Aortic Root Enlargement and Aortic Valve Replacement for Calcified Supravalvular and Valvular Aortic Stenosis in Homozygous Familial Hypercholesterolemia: A Case Report.

Familial homozygous hypercholesterolemia is a rare disease with diverse clinical presentations ranging from premature ischemic heart disease to aortic root stenosis but rarely presents with anginal symptoms due to supravalvular and valvular aortic stenosis. We report a 19-year-old male patient with familial homozygous hypercholesterolemia with progressive supravalvular and valvular aortic stenosis that ultimately required aortic root enlargement and aortic valve replacement using a mechanical prosthesis, despite aggressive medical therapy. Surgical importance of this rare condition is highlighted.

Successful Use of Intra-aortic Balloon Counterpulsation for Systemic Ventricular Failure Following Total Pericardiectomy for Calcific Chronic Constrictive Pericarditis.

We report two male patients aged 18 and 19 years, respectively, undergoing total pericardiectomy for chronic calcific constrictive pericarditis who developed systemic ventricular failure unresponsive to medical management following surgery. The failing circulation was successfully reestablished using intra-aortic balloon counterpulsation. Aortic counterpulsation facilitates recovery of ventricular function and appears to be a reasonable alternative in select instances of refractory cardiac failure following pericardiectomy.