Peripartum outcomes in a large population of women with pulmonary arterial hypertension associated with congenital heart disease

Abstract

Aims Pulmonary arterial hypertension is a severe complication in patients with congenital heart disease and poses a significant risk to women wishing to become pregnant. This study describes the clinical presentation, maternal outcomes and risk factors for the peripartum period in women with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD). Methods All pregnant women with PAH-CHD who were admitted for delivery in a tertiary center between February 2011–September 2016 were included. Logistic regression analysis was used to identify predictors of the combined endpoint of maternal death, severe heart failure requiring treatment, or pulmonary hypertensive crisis. Results Ninety-three women (94 pregnancies) were included. Average age was 27.5 ± 4.4 years. Thirty (31.9%) patients had Eisenmenger syndrome, 51 (54.3%) had pulmonary arterial hypertension associated with systemic-to-pulmonary shunts, and 13 (13.8%) had pulmonary arterial hypertension with corrected congenital heart disease. Twenty-three (24.5%) women required admission for delivery within two days from presentation. Elective Cesarean section was performed in 95.7% of women, with intravertebral anesthesia in 93.6%. Fifty-one (54.2%) patients received pulmonary arterial hypertension therapies during pregnancy. Six (6.4%) women died, 33 (35.1%) developed heart failure and 10 (10.6%) had a pulmonary hypertensive crisis. Patients who met the combined endpoint ( n = 34, 36.2%) were more likely to have Eisenmenger syndrome or repaired defects ( p < 0.001). Other risk factors in the multivariate model included lower arterial blood oxygen saturation, higher brain natriuretic peptide, and pericardial effusion on echocardiography. Conclusion Maternal mortality and morbidity remain high in PAH-CHD patients, who should be counseled on the risks of pregnancy and managed in a tertiary multidisciplinary environment to improve prognosis.

Pregnancy still contraindicated in pulmonary arterial hypertension related to congenital heart disease: True or false?

“…Independent predictive factors of the composite endpoint of maternal death, heart failure and pulmonary hypertension crisis were low arterial blood oxygen saturation, elevated brain natriuretic peptide levels and presence of pericardial effusion on echocardiography. 8 In general, their real-life data support that the mortality is not as high as in the past and PAH targeted treatment might have contributed to this decreased rate. However, mortality and morbidity still remain high.…”

“…1 In this issue of the European Journal of Preventive Cardiology, Li et al present the outcomes of pregnancy in 93 patients (94 pregnancies) with PAH-CHD who were admitted for delivery in a tertiary centre during a period of 5.5 years. 8 The study was single-centre retrospective in design. However, it included the largest cohort of pregnancies in this population until now and is the first that used the current clinical classification of PAH related to CHD according to European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension.…”

“…10,11 Notably, some of the patients were referred late in their pregnancies as emergencies in the tertiary centre. 8 In total, 6.4% of pregnant women died, 35.1% developed heart failure and 10.6% experienced a pulmonary hypertension crisis. Patients with left-to-right shunt had more favourable outcome compared with Eisenmenger patients and those with repaired defects.…”

“…10,11 Notably, some of the patients were referred late in their pregnancies as emergencies in the tertiary centre. 8…”

Síndrome de eisenmenger y embarazo – reporte de 2 casos

“…Un estudio prospectivo evalúo el efecto del sildenafil en pacientes con hipertensión pulmonar severa asociada a SE, después de 6 meses de tratamiento se evidencio recuperación en la capacidad funcional, mejoría hemodinámica pulmonar por reducción en la presión arterial pulmonar media, presión sistémica, adecuada tolerancia y escasos efectos adversos 24 . Li et al, reportaron que el 86.7% de las pacientes con SE, recibieron tratamiento con vasodilatador pulmonar, mejorando las condiciones clínicas de pacientes y obteniendo resultados materno fetales favorables 25 . De igual manera, Cartago et al encontró que la monoterapia con sildenafil contribuyó a mejorar la condición materna en la gestante con SE 26 .…”