Coral reef aorta: a systematic review and proposal for a novel anatomical classification

BACKGROUND: Coral reef aorta (CRA) is a rare condition characterized by exophytic, heavily calcified aortic stenoses. Limited evidence is available concerning epidemiology, pathophysiology and management. This study aims to consolidate current knowledge on CRA and to propose a first classification system. METHODS: A wide systematic literature review was conducted according to PRISMA guidelines including all original reports of lesions consistent with CRA features. Extracted data included demographics, lesion location, clinical presentation, treatment techniques and outcomes. RESULTS: A total of 368 patients from 141 publications were included (133 case reports and 8 retrospective cohort studies). Women represented 64.6% and were older at diagnosis than men (62.2 vs. 55.1 years, P1). Lesion extent was analyzed in 164 patients, allowing the proposal of a new classification system distinguishing thoracic (type I, 31.7%), paravisceral (type II, 36.0%), and infrarenal (type III, 32.3%) CRA, as well as their subtypes. CONCLUSIONS: CRA has distinct epidemiological features that raise questions about its pathophysiological origin. While open surgery remains the mainstay of treatment, endovascular repair appears to offer better short-term outcomes, although long-term data remain scarce. The proposed classification provides a framework for standardized reporting and future research.