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Double Outlet Right Ventricle Syndrome in an Obstetric Patient
Abstract Number: SAT-69
Abstract Type: Case Report/Case Series
Double Outlet Right Ventricle (DORV) characterizes a heterogeneous group of congenital malformations in which the pulmonary artery and aorta both arise from the right ventricle. Additionally, a ventricular septal defect (VSD) is almost always present. Classifying the subtype of DORV is essential to developing an appropriate anesthetic plan. Multiple classification systems exist, and they are broadly classified according to the location of the VSD in relation to the great arteries including: subaortic, subpulmonary, doubly committed, and non-committed. The Society of Thoracic Surgeons database further classifies these conditions into 5 subtypes according to the physiology they mimic: VSD Type, Tetralogy of Fallot (TOF) Type, Transposition of the Great Arteries (TGA) Type, Remote VSD Type, and VSD with Atrioventricular Septal Defect (ASVD) Type. Each subtype has a series of implications for an obstetric patient that would depend on the type of correction, possible presence of pulmonic outflow obstruction, pulmonary hypertension, subaortic stenosis and/or aortic arch outflow obstruction, and congestive heart failure. Thus, it is essential to investigate the subtype of DORV and the correction status to guide anesthetic management.
A 21yo G2P1 at 37 weeks gestation with corrected DORV of unknown subtype presented for labor epidural and planned vaginal delivery with a passive second stage. During the anesthesia interview, she revealed that she had a Blalock-Taussig shunt shortly after birth and a subsequent Rastelli Procedure for pulmonary hypertension. She had been asymptomatic during pregnancy and denied any functional limitations. Electrocardiogram at the time of admission showed normal sinus rhythm with mild right axis deviation. Oxygen saturations were 100% on room air and no increased work of breathing was appreciated. Further cardiology records were obtained, and an echocardiogram prior to pregnancy revealed normal left ventricular function with an ejection fraction of 55% and normal pulmonary artery pressures. A clinic visit record noted that she was believed to have TGA type DORV status post correction of the transposition. After further delineating the nature of her cardiac anatomy following the outlined corrections, it was determined that her physiology should mimic natural flow and would therefore be amenable to a slowly titrated epidural. Prior to placement of the epidural, a second large bore IV was started and a one liter normal saline bolus was administered. The patient’s epidural was placed without complication. Following a negative test dose, 0.25% Bupivacaine was administered slowly in 2mL doses for a total of 8mLs. No hemodynamic compromise was noted and a T10 sensory level was achieved. After 20 minutes, a continuous infusion epidural pump was started with 0.125% Bupivacaine with 2mcg/mL of Fentanyl at rate of 8cc/hr. The patient had an uncomplicated passive second stage delivery with adequate analgesia.