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