31 Congenital Heart Disease

• central cyanosis: seen in congenital heart disease in babies
• warm extremities: perfused with warm blood, just not enough O2

• 5 T's

Tetralogy

Pathogenesis

• pic: aorta shifted to right, override ventricular septum with VSD, thick RV walls, stenotic PV

• all abnormalities caused by infundibulum/conus arteriosus "monology of fallot"

• pic: white infundibulum moving towards RV, dragging aorta with it to cause aorta to override RV, also creating VSD

• restricts flow of pulmonary artery

• deoxygenated blood diverted away from lung to LV and systemic circulation
• pink tets: mild obstruction, less cyanosis

Symptoms

• boot shaped heart
• hypertrophic RV and apex misplaced

• knit femoral arteries when squatting

Truncus Arteriosus

• normally truncus divides into aorta or PA
• persists = common trunk
• mixing of blood = cyanosis

Transposition of great vessels

• switched aorta and PA

• relative position normal

• D transposition
• VSD: allow RV/LV mix
• PDA: allow Ao PA mix

• rare variant
• RV fail from not being able to take aorta pressure

• transposition most common

Tricuspid Atresia

• only case to be compatible with life
• 2 shunts to allow blood flow

TAPVR

• mixed blood with low O2, cyanosis

Ebstein's

• cyanosis and HF from tricuspid regurge

Pulmonary Atresia

• in utero not a problem
• VSD: allows blood to exit ventricle

• keeps connection open

Conotruncal Heart Defect

• arteriosus: form outflow of LV and RV
• classic: DiGeorge, develop any conotruncal problems

Coarctation

• preductal: before ductus arteriosus insertion

Preductal

• DA supplying lower body = no need for collateral vessel development in womb

• cyanosis: blood bypass lung via DA and to lower extremities

• all blood has to go through narrowing
• baby into heart failure/shock in day 2 of life

• narrowing of aorta usually in thoracic region

Postductal

• good: no HF in babies
• bad: asymptomatic until adult

• delay between when feel brachial vs femoral pulse

• young person with htn
• rarely, poor blood flow to lower body: pain/ischemia with walking

• xray: aortic knob and below

• not flushed in upper extremities or cyanotic in lower extremities because of autoregulation

• endovascular infection: infection inside vascular tree
• endocarditis: heart valves
• endarteritis: artery

• coarctation part of a bigger problem of vasculature