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A High Pressure Delivery: Multispecialty Care for a Parturient with Primary Ciliary Dyskinesia
Abstract Number: F-21
Abstract Type: Case Report/Case Series
Primary ciliary dyskinesia (PCD) is a rare genetic condition resulting in poor ciliary function, leading to bronchiectasis and chronic lung disease. Associated parenchymal changes and decrease in nitric oxide (NO) may lead to pulmonary hypertension (PH). These conditions carry significant peripartum mortality risk, partly due to the unique physiologic changes of pregnancy.1
A 30 year-old G1P0 with PCD complicated by end-stage obstructive and restrictive lung disease and suspected PH presented for care. For several years, she required home O2 and multiple admissions for pneumonia. With pregnancy, her functional status deteriorated to dyspnea at rest. Chest CT prior to pregnancy showed diffuse bronchiectasis. PFTs obtained during 3rd trimester of pregnancy, were FEV1 25%, FVC 33%, and FEV1/FVC 0.77, comparable to pre-pregnancy values, which included DLCO 16%. TTE revealed mild PH with estimated RVSP 37-47mmHg. Right heart cath was aborted after iatrogenic pneumothorax. At 36 weeks GA, she presented with preterm contractions. She was not in labor but was admitted for monitoring and aggressive pulmonary toilet to prepare for labor induction. Due to concern for rapid decline in cardiopulmonary status during labor, she was transferred to the SICU with invasive BP and CVP monitoring. A lumbar epidural was placed and vasopressin was used to maintain MAP>60mmHg. In the event of pulmonary collapse or right ventricular failure, NO, milrinone, and ECMO were on standby. At 37 weeks GA, in the SICU, she had a successful vaginal delivery. She required diuresis during stage II of labor but remained stable. She stayed in the SICU for 5 days of postpartum monitoring and repeated doses of diuretics for fluid management.
Early pulmonary optimization, avoidance of fluid overload, and hemodynamic stability were emphasized in the care of this patient. To accomplish these goals, adequate neuraxial labor analgesia was critical to minimize the sympathetic response to pain while avoiding high sensorimotor block that could be detrimental for her pulmonary status. This helped avoid systemic opioids, which blunt airway reflexes and respiratory drive, and avoid general anesthesia and intubation in a patient who would likely do poorly with positive pressure ventilation and ventilator weaning. Vaginal delivery was recommended to minimize fluid shifts associated with C-section, the risk for intubation, and post-operative morbidities. Vasopressin was used due to its relatively favorable effects on pulmonary vasculature versus other vasopressors. General anesthesia and ECMO were available as part of contingency planning. Management of such a complex parturient requires coordinated multidisciplinary care to mitigate risks peripartum.2
1. Storm van’s Gravesande K, Omran H. Primary ciliary dyskinesia: clinical presentation, diagnosis, and genetics. Ann Med. 2005;37(6):439-49.
2. Obican SG, Cleary KL. Pulmonary arterial hypertension in pregnancy. Semin Perinatol. 2014;38(5):289-294.