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Adult Congenital Heart Disease, Development of Heart, Hypoplastic Left Heart Syndrome, Complex Chd, Spiral Truncoconal Septum, Subpulmonic Stenosis, Palliative Surgical Shunts are some points in Introduction to General Medicine lecture. This lecture is one of 61 lectures you can find here for this course.
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Describe the changing profile of ACHD
Describe the challenges in the transfer of care from pediatric to adult practitioners
Review the development of the heart
Describe the anatomy, natural history, surgical repair, and common adult presentations of tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome
A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance
Overall incidence: 75 of every 1000 live births
Incidence of moderate and severe CHD: 8 of every 1000 live births
A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance
Overall incidence: 75 of every 1000 live births
Incidence of moderate and severe CHD: 8 of every 1000 live births
Over 90% of children survive to adulthood
20,000 operations for CHD in the US each year
Over 1 million adults in the US with CHD
32 nd^ Bethesda Conference (2000)
Named by Etienne-Louis Arthur Fallot in 1888
Approximately 10% of all complex CHD
Single developmental error of the terminal portion of the spiral truncoconal septum
Four distinct components: subpulmonic stenosis, VSD, overriding aorta, and RV hypertrophy
Often accompanied by other anomalies
Longest surgical history/most studied outcomes
Palliative surgical shunts
Classic/complete repair
Infundibular muscle resection, VSD patch, & RVOT repair Typically preformed between 4-6 months of age Surgical risk: < 5%
Survival rates: 85% at 30+ years
Repaired
RVOT obstruction Pulmonary or tricuspid regurgitation
Complete TGA or D-TGA
Embryological inversion of the great arteries Ventriculoarterial discordance
Balloon atrial septostomy
Developed by Rashkind (1965) Enlarges the atrial communication
Atrial switch
Performed first by Senning (1958) and later modified by Mustard (1964) Atrial baffle is created to direct venous return to the contralateral ventricle
RV dysfunction
Tricuspid insufficiency
Bradyarrhythmias
Atrial tachycardias
Obstructed/leaky atrial baffle
Coronary perfusion issues
Supravalvar aortic and pulmonic stenosis
Aortic root dilatation and valvular insufficiency
Branch pulmonary stenosis
Hypoplastic left-heart syndrome
Hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum)
Tricuspid valve atresia
Mitral valve atresia
Double-inlet ventricle
Atrioventricular canal defects
Double outlet right ventricle