Konno Ross procedure, coronary artery bypass graft and mitral valve replacement in a 12-year-old girl with homozygous familial hypercholesterolemia

Background: Familial hypercholesterolemia (FH) is a genetic disorder caused by a mutation of the gene for the low density lipoprotein receptor. This mutation can lead to elevated plasma cholesterol levels and subsequently to premature coronary artery disease. Management of patients with FH is complicated and surgery is accompanied by high risk, even in skillful hands. Case presentation: A 12-year-old female patient was referred to our department in January 2013 with chest pain and dyspnea. Her history showed that he had documented evidence of homozygous HF (HFH) since 2 years of age and that she underwent a Ross–Konno procedure for valvular aortic stenosis, 3 years ago. Electrocardiography showed ST depression in the inferolateral derivations and ST elevation in aVr. The echocardiography showed LV systolic dysfunction and important mitral regurgitation. Coronary angiography demonstrated stenosis in the distal part of the left main and severe three vessel coronary artery disease. The patient presented critical acute myocardial ischemia immediately after coronary angiography. She was referred for surgery. The left anterior descending artery was bypassed using saphenous vein and both right coronary artery and marginal artery using sequential saphenous vein. The mitral valve was replaced with mechanic prosthesis. The postoperative course was uneventful. She was prescribed atorvastatin accompanied by cholestyramine and diet modulation. Conclusion: HFH patients are at increased risk of developing coronary artery disease and also sudden death unless the condition is recognized and treated promptly. Surgery remains the most effective means of prolonging the life of these patients