Shone's Syndrome and Transplant

"Shone's syndrome" was identified in 1963 as a constellation of 4 coexisting lesions leading to left ventricular inflow and outflow obstruction. This congenital heart disease syndrome has hemodynamic consequences throughout the lifetime of patients with this syndrome, and these patients often progress to requiring advanced therapies. This case series reviews the complex decision-making involved in advanced therapy evaluation for this specific patient population. We present 8 cases of patients with Shone's syndrome who were referred for advanced therapies. Of these patients, 6 patients went on to heart transplant alone and 2 patients underwent dual organ transplant. Two patients died after transplant. These cases each highlight important considerations for transplant in this patient population, including the development of pulmonary hypertension, potential need for single vs dual organ transplant, and complex pre- and postoperative courses. We emphasize the importance of early referral for advanced therapies, multidisciplinary evaluation, and individualized care for these complex patients.