The Complexities of Managing Acute Coronary Syndrome in a Patient With Hereditary Hemorrhagic Telangiectasia: A Case Report and Literature Review

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant genetic disorder characterized by mucocutaneous and gastrointestinal telangiectasias and visceral arteriovenous malformations (AVMs). Patients with HHT have an increased risk of both bleeding and arterial and venous thrombosis. Due to the increased risk of bleeding, these patients generally cannot tolerate antiplatelet or anticoagulant therapies. This poses a particular hurdle when treating acute coronary syndrome (ACS). Our case involves a 79-year-old male patient with a past medical history of HHT, major gastrointestinal bleeding (GIB), and coronary artery disease who presented with a non-ST-segment elevation myocardial infarction (NSTEMI) and acute-on-chronic anemia. Our treatment options were limited given his intolerance to antiplatelet and anticoagulant therapies which resulted in major GIB, making him a poor candidate for percutaneous coronary intervention. We consulted cardiology and treated him with beta-blockers to decrease oxygen demand, packed red blood cell (PRBC) transfusion to increase oxygen supply, and ranolazine for symptom relief. His symptoms improved and he was discharged. Four weeks later, he suffered a cardiac arrest due to ventricular fibrillation. His family chose to pursue comfort measures, and he was transferred to an inpatient hospice. By reporting this case, we aim to highlight the unique challenges faced when managing ACS in patients with HHT. We underscore the importance of mitigating the risks of coronary artery disease in these patients. Given the limited treatment options and low tolerance to treatment modalities used for ACS, early detection of HHT and implementing effective primary prevention strategies are crucial in these patients.