Adult Congenital Heart Disease - Introduction to General Medicine - Lecture Slides, Slides of Medicine

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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)

 Regular evaluation at an ACHD center

 Health care passport

 Structured transfer/transition plan

 Co-management with cardiologists

having undergone advanced training

 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