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Anesthetic Management of a Parturient with Heterotaxy Syndrome: A Case Report
Abstract Number: F 72
Abstract Type: Case Report/Case Series
INTRODUCTION: Heterotaxy syndrome (HS) is infrequently found in adults, especially in the pregnant population. It is a congenital disorder caused by failed embryonic development of normal left-right asymmetry and is associated with a wide range of cardiac and extracardiac congenital anomalies in the gastrointestinal and bronchopulmonary systems, the axial skeletal and the CNS (1). Pregnancy outcomes in women with heterotaxy syndrome have not been reported (2), but require a multidisciplinary approach.
CASE: A 25-year-old primigravida at 34 weeks EGA presented for anesthetic evaluation. Her medical history was significant for HS characterized by right pulmonary agenesis, tracheal defect requiring patch repair as a neonate and tracheostomy from age 2 months to 2 years old. Other abnormalities included dextrocardia, asplenia, deafness, and microgastria with recurrent aspiration pneumonia. Her most recent episode of pneumonia was 3 days prior to admission. Echocardiography demonstrated an EF of 70% with mild tricuspid regurgitation and mild pulmonary hypertension. PFTs indicated FEV1 at 26% of predicated, and FVC at 47%. Chest x-ray revealed complete opacification of the right hemithorax with compensatory hyperinflation of the left lung and mild right tracheal deviation. She was a thin frail female at 145cm (4’7”) and 41.4kg with a marked kyphoscoliosis.
A multidisciplinary approach to our patient’s care was developed by Anesthesiology, OB and social services. Given her history of tracheomalacia and prior tracheostomy, ENT was consulted for lower airway evaluation. The risks and benefits of vaginal delivery vs C/S and GA vs neuraxial analgesia were discussed with her; vaginal delivery under epidural was chosen. An epidural was placed at 5cm cervical dilation without difficulty. The epidural was initiated and maintained with a reduced amount of local anesthetic due to her short stature, but a second epidural was necessary because of intravascular migration. She had an uncomplicated SVD of a baby boy without obvious anomalies. She required oxygen after delivery but did not again develop pneumonia .
DISCUSSION: Our patient’s history of recurrent pulmonary aspiration, right lung agenesis, prior tracheostomy and mild pulmonary hypertension predisposed her to airway difficulties and pulmonary complications if C/S under GA became necessary (short stature, small unproven pelvis). Early epidural placement was essential to ensure time for troubleshooting (3) in an effort to avoid GA. Our understanding of her cardiac, pulmonary and skeletal defects along with an informed delivery plan led to a favorable outcome.
1. J of Cardiothoracic and Vascular Anesthesia 2010; 24:834-844.
2. Diagnosis and Management of Adult Congenital Heart Disease, 2nd ed. Gatzoulis MA, et al editors. Saunders, 2011.
3. Anesth Analg 2009;109(6):1930-4.