Pregnancy outcomes in patients with pulmonary arterial hypertension have not been documented in our local setting. In our institution, access to therapies like nitric oxide, inhaled prostacyclin and iloprost is limited. We describe two such women given sildenafil monotherapy between April and May 2011. Both had an atrial septal defect complicated by Eisenmenger syndrome. Both survived -one after elective Caesarean section for malpresentation (breech) under general anaesthesia, the other after an assisted vaginal delivery. In addition to oral sildenafil, both received oxygen supplementation. To date, there have been three reported cases of pulmonary arterial hypertension in pregnancy treated with sildenafil in combination with another drug. Our two cases demonstrate that treatment for pulmonary arterial hypertension using sildenafil as monotherapy may allow stabilization of the maternal condition and improve clinical outcomes for both mother and baby. However, pregnancy is still discouraged in women with pulmonary arterial hypertension.
Keywords
High-risk pregnancy, complications
Case reportsCase 1 I.D., a 33-year-old woman with known atrial septal defect (ASD) was admitted to our institution at 34 weeks' gestation in her second pregnancy following the onset of uterine contractions. She gave a history of cyanosis when crying during childhood. During her first pregnancy six years prior, she developed exertional dyspnoea and although she delivered at term, there was a neonatal death on day seven, allegedly due to congenital heart disease (CHD).In this second pregnancy she again described exertional dyspnoea from 16 weeks but the patient had not sought medical advice until the admission.On examination, there was digital clubbing, a grade 3/6 systolic murmur audible in the second to fourth intercostal space at the right parasternal border, a split second heart sound, with a palpable pulmonary component, a right ventricular heave and clear breath sounds. Oxygen saturation on room air ranged from 78% to 81%.Echocardiography confirmed a 1.3 cm ASD, with pulmonary artery pressure (PAP) 122 mmHg (Figure 1). Oxygen support via face mask at 10 l/min improved the saturation to between 81% and 89%. She was commenced on sildenafil 50 mg 8 hourly, together with nifedipine 10 mg every 4 h to inhibit the premature labour. The nifedipine was reduced after 48 h to 10 mg every 6 h and discontinued after 72 h upon cessation of uterine contractions. Four doses of dexamethasone 6 mg IM were administered to improve foetal lung maturity. She underwent elective lower segment Caesarean section under general anaesthesia for malpresentation (breech) as on the fourth hospital day delivering a live baby (1.6 kg) APGAR 9, 9, by elective. At that time bilateral tubal ligation was performed at operation. The patient was extubated after delivery. Oxygen saturations improved to between 89% and 92% on oxygen support 2-4 l/min via nasal cannulae. She was discharged on the 15th hospital day -although tolerating room air, she was advised to con...