Interact CardioVasc Thorac Surg 2008;7:740-741. doi:10.1510/icvts.2007.171397
© 2008 European Association of Cardio-Thoracic Surgery
Case report - Vascular thoracic |
Hemiarch aortic replacement for acute type A dissection in a Marfan patient with twin pregnancy
Sebastian Pagnia,*,
Brian L. Ganzela and
Thomas Tabbb
a Department of Surgery, Division of Thoracic and Cardiovascular Surgery, University of Louisville, 201 Abraham Flexner Way, Suite 1200, Louisville, KY 40202, USA
b Department of Obstetrics and Gynecology, University of Louisville, Louisville, KY, USA
Received 7 November 2007;
received in revised form 19 February 2008;
accepted 20 February 2008
*Corresponding author. Fax: +1-502-561-2180/1-502-561-2190.
E-mail address: spagni{at}ucsamd.com (S. Pagni).
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Abstract
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Type A aortic dissection is a high risk surgical emergency. Its occurrence during pregnancy represents an extremely high risk for death and sequelae for both the mother and the fetus. Surgical treatment in the form of cesarean delivery and aortic repair using hypothermia and circulatory arrest is mandatory in most patients. We report the case of a 29-year-old Marfan female with an acute type A aortic dissection at 34 weeks of a twin pregnancy. She underwent cesarean delivery of male twins followed by repair of the ascending aorta and proximal arch using deep hypothermia and circulatory arrest. The mother and twin babies survived without sequelae and are alive at two years.
Key Words: Marfan syndrome; Aortic dissection; Twin pregnancy
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1. Introduction
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The simultaneous occurrence of acute type A aortic dissection and pregnancy is rare and commonly results in catastrophic outcomes unless emergent surgical intervention is undertaken [1]. Marfan syndrome is a distinct predisposing factor for aortic dissection, especially if the aortic root is already dilated. We report the case of successful aortic root and hemiarch replacement and cesarean delivery in a Marfan patient with twin pregnancy.
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2. Case report
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A 29-year-old female with known Marfan syndrome and pregnant at 34 weeks presented to the emergency room with severe chest pain, hypertension and increased painful uterine contractions. She had the classic stigmata of Marfan Syndrome. She was followed at the high risk obstetric clinic due to her aortic root dilatation (4 cm), however, with poor compliance. A CT-scan with IV contrast showed the thoracic aorta dissected with a diameter of 6 cm and extension of the dissection into the arch vessels and coronary sinuses.
Fetal viability and presence of twins was confirmed by ultrasound. She underwent fentanyl-based general anesthesia with invasive arterial and pulmonary artery monitoring. The intraoperative TEE showed severe AI and the intimal flap extending into the non-coronary sinus and right coronary ostium (Fig. 1). A cesarean section and twin males were delivered with low Apgar scores (1–7 and 2–8 at 1 and 5 min, respectively). This was most likely related to the use of fentanyl and maternal stress and pain. Babies' weights were 2.18 kg and 1.79 kg. After the abdominal closure, a median sternotomy incision was performed and a large hematoma at the root of the aorta was found with impending rupture. Cardiopulmonary bypass (CPB) was instituted through the right femoral artery and right atria cannulation using circulatory arrest (31 min) to complete the hemiarch anastomosis with velveted #28 mm Dacron graft (Vascutek, Terumo Cardiovascular Systems Corp., Ann Arbor, USA). Biologic glue (Bioglue, Cryolife, Inc., Kennesaw, USA) was used to reconstruct the layers of aorta before suturing. Circulatory arrest was started with a nasopharyngeal temperature of 17 °C with a flat EEG. Retrograde cerebral perfusion was implemented through an SVC cannula and monitored with transcraneal Doppler and cerebral oximetry to confirm symmetric delivery of cold blood. The technique of cerebral protection is described elsewhere [2].
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Fig. 1. Intraoperative TEE long axis view shows proximal extension of the type A dissection into the coronary ostia and sinuses.
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While rewarming, the coronary ostia patches were developed and reconstructed with glue, and a composite mechanical valved conduit (St Jude Medical Inc., Minneapolis, USA) #27 mm was implanted with the coronary ostia were attached individually. At a temperature of 36.5 °C the patient was weaned from CPB using a low dose of epinephrine (0.05 µg/kg/min). Myocardial arrest times were 208 and 170 min, respectively.
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3. Discussion
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Type A aortic dissection complicating pregnancy is a rare occurrence and life threatening for both the mother and the fetus, and requires immediate surgical intervention. The association of Marfan syndrome, pregnancy and aortic dissection is extremely rare. An English literature review [1] of dissection in pregnancies (n=16) showed the majority of pregnancies (1983–2002) in the third trimester patients with enlarged aortic roots (>4 cm) or increasing aortic size during pregnancy were at higher risk for dissection. Weiss et al. [3] analyzed 32 patients reported worldwide with aortic-arterial dissection (type not specified) and pregnancy. Maternal morbidity and mortality were 34% and 22%, respectively. It was this point on the higher risk for maternal death when surgery was performed late in pregnancy, most likely related to the hemodynamics burden that peak in the antepartum period [4]. The aortic repair in the mother has a reported mortality higher to the one in the non-pregnant patient.
Overall reported fetal outcomes are relatively poor [5, 6], likely secondary to factors associated with delivery, fetal maturity and condition of the fetus in situ. The mechanisms underlying fetal distress include hypothermia, placental vasoconstriction and increased uterine contraction resulting in hypoxia and potentially fetal demise. Fetal outcome is superior in later pregnancy. At 28 weeks of gestation surgical strategy mandates cesarean delivery immediately followed by aortic repair. If the dissection occurs before the 28th week, fetal outcome is likely poor, but newer reports such as Sakaguchi et al. and others [7, 8] have successfully addressed the use of deep hypothermic circulatory arrest in early pregnancy with fetal viability in term. Matsuda et al. [9] reported a hemiarch aortic replacement at 19 weeks of pregnancy using normothermic selective cerebral perfusion and high flow pulsatile systemic perfusion restuling in good materno-fetal outcome.
Immer et al. [1] pointed out the importance of counseling in pregnant Marfan patients as well as in those with bicuspid aortic valves or enlarged aortas. In patients who are diagnosed with an enlarged aorta during pregnancy, a close echocardiographic follow-up (biweekly) is recommended. Development of the fetus should be closely monitored and fetal lung maturation at 26 weeks and/or early hospitalization in the third trimester should be considered, selectively.
To our knowledge, this is the first report in the English literature describing the occurrence of acute type A aortic dissection, Marfan syndrome and twin pregnancy. This report emphasizes the importance of counseling of all Marfan patients undergoing pregnancy.
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References
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- Immer FF, Bansi AG, Immer-Bansi AS, McDougall J, Zehr KJ, Schaff HV, Carrel TP. Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg 2003;76:309–314.[Abstract/Free Full Text]
- Ganzel BL, Edmonds HL, Pank JR, Goldsmith LJ. Neurophysiologic monitoring to assure delivery of retrogarde cerebral perfusion. J Thorac Cardiovasc Surg 1997;113:748–755; discussion 755–757.[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;178:1643–1653.
- Mone SM, Sanders S, Colon SD. Control mechanisms for physiological hypertrophy of pregnancy. Circulation 1996;94:667–672.[Abstract/Free Full Text]
- Jahangiri M, Clark J. Surgery for Type A aortic dissection in pregnant patients with Marfan Syndrome. Eur J Cardiothoracic Surg 2006;29:264.[Free Full Text]
- Zeebregts CJ, Schepens MA, Hameeteman TM, Morshuis WJ, Brutel de la Riviere A. Acute aortic dissection complicating pregnancy. Ann Thorac Surg 1997;64:1345–1348.[Abstract/Free Full Text]
- Buffalo E, Palma JH, Gomes WJ, Vega H, Born D, Moron AF, Carvalho AC. Successful use of deep hypothermic circulatory arrest in pregnancy. Ann Thorac Surg 1994;58:1532–1534.[Abstract]
- Sakaguchiu M, Kitahara H, Seto T, Furusawa T, Fukui D, Yanagiya N, Nishimura K, Amano J. Surgery for acute Type A aortic dissection in pregnant patients with Marfan syndrome. Eur J Cardiothorac Surg 2005;28:280–283.[Abstract/Free Full Text]
- Matsuda H, Ogino H, Neki R, Kitamura S. Hemiarch replacement during pregnancy (19 weeks) utilizing normothermic selective cerebral perfusion. Eur J Cardiothorac Surg 2006;29:1061–1063.[Abstract/Free Full Text]
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Abstract
1. Introduction