Cardiovasc Pathol. 2026 Oct 16:107866. doi: 10.1016/j.carpath.2026.107866. Online ahead of print.
ABSTRACT
BACKGROUND: Bicuspid aortic valve, the most common congenital valvular disease, frequently coexists with aortic pathologies. Symptomatic arrhythmias from unruptured giant aortic sinus aneurysm compressing the sinoatrial node are exceedingly rare. The coexistence of bicuspid aortic valve, arrhythmogenic sinus aneurysm, coarctation of the aorta, and right coronary artery occlusion represents an extraordinarily complex malformation with scarce documentation and controversial management strategies.
CASE PRESENTATION: A 67-year-old male patient presented with chest tightness and palpitations. Echocardiography and computed tomography angiography revealed Type 0 bicuspid aortic valve (two-sinus type) with giant unruptured non-coronary sinus aneurysm (52 mm × 48 mm), descending coarctation of the aorta (14 mm), post-stenotic aneurysm (42 mm), and right coronary artery occlusion. Imaging confirmed direct sinoatrial node compression. First-stage surgery comprised biological aortic valve replacement, sinus repair, and coronary artery bypass grafting. The coarctation of the aorta was managed conservatively based on borderline transcoarctation gradient and favorable clinical status.
CONCLUSION: Direct mechanical sinoatrial node compression combined with compromised nodal arterial supply constituted the pathophysiological basis for arrhythmia. For bicuspid aortic valve with multisegmental aortic disease, individualized strategy based on anatomical characteristics, hemodynamic parameters (such as transcoarctation gradient, aortic isthmus ratio, and extremity blood pressure difference), and systemic condition is essential. Staged repair prioritizing hemodynamically threatening lesions reduces surgical trauma and spinal ischemia risk, providing clinical guidance for managing such rare complex aortic pathologies.
PMID:42887709 | DOI:10.1016/j.carpath.2026.107866