Anomalous aortic origin of a coronary artery
Encyclopedia
Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva with an interarterial, intraconal, or intramural course is a rare heart defect associated with an increased risk of sudden death
in children.
(CHSS) established a North American Registry in order to study a large multi-institutional cohort of patients with AAOCA. This initiative is intended to generate new knowledge concerning the natural history of AAOCA, to describe the outcomes of surgical intervention versus observation in children and young adults with AAOCA, and to generate evidence to support risk stratification among patients with AAOCA and eventually suggest evidence-based guidelines for management.
.
Children and young adults with these defects can die suddenly, especially during or just after exercise. In fact, AAOCA is the second leading cause of sudden cardiac death
in children and adolescents in the United States behind hypertrophic cardiomyopathy
. The prevalence
is estimated at 0.1% to 0.3% of the general population. Neither the true risk of sudden death nor the best way to treat these patients is known with certainty. Because of the risk of sudden death, doctors face the pressure to “do something” but in the absence of long-term follow-up data, the risks and benefits of different management options are unconfirmed. This study will create a pool of information that may guide future choice of treatment options for these children and young adults.
This study will be ongoing for 15 years. It is expected that approximately 1000 patients will be enrolled.
This funding to start the registry was provided by The Children's Heart Foundation
, The Cardiac Center at The Children's Hospital of Philadelphia
and from CHSS member institutions.
, Ventricular septal defect
, Patent ductus arteriosus
, bicuspid aortic valve
, mild pulmonary stenosis etc .
Patients who have other major heart problems that require operations are currently not included in this Cohort study
. Any other problems with coronary arteries are also not included.
. Hospitals from across North America continue to join the study group and enroll patients. Over 140 patients with AAOCA have been enrolled by June 2011, making it the largest cohort ever assembled of this anomaly.
Sudden death
In a sport or game, sudden death is a form of competition where play ends as soon as one competitor is ahead of the others, with that competitor becoming the winner. Sudden death is typically used as a tiebreaker when a contest is tied at the end of the normal playing time or the completion of...
in children.
CHSS AAOCA study
In 2009, The Congenital Heart Surgeons' SocietyCongenital Heart Surgeons' Society
Congenital Heart Surgeons' Society is a professional membership organization of heart surgeons who specialize in treating congenital heart defects...
(CHSS) established a North American Registry in order to study a large multi-institutional cohort of patients with AAOCA. This initiative is intended to generate new knowledge concerning the natural history of AAOCA, to describe the outcomes of surgical intervention versus observation in children and young adults with AAOCA, and to generate evidence to support risk stratification among patients with AAOCA and eventually suggest evidence-based guidelines for management.
Rationale and Objectives
The AAOCA is a rare birth defect in the heart that occurs when a coronary artery arises from the wrong location on the main blood vessel, the aortaAorta
The aorta is the largest artery in the body, originating from the left ventricle of the heart and extending down to the abdomen, where it branches off into two smaller arteries...
.
Children and young adults with these defects can die suddenly, especially during or just after exercise. In fact, AAOCA is the second leading cause of sudden cardiac death
Sudden Cardiac Death
Sudden cardiac death is natural death from cardiac causes, heralded by abrupt loss of consciousness within one hour of the onset of acute symptoms. Other forms of sudden death may be noncardiac in origin...
in children and adolescents in the United States behind hypertrophic cardiomyopathy
Cardiomyopathy
Cardiomyopathy, which literally means "heart muscle disease," is the deterioration of the function of the myocardium for any reason. People with cardiomyopathy are often at risk of arrhythmia or sudden cardiac death or both. Cardiomyopathy can often go undetected, making it especially dangerous to...
. The prevalence
Prevalence
In epidemiology, the prevalence of a health-related state in a statistical population is defined as the total number of cases of the risk factor in the population at a given time, or the total number of cases in the population, divided by the number of individuals in the population...
is estimated at 0.1% to 0.3% of the general population. Neither the true risk of sudden death nor the best way to treat these patients is known with certainty. Because of the risk of sudden death, doctors face the pressure to “do something” but in the absence of long-term follow-up data, the risks and benefits of different management options are unconfirmed. This study will create a pool of information that may guide future choice of treatment options for these children and young adults.
This study will be ongoing for 15 years. It is expected that approximately 1000 patients will be enrolled.
This funding to start the registry was provided by The Children's Heart Foundation
Children's Heart Foundation
The Children’s Heart Foundation is a United States health charity, whose mission is to fund the most promising research to advance the diagnosis, treatment and prevention of congenital heart defects in children...
, The Cardiac Center at The Children's Hospital of Philadelphia
Children's Hospital of Philadelphia
The Children's Hospital of Philadelphia is one of the largest and oldest children's hospitals in the world. CHOP has been ranked as the best children's hospital in the United States by U.S. News & World Report and Parents Magazine in recent years. As of 2008, it was ranked #1 in the nation for...
and from CHSS member institutions.
Enrollment criteria
Patients who are diagnosed with AAOCA at or before age 30 years are eligible for this study. They should have otherwise normal heart or only minor defects such as Atrial septal defectAtrial septal defect
Atrial septal defect is a form of congenital heart defect that enables blood flow between the left and right atria via the interatrial septum. The interatrial septum is the tissue that divides the right and left atria...
, Ventricular septal defect
Ventricular septal defect
A ventricular septal defect is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart.The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.The membranous...
, Patent ductus arteriosus
Patent ductus arteriosus
Patent ductus arteriosus is a congenital disorder in the heart wherein a neonate's ductus arteriosus fails to close after birth. Early symptoms are uncommon, but in the first year of life include increased work of breathing and poor weight gain...
, bicuspid aortic valve
Aortic valve
The aortic valve is one of the valves of the heart. It is normally tricuspid , although in 1% of the population it is found to be congenitally bicuspid . It lies between the left ventricle and the aorta....
, mild pulmonary stenosis etc .
Patients who have other major heart problems that require operations are currently not included in this Cohort study
Cohort study
A cohort study or panel study is a form of longitudinal study used in medicine, social science, actuarial science, and ecology. It is an analysis of risk factors and follows a group of people who do not have the disease, and uses correlations to determine the absolute risk of subject contraction...
. Any other problems with coronary arteries are also not included.
Current status
The Registry has been enrolling new patients from participating institutions that are member of the Congenital Heart Surgeons' SocietyCongenital Heart Surgeons' Society
Congenital Heart Surgeons' Society is a professional membership organization of heart surgeons who specialize in treating congenital heart defects...
. Hospitals from across North America continue to join the study group and enroll patients. Over 140 patients with AAOCA have been enrolled by June 2011, making it the largest cohort ever assembled of this anomaly.
See also
- Congenital Heart Surgeons' SocietyCongenital Heart Surgeons' SocietyCongenital Heart Surgeons' Society is a professional membership organization of heart surgeons who specialize in treating congenital heart defects...
- Children's Hospital of PhiladelphiaChildren's Hospital of PhiladelphiaThe Children's Hospital of Philadelphia is one of the largest and oldest children's hospitals in the world. CHOP has been ranked as the best children's hospital in the United States by U.S. News & World Report and Parents Magazine in recent years. As of 2008, it was ranked #1 in the nation for...
- The Hospital for Sick Children, Toronto, Canada
- Children's Heart FoundationChildren's Heart FoundationThe Children’s Heart Foundation is a United States health charity, whose mission is to fund the most promising research to advance the diagnosis, treatment and prevention of congenital heart defects in children...
External links
- Congenital Heart Surgeons' Society (CHSS)
- CHSS Data Center - the research organization conducting the study