Left Main Coronary Artery Disease Due to Primary Hypoalphalipoproteinemia in a 17-Year-Old Female

Low high density lipoprotein (HDL-C) levels are inversely related to risk for coronary artery disease (CAD). Some forms of hypoalphalipoproteinemias characterized by extremely low levels of serum HDL are associated with premature CAD. We report a 17-year-old female with acute myocardial infarction associated with total occlusion of the left main coronary artery at coronary angiography. A successful angioplasty and stenting was performed and flow was restored. She died 4 months after miyocardial infarction due to heart failure. No cause of CAD, besides atherosclerosis, was determined, in that case. She had extremly low levels of serum HDL-C and normal LDL-C, triglycerides (HDL-C: 2 mg/dL, LDL-C: 92 mg/dL, total cholesterol: 130 mg/dL, triglycerides: 180 mg/dL). The absence of low levels of HDL-C in her family members suggested that she might have primary hypoalphalipoproteinemia. Although we could not perform genetic analysis, we think she might have apo AI gene disarrengement, apo AI frameshift mutation or specific Apo-A1 point mutation like Apo-A1ZAVALLA (Leu159ÆPro ) and Apo-A1PISA (Leu141ÆArg) because the other forms of hypo-alphalipoproteinemias are not associated with CAD.