2001). continuation of therapy. strong class=”kwd-title” Keywords: Pregnancy, Pulmonary hypertension Case Presentation A 27-year-old woman with a history of moderate but well-controlled pulmonary hypertension was seen on October 12, 2009, by her pulmonologist for worsening symptoms of her disease. The results BTZ043 of a routine pregnancy test revealed she was seven-and-a-half weeks pregnant. The pulmonologist counseled that her safest course of action was to BTZ043 end the pregnancy, since in the best case, pregnancy with pulmonary hypertension carries a 10C15% risk of mortality for a pregnant woman trying to carry to term, and because of the severity of her disease, her own prospects were closer to 50% risk of death. The woman, a Catholic with four children, decided not to terminate. On November 3, 2009, the woman was admitted to St. Josephs Hospital and Medical Center with worsening symptoms. At this time, the woman was 11 weeks pregnant. A cardiac catheterization revealed that she now had very severe pulmonary arterial hypertension with profoundly reduced cardiac output; in another part of the record, a different physician confirmed severe, life-threatening pulmonary hypertension, right heart failure, and cardiogenic shock. The chart noted that she had been informed that her risk of mortality approaches 100%, is near 100%, and is close to 100% if she were to continue the pregnancy. BTZ043 The chart also noted that surgery is absolutely contraindicated. The Ethics Committee at St. Josephs Hospital and Medical Center was asked for a determination if a D&C would be morally appropriate according to Catholic teaching. Per their reading of the Ethical and Religious Directives for Catholic Health Care Services (4th edition) and their understanding of the Catholic moral tradition, the Ethics Committee determined that the intervention would not be considered a direct abortion. They therefore approved the intervention, which was carried out on November 5, 2009. Introduction Pulmonary arterial hypertension (PAH) has been reported as a rare but significant complication in pregnancy. Idiopathic pulmonary arterial hypertension (IPAH) is a rare condition that has a reported incidence of one or two cases per million per year (Rich et al. 1987). Several noted characteristics of the disease include: vaso-constriction, thrombosis, and vascular proliferation with remodeling. PAH may be idiopathic, as in SIRT6 this patient, or associated with collagen vascular disease, congenital heart disease, portal hypertension, HIV infection, the use of certain medications, and thyroid disorders (Rich et al. 1987). The diagnosis is made hemodynamically with a systolic pulmonary artery pressure 30 mm Hg or a mean pulmonary arterial pressure of 20 mm Hg (McMillan et al. 2002). Patients with severe IPAH have a previously reported 5-year survival of only 27% with supportive treatment, and a previously reported increased survival rate of 54% with targeted therapy (McMillan et al. 2002). The only known curative therapy for PAH is a heart-lung transplant. Pregnancy with PAH is a morbid condition and carries a serious prognosis. Patients with pre-existing PAH have a high-risk of maternal mortality (30C55%) (Weiss et al. 1998). A systematic review by Bedard, Dimopoulos, and Gatzoulis (2009), found a somewhat decreased overall mortality rate of 28%, and with idiopathic pulmonary hypertension, as in this patient, a lower (17%) mortality in their review. A review of the literature by Archer and Michelakis in 2006 found several classes of medications and.