Conference: 2018 PHA International PH Conference & Scientific Sessions
Release Date: 06.28.2018
Presentation Type: Abstracts
File Download: Conference 2018_1064
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Abstract presented at the 2018 International PH Conference and Scientific Sessions in Orlando, Fla., June 28-July 1, 2018.
Our case illustrates a patient who was diagnosed with severe Methamphetamine Associated PAH at the 27th week of gestation, who was successfully treated with parenteral prostacyclin therapy culminating in a successful outcome.
Despite advanced therapies, maternal mortality in women with WHO Group I Pulmonary Arterial Hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. Early termination of pregnancy is generally recommended. There is scant data pertaining to the phenotype of Methamphetamine Associated PAH (MAPAH) and its related pregnancy outcomes.
38-year-old woman G5P4 at 28 weeks and 5 days gestation with past medical history of chronic methamphetamine use presented to outside hospital complaining of worsening dyspnea on exertion. Dyspnea preceded the pregnancy by 1 year, but symptoms worsened significantly as her pregnancy progressed, with dramatic reduction in exercise tolerance. Patient was referred and admitted to our institution to allow for urgent evaluation for Pulmonary Hypertension. Echocardiogram showed severely dilated right ventricle, septal flattening, moderately dilated right atrium, and right ventricular systolic pressure 80 mmHg. Right heart catheterization showed right atrial pressure 18 mmHg, pulmonary artery pressure 80/38 mmHg, mean pulmonary artery wedge pressure of 15 mmHg, cardiac output by Fick’s method of 6.3 L/min, cardiac index 3.15 L/min/m2, and pulmonary vascular resistance of 5.87 woods units. Patient was admitted to the Intensive Care Unit and started on intravenous Treprostinil. After multidisciplinary family meeting with Pulmonology, High Risk OB/GYN, Neonatology, ECMO team, and Cardiac Anesthesiology it was decided that patient would be managed expectantly with plans for a scheduled caesarian section at 31 weeks gestation. The planned C-section was uncomplicated, and results in the delivery of a baby girl who required minimal neonatal intensive care monitoring. After delivery, patient was transitioned to subcutaneous Treprostinil. Mother and baby were discharged home 11 days after delivery. Patient’s functional status has been steadily improving since discharge from hospital. Now 7 months after her pregnancy, she is WHO functional Class II on Subcutaneous Treprostinil.
Despite the detection of PAH late during her pregnancy, a methodical, systematic and multidisciplinary “teamwork” approach along with patient education resulted in a successful outcome in this young mother with MAPAH.