Endocrine Abstracts

A 33-year-old lady reported a 6 months history of sweating and worsening palpitations especially after taking sotalol. She had a history of congenital heart disease (double inlet left ventricle, pulmonary valvular stenosis, ventricular septal defect, and Fontan connection surgery at 18 years); Blue Bleb Syndrome with chronic gastrointestinal blood loss, and recurrent pulmonary emboli. She required long-term warfarin treatment and regular blood transfusions. She was found to have new hypertension. Her cardiac performance and right ventricular ejection fraction (44%) had reduced which serves her systemic circulation. Her symptoms and modestly elevated urinary noradrenaline levels were initially felt to be in keeping with worsening intrinsic cardiac function (596–892 nmol/24 h; n=50–560). A clonidine suppression test and genetic screening were negative. However plasma normetanephrines were significantly elevated at 5415 and 5661 pg/ml (n<1180) and a MIBG with spect CT scan (meta-iodobenzylguanidine with single-photon emission computed tomography) demonstrated a hot spot at the left para-aortic region. She had a prolonged admission for withdrawal of sotalol and frusemide and introduction of alpha-blockade which she tolerated well. Inputs were sought from cardiac, endocrine and vascular surgery, anaesthetics, cardiology, haematology and gastroenterology. She proceeded to elective surgery which was uncomplicated apart from modest postoperative bleeding. Follow-up urinary catecholamines normalised and the patient’s presenting symptoms partially abated. This case illustrates the difficulty of diagnosing primary catecholamine excess in the setting of severe cardiac disease and also the success of the multidisciplinary approach for patients with multiple threatening co potentially life-morbidities.