Hemodynamics in Adults With the Shone Complex

Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.