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Respiratory Decompensation in a Parturient with Hamman-Rich Syndrome
Abstract Number: T-15
Abstract Type: Case Report/Case Series
Introduction: Anesthetic management of a parturient with restrictive lung disease can be challenging when faced with the cardiopulmonary changes of pregnancy. We present a rare case of respiratory decompensation in a parturient with Hamman-Rich Syndrome, involving worsening pulmonary edema and hypoxia in the setting of baseline pulmonary fibrosis.
Case Presentation: A 42 year old G2P0 female with history of gestational hypertension and Hamman-Rich Syndrome was initially seen in a consultation visit. She was requiring 3L/min oxygen during pregnancy, increased from her nocturnal 2 L/min baseline and using a scheduled ipratropium inhaler. With advancing gestation she required a wheelchair secondary to increasing dyspnea. Recent PFTs showed moderate to severe restrictive ventilatory defect with FEV=48(% predicted); FEV1=48%; FEV1/FVC=101%; FEF25-75=42%; DLCO47%. Initial anesthetic plan was for early placement of an epidural with slow dosing upon onset of labor. The patient presented a week later at 35 weeks with acute worsening of hypoxia, non-productive cough, lower extremity edema and concern for preeclampsia. On admission oxygen requirements were increased to 6L/min and ABG showed a PaO2 of 61. Notably, she was unable to lay flat without having severe coughing spells with oxygen desaturations to the 80's. Chest X-ray confirmed worsening pulmonary edema and small pleural effusions. She required urgent cesarean delivery due to her worsening respiratory status as well as non-reassuring fetal heart tones. Due to her inability to lay supine, she was preoxygenated in an upright position and only after rapid sequence induction was the patient flattened and intubated. After transfer to the intensive care unit her oxygenation showed marked improvement with positive pressure ventilation, PEEP, and small incremental doses of furosemide. Echocardiogram exhibited moderate pulmonary hypertension with calculated RVSP of 55mmHg. Following extubation on postoperative day one her respiratory status continued to improve and she was discharged home four days later without further complications.
Discussion: Hamman-Rich syndrome is a rare form of diffuse lung injury resembling acute respiratory distress syndrome. Diagnosis is based on pathologic evidence of diffuse alveolar damage along with associated clinical findings. Treatment is supportive and prognosis is very poor despite aggressive treatment efforts (1). Management of patients requires close monitoring, planning and supplemental oxygen, diuretics and corticosteroids when appropriate. Physiologic changes in pregnancy including an increase in oxygen consumption, minute ventilation, intravascular volume, cardiac output and a decrease in lung volumes acutely worsened the already compromised lung function of this patient.
1. Avnon LS et al. Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations. Anesth Analg. 2009 Jan;108: 232-7