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CASE REPORT
Year : 2023 | Volume
: 13
| Issue : 1 | Page : 106-108
Combined spinal-epidural anesthetic management of delivery for marfan syndrome: Case report
Matea Malinovic, Kimberly Babiash, Felecia Newton
Department of Anesthesiology, University of Kansas School of Medicine, Wichita, KS, USA
Correspondence Address:
Dr. Matea Malinovic
Department of Anesthesiology, University of Kansas School of Medicine, Wichita, KS
USA
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/JOACC.JOACC_31_22
Pregnancy in a patient with Marfan syndrome is associated with risks, including cardiovascular complications. The hemodynamic changes of pregnancy during delivery are deleterious to Marfan syndrome patients. This case describes a Marfan syndrome parturient with dilated aortic root >45 mm who underwent neuraxial anesthesia and cesarean delivery. Anesthetic management focused on reducing hypertension and shear forces on the ascending aorta. A combined spinal-epidural with a reduced dose of hyperbaric bupivacaine was utilized for cesarean delivery. Multidisciplinary approaches are vital in the management of Marfan syndrome pregnancy and cardiovascular complications must be considered when planning for delivery and postpartum management. Anesthetic management must consider the hemodynamic changes in pregnancy and severity of Marfan syndrome cardiovascular involvement in the parturient as well as neonatal risks. Guideline development concerning anesthetic management for both the timing of delivery and aortic repair postpartum would be beneficial to Marfan syndrome parturients.
Keywords: Aortic dissection, case report, dilated aorta, high-risk pregnancy, Marfan syndrome
Marfan syndrome (MFS) is a connective tissue disorder that involves cardiovascular and hemodynamic consequences.[1] Increased cardiac output, heart rate, progesterone, and estrogen during pregnancy further compromise the aorta, increasing the risk of aortic dilation and dissectio n.[2] Thus, a multidisciplinary approach is crucial to ensure the safest outcome for the parturient and neonate. This case report was published with the written consent of the patient and adheres to the CARE guidelines.
Case ReportA 40-year-old parturient at 34 weeks gestation presented with the history of MFS with a dilated aortic root. This was her fourth pregnancy; after her three prior vaginal births, she had been advised against future pregnancies due to aortic enlargement. Labetalol was used for management of hypertension and vascular shear forces. Transthoracic echocardiogram (TTE) three months prior indicated an ejection fraction of 60%, moderate aortic regurgitation, and both dilated aortic root at 4.8 cm and ascending aorta at 4.96 cm. She denied angina, dyspnea, or dizziness.
TTE on admission showed a sinotubular junction of 5.3 cm and aortic root of 4.6 cm. She underwent a preterm (35 weeks, 6 days), low transverse cesarean section (c-section) and postpartum tubal ligation with cardiothoracic surgery on standby. Before surgery, radial arterial and internal jugular central lines were placed without complications using 3 mg midazolam and 60 μg remifentanil. After a co-load with 500 mL lactated ringers, anesthesia was initiated with a combined spinal-epidural (CSE) using 10 μg fentanyl, 0.1 mg morphine, and 4.75 mg hyperbaric bupivacaine. Due to the low dose CSE, she initially displayed thoracic 9 dermatome levels to Allis clamp which provided insufficient anesthesia for c-section. Therefore, the block was slowly raised using 2% lidocaine boluses (35 mL) over 45 min to obtain thoracic 4 dermatome levels. During this time, she was continuously assessed for changes in vital signs and for local anesthetic toxicity, but she remained stable.
Systolic blood pressure (SBP) was maintained at <120 mmHg and heart rate at <100 bpm with esmolol and nicardipine infusions, along with intravenous 5 mg metoprolol and 15 μg nitroglycerin. Prior to incision, 1 g tranexamic acid was given prophylactically for vasodilatory effects on endometrial smooth muscle. Intrauterine 600 μg misoprostol was given at delivery. The oxytocin bolus was reduced to 5 units over 20 min to avoid reflex tachycardia and maintain stable hemodynamics, followed by a maintenance rate of 80 milliunits/min postpartum. A Bakri balloon was placed to reduce potential postpartum atony and hemorrhage, with blood loss estimated at 600 mL. The epidural catheter was removed before transport to the ICU, without surgical or anesthetic complications. The preterm newborn was resuscitated by the NICU team.
Postoperatively, hypertension was treated with infusions of nitroglycerin, nicardipine, and esmolol. Oral labetalol was increased to 200 mg twice daily, and 10 mg oral amlodipine was initiated. The patient was weaned from antihypertensive infusions on postoperative day (POD) 1. On POD 2, a computed tomography angiogram revealed an aneurysmal dilatation of the thoracic aortic root measuring 5.7 cm [Figure 1]. Labetalol was subsequently increased to 300 mg twice daily. She was stable upon discharge on POD 4 and remained on antihypertensive agents. She underwent replacement of her ascending aorta four months postpartum.
Figure 1: Coronal (a) and axial (b) chest computed tomography scan performed postoperatively, indicating aortic aneurysmal dilation Discussion Cardiovascular involvement is found in 80% of MFS patients, with serious complications including aortic root dilation and rupture. Pregnancy-related physiologic changes exacerbate these complications, resulting in weakening of the tunica media of the aorta.[2],[3] Anesthetic management involving hemodynamic parameters and the potential for dural ectasia (DE) are important considerations, and multidisciplinary approaches with attention to maternal and fetal physiology are critical.
The World Health Organization divides MFS parturients into four risk categories based on their aortic root diameter.[4],[5] Vaginal delivery is recommended with an aortic root diameter <40 mm. C-section is recommended in an aortic root diameter >40 mm since the risk of dissection increases to 10%. Aortic repair before pregnancy is advised for patients with an aortic root diameter >45 mm, and managed with serial echocardiograms during pregnancy.[4],[5] Our patient's aortic root diameter exceeded 45 mm without aortic repair, but she was closely monitored with TTEs during pregnancy. C-section was scheduled to reduce fluctuating hemodynamics associated with vaginal birth. The plan and associated risks were discussed with the patient, who elected to proceed.
CSE was chosen because of the risks associated with hemodynamic changes and aspiration during anesthetic induction, and inhalational anesthetic agents effects on uterine atony, blood loss, and neonatal exposure and depressant effects.[6] While spinal block failure due to DE occurs in >50% of MFS patients, our patient had no history of DE and a regional technique had hemodynamic advantages over general anesthesia.[7] A case series utilizing neuraxial techniques on MFS patients with DE uses a reduced dose of hyperbaric bupivacaine injected into the spinal space, with the height of the block slowly increased to thoracic 4 dermatome levels using 2% lidocaine.[8] Standard spinal doses for c-section creates a sudden reduction in preload causing increased shear forces on aortic vasculature through reflex tachycardia. Consequently, we reduced bupivacaine dosage to ensure a denser surgical block, slowly raising the block with constant hemodynamic monitoring. An unsatisfactory epidural would have made hemodynamic control challenging without performing general anesthesia.
There is no consensus about anesthetic management for reducing shear forces on the aorta during delivery.[9] In this case, esmolol and nicardipine drips were utilized to manage SBP <120 mmHg. Esmolol helped avoid tachycardia, and nicardipine, despite uterine relaxation effects was beneficial for arterial vasodilation. Remifentanil infusion was used to provide analgesia and anxiolysis, reduce heart rate and blood pressure fluctuations, and lessen neonatal depression.[6] Using β-blockers, such as labetalol, decreases the rate of aortic aneurysm growth by reducing sympathetic activity, consequently lowering heart rate and shear stress on the aorta.[10] For postpartum management, close follow-up is pivotal because aortic dilation occurs in approximately 26% of patients.[3] Our patient experienced this, with the aortic root measuring 5.7 cm on POD 2. She was transitioned to oral antihypertensive agents, discharged on POD 4, and successfully replaced her ascending aorta four months postpartum.
While there are clear recommendations for the methods of delivery depending on aortic root diameter in MFS, guidelines concerning anesthetic management and both timing of delivery and aortic repair postpartum need further research.
Presentations
The University of Kansas School of Medicine Wichita Research Forum, Wichita, KS. The paper was virtually presented as a poster on April 21-22, 2022.
Author contributions
M.M. contributed to the conception of the work, drafted the manuscript, provided final approval of the version to be published, and agreed to be accountable for all aspects of the work. K.B. contributed to the conception of the work, edited the manuscript, provided final approval of the version to be published, and agreed to be accountable for all aspects of the work. F.N. contributed to the conception of the work, edited the manuscript, provided final approval of the version to be published, and agreed to be accountable for all aspects of the work.
Acknowledgments
This case report was published with the written consent of the patient. The authors would like to thank William Krogman, M.S., for help with editing and formatting the manuscript for submission. This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Conflicts of interest
No external funding and no competing interests declared.
References
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