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.

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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.

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The present perspective is a synthesis of 108 published investigations in the setting of different types of left ventricular rupture following mitral valve replacement (MVR). We identified 109 investigations and reviewed the clinical presentation, diagnostic modalities, surgical techniques and outcomes. Clinical presentation, roentgenography, cross-sectional transthoracic/transesophageal echocardiography and computerized tomography provided the diagnostic information and defined the causative mechanism. Magnetic resonance imaging had been used for further clarification of the native ventricular anatomy in high-risk subset of patients, undergoing non-traditional transapical off-pump mitral valve repair with neochordal implantation. In this article, we have attempted to address several concerning issues and controversies with reference to the possible causative mechanisms, preventive measures, the issue of chordal preservation during MVR, the degree of decalcification required in cases of heavily calcified mitral annulus, selection of appropriate sized prosthetic valves, the surgical importance of a small left ventricle, various techniques of repair, role of cardiopulmonary bypass and cardioplegic arrest during the repair of left ventricular rupture, the role of biodegradable epicardial tissue sealants, to repair or not to repair the atrio-ventricular groove hematoma during mitral valve surgery, and the role of intra-aortic balloon counterpulsation in the perioperative period. Additionally, we have highlighted a new type of left ventricular rupture described in the literature located between the base of the papillary muscle and the apex, which can be categorized as a complication of new technologies of mitral valve repair such as NeoChord device artificial chordal implantation. We have classified this category as type IV ventricular rupture. Overall, this review attempts to address the guidelines for different surgical approaches and techniques of repair of different types of left ventricular rupture for a successful outcome. We submit that an increased appreciation of the causative mechanisms of different types of left ventricular rupture and its prevention may well contribute to improved surgical management.