Myocardial infarction in a patient with hereditary haemorrhagic telangiectasia in a remote location

Abstract

Context: Prompt reperfusion therapy in acute ST-segment elevation myocardial infarction (STEMI) improves outcomes and is therefore a key part of the management of affected patients. Primary percutaneous coronary intervention (PPCI) is preferred over thrombolysis; however, when PPCI is not available within an acceptable time frame, thrombolysis should be administered without delay. When thrombolysis is contraindicated, this can lead to delays in achieving coronary artery reperfusion, and making therapeutic decisions is more challenging. Issues: A 57-year-old woman with hereditary haemorrhagic telangiectasia (HHT) presented with chest pain to local medical services on a Scottish island. Initial assessment including 12-lead ECG was performed without delay and led to a diagnosis of infero-lateral STEMI. Initial treatment was with antiplatelets, nitrate and opiate analgesia. Immediate reperfusion therapy with thrombolysis was considered; however, this was contraindicated due to the diagnosis of HHT. Following discussion with the regional centre she was treated with heparin and urgently transferred to the nearest catheterisation laboratory for PPCI. Unfortunately delays in transfer were caused by inclement weather conditions, which precluded the use of an air ambulance. The patient reached a cardiac catheterisation laboratory by road. A total occlusion of a distal branch of the circumflex artery was found to be the culprit lesion and despite wiring and ballooning good blood flow was not restored. However, she made a good recovery and was discharged home on secondary prevention therapy, which was modified to take into account her increased bleeding risk associated with her pre-existing HHT. Lessons learned: In this case, the patient presented early to medical services following the onset of symptoms and was immediately assessed, resulting in a prompt accurate diagnosis at first medical contact. Despite this, the presence of HHT, and the particularities of living in a remote area compounded by unfavourable weather conditions, resulted in a delay in definitive treatment that would have been available to a similar patient presenting in an urban setting. In remote regions where reperfusion therapy for acute STEMI relies more often on thrombolysis, an increased awareness of the issues around the contraindication of thrombolysis, together with early discussion with a regional cardiology service leads to the most efficient way of establishing the best treatment plan for individual patients. Despite this, rural patients may remain at a disadvantage.