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Successful near-term pregnancy outcome after repair of a dissecting thoracic aortic aneurysm at 14 weeks gestation

[Dénouement heureux d’une grossesse menée presque à terme à la suite de la réparation d’un anévrysme disséquant de l’aorte thoracique à 14 semaines de gestation]

From the Department of Anesthesiology, University of Oklahoma Health Sciences Centre, Oklahoma City, Oklahoma, USA.

Address correspondence to: Dr. Monala Tilak, Department of Anesthesiology, University of Oklahoma Health Sciences Centre, 920 Stanton L. Young Blvd., Room WP 2530, Oklahoma City, OK 73104, USA. Phone: 405-271-4351; Fax: 405-271-8695; E-mail: Monala-tilak{at}ouhsc.edu

	Abstract

TOP Abstract Introduction Clinical features Discussion Conclusion References

 
Purpose: We report the anesthetic management for a scheduled Cesarean section of a 29-yr-old female who previously had a repair of her dissecting thoracic aortic aneurysm during the 14^th week of her pregnancy.

Clinical features: A 29-yr-old female with a history of hypertension and previously diagnosed aortic dissection secondary to suspected Marfan’s syndrome, presented to our institution for the first time after she became pregnant. A transesophageal echocardiogram revealed a dissecting aortic aneurysm greater than 8 cm in diameter beginning distal to the left subclavian artery and extending into the descending thoracic aorta. The patient was counseled in great detail about the risk of rupture with continuing pregnancy. She refused termination and chose elective repair of the aneurysm, with continuation of the pregnancy. Partial repair of the thoracic aneurysm was undertaken when the pregnancy was 13^5/7 weeks by ultrasound dates. She was subsequently maintained on labetolol and hydralazine for blood pressure control. A decision was made to proceed with a Cesarean section at 32 weeks. After placement of a radial artery catheter and two large peripheral iv catheters, she received a of 7 and 7 at one and five minutes was delivered.

Conclusions: Aortic dissection in pregnancy may have catastrophic results. Undoubtedly, it presents unique challenges for anesthetic and obstetrical management. With appropriate care and surgical correction of the dissecting aneurysm early in pregnancy, a successful outcome for the pregnancy was possible.

	Introduction

TOP Abstract Introduction Clinical features Discussion Conclusion References

 
AORTIC dissection is often cited as a complication of Marfan’s syndrome, being reported in as many as one third of the patients at autopsy.¹ In these patients, aortic dissection occurs at an early age and is usually unaccompanied by hypertension. Aortic dissection in pregnancy can be catastrophic. It has been estimated that 50% of all aortic dissections in women less than 40 yr of age occurs during pregnancy, more commonly in the later months, leading to the speculation that the hemodynamic alterations during pregnancy may play a role.² While systemic hypertension is the most common etiological risk factor for aortic dissection, Marfan’s syndrome, other congenital cardiovascular abnormalities and pregnancy-induced changes in the vessel wall are also associated with this entity.³ Several authors have suggested that physiologic, cardiovascular and hormonal changes in pregnancy may result in an increase of the shearing stress on the aortic wall accompanied by weakening of some of its elements, thus predisposing the pregnant patient to aortic dissection.⁴ We report a case of a 29-yr-old woman with a known aortic dissecting aneurysm for two years who presented to us initially, for the management of her pregnancy. She had partial repair of her dissecting aneurysm during the 14^th week of her pregnancy, and successfully proceeded to 32 weeks when she delivered a live infant by Cesarean section.

	Clinical features

TOP Abstract Introduction Clinical features Discussion Conclusion References

 
All personal identifying details have been excluded and consent for publication of this manuscript obtained in accordance with our institutional guidelines. A 29-yr-old, primigravid female, 180 cm tall and weighing 135 kg, with a history of chronic hypertension and suspected Marfan’s syndrome, underwent partial repair of a type III thoracic aortic dissecting aneurysm during her 14^th week of pregnancy. The dissecting aneurysm was identified two years previously when the patient presented at another institution for complaints of "burning" back pain and left upper quadrant pain, which led to serial imaging studies. She had previously lost a fetus at eight weeks. Her father, two uncles and a brother had died from acute cardiac events. The patient was counseled in great detail about the risk of continuing the pregnancy, but she chose elective repair of the aneurysm with continuation of the pregnancy. Partial repair of the thoracic aneurysm was undertaken when the pregnancy was 13^5/7 weeks by ultrasound dates. The surgical procedure involved replacement of the proximal area of the dissection with a synthetic graft during atrialfemoral bypass without circulatory arrest. The surgeon elected not to proceed with repair of the distal aorta to protect the pregnancy and minimize the risk of spinal cord damage. Repair of the distal aorta would have needed an extension of the thoracotomy to a laparotomy, prolonged the length of the operation, required larger doses of anticoagulation and increased the risk for hypothermia, which would have been dangerous to the fetus.⁴ Only when the entire descending thoracic aorta is grossly aneurysmal should one consider replacing the entire descending aorta.⁵ If resection of the distal thoracic aorta can be avoided, the perfusion to the spine is less likely to be affected, minimizing the risk of spinal cord ischemic injury.⁶ During the procedure, the patient received two units of packed red blood cells, 1 L of fresh frozen plasma, one apheresis unit (700 mL) of platelets, 20 units of cryoprecipitate and four units of cell saver blood. The postoperative course was noteworthy for lower visual field loss in her right eye consistent with posterior ischemic optic neuropathy and left otitis externa, both of which resolved without sequelae.

Following the surgery, the patient was followed-up in the high-risk obstetric clinic and experienced no further problems. Her blood pressure was adequately controlled with labetalol and hydralazine. Three weeks after surgery, at 16^4/7 weeks of gestation, the patient returned with complaints of "tearing" chest pain. A computerized tomography (CT) scan with contrast was done to rule out an extension of the dissection. This showed a type III dissecting aortic aneurysm with a maximal diameter of 8 cm (Figure) extending into the abdominal and pelvic aorta with a stable configuration of the true and false lumen, as well as chronic hematoma over the graft with no active extravasation. The maximal diameter obtained on transesophageal echocardiography during the 12^th week of gestation was 6.7 cm. The patient remained asymptomatic after that episode.

View larger version (119K): [in this window] [in a new window]   FIGURE Contrast computed tomography scan showing the residual type III dissecting aneurysm of the aorta (arrow) with a maximal diameter of 8 cm. The arrow points to the intimal flap with a smaller true lumen above it, and a larger false lumen below.

	Discussion

TOP Abstract Introduction Clinical features Discussion Conclusion References

 
A literature search did not reveal any published cases of aortic dissection in pregnancy that was repaired as early as 14 weeks of gestation, which proceeded to a live birth.

Aortic dissection is commonly associated with chronic hypertension, however, other risk factors such as atherosclerosis, endocrine abnormalities, trauma, pregnancy and diseases involving abnormalities of elastic tissues (Marfan’s syndrome) can play a role in the development of a dissecting aneurysm.¹ Prior studies have found that approximately half of aortic dissections reported in females younger than 40 yr of age are associated with pregnancy.² Pregnancy associated changes related to the cardiovascular system, including a sustained increase in cardiac output, pulse pressure, as well as the histological changes that alter the media of the aorta, are very likely related to the increased risk of aortic dissection. These changes can be catastrophic in a female with Marfan’s syndrome.⁷ With pregnancy, the histological changes that have been described with reference to the aorta, include fragmentation of the reticulum, attenuation of the elastica with increased corrugation, and a decrease in the amount of acid mucopolysaccharides. These changes are likely to increase the vulnerability of the aorta to dissection.⁸ Symptoms such as a tearing or ripping sensation in the chest, abdominal or back pain should alert clinicians to the diagnosis of aortic dissection. Our patient had presented two years previously to another institution with burning back pain and left upper quadrant pain, and the diagnosis was made with the aid of an aortogram and an angiocardiogram. Definitive diagnosis and sequential monitoring during pregnancy is best performed with transesophageal echocardiography, CT scan or magnetic resonance imaging. The diagnostic accuracy of transesophageal echocardiography is extremely high (up to 99%). The use of contrast makes the CT scan less favourable in pregnancy. Angiography, though sensitive, is an invasive procedure, requiring a significant dose of radiation as well as iv contrast material. Therefore it is not the preferred diagnostic procedure in pregnancy.⁴ It also carries a substantial risk of procedural complications and diagnostic pitfalls.⁹

The treatment of aortic dissection in pregnancy is based on its location and severity, and the gestational age. Aortic dissections that occur in the ascending aorta (Debakey types I and II) need surgical repair, while aneurysms of the descending aorta (Debakey type III) allow for consideration of medical management.² In a patient with a fetus of less than 28 weeks gestation, aortic repair without delivery is the suggested treatment and in a fetus with a gestational age greater than 32 weeks, Cesarean section coupled with subsequent aortic repair is recommended. Gestational age of 28 to 32 weeks is an unclear area where the condition of the mother and the fetus must be evaluated at length.¹⁰ The cardiovascular changes during pregnancy can be particularly dangerous in this situation because of the increased pulsatile shear stress on the aortic wall.¹¹ Correct medical management consists of reduction of arterial pressure and velocity of ventricular contractions with antihypertensive agents and ß-blockers. Beta-blockers may have important maternal and fetal side effects and should be used cautiously. They can increase the uterine tone and contractility and decrease umbilical blood flow.¹² Propranolol may be associated with intra-uterine growth retardation. Hydralazine, labetalol, and nifedipine have been used safely in pregnant patients. Nitroprusside and nitroglycerine have also been used, but carry the risk of fetal cyanide toxicity and loss of fetal heart beat-to-beat variability, respectively.² This patient was treated for hypertension using labetalol and hydralazine following her initial repair, early in pregnancy.

The current literature offers minimal information with regard to the induction of anesthesia for obstetrical delivery in the pregnant patient with an incompletely repaired dissecting aortic aneurysm. The anesthetic considerations of an existing aortic aneurysm applied during the management of the Cesarean section. The goal of anesthetic management is to maintain a stable hemodynamic status avoiding both hypotension, which would jeopardize uteroplacental flow, as well as hypertension which might increase the risk of the aneurysm rupturing. General anesthesia carries the risk of a hypertensive response to laryngoscopy and intubation which could have disastrous consequences, although this can be prevented with the use of appropriate pharmacology.³ Regional anesthesia avoids this problem, although there is a risk of an abrupt sympathectomy that can be induced with spinal anesthesia in a patient on concurrent treatment with ß-blockers.² The patient’s hemodynamic status must be monitored closely and controlled to limit the risk of further dissection or rupture. Vascular changes do not normalize after pregnancy, thus increasing the risk for subsequent pregnancies. Therefore, aortic dissection may be considered a contraindication to further pregnancy, thus necessitating effective contraception.¹⁰

We chose a regional technique for this patient’s Cesarean section which was performed at 32 weeks gestation because the aneurysm was partially repaired and the situation was reasonably well controlled, as opposed to a patient presenting with an acute dissecting aortic aneurysm at that gestational age. The surgery was performed in the cardiac operating room with drugs to manipulate the heart rate and blood pressure readily available. The anesthesia team involved a cardiac anesthesiologist and an obstetric anesthesiologist adept at caring for high-risk obstetric patients. Although epidural anesthesia is more controlled, it is slower in onset and has a risk of inadequate block which could be disastrous in this patient. Hence we chose spinal anesthesia as our predominant anesthetic. The combined spinal-epidural technique would provide us with the capability to extend the duration of the block with the epidural catheter, in the event of an unforeseen delay in the completion of the surgery, and also facilitate postoperative analgesia. This patient also had an elective sterilization following the Cesarean section.

	Conclusion

TOP Abstract Introduction Clinical features Discussion Conclusion References

 
A pregnant patient with a dissecting aortic aneurysm presents a considerable challenge for anesthesiologists, obstetricians and cardiovascular surgeons. Proper management required consultations among the above departments, including cardiology and perinataology physicians. With appropriate care, a successful out-come for the mother and the baby can be achieved as in our case, where the dissecting aneurysm was partially repaired during the 14^th week, with successful continuation of the pregnancy to 32 weeks.

	Acknowledgments

 
The authors gratefully acknowledge the invaluable help provided by Ms. Sonia Perinovic in the preparation of the manuscripte. We thank Jane C.K. Fitch MD for her expert suggestions and editorial comments.

	Footnotes

 
There are no funding sources (departmental, hospital, institutional, commercial, etc.) supporting the submitted work, any commercial or non-commercial affiliations that are or may be received to be a conflict of interest with the work, and any other associations such as consultancies.

Accepted for publication November 18, 2004. Revision accepted May 2, 2005. Final revision accepted July 6, 2005.

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This Article Abstract Résumé de cet Article Full Text (PDF) Submit a scholarly reply Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Tilak, M. Articles by Peyton, M. Search for Related Content PubMed PubMed Citation Articles by Tilak, M. Articles by Peyton, M.