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Anesthetic Management of Parturient with Shone's Syndrome
Abstract Number: S4D-8
Abstract Type: Case Report/Case Series
Shone’s syndrome is a rare congenital cardiac condition that consists of four major obstructive lesions that involve the left side of the heart. These defects include supravalvular mitral ring, parachute deformity of the mitral valve, subaortic stenosis and coarctation of the aorta. Shone’s syndrome can be complete and present with all four lesions, or more commonly incomplete. Rapid clinical deterioration can occur in symptomatic pregnant patients with heart disease such as Shone’s due to the known changes in HR, CO and SVR. Thus it is important to understand the physiology and effects of pregnancy in this patient population when developing an anesthetic plan. We discuss the successful use of labor epidural anesthesia for vaginal delivery in a patient with Shone’s syndrome.
21 year old G1P0 at 41 weeks gestation with h/o Shone’s syndrome presented for scheduled IOL and assisted vaginal delivery. Patient’s history included mitral valve stenosis, coarctation of the aorta, s/p repair at two months of age, parachute mitral valve and bicuspid aortic valve. She was reported to have intermittent SOB that improved with inhalers before and during pregnancy. She also had baseline substernal chest pain at least once but had >4 METS activity level. Cardiology had been following and managed her symptoms with acebutalol and furosemide. TTE during her third trimester revealed normal LVF with an EF of 55% and normal PAP. Based on this preoperative collaboration and evaluation, we planned for a labor epidural which was placed uneventfully. Non-invasive BP monitoring was used during the placement of the epidural catheter and initial dosing. Following a negative test dose, 3mLs 0.25% bupivacaine and 100mcg fentanyl were administered through the epidural. After which an epidural infusion was started with 0.125% Bupivacaine with 2mcg/ml Fentanyl at a rate of 10ml/hr. FHR and maternal HR and pulse oximetry were continuously monitored. A T10 level was achieved and the patient remained hemodynamically stable throughout. Patient was taken back to the OR for anticipated forceps delivery and delivered a healthy baby boy and she was discharged home two days later.
In pregnancy, stenotic or obstructive heart lesions, such as those with Shone’s syndrome are less tolerated due to the fixed stroke volume and the unfavorable effects of tachycardia. The obstetrical plan for assisted vaginal delivery with appropriate analgesia has been recommended in the literature for patients who are asymptomatic or have mild disease. However, the concerns for hemodynamic compromise still remain in the setting of neuraxial anesthesia and analgesia that may lead to afterload reduction, hypotension and tachycardia.
1. African Journal of Anaesthesia and Analgesia, 22:1, 33-36, DOI: 10.1080/22201181.2015.1111676
2. Anesthesia & Analgesia. 107(5):1652-1654, November 2008. DOI: 10.1213/ane.0b013e3181864d6e