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Labor analgesia for a patient with combined congenital macrothrombocytopenia and Ebstein’s anomaly
Abstract Number: T4C-8
Abstract Type: Case Report/Case Series
The peripartum management of women with pre-existing hematologic and cardiac disease present unique challenges to obstetric anesthesiologists. We present the anesthetic management of a woman intending vaginal delivery who had significant thrombocytopenia due to congenital macrothrombocytopenia (CMTP) and Ebstein’s anomaly (EA).
A 29 yr-old G7P0 with history of 6 prior miscarriages, CMTP, and corrected EA was referred to the high-risk obstetric anesthesia clinic at 18 weeks’ gestation. She had undergone multiple tricuspid valve replacements, aortic coarctation repair, and a ventricular septal defect (VSD) repair for EA. Due to severe arrhythmia burden from EA and subsequent ablation, she was also pacemaker-dependent. She had been stable from a cardiac perspective with echo showing mildly decreased biventricular function, mild RV enlargement, and a persistent VSD. The patient also had a lifelong history of thrombocytopenia (PLT count range: 25-60x10^9/L) and no history of significant bleeding after multiple cardiac surgeries. In 2017, a gene panel for platelet disorders revealed a definitive diagnosis of CMTP from a mutation in the actin 1 gene. A multidisciplinary input from MFM, cardiology, hematology, and anesthesia was needed for her delivery plan. The patient had no absolute cardiac or obstetric contraindications to vaginal delivery. Given her complex cardiac comorbidity, early neuraxial labor analgesia was a key consideration. However, due to thrombocytopenia, platelet transfusion was recommended prior to potential epidural placement.
Labor was induced at 37 weeks; her PLT count was 41x10^9/L and TEG parameters were normal (MA=63.1 mm and α angle=60.4 degrees). IV opioid analgesia was initially used, but because of breakthrough pain and concern about severe dyspnea during contractions, an epidural was advised. Prior to epidural placement, 1 unit PLT was transfused and successful labor epidural analgesia was achieved. She underwent spontaneous vaginal delivery. Her epidural was removed on PPD1 after an additional 1 unit PLT transfusion. She was discharged without any significant postpartum complications.
The obstetric anesthetic management of patients with corrected EA generally involves telemetry monitoring, early neuraxial analgesia to prevent sympathetic surges from pain, and careful attention to fluid balance. For our patient, management was further complicated by significant thrombocytopenia due to CMTP. There is no consensus on an acceptable PLT count to safely perform neuraxial techniques.1 Most guidelines recommend a PLT threshold above 70-80x10^9/L for epidural placement to limit the possible risk of epidural hematoma.2 Our decision to transfuse PLT and perform epidural analgesia was influenced by her lack of significant bleeding events, reassuring baseline TEG indices, and her pre-existing cardiac condition.
Obstet Gynecol. 2016;128:e43–53.