Pregnancy In Pulmonary Arterial Hypertension Research Articles

Maternal and Fetal Outcomes in Pregnant Women with Pulmonary Arterial Hypertension: A Single-Center Experience and Review of Current Literature.

Although pregnancy in women with pulmonary arterial hypertension has been considered a high-risk condition, current data regarding pregnancy with pulmonary arterial hypertension are scarce. In this study, we aimed to evaluate our single-center data on maternal and fetal outcomes in pregnant women with PAH and review currently available risk-based management strategies. Our single-center study group comprised 35 women who became pregnant after the diagnosis of pulmonary arterial hypertension or in whom pulmonary arterial hypertension was diagnosed within early post-partum period. Clinical, laboratory, echocardiographic, and hemodynamic characteristics of pregnant and non-pregnant productive women with pulmonary arterial hypertension were compared, and similar comparison was also repeated for survivors and non-survivors in pregnant patient group. Pregnancy was noted in 15% of the 228 females with pulmonary arterial hypertension who were of hormonally productive ages, generally well-tolerated until delivery. Elective abortion and pre-term delivery were documented in 1 (2.8%) and 12 (35.3%) pregnant women, respectively. Switching to sildenafil was the standard medication during pregnancy. Cesarian section was the preferred method of delivery in all pregnant women with pulmonary arterial hypertension and was performed without any complication. Clinic deteoriation within the first week of delivery was observed in 5 (41.6%) patients. Maternal mortality was noted in 13 (37.1%) patients and was documented to cumulate within the first month of delivery. However, any sign predicting post-partum clinical deterioration was not found. No fetal mortality was observed. Despite the development of advanced therapies, pregnancy in pulmonary arterial hypertension still carries a high mortality risk and requires multi-disciplinary expert center care with more proactive management strategies.

Pregnancy in Pulmonary Arterial Hypertension: A Multidisciplinary Approach

Pulmonary arterial hypertension (PAH), a female predominant disease, carries a high maternal and fetal mortality in pregnancy despite improved insight and the development of novel therapies. The high risk is attributed to the adaptive changes that take place to promote healthy fetal development during pregnancy, which can adversely affect the already compromised right ventricle in patients with PAH. While in the prior era emphasis was placed on termination of pregnancy, here we will illustrate through a multidisciplinary approach and meticulous planning at an expert center, these high-risk women can undergo successful childbirth.

Pregnancy and pulmonary arterial hypertension: a case series and literature review

Despite the development of advanced therapies for pulmonary arterial hypertension, pregnancy remains contraindicated in these patients owing to high maternal and fetal morbidity and mortality. Limited data exist regarding pregnancy management and outcome in this unique patient population. We describe a series of pregnant patients diagnosed as having pulmonary arterial hypertension before or during pregnancy who delivered at a tertiary center with a comprehensive and established pulmonary vascular disease program. This study aimed to describe a single institution's experience and review the existing literature for pregnancy management and outcomes in patients with pulmonary arterial hypertension. A review of all patients with pulmonary arterial hypertension who were admitted for delivery between 2005 and 2019 at our institution was performed. All data were extracted from the electronic health record and included patient demographics, pulmonary arterial hypertension subtype, pulmonary arterial hypertension-targeted therapies, and mode of delivery and anesthesia. A total of 7 patients were identified; 5 patients had a prepartum diagnosis of pulmonary arterial hypertension, whereas 2 patients were diagnosed as having pulmonary arterial hypertension during the third trimester. All patients were started on prostacyclins and the majority were on combination pulmonary arterial hypertension-targeted therapy. The maternal mortality rate was 29%. Elective cesarean delivery was performed in more than 70% of cases, whereas 1 patient required an urgent cesarean delivery and 1 patient had a successful vaginal delivery. Most patients had epidural anesthesia. Notably, 2 patients required extracorporeal membrane oxygenation after delivery and both died. There were no cases of neonatal mortality. Our cases series and the published literature to date show that pregnancy in pulmonary arterial hypertension remains poorly tolerated despite marked advancements in pulmonary arterial hypertension-targeted therapies and postpartum care. A multidisciplinary team approach remains essential for the management of these patients.

Pregnancy in pulmonary arterial hypertension: Midterm outcomes of mothers and offspring

Abstract Current guidelines recommend that patients with pulmonary arterial hypertension (PAH) avoid pregnancy because of a high mortality risk for the patients and their offspring. Still, some PAH patients become pregnant. We herein report the mid-term maternal and offspring outcomes of 25 consecutive pregnancies in 16 patients with PAH. Five patients had a total of 8 abortions (5 spontaneous, 3 induced; one of these patients developed right ventricular failure and underwent urgent lung transplantation). Thirteen patients delivered a total of 18 healthy children. One of these patients developed right heart failure in the postpartum period, requiring urgent lung transplantation. All other patients did well and were alive after a follow-up period of 1-12 (median, 6) years. Their infants were also doing well, and all had normal results on a standardized infant developmental questionnaire. These data suggest that nowadays, pregnancy can be associated with good outcomes in selected patients with PAH.

Pregnancy in pulmonary arterial hypertension.

Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who become pregnant. Early termination should be discussed. Women who choose to continue with their pregnancy should be treated at specialised pulmonary hypertension centres with experience in managing PAH during and after pregnancy.

Pregnancy in pulmonary arterial hypertension

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.

Pulmonary arterial hypertension in pregnant women

Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling that limits the ability of the pulmonary vascular bed to withstand the physiological changes of pregnancy. Historically, pregnancy in PAH carries a high risk to the parturient. Normal pulmonary vasculature can withstand the hemodynamic and physiological changes associated with pregnancy without the development of respiratory symptomatology. However, in the presence of pulmonary vascular remodeling the capacity to handle these changes is compromised. During pregnancy, increase in cardiac output from the increased intravascular volume can lead to right heart failure. Therefore, all patients with PAH of childbearing potential should receive preconception counseling and be advised to use two methods of contraception. Patients with PAH should be advised against continuing pregnancy if they do become pregnant. According to the literature, deterioration in pregnancy mainly occurs in the second trimester and early in the third trimester; immediately postpartum is the most critical time for patients with PAH. In this review, we will discuss the recent advances in the management of parturient patients with PAH.

Pregnancy outcomes in pulmonary arterial hypertension in the modern management era

Previous studies have reported mortality rates of up to 56% associated with pregnancy in pulmonary arterial hypertension (PAH) but the management of this disease has changed considerably in recent years. We compiled a multinational, prospective registry to examine the contemporary outcome of pregnancies in patients with PAH. During a 3-yr period, the 13 participating centres reported 26 pregnancies. Three (12%) females died and one (4%) developed right heart failure requiring urgent heart-lung transplantation. There were eight abortions; two spontaneous and six induced. 16 (62%) pregnancies were successful, i.e. the females delivered healthy babies without complications. These females had well controlled PAH (pulmonary vascular resistance (PVR) 500 ± 352 dyn·s·cm(-5)); eight of them were long-term responders to calcium channel blockers. In contrast, the females who died or required transplantation had poorly controlled PAH (PVR 1,667 ± 209 dyn·s·cm(-5)). Pregnancy remains associated with a substantial mortality rate in PAH. However, our results indicate that the outcome of pregnancy in PAH has improved, at least when PAH is well controlled, and particularly in long-term responders to calcium channel blockers. These data must be confirmed by larger series before the general recommendation to avoid pregnancy in all patients with PAH is reconsidered.

Successful pregnancy in pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report

IntroductionPulmonary arterial hypertension is a complication of systemic lupus erythematosus. Mortality in pregnant patients with pulmonary arterial hypertension related to connective tissue disease is as high as 56%. The authors report the first case of a successful maternal-fetal outcome in a pregnant patient with systemic lupus erythematosus-associated pulmonary arterial hypertension treated with sildenafil and inhaled iloprost during pregnancy and until several weeks after caesarean section.Case presentationThe case presented is of a 29-year-old woman with systemic lupus erythematosus and associated severe pulmonary arterial hypertension. Vasodilator therapy with bosentan and sildenafil, immunosuppressive therapy with prednisone, hydroxychloroquine and azathioprine and oral anticoagulation (phenprocoumon) had normalized her right ventricular over right atrial pressure when she was diagnosed in her 5th week of pregnancy. The teratogenic drugs bosentan and phenprocoumon were stopped, the latter replaced by low molecular weight heparin. During the 35th week, a slight increase in pulmonary pressure was found. Therapy with inhaled iloprost was established. A caesarean section was performed in the 37th week and a healthy baby was delivered. The patient remained stable until 11 weeks after delivery, when an increase in right ventricular over right atrial pressure was noted. Bosentan was reintroduced and prednisone and azathioprine doses were increased. The patient has remained stable until the present time.ConclusionPulmonary arterial hypertension has been considered a contraindication for pregnancy. Novel vasodilator therapy, combined with immunosuppressants in this patient with systemic lupus erythematosus, may "cure" pulmonary arterial hypertension and permit pregnancy with successful outcome. However, postpartum exacerbation of systemic lupus erythematosus and pulmonary arterial hypertension have to be considered.