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Use of Neuraxial Anesthesia and Epoprostenol for Cesarean Delivery for a Patient with CREST Syndrome and Pulmonary Hypertension
Abstract Number: T 40
Abstract Type: Case Report/Case Series
A 26 year old G2P0101 presented for cesarean delivery due to increasing shortness of breath and orthopnea associated with CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, telangiectasia) diagnosed in the second trimester.
Pulmonary function tests showed a mild restrictive ventilatory defect and severely reduced diffusion capacity. CT scan showed diffuse esophageal dilatation. Cardiac echocardiography revealed a moderately to severely dilated right ventricle, moderate pulmonary hypertension with right ventricular systolic pressure (RVSP) of 50-60 mmHg, and an ejection fraction of 50%. Right heart catheterization showed an RVSP of 52 mmHg, PAP 53/25 mmHg and mean PAP 37 mmHg. The trans-pulmonary gradient and pulmonary vascular resistance were significantly elevated, and cardiac output significantly decreased (4.2 L/min). Because the right ventricle showed poor function on echocardiography, there was concern for right ventricular failure immediately after delivery with the increased preload from autotransfusion. Five days prior to surgery, a Swan-Ganz catheter was placed and epoprostenol was titrated to a low dose of 3.92 ng/kg/min to improve pulmonary pressures and cardiac output.
Low-dose combined spinal-epidural anesthesia was elected to avoid intubating a potentially friable airway, and to avoid large changes in pulmonary pressures during induction and intubation. TEE monitoring was not considered due to risks associated with placing the probe in a patient with esophageal dilatation. Cesarean delivery was performed without maternal or neonatal complications. Pulmonary pressures remained stable throughout the procedure without changing the epoprostenol rate.
Maternal mortality from pulmonary arterial hypertension remains at 30-50%(1), primarily due to right ventricular dysfunction secondary to increases in pulmonary pressures and pulmonary vascular resistance following delivery. Currently there is no standardized care for these patients. However, in recent case reports, the use of epoprostenol in the peripartum period have been shown to improve maternal outcome with no adverse fetal effects(2). In addition, the patients with pulmonary hypertension associated with CREST syndrome may have pulmonary fibrosis, esophageal dilation, automonic neuropathy, and Raynaud’s phenomenon(3). Airway challenges include restricted mouth opening and neck extension due to dermal fibrosis, and potential airway bleeding from telangiectasias(3).
1.Bonnin M. Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology 2005;102:1133-7.
2.Goland S et al. Favorable outcome of pregnancy with an elective use of epoprostenol and sildenafil in women with severe pulmonary hypertension. Cardiology 2010;115:205-8.
3.Dempsey ZA et al. The role of regional and neuroaxial anesthesia in patients with systemic sclerosis. Local Reg Anesth. 2011;4:47-56