Angina Pectoris or Myocardial Infarctions, Pulmonary Arteriovenous Malformations, Hereditary Hemorrhagic Telangiectasia, and Paradoxical Emboli

Abstract

Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder characterized by vascular malformations, and many clinical complications are related to pulmonary arteriovenous malformations (PAVMs) because they provide direct right-to-left shunts. Paradoxical emboli through these shunts are a well-recognized cause of transient ischemic attack, stroke, and cerebral abscess. The aim of this study was to determine whether paradoxical emboli through PAVMs could manifest as cardiac ischemic events. The study included a single-center population of 98 patients with PAVMs. Eighty-four had undergone PAVM embolotherapy, and the remaining 14 patients had PAVMs too small to require embolization. Patients were interviewed by telephone and surveyed regarding their cardiopulmonary symptoms and histories of cardiac diagnoses. We found that 6 patients (which is 18% of patients with symptomatic PAVMs, n = 33, and 6% of the total cohort, n = 98) reported that they had experienced typical angina pectoris-like chest pain or had a myocardial infarction before PAVM embolotherapy. Five patients had had a cardiac catheterization, 4 had normal coronary arteries, and 1 had a single artery occlusion. In conclusion, we suggest that in patients with untreated PAVMs, cardiac ischemia can occur because of a paradoxical embolus through PAVMs to a coronary artery.