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Anesthetic Management of Cesarean Section in a Parturient with Heterotaxy Syndrome and Severe Mitral Stenosis, a Case Report
Abstract Number: F-03
Abstract Type: Case Report/Case Series
INTRODUCTION: Heterotaxy syndrome is a rare congenital disorder caused by embryonic failure to differentiate left-right asymmetry. There is little data regarding outcomes in pregnant women with this disorder. We present a case of a pregnant female with heterotaxy syndrome complicated by severe mitral stenosis for elective cesarean delivery (C/S) using combined spinal/epidural (CSE) anesthesia.
CASE: A 31 y/o G3P1101 female presented for C/S at 37 4/7 weeks with a history of heterotaxy syndrome and increasing shortness of breath. She had polysplenia, misaligned gastrointestinal organs (volvulus repair at 34 days), and an endocardial cushion defect (mitral valve annuloplasty as a child). She developed severe stenosis of the mitral annuloplasty site, moderate pulmonary hypertension, chronic congestive heart failure, and atrial flutter necessitating pacemaker placement and anticoagulation (SQ heparin). An ECHO showed an EF 40%, RVH, decreased RV function, mean PAP 55 mmHg, and elevated peak and mean mitral valve gradients (MV area 2.1 cm2 ). She was morbidly obese (BMI 37) with obstructive sleep apnea (OSA) requiring nasal CPAP. Prior to delivery, the abdominal pacemaker was converted from VVI to VOO mode and an arterial catheter was inserted. We placed a CSE (intrathecal bupivacaine 0.75% 10 mg, fentanyl 10 mcg, hydromorphone 100 mcg) and maintained her MAP within 20% of baseline during the uneventful C/S.
DISCUSSION: Heterotaxy syndrome is a serious congenital defect resulting from disorders of left-right axis determination during embryonic development (1). The incidence is 1 in 5,000-7,000 live births and factors that worsen prognosis include increasing A/V valve regurgitation, elevated pulmonary vascular resistance, and impaired ventricular function (2). Cardiac manifestations involve meso or dextrocardia, atrioventricular discordance, single ventricle physiology, AV septal defects, AV valve regurgitation, hypoplastic sinus node, congenital AV block, partial anomalous pulmonary venous drainage, and pulmonary stenosis or atresia. Extra-cardiac characteristics include symmetrically lobed lungs and bronchi, asplenia or polysplenia, malrotation of the intestine, midline liver, and bronchial cilia dysfunction.
Most patients with severe mitral stenosis are delivered via C/S using epidural anesthesia because of its hemodynamic stability (3,4). We chose a CSE using a reduced dose of intrathecal bupivacaine to minimize maternal hemodynamic fluctuations, provide intense motor/sensory block, and to allow an extended duration of action. We recovered our patient in the ICU, carefully monitoring cardiac function, volume status, and postoperative pain, instituting nasal CPAP to minimize airway obstruction.
REFERENCES: 1. Circulation Journal 76, 2012: 2066-075 2. Eur J Cardiothorac Surg, 2010; 38:721-727 3. J Cardiothoracic Vasc Anesth, 2010 Dec 24(6): 1022-3 4. Reg Anesth Pain Med, 2004 Nov-Dec; 29(6): 610-5