Lyana Labrada, Tripti Gupta, Christiane Haeffele, Adam D. DeVore, Jonathan Menachem
Temple University Hospital. University of San Diego. Stanford University. Duke University School of Medicine. Vanderbilt University Medical Center.
United States
Journal of the American College of Cardiology Case Reports
JACC Case Rep 2025;
DOI: 10.1016/j.jaccas.2025.105000
Abstract
“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.
Category
Class II. Pulmonary Hypertension Associated with Valvular Disease of the Left Side of the Heart
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes