Surgical Considerations in Shone Complex

Abstract

Shone complex is defined by 4 anomalies: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and coarctation of the aorta. Establishing a clear definition is one of the principal challenges in the study of Shone complex as not all patients have all lesions. The essential feature of Shone complex is multilevel left-sided obstruction involving both the left ventricular inflow and outflow. This anatomic variability is reflected in the clinical presentation as signs of left ventricular inflow obstruction are often masked by outflow obstruction and the multilevel nature of the condition is thus underappreciated. Surgical treatment is often stepwise addressing the outflow obstruction first. In this review, geared to the pediatric cardiac anesthesiologist, we review the pathophysiology, diagnosis, treatment, and outcomes of Shone complex.