P057: ANESTHETIC MANAGEMENT OF A PARTURIENT WITH MARFAN SYNDROME AND CHRONIC TYPE B AORTIC DISSECTION UNDERGOING CESAREAN SECTION
Karolain Garcia, MD; Vadim Katsenovich, DO; University of Florida
Introduction/Background: Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder caused by a mutation in the fibrilin-1 gene on chromosome 15. Prevalence rates range from 1.5 to 17.2 per 100,000 individuals. Most patients have cardiovascular manifestations including aneurysmal dilation of the aorta, mitral valve prolapse, and mitral regurgitation. Abnormalities of the musculoskeletal and ocular system are other manifestations. Major causes of death are aortic aneurysm, dissection, and rupture. Neuraxial anesthesia is the recommended modality for cesarean section (CS) for patients with MFS, although spinal anesthesia failure has been widely reported in the literature due to the presence of dural ectasia in patients with MFS. Dural ectasia causes an increase in capacity of CSF in the dural space of the lumbosacral spine causing inadequate spread of intrathecal local anesthetics. We report the perioperative management of a patient with a history of MFS and chronic Type B aortic dissection who underwent CS using combined spinal epidural (CSE) anesthesia.
Case Presentation: A 36-year-old female G5P2113 at 32-week gestation presented for elective repeat CS and bilateral tubal ligation. The patient had a history of MFS, aortic valve and aortic arch replacement, and medically managed Type B aortic dissection. Computerized Tomography scan during pregnancy showed a Type B dissection extending into the bilateral common iliac arteries and a 4.8 cm distal aortic arch aneurysm. Transthoracic echocardiogram showed a functioning bioprosthetic aortic valve, normal left ventricular ejection fraction, and no regional wall motion abnormalities.
Standard monitors and a left radial arterial line were placed in the operating room. A CSE was used to provide neuraxial anesthesia. A mixture of 1.6 ml of 0.75% hyperbaric bupivacaine, 20 mcg of fentanyl, 0.1 mg of morphine, and 20mcg of clonidine were injected intrathecally. The spinal needle was withdrawn and the epidural catheter was threaded. No medications were given through the catheter. Sensory block up to the level of T4 was achieved within five minutes. The case proceeded with uneventfully delivery of a female infant with APGAR scores of 8 and 8 at 1 and 5 minutes, respectively. Spinal anesthesia started to recede approximately three hours later and the patient ambulated without difficulty the next day.
Discussion/ Conclusion: Parturients with MFS are at increased risk of aortic dissection due to the physiologic changes of pregnancy as well as the hemodynamic strain associated with vaginal delivery. Patients with aortic dilation >4 cm, increasing dilation of aorta during pregnancy, or a history of prior aortic repair are recommended to have a CS. CSE is preferred over general anesthesia for CS in patients with MFS. It avoids the detrimental risks of general anesthesia such as significant hemodynamic response to laryngoscopy, aspiration risk during induction, and neonatal depression caused by maternally administered opioids. The primary goal in management of MFS patients with aortic aneurysms and dissections is reduction of cardiovascular stress and strict blood pressure control.
Our plan included the potential management of a failed spinal anesthetic, maintenance of hemodynamic instability, and emergent treatment of a ruptured aorta.