Life with an anomalous left coronary artery originating from the pulmonary trunk
Ruiz, Miriam I.
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The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect occurring approximately one out of every 300,000 live births. ALCAPA is a coronary artery anomaly in which the left coronary artery originates anomalously from the pulmonary trunk rather than from its usual origin, the aorta. It is a serious heart defect with a presentation range from severe congestive heart failure and left ventricular dysfunction in infants to asymptomatic survival into adulthood. ALCAPA is one of the most common causes of myocardial ischemia and infarction in infants and children, and if untreated can cause death in up to 90% of cases due to heart failure. With technological advances in diagnosis and treatment, there are increasing numbers of ALCAPA patients surviving into adulthood. This trend is seen with CHD in general with over 85% of treated children born with CHD surviving into adulthood today with an estimated increase in the next few decades. Although ALCAPA patients undergo "corrective" surgery, they are never truly cured, and patients must manage their condition as a mild, moderate, or potentially severe chronic disease. Through a review of current literature about ALCAPA patients and adults with CHD in general, this thesis explores the postoperative physiological outcome, psychosocial effects, and health care options of treated pediatric ALCAPA patients that survived into adulthood. We have found that there are still significant unknowns about the future of adult ALCAPA survivors. First, earlier diagnosis and treatment of ALCAPA can lead to good outcomes, yet it is unknown whether it is feasible to find the defect before it manifests and treat it before any damage has occurred. Secondly, long-term studies of patients are currently insufficient to determine the health of these patients past a decade or two. Lastly, the current health care options for ALCAPA and adults with CHD in general do not currently meet the needs of this unique population, as they require highly specialized services in order to ensure their health.
Thesis (M.A.)--Boston University
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