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Safe delivery of a preterm infant in a parturient with end stage pulmonary fibrosis and pulmonary hypertension
Abstract Number: 34
Abstract Type: Case Report/Case Series
A 28 year old multi-gravid lady (para 3+0) presented to the Emergency Room at 26 weeks gestation. She was tachypnoeic and breathless, and oxygen saturations measured 71%. Arterial Blood Gas sampling revealed a pO2 of 5.1kPa, and a pCO2 of 3.9kPa. Her past history was significant for severe idiopathic pulmonary fibrosis. She had been assessed for lung transplant, fitted the criteria, but had not yet received a transplant. She had developed secondary pulmonary hypertension with a mean Pulmonary Artery Pressure(PAP) of 35mmHg prior to admission.
Initial therapy was with oxygen and her functional status gradually improved. Investigations revealed the cause of her acute deterioration to be a mild Lower Respiratory Tract infection. Once stabilised, her delivery was planned by a multidisciplinary team with the goal to get to 30 weeks fetal gestation.
Repeat echocardiogram showed stable pulmonary hypertension at 37mmHg mean PAP. The patient continued to remain oxygen dependant, with desaturations to 75% on walking.
A non-invasive circuit capable of delivering Nitric Oxide was arranged for the perioperative period, aiming to avoid intubation and provide pulmonary vasodilatation if required.
She had another mild respiratory deterioration at 30+1 weeks. The decision was taken to deliver the baby by Caesarean Section.
An arterial line was placed. An epidural was sited and carefully established with a total of 10mls of 5mg/ml L-bupivicaine. It was gradually topped up with 2% lignocaine to provide surgical anaesthesia. Minimal amounts of phenylephrine were used for maintenance of mean arterial pressure > 65mmHg, while limiting further pulmonary vasoconstriction. The patient tolerated the delivery, with the aid of high flow oxygen, and a well 1.4kg infant was safely delivered. She was subsequently monitored in the intensive care unit(ICU) where she recovered well.
The patient returned to the ICU 6 weeks later with a further respiratory deterioration and severe hypoxia. Despite maximal therapy, including 6 days spent on an oscillator, she expired from her pulmonary fibrosis.
Careful planning and management enabled the safe delivery of a healthy infant in a multigravid mother with end stage idiopathic pulmonary fibrosis and secondary pulmonary hypertension, who died soon after from her primary pathology.