndrome and displayed almost all the classic clinical manifestations of the disease (tall, thin appearance, arachnodactyly, funnel chest, ectopia lentis) as well as a family history of aortic dissection. Two patients were diagnosed with Marfan syndrome during childhood, and all patients were referred for routine prenatal care. However, the diagnosis of Marfan syndrome was not recognized by the obstetrician at the time of referral to our hospital. Thus, none of the patients had received preconceptional counseling regarding the risks of pregnancy at their institutions nor underwent echocardiographic monitoring of aortic root size. The preoperative condition of all patients is summarized in Table 1
. Mean patient age was 31.8±2.28 years, with a range of 28–34 years old. The mean gestational period at the time of operative repair was 31 weeks, with a range of 26–34 weeks. One patient experienced threatened premature birth. Three of four cases consisted of DeBakey type I aortic dissection, while the remaining case was classified as DeBakey type II aortic dissection. The diagnosis was confirmed by echocardiography and/or computerized tomography, which demonstrated an enlarged aortic root (diameter range 35–85mm).
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3.1. Operations
All cases underwent operative repair within 1 day of referral to our institution. Treatment and operative procedures are summarized in Table 2
. Two of the four patients underwent a combined operation, consisting of cesarean section followed by aortic repair. One patient underwent operative repair following spontaneous delivery, because the patient's cervix was dilated and effaced, and premature rupture of the membrane had already occurred (case 3). The patient with threatened premature birth progressed rapidly, leaving insufficient time to establish epidural anesthesia. The remaining patient underwent operative repair with the fetus remaining in situ, because the patient refused cesarean section (case 2). Fentanyl anesthesia was selected by all the patients for cardiovascular repair, and general anesthesia with fentanyl was used for cesarean section in two patients. In one case, a baby was delivered with a 1-min Apgar score of 1, necessitating intubation. This outcome is likely related to the high-dose fentanyl anesthesia.
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3.2. Morbidity and mortality
Outcomes following operative repair are summarized in Table 3 . One mother and baby died (case 2); this patient underwent operative repair with the fetus remaining in situ and experienced fetal loss just after completing surgery. During cardiopulmonary bypass, maternal blood pressure was controlled between 70 and 80mmHg with a pump blood flow rate of 2.6–3.0L/min per m2 per min, but the fetal heart rate showed gradual deceleration with subsequent arrest. Total aortic arch replacement and aortic root replacement proceeded with antegrade cerebral perfusion and deep hypothermia. Fetal monitoring failed to detect a sustained heart rhythm after weaning from cardiopulmonary bypass and completion of cardiovascular repair. Fetal demise was diagnosed, and a stillborn fetus was delivered spontaneously on the following day. Postoperatively, her laboratory data demonstrated severe hepatic, renal dysfunction, acidosis, and coagulopathy. And the patient developed multi-organ failure and disseminated intravascular coagulation, and she died 4 days later.
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The three surviving women were healthy at an average follow-up time point of 86.3 months after their date of discharge (range 18–168 months). Subsequent pregnancies did not occur in any of the patients. One patient required graft replacement of the descending aorta 2 year later, thoraco-abdominal graft replacement 2 year later, and graft replacement of the right subclavian artery 12 years later secondary to chronic dissection. The three children that were discharged were alive and healthy and did not experience any operation-related adverse events.
4. Discussion
Aortic dissection is rare in young women but is more frequent in the context of pregnancy secondary to the hyperdynamic and hypervolemic circulatory state associated with pregnancy [1]. Marfan syndrome is a particularly important predisposing factor for aortic dissection in pregnancy, and women with this disease should receive preconception counseling regarding the risks to both the mother and the fetus [1,2]. Review of more than one million patient records between 1991 and 2003 at our institution identified five patients that developed acute aortic dissection during pregnancy. The four patients with type A dissection were described in the present study, while identification of one patient with type B dissection (33-year-old female, 38th week of gestation) resulted in the safe delivery of a healthy newborn without complications to the mother.
Another study [3] identified five cases of aortic dissection in a total of 44 women with Marfan syndrome (78 pregnancies beyond 24 weeks of gestation). Unfortunately, the prevalence of acute aortic dissections in pregnant patients with Marfan syndrome could not be determined from retrospective review of our records, because: (1) patients were either unaware of their diagnosis of Marfan syndrome or simply uninterested in the implications of the disease towards and their pregnancy, or (2) many gynecologists in Japan are unaware of the implications of a diagnosis of Marfan syndrome in pregnant patients and, thus, do not perform proper screening for the disease.
Rossiter et al. [2] and Immer et al. [4] reported that favorable maternal and fetal outcomes in the context of Marfan syndrome were associated with an aortic root diameter of <40mm and the absence of subsequent aortic root dilatation. However, in the present study, one patient presented with aortic dissection despite having an aortic root diameter of <40mm. Thus, smaller aortic root diameter cannot rule out the occurrence of aortic dissection in pregnant Marfan parents.
Surgery for acute aortic dissection during pregnancy has been described by other investigators [4,5], and favorable outcomes for pregnant women with acute type A dissection occurring late in pregnancy have been reported [4,5]. However, in most cases, the fetal outcome was relatively poor [4], likely secondary to variables associated with delivery of the fetus, fetus maturity and the condition of the fetus in situ. In combination with the present cases series, these data suggest that, in cases of fetal maturity, cesarean section should be performed before or in combination with cardiovascular surgery [4]. However, the appropriate surgical management for acute type A dissection with an immature fetus in utero remains unclear. Review of the literature suggests that cardiopulmonary bypass during pregnancy is associated with maternal mortality of only 3% but a fetal poor mortality of 20% [6]. Thus, cardiopulmonary bypass with the fetus in utero may itself represent a risk factor. Further, studies have demonstrated that hypothermia during cardiopulmonary bypass results in uterine contraction and reduces placental blood flow [6], while re-warming from hypothermia may induce uterine contractions and premature labor. By contrast, Becker [7] suggested that high-flow, high-pressure normothermic perfusion during cardiopulmonary bypass is probably safer for the fetus than the use of hypothermia during cardiopulmonary bypass.
Cardiovascular operation using deep hypothermia with total circulatory arrest for aortic repair may be associated with an even higher risk of fetal mortality. Mul et al. [8] reviewed a case of acute type A aortic dissection at 29 weeks' gestation. The patient underwent aortic root replacement under deep hypothermia and circulatory arrest, resulting in delivery at 38 weeks' gestation of a female infant with progressive brain atrophy. However, another study described a case of successful repair of a ruptured aortic arch aneurysm using deep hypothermic circulatory arrest at 21 weeks' gestation [9]. Both the mother and the fetus survived, and a normal infant was delivered at 39th weeks of gestation.
The case in which operative repair was attempted with the fetus remaining in situ resulted in death of the mother and fetus. During cardiopulmonary bypass in this case, maternal blood pressure was controlled, but the fetal heart rate showed gradual deceleration with subsequent arrest. Of note, the cardiopulmonary bypass and deep hypothermia with circulatory arrest times were relative long (Table 2). Further, the aortic dissection extended from the aortic root to the iliac bifurcation. This, combined with the face that the femoral artery was used for cardiopulmonary bypass, raises the possibility that the patient developed interruption of splanchnic blood flow, resulting in bowel, hepatic, renal and placental ischemia and necrosis. Moreover, despite fetal immaturity, cesarean section at gestational week 26 may have produced improved outcomes for both the fetus and the patient, as postnatal therapy for immature infants has rapidly improved over the past decade. Thus, in retrospect, the patient may have benefited from cardiopulmonary bypass with cannulation of the right axillary artery and femoral artery for prevention of malperfusion and/or from cesarean section before aortic repair.
In conclusion, emergent delivery of the fetus is recommended for pregnant women with type A aortic dissection prior to emergent aortic repair. Other strategies, including minimization of deep hypothermic circulatory arresting time and institution of concomitant right axillary and femoral artery cannulation for cardiopulmonary bypass may be of benefit in cases in which the fetus remains in situ.
References
This article has been cited by other articles:
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  S. Goland and U. Elkayam Cardiovascular Problems in Pregnant Women With Marfan Syndrome Circulation, February 3, 2009; 119(4): 619 - 623. [Full Text] [PDF]
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 S. Pagni, B. L. Ganzel, and T. Tabb Hemiarch aortic replacement for acute type A dissection in a Marfan patient with twin pregnancy Interactive CardioVascular and Thoracic Surgery, August 1, 2008; 7(4): 740 - 741. [Abstract] [Full Text] [PDF]
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 H. Wakiyama, M. Nasu, H. Fujiwara, A. Kitamura, and Y. Okada Two Surgical Cases of Acute Aortic Dissection in Pregnancy with Marfan Syndrome Asian Cardiovasc Thorac Ann, October 1, 2007; 15(5): e63 - e65. [Abstract] [Full Text] [PDF]
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 J. Seeburger, F. W. Mohr, and V. Falk Acute Type A Dissection at 17 Weeks of Gestation in a Marfan Patient Ann. Thorac. Surg., February 1, 2007; 83(2): 674 - 676. [Abstract] [Full Text] [PDF]
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 H. Matsuda, H. Ogino, R. Neki, and S. Kitamura Hemiarch replacement during pregnancy (19 weeks) utilizing normothermic selective cerebral perfusion. Eur. J. Cardiothorac. Surg., June 1, 2006; 29(6): 1061 - 1063. [Abstract] [Full Text] [PDF]
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M. Jahangiri and J. Clark Surgery for type A aortic dissection in pregnant patients with Marfan syndrome Eur. J. Cardiothorac. Surg., February 1, 2006; 29(2): 264 - 264. [Full Text] [PDF]
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M. Sakaguchi Reply to Jahangiri and Clark Eur. J. Cardiothorac. Surg., February 1, 2006; 29(2): 264 - 265. [Full Text] [PDF]
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