Anaesthetic management of a parturient with hereditary haemorrhagic telangiectasia (HHT) and pulmonary haemorrhage requiring urgent caesarean section

Abstract

A 25-year-old gravida 3 para 3 with a history of hereditary haemorrhagic telangiectasia (HHT) and embolised pulmonary arteriovenous malformations (PAVMs) was admitted at 36 weeks gestation with haemoptysis, epistaxis and CT evidence of recent alveolar haemorrhage. An urgent caesarean section was planned. Both previous pregnancies had been delivered by elective lower segment caesarean section (LSCS) under subarachnoid block (SAB) at term. Preanaesthetic planning involved consultation with our tertiary maternity referral centre, the national HHT centre and our tertiary adult referral centre, which has interventional radiology and cardiothoracic capabilities. A whole spine MRI was carried out to rule out vascular malformation. Following multidisciplinary discussion, the decision was made to proceed with caesarean section in our hospital under SAB. Wide bore intravenous access was sited and blood product availability was ensured in case of acute pulmonary haemorrhage. The LSCS was uneventful. Postoperatively following discharge from the hospital, the patient experienced recurrent episodes of small volume haemoptysis, and had further PAVM embolisation in the national HHT centre. This report highlights the difficulties in managing complex parturients in a non-tertiary referral centre and underlines the importance of communication and multidisciplinary team discussion to determine the most appropriate management.