Cookiecutter Heart

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The term "cookiecutter heart" refers to a specific type of congenital heart defect known as atrioventricular septal defect (AVSD). AVSD is characterized by an abnormal opening or hole between two chambers of the heart, typically the left and right ventricles. This defect can lead to various complications, including heart failure, arrhythmias, and increased risk of endocarditis. Individuals born with a cookiecutter heart often experience symptoms from infancy onward. These may include rapid breathing rate, fatigue, poor weight gain, and bluish discoloration of the skin due to inadequate oxygen delivery to the body's tissues. In severe cases, the defect can cause death in early childhood if left untreated. Treatment options for AVSD depend on the severity of the condition and the patient's overall health. Pediatric cardiologists often recommend surgery to repair or replace affected heart valves and close any openings between chambers. The goal is to restore normal blood flow and prevent further complications. While surgical intervention can significantly improve quality of life, individuals with a cookiecutter heart may require ongoing medical care and monitoring throughout their lives. Regular check-ups with a pediatric cardiologist are essential to ensure that the repair holds and that no new issues arise.