Black Oesophagus as the Terminal Event in Severe Pulmonary Arterial Hypertension

Abstract

We present the case of a 74-year-old nurse practitioner, who had suffered from breathlessness since December 2014, and was diagnosed with idiopathic pulmonary arterial hypertension in May 2016. Right heart catheterisation in August 2016 showed a right atrial pressure of 12 mmHg, a main pulmonary artery pressure of 51 mmHg, and a pulmonary vascular resistance of 9.0 Wood units. She was treated with tadalafil (30 mg, Septenber 2016) and ambrisentan (10 mg, October 2016), and iloprost added (April 2017). Iloprost was switched to an intravenous infusion of epoprostenol in October 2017, (peak dose 26 ng/kg/min) and continuous home oxygen. Pertussis bronchitis in July 2017 was accompanied by a reactive pleural effusion and heralded clinical deterioration with subsequent pleural effusions (transudates) requiring drainage of 1.5–2 L per week on 10 occasions. Her final admission was precipitated by a fall. She was found to be in worse right heart failure, despite taking heart failure drugs. She was treated by diuresis, paracentesis, pleurocentesis, and dobutamine for hypotension. At day 4 of treatment she reported odynophagia, her haemoglobin fell from 166 g/L to 47 g/L, and melena became manifest (despite long-term pantoprazole). Urgent gastroscopy showed black oesophagus (necrosis of distal third of the oesophagus), and diffuse duodenal bleeding and haemostasis was unable to be achieved. She died in palliative care 3 days later. Autopsy confirmed the diagnosis of idiopathic pulmonary arterial hypertension, with right ventricular hypertrophy and dilatation and ulceration of the distal oesophagus (with black slough) and duodenal ulceration. Black oesophagus is rare, and has not been reported previously in pulmonary arterial hypertension or in patients on multiple therapies for pulmonary arterial hypertension. It, nevertheless, was the terminal event in this patient with idiopathic pulmonary arterial hypertension.