structural heart defects Flashcards
define congenital heart disease
A general term for a range of birth defects that affect the normal way the heart works.
-Vary from minor to incompatible with life ex-utero
-Many lesions used to have very poor prognosis
describe the physiology of congenital heart disease
-The stenosis of the RV outflow leads to the RV being at a higher pressure than the left
-There blue blood passes from the RV to the LV
-The patients are blue
name 7 structural heart defects
-Ventricular septal defect
-Atrio-ventricular septal defects
-Patent ductus arteriosus
-Coarctaction of the aorta
-Bicuspid aortic valve and aortopathy
-Pulmonary stenosis
-Eisenmenger syndrome (any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, cyanosis, reversal of flow).
what is the Tetralogy of Fallot
- ventricular septal defect
- pulmonary stenosis
- hypertrophy of Rt ventricle
- overriding aorta
describe the physiology of tetralogy of fallot
-The stenosis of the RV outflow leads to the RV being at higher pressure than the left
-Therefore blue blood passes from the RV to the LV
-The patients are BLUE
describe what ventricular septal defects are
-Abnormal connection between the two ventricles
-Many close spontaneously during childhood
describe the physiology of ventricular septal defects
-High pressure LV
-Low pressure RV
-Blood flows from high pressure chamber to low pressure chamber
-Therefore NOT blue
-Increased blood flow through the lungs
what are the symptoms of ventricular septal defects?
- very high pulmonary blood flow in infancy
- breathless
- poor feeding
- failure to thrive
what are the clinical signs of ventricular septal defects?
- small, breathless, skinny baby
- increased respiratory rate
- tachycardia
- big heart on chest x- ray
- murmur varies in intensity
what is Eisenmengers syndrome
- high pressure pulmonary blood flow
- damages to delicate pulmonary vasculature
- the resistance to blood flow through the lungs increases
- the RV pressure increases
- the shunt direction reverses
-the patient becomes blue
( untreated tetralogy of fallot may result in eisenmengers syndrome )
define atrial septal defects ASD
an abnormal connection between the two atria
describe the physiology of ASD
-slightly higher pressure in the LA than the RA
- shunt is left to right ( therefore not blue)
- increased flow into right heart and lungs
what are the symptoms of ASDs?
- significant increased flow though the right heart and lungs in childhood
- right heart dilation
- shortness of breath on exertion
- increased chest infections
what are the clinical signs of ASDs?
- pulmonary flow murmur
- fixed split second heart sound- delayed closure of PV because more blood has to get out0
- big pulmonary arteries on CXR
- big heart on chest x ray
describe atrio-ventricular septal defects- AVSD
-a hole in the very centre of the heart
- involves the ventricular septum, atrial septum, mitral and tricuspid valves
- can be complete or partial
In atrio ventricular septal defects- AVSD what do the AV valves look like?
instead of two separate valves, there is one big malformed one
what do those with a complete defect in AVSD look like?
- poor feeding. poor weight gain
- torrential pulmonary blood flow
- breathless as neonate
- needs repair or PA band in infancy
what do those with a partial defect in AVSD look like?
- presents like a small VSD/ASD
- can present in late adulthood
-may be left alone if there is no right heart dilation
what is a patent ductus arteriosus?
when the ductus arteriosus fails to close after birth, leaving a vessel connecting the aorta and pulmonary artery.
what are the clinical signs of patent ductus arteriosus?
- continuous murmur
- if large- big heart, breathless
- eisenmenger’s syndrome- cyanosis
what is the physiology of patent ductus arteriosus?
- breathless, poor feeding, failure to thrive
-torrential flow from the aorta to the pulmonary arteries in infancy
how is closure usually done in someone with the patent ductus arteriosus?
usually done through surgery
under local anaesthetic
- there is a low risk of complications
what is the coarctation of the aorta?
the narrowing of the aorta- usually at the site of insertion of the ductus arteriosus
describe the physiology behind severe coarctation of the aorta
- complete or almost obstruction to aortic flow
- collapse with heart failure
- needs urgent repair
describe the physiology behind mild coarctation of the aorta
- presents with hypertension
- incidental murmur
- should be repaired to try to prevent problems in the long term
what are the clinical signs of coarctation of the aorta?
- right arm hypertension
- Bruits ( buzzes) over the scapulae and back from collateral vessels
- murmur
what are the long term problems with coarctation of the aorta?
- hypertension
- early CAD
- Early strokes
- sub arachnoid haemorrhage - re- coarctation requiring repeat intervention
- aneurysm formation at the site of repair
what is pulmonary stenosis?
the narrowing of the outflow of the right ventricle
describe severe pulmonary stenosis
- right ventricular failure as a neonate
-collapse - poor pulmonary blood flow
- tricuspid regurgitation
what is the treatment for pulmonary stenosis?
-Balloon valvuloplasty
-Open valvotomy
-Shunt (to bypass the blockage)
