Pulmonary arterial hypertension and pregnancy
A previously healthy, 30-year-old primiparous pregnant woman with progressive respiratory distress and oxygen need was diagnosed to have PAH in her country of origin at her 24th week of gestation. She was counseled to terminate the pregnancy due to her grave disease, but refused the termination. She applied to our center and was hospitalized in cardiac intensive care unit (Ege University Hospital, Izmir, Turkey, 2011) as a 24 weeks pregnant woman with PAH. Echocardiographic examination showed the findings of severe pulmonary hypertension: enlarged right ventricle (45 mm) and D-shaped left ventricle due to increased pressure of the right side. Right ventricular ejection fraction was calculated as 45% and severe tricuspid failure with a tricuspid regurgitant flow velocity of 4.4 m/ sec (normal range: 2.8 m/sec) was detected. Pulmonary artery systolic pressure was calculated as 80 mm Hg (normal range: 25–30 mm Hg). Cardiac catheterization showed a mean pulmonary arterial pressure of 50 mm Hg (normal range: 15–20 mm Hg), pulmonary wedge pressure of 9 mm Hg (normal range: 8–12 mm Hg), pulmonary vascular resistance of 880 dynes-sec/cm5 (11 Wood Units) (normal range: 100–250 dynes-sec/cm5), and cardiac output of 3.7 L/ min (normal range: 5–7 L/ min). Additional laboratory work-up for the differential diagnosis excluded the causes other than idiopathic pulmonary hypertension, including cardiac disorders, lung disease, thyroid disease, human immunodeficiency virus, thromboembolic disorders, connective tissue diseases, and deep venous thrombosis, as described in 2009 guidelines for PAH.[] The patient was in functional class 3 according to New York Heart Association classification, which may correspond to 2.5 years of median survival expectation.[] She was once again counseled about the severity of her illness and the poor prognosis, but she refused the termination of the pregnancy. The treatment of the patient was then programmed in a multidisciplinary approach by a team of cardiologists, obstetricians, pulmonologists, and neonatologists. Treatment with inhaled iloprost (10–20 mcg 7–9 times daily) and oral sildenafil (10–20 mg three times a day) was simultaneously started in lowest doses and gradually increased to target the maximum tolerated dose carefully because of the risk of lowering the cardiac output. Patient was kept in bed rest in left lateral decubitus position. Anticoagulant therapy with low molecular weight heparin was started for thromboprophylaxis. Arterial oxygen saturation was around 80% despite oxygen treatment via nasal cannula. Delivery was planned for the 31st–32nd week because the patient was decompensating. Fetal well-being was pregnancy evaluated with biophysic scoring and sonographic fetal biometry. Inhaled iloprost was replaced with intravenous iloprost infusion (2 ng/kg/min) 5 days before delivery because of increased oxygen needs and as a prophylaxis for the very high risk peripartum period. Antenatal betamethasone therapy was given for the lung development of the infant.\nAt 31 weeks + 6th day of pregnancy, she delivered a male infant with good Apgar scores of 8 in the first minute and 9 in the 5th minute by Cesarean section under epidural anesthesia. Birth weight was 1700 g (75–90P), birth height was 41 cm (25–50P), and head circumference was 30 cm (75–90P). Clinical findings were normal and he had a sinusal tachycardia and mild respiratory distress syndrome with oxygen need. Echocardiography, cranial and abdominal ultrasounds were normal. Formula feeding was initiated because of the mother's poor health conditions and the ongoing treatment agents with unknown effects on breastfed infant. At 18th day of life, the infant was having full feeds enterally.\nOxygen needs of the infant around 30% of fractional inspired concentration continued up to 36th week despite negative X-ray findings. Anemia and infectious findings which might explain this oxygen need were negative clinically and on blood tests. The infant was then diagnosed of having mild bronchopulmonary dysplasia (BPD) and a short duration of dexamethasone therapy helped to resolve the oxygen requirement and the infant was weaned to room air. The infant was discharged home at the 36th day of life with a weight of 2360 g. The mother was followed in cardiac intensive care unit and required oxygen of 4 L/min and pulmonary vasodilator therapy with the same dose of inhaled iloprost and oral sildenafil as in pregnancy. She was discharged home at 2 months of delivery with oxygen and inhaled iloprost treatment. She was advised regarding contraception; however, no sterilization procedure was performed during the Cesarean operation due to her refusal. At the time of writing the manuscript, she was alive for 10 months after delivery with the same echocardiographic findings under the same vasodilator treatment and oral anticoagulation with warfarine sodium.