31 Congenital Heart Disease

Cyanosis

[_](central vs peripheral cyanosis. 5T's of central cyanosis)..

• central cyanosis: seen in congenital heart disease in babies

• warm extremities: perfused with warm blood, just not enough O2

• 5 T's

Tetralogy

Pathogenesis

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• 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

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• boot shaped heart

• hypertrophic RV and apex misplaced

• knit femoral arteries when squatting

Truncus Arteriosus

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• normally truncus divides into aorta or PA

• persists = common trunk

• mixing of blood = cyanosis

Transposition of great vessels

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• 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

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• mixed blood with low O2, cyanosis

Ebstein's

[_](Ebstein's is, characteristics, cause, EKG)..

• 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

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• arteriosus: form outflow of LV and RV

• classic: DiGeorge, develop any conotruncal problems

Coarctation

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• preductal: before ductus arteriosus insertion

Preductal

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• 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

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• 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