INTRODUCTION: Pulmonary arterial hypertension (PAH) in pregnancy carries high maternal mortality. In non-pregnant patients with PAH, inhaled treprostinil has been demonstrated to sustained effect on pulmonary vascular resistance with a longer duration of action. We report a successful maternal-fetal outcome in a pregnant woman with PAH treated with inhaled treprostinil.
CASE PRESENTATION: A 17 year-old female with Mixed Connective Tissue Disease presented to the emergency department with dyspnea at 33 weeks gestation. Medications included plaquenil, prednisone, and albuterol. Physical exam revealed a respiratory rate of 22 and an oxygen saturation of 91%. She had mild bibasilar crackles, regular heart tones, no peripheral edema, and a gravid uterus. Chest x-ray showed bilateral hilar prominence. Computed tomography of the chest showed enlargement of the pulmonary arteries. Transthoracic echocardiogram (TTE) showed normal left ventricular (LV) function, with mildly dilated right atrium (RA) and right ventricle (RV). Estimated systolic pulmonary artery (PA) pressure was 60 mmHg. She was admitted to the hospital, and a right heart catheterization with vasoreactivity was performed, revealing moderate fixed pulmonary hypertension, along with Class III symptoms. Treatment was initiated with diuretics and inhaled treprostinil. Her symptoms improved, and she was discharged home. She was re-admitted 12 days later in active labor at 36 weeks gestation. Inhaled nitric oxide and supplemental oxygen were initiated for desaturations during early labor. Inhaled treprostinil and furosemide were continued. TTE showed normal LV and RV function. Labor and delivery was uneventful, with birth of a healthy baby girl via vaginal forceps-assisted delivery. Postpartum, the inhaled nitric oxide and oxygen were quickly discontinued. She remained on furosemide and inhaled treprostinil, and was discharged on Postpartum Day 4. TTE at 3 months showed improved RA and RV size, normal RV systolic function, and an improved systolic PA pressure. At 6-month follow-up she had Class I symptoms, and six-minute walk distance (6MWD) had improved from 351 to 410 meters.
DISCUSSION: PAH is a devastating complication of Mixed Connective Tissue Disease. Normal pregnancy causes a wide range of physiologic and hemodynamic changes including volume overload. Patients with PAH have a limited ability to compensate for these changes. Maternal mortality in pregnant patients with severe PH has been reported as high as 56%. Management of PAH during pregnancy must evaluate possible toxic effects of medications on the mother and fetus. Aggressive diuresis is key to maintaining appropriate RV volume postpartum. There is a paucity of data regarding management of this patient population, and no reported cases using inhaled treprostinil, which was chosen for our patient due to its longer duration of action, and safer pregnancy category. Management of our patient was successful as evidenced by peripartum adherence to therapy, successful maternal-fetal outcome, improved RA and RV size, improved PA pressure, and improvement in 6MWD.
CONCLUSIONS: PAH in pregnancy carries high mortality, and there is limited data regarding management of this patient population. Our patient was successfully managed using inhaled treprostinil.
Reference #1 Bedard E, Dimopoulos K, Gatzouls MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30(3)256-265.
Reference #2 Voswinckel R, Enke B, Reichenberger F, et. al. Favorable effects of inhaled treprostinil in severe pulmonary hypertension. J Am Coll Cardiol 2006; 48:1672-1681.
Reference #3 Weiss BM, Zemp L, Seifert B, et. al. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998; 31: 1650-1657.
DISCLOSURE: The following authors have nothing to disclose: Carmel Goudzwaard, Rajive Tandon, Elaine Chen
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