Journal of Rare Cardiovascular Diseases

Pulmonary arterial hypertension (PAH) is a progressive disease leading to right ventricular failure and death, if not treated adequately. Pregnancy in women with PAH is associated with significantly high mortality, thus current guidelines strongly recommend, that pregnancy in PAH female patients should be avoided and in the case of pregnancy, termination at early stage is recommended. We present results of a study aimed at assesing maternal and newborn outcomes in gravidas with PAH. Five pregnant females with PAH in the mean age of 29.6 ±8.23 years were included in the study. In two patients idiopathic PAH (IPAH) and in three PAH due to congenital heart defects and Eisenmenger syndrome were diagnosed. Three patients received PAH-specific therapy (monotherapy) prior to pregnancy. During pregnancy two women received sildenafil, then treprostinil (one started in 33rd week of gestation, second few hours before labour). Two patients did not received PAH specific therapy due to non-availability in Poland at that time and one according to treatment denial. In one case spontaneous abortion at the 8th week of pregnancy occurred and one of the patients reported 4 previous miscarriages. Two patients are still alive, one is lost to follow-up and two of them died: one in the postpartum period and the second ca. 18 month after abortion. All newborns survived.Considering the high mortality rate among pregnant women with PAH, pregnancy is strongly contraindicated. If the patient decides to continue pregnancy she should be under medical care in a specialist centre with a multidisciplinary approach and PAH specific treatment should be administered.

rare disease; right ventricular failure; echocardiography; heart catheterization; congenital heart defect; Eisenmenger syndrome; medical therapy; caesarean section

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