Ann Thorac Surg 2007;83:674-676
© 2007 The Society of Thoracic Surgeons
Case Reports
Acute Type A Dissection at 17 Weeks of Gestation in a Marfan Patient
Joerg Seeburger, MD*,
Friedrich Wilhelm Mohr, MD, PhD,
Volkmar Falk, MD, PhD
Accepted for publication June 5, 2006.
* Address correspondence to Dr Seeburger, Heartcenter, Leipzig University, Struempelstrasse 39, 04289 Leipzig, Germany. (Email: j.seeburger{at}web.de).
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Abstract
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A 29-year-old Marfan patient at 17 weeks of gestation was diagnosed with acute type A aortic dissection and severe aortic regurgitation. Aortic valve and aortic arch replacement was successfully performed under circulatory arrest with deep hypothermia. At 34 weeks of gestation, the patient underwent a cesarean section and delivered a healthy baby.
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Introduction
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Acute aortic type A dissection in pregnancy is of high risk for the mother and the fetus. Concomitant disease of Marfan syndrome is of special interest.
A 29-year-old asymptomatic Marfan patient booked at 17 weeks of gestation in her first pregnancy was brought to our hospital with echocardiographic evidence of acute type A dissection and severe aortic regurgitation. The dissection, including the aortic arch, supraaortic vessels, and the aorta descendens, was verified by a computed tomography (CT) scan (Fig 1). An 80-mm aorta ascendens aneurysm was also diagnosed. This patient had a negative history of vascular events, high blood pressure, and previous vascular surgery.
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Fig 1. Complete dissection of the aortic arch (top) and the aortic root aneurysm (bottom) in a 29-year-old Marfan patient at 17 weeks of gestation.
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Because of the age of the patient and despite the possibility of future pregnancies, the patient insisted on a mechanical valve. Owing to dissection of both the truncus and the right axillary artery, our standard cannulation technique was altered, and the nondissected left carotid artery was selected and cannulated with an 8-mm Hemashield prosthesis (Boston Scientific, Natick, MA).
A median sternotomy was performed. Aortic surgery was conducted with a brief period of circulatory arrest and deep hypothermia (22.2°C). A Hemashield prosthesis was distally anastomosed as an elephant trunk, followed by anastomoses of the supraaortic vessels in 20 minutes of distal circulatory arrest. Because of dissection of the noncoronary and right coronary sinus and the massive root enlargement, aortic valve reconstruction was not possible. A 25-mm ATS Aortic Valve Graft (ATS Medical, Minneapolis, MN) was implanted, with reimplantation of the coronary ostia. Clamp time was 173 minutes. Total circulatory arrest time was 20 minutes. Isolated cerebral perfusion was performed for 35 minutes. Reperfusion time was 71 minutes. There were no intraoperative complications.
Postoperative sonography demonstrated a healthy fetus with normal heart rate and motility. Intensive care unit stay was 66 hours. Postoperative medication consisted of ß-blockers, heparin, and diuretics. Paroxysmal atrial fibrillation occurred throughout the hospital stay.
On day 8 after surgery, a left-sided chylothorax was diagnosed. Subsequently, nutrition was switched to parenteral substitution for 5 days, followed by a 16-day medium-chain triglyceride diet containing mainly middle-length fatty acids. After chest drainage for 24 days the chyle secretion was self-limiting, with no recurrence.
The patient was released from hospital on day 39 after surgery for rehabilitation. She was in good physical condition, with no neurologic impairment except a persisting slight anisocoria and dysphonia. Subsequent anticoagulation until caesarian section was achieved with phenprocoumon (international normalized ratio, 2 to 3).
In week 34 of gestation, the patient gave birth to a healthy boy through caesarian section (weight, 1900 grams; length, 45 cm; Apgar score, 8/9/9). Subsequent clinical examination and a CT scan of the mother showed no abnormalities. At 12 weeks after the caesarian section, the mother and baby are in good health.
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Comment
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Aortic dissection is a life-threatening disease with the need for immediate surgical intervention and is not uncommon during pregnancy, especially labor [1]. Immediate aortic surgery is indicated in pregnant women with acute aortic type A dissection, despite the risk of poor fetal outcome [1]. Aortic root enlargement that exceeds 4 cm or an increase of aortic size during pregnancy are identified risk factors for the occurrence of type A dissection in Marfan patients [2].
Only very limited data exist on the coincidence of Marfan syndrome, pregnancy, and aortic dissection. For the most part, these patients experienced dissection during labor and underwent aortic surgery after caesarian section or natural birth. To our knowledge, only two cases of pregnant Marfan patients undergoing cardiovascular surgery before labor and birth have been reported [3, 4]. Sakaguchi and colleagues [3] recently reported four pregnant Marfan patients with acute type A dissection. Three underwent caesarian section, followed by aortic surgery with acceptable results. One patient had surgery with the fetus in utero but died 4 days later from multiorgan failure. In the case reported by Naito and colleagues [4], a pregnant Marfan patient with acute aortic dissection and aortic ectasia underwent a Bentall procedure at 24 weeks of gestation and delivered a healthy baby by caesarian section in week 33.
Aortic arch surgery is a clear indication for circulatory arrest with deep hypothermia and simultaneous brain perfusion. But the use of cardiopulmonary bypass (CPB) is associated with a maternal mortality of 3% and a poor fetal outcome, with a mortality of 20% [5]. The use of CPB itself can be a risk factor, especially in pregnant patients, and may lead to further complications because it is known to induce a systemic inflammatory response [6]. So far, no guidelines exist to the use of normothermia, or moderate or deep hypothermia in pregnant patients. Hypothermia has been described to reduce placental blood flow and increase uterine contraction [5]. In the present patient, circulatory arrest and deep hypothermia with selected perfusion of the head were applied, with a highly satisfying outcome and no major intraoperative or postoperative complications.
No long-term data on the baby’s health status, especially his neurological outcome, are available at this time, but at 12 weeks, the neurological development is normal. Mul and colleagues [7] reported a case of aortic arch replacement under deep hypothermia in a pregnant patient who delivered an infant presenting with brain atrophy [7]. This contrasts with one other case reported by Buffolo and colleagues [8] in which a pregnant patient at 21 weeks of gestation with a ruptured aortic arch aneurysm that was repaired under deep hypothermia and circulatory arrest delivered a normal infant at 39 weeks of gestation [8]. The potential of cerebral, neurologic, and other deficiencies of the unborn child can only be estimated after close long-term follow-up on these very rare patients.
Currently, most pregnancies are terminated. If favorable outcomes are reported more frequently, it may affect the perioperative counselling. This report should also underline the importance of counselling of all Marfan patients in pregnancy to avoid life-threatening complications such as acute type A dissection.
Marfan syndrome, pregnancy, and aortic dissection is a very rare but highly challenging coincidence with no standardized therapeutic concept. In our opinion, surgical repair of acute type A dissection in pregnant patients using CPB and deep hypothermia with the fetus in utero seems to be an adequate therapeutic regime, even in Marfan patients. We suggest that a more aggressive approach in selected cases, maybe elective treatment, will lead to favorable results at a reasonable risk.
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References
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- Immer FF, Bansi AG, Immer-Bansi AS, et al. Aortic dissection in pregnancy: analysis of risk factors and outcome Ann Thorac Surg 2003;76:309-314.[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;173:1599-1606.[Medline]
- Sakaguchi M, Kitahara H, Seto T, et al. Surgery for acute type A aortic dissection in pregnant patients with Marfan syndrome Eur J Cardiothorac Surg 2005;28:280-285.[Abstract/Free Full Text]
- Naito H, Naito H, Tada K. Open heart operation for a pregnant patient with Marfan syndrome Masui 2005;54:525-529.[Medline]
- Pomini F, Mercogliano D, Cavalletti C, Caruso A, Pomini P. Cardiopulmonary bypass in pregnancy Ann Thorac Surg 1996;61:259-268.[Abstract/Free Full Text]
- Cremer J, Martin M, Redl H, et al. SIRS—the systemic inflammatory response syndrome after cardiac operations Ann Thorac Surg 1996;61:1607-1608.[Free Full Text]
- Mul TF, van Herwerden LA, Chen-Overbeck TE, Catsman-Berrevoets CE, Lotgering FK. Hypoxic-ischemic fetal insult resulting from maternal aortic root replacement, with normal fetal heart rate at term Am J Obstet Gynecol 1998;179:825-827.[Medline]
- Buffolo E, Palma JH, Gomes WJ, et al. Successful use of deep hypothermic circulatory arrest in pregnancy Ann Thorac Surg 1994;58:1532-1534.[Abstract]
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Abstract
Introduction
