16 year male with sarcomere mutation and family history of hypertrophic cardiomyopathy. He is referred for MRI as part of the VANISH trial. Left VentricleThe left ventricle is normal in size and systolic function. The interventricular septal thickness is normal (9mm). There are deep crypts/ recesses in the basal to mid inferior wall, these have been described to be present in family members of patients with hypertrophic cardiomyopathy. The overall LV ejection fraction is 56%, the LV end diastolic volume index is 92 ml/m2 (normal range: 74+/-15), the LVEDV is 184 ml (normal range 142+/-34), the LV end systolic volume index is 41 ml/m2 (normal range 25+/-9), the LVESV is 82 ml (normal range 47+/-19), the LV mass index is 41 g/m2 (normal range 85+/-15), and the LV mass is 81 g (normal range 164+/-36). There are no regional wall motion abnormalities present. There is no late gadolinium enhancement to suggest the presence of an underlying fibrosing, infiltrative, or inflammatory process.Left AtriumThe left atrium is normal in size. Right VentricleThe right ventricle is normal in size and systolic function. The overall RV ejection fraction is 51%, the RV end diastolic volume index is 94 ml/m2 (normal range 82+/-16), the RVEDV is 188 ml (normal range 142+/-31), the RV end systolic volume index is 46 ml/m2 (normal range 31+/-9), and the RVESV is 92 ml (normal range 54+/-17).Right AtriumThe right atrium is normal in size.Aortic ValveThe aortic valve opens widely and there is no significant aortic regurgitation.Mitral ValveThe mitral valve opens widely and there is no significant mitral regurgitation.Pulmonic ValveThe pulmonic valve opens widely. There is no significant pulmonic regurgitation.Tricuspid ValveThe tricuspid valve opens widely. There is no significant tricuspid regurgitation.AortaThere is a left sided aortic arch with a normal brachiocephalic branching pattern. The aortic root is normal in size.Pulmonary VeinsAll four pulmonary veins drain normally into the left atrium.Pulmonary ArteryThe main pulmonary artery is normal in size.Venous AnatomyThe SVC and IVC are normal in size and drain normally into the right atrium.PericardiumThere is no obvious pericardial disease.Extracardiac FindingsThis study is not designed for the specific evaluation of extra-cardiac findings; therefore, the differentiation between artifacts and real extracardiac pathology may be difficult. If clinically indicated, a separate dedicated evaluation should be considered.
1. Normal LV size and systolic function (LVEF 56 %) without evidence of regional wall thickness. Deep recesses and crypts are noted in the basal to mid inferior wall, which have been described in gene positive, phenotype negative hypertrophic cardiomyopathy patients.2. No evidence of underlying myocardial fibrosis, inflammation, or infiltration.3. Normal RV size and systolic function (RVEF 51 %).