PREGNANCY MANAGEMENT AND OUTCOME IN A WOMAN WITH CONGENITAL UNILATERAL LUNG AGENESIS: A CASE REPORT

Abstract

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Congenital unilateral lung agenesis is a rare condition characterized by the complete absence of a lung and the bronchovascular tree. It is often associated with cardiac and bronchopulmonary malformations, resulting in significant morbidity and mortality. Pregnancy, due to the physiologic changes during gestation and childbirth, poses additional risks to these patients. We report a case of a woman with congenital right lung agenesis (CRLA) whose successful pregnancy could offer guidance to clinicians in the interdisciplinary management of a high-risk condition (1). CASE PRESENTATION: A 26-year-old G1P0 was referred to our Maternal Fetal Medicine (MFM) clinic for her CRLA with resultant dextrocardia and left lung hyperinflation. She was asymptomatic and was able to tolerate daily activities without concerns. Pre-conceptional imaging studies showed results consistent with her medical history.There were no obstetric concerns with normal prenatal labs and normal fetal anatomy ultrasound. She was recommended to undergo cardiopulmonary evaluation and follow up with the relevant specialists.The patient remained clinically stable throughout the gestation. Delivery and postpartum planning was done in the early third trimester at a multidisciplinary conference with the MFM, Anesthesia, Cardiology, Pulmonology, Obstetrics, and Critical Care teams.The patient was electively induced at 39 weeks resulting in an uncomplicated vaginal delivery. Careful monitoring of the patient's hemodynamic status was performed during the intra- and postpartum periods due to the concern of volume overload from intravenous fluid infusions during and autotransfusion following delivery. Her postpartum course was uncomplicated. DISCUSSION: Several cardiopulmonary physiological changes occur in pregnancy to accommodate the growing fetus. An increase in cardiac output with a decrease in pulmonary and systemic vascular resistance leads to an increase in pre-load and increased pulmonary capillary permeability. The risk of pulmonary edema is highest in the second stage of labor due to the autotransfusion into the systemic circulation and the relief of pressure on the inferior vena cava (2). During pregnancy, there is a 40% increased oxygen requirement by the growing fetus, placenta, and maternal organs with a decreased reserve secondary to the increased consumption and decreased functional residual capacity. These changes are often the source of significant morbidity and mortality in women with compromised systems (3). For these reasons, a multidisciplinary care approach was of utmost importance for this patient. CONCLUSIONS: We advocate for the utility of a multidisciplinary approach with frequent follow-up and testing with each specialty to ensure the best care for a successful pregnancy outcome in this patient with CRLA. REFERENCE #1: Wert S. E. (2016). Normal and Abnormal Structural Development of the Lung. In Polin R. A., Abman S. H., Rowitch D. H., Benitz W. E., & Fox W. W., Fetal and Neonatal Physiology (pp. 627-641). Elsevier. https://doi.org/10.1016/B978-0-323-35214-7.00061-5 REFERENCE #2: Silversides C. K. & Colman J. M. (2007). Physiological changes in pregnancy. Oakley, C. & Warnes C. A. (2007). Heart Disease in Pregnancy (pp. 6-16). Blackwell Publishing Limited. REFERENCE #3: Gabbe, S. G., Niebyl, J. R., Simpson, J. L., Landon, M. B., Galan, H. L., Jauniaux, E. R., ... & Grobman, W. A. (2016). Obstetrics: normal and problem pregnancies e-book. Elsevier Health Sciences. DISCLOSURES: No relevant relationships by Kajal Angras, source=Web Response No relevant relationships by Kelly Ca, source=Web Response No relevant relationships by Cherise Hatch, source=Web Response No relevant relationships by VANI MOVVA, source=Web Response