Congenital Heart Disease Flashcards
What are congenital heart diseases?
During what time frame do they most commonly develop?
Most common in what demographics?
When are they most frequently diagnosed?
- Congenital Heart Disease- present at birth
- Development
- most during weeks 3-8 of gestation
- More common in premature infants & stillborn
- Diagnosed
- 1/2 during first year
- the rest may not manifest until later
What is the major known causes of congenital heart disease?
sporadic genetic abnormalities
12 disorders account for ~85% of all cases
Almost all congenital heart abnormalities cause a predisposition for what problem?
bacterial endocarditis
because they will result in abnormal/turbulant blood flow, which will cuase damage to the endocardium
What is the most common congenital heart disease?
Second most common?
what is the difference in incicence between these two?
- ventricular septal defect (4x)
- Atrial septal defect
What is the major change that happens from fetal circulation to a newborn baby’s circulation?
once the lungs expand & the pulmonary system becomes a low pressure system - we lose the need for all of the shunts that exist in the fetal heart & they will close
What are the 3 functional classifications of congenital heart defects?
Explain the reasoning behind their presentation
-
Presenting as left-to-right shunt (usually acyanotic)
- b/c oxygenated blood mixing with the deoxygenated blood on the right heart that will go back through the lungs & be oxygenated again
-
Presenting as right-to-left shunt (usually cyanotic)
- deoxygenated blood from the right heart being shunted into the left heart and it is going out into the systemic circulation, by-passing the lungs
-
Causing an obstruction
- like stenosis of a valve
Describe the sequence of events that occurs with left-to-right shunts
- Initially acynotic
- oxygenated blood from left heart is mixed with unoxygenated blood from right
- increases flow volumes & pressure in right heart and lungs
- chronically increase the pressure in the lungs, which will cause pulmonary hupertension eventually causing reversal (months-to-years-to-decades); right-to-left shunt
- at this point will present with cyanosis
What are the 4 congenintal disorders that cause left-to-right shunts?
- Ventricular Septal Defect (VSD)
- Atrial Septal Defect (ASD)
- Patent Ductus Arteriosus (PDA)
- Atrioventricular Septum Defect (AVSD)
**they all have a “D” in them- unlike me
What defect occurs with Ventricular Septal Defect?
Incomplete closure of interventricular septum
What defect is shown in the provided image?
Ventricular Septal Defect
- hole in the inerventricular septum
- notice it is smooth around the edges, indicating it is not a rip or a tear
- can be small or large
How does the heart respond to a Ventricular Septal Defect?
-
Pressures are the same in both ventricles
-
Pressure hypertophy of R ventricle
- myocytes respond to increased pressure by concentric hypertrophy
- large, untreated VSDs almost always lead to irreversible pulmonary hypertension
- right heart pressure eventually exceeds that of the left heart & shunt will be reversed, with unoxygenated blood flowing from right to left, then out to the systemic circulation
- Volume hypertrophy of L ventricle are usuall present
-
Pressure hypertophy of R ventricle
What defect occurs with Atrial Septal Defect?
Where does it usually occur?
Persistent opening in the interatrial septum
most common site is foramen ovale
What is the most common congenital heart abnormality to present in adulthood?
atrial septal defect
usually asymptomatic until after age 30
What defect is shown in the provided image?
Atrial Septal Defect
- notice smooth margins of hole, inidicating it is not a rip nor tear
What defect occurs with Patent Ductus Arteriosus?
How does it present at birth?
it is most common within what demographics?
Treatment?
Persistent opening of ductus arteriosus after birth
- abnormal blood flow between aorta and pulmonary artery
- ductus arteriosus connects aorta & pulmnomary artery
- usualy asumptomatic at birth
- can result in cyanosis when the shunt reversed due to pressure imbalances
- may have a machinery murmur on physical examination
- demographics
- F > M
- premature infants
- infants with neonatal respiratory distress syndrome
- 90% are isolated finding - some can be associated with clinical syndromes
- Treatment:
- endomethicin, other NSAIDS
- large enough, may have to do surery
What defect is shown in the provided image?
Identify the features outlined in blue, green, and red.
What defect occurs with Atrioventricular Septal Defect?
What are the different severities that can exist?
Commonly affected demographics?
Large, combined AV septal defect and large common AV valve. A “hole” in the center of the heart
superior & inferior endocardial cushions don’t fuse in embryogenesis - partial or complete
- Partial - mostly involves the atrial & a lesser extent the ventricles
- Complete- a 4 chamges will freely communicate
- Demographics
- >1/3 of people with complete AVSD have Down Syndrome
Describe the sequence of events that occurs with Right-to-Left shunts
- Unoxygenated blood from right side flows directly inot the left side
- hypoxemia & cyanosis result
- Complications
- paradoxical emboli
- infarcts & abscesses
- hypertrophic osteoarthropathy (clubbing of fingers)
- polycythemia
- kidneys respond to hypoxemia by producing more erythropoietin
- paradoxical emboli
What pathology is shown in the provided image?
This is associated with what type of congenital heart defect?
Hypertrophic osteoarthoropathy
“clubbing of the fingers” & curling of the nails
associated with right-to-left shunts
What type of defect occurs with Patent Foramen Ovale?
How common are they?
Symptoms?
Transient Right-to-left shunt
common
most are asymptomatic
increased risk of paradoxical emboli - w/ increased pressure w/ cough or straining to have a bowel movement; embolus can go from right side of heart to left side of heart & out into systemic circulaiton- infarcts
possiblly the cause for people with unexplained strok, migraines or MI
What are the five congenital abnormalities presenting with right-to-left shunts?
- Tetralogy of Fallot
- Transposition of the Great Arteries
- Persistent Truncus Arteriosus
- Tricuspid Atresia
- Total Anomalous Pulmonary Venous Connection
** All have a “T” in the name – “tree frog” clubbed fingers
What abnormality occurs with Tetralogy of Fallot?
- Results from a single developmental defect
- abnormal anterior & cephalad displacement of infundibular (outflow tract) portion of interventricular septum
- overriding aorta
- pulmonic stenosis
- ventricular septal defect
- right ventricular hypertrophy
What abnormality is shown in the provided radiograph?
Tetrology of Fallor
boot-shaped heart on radiograph
What symptoms are associated with Tetrology of Fallot?
cyanosis worsens as the stenosis worsens
infants may turn blue during crying spells
children often squat during exercise
What defect is seen with Transposition of the Great Arteries?
What additional defect is required for survival?
Treatment?
Aorta coming out of right ventricle & pulmonary trunk coming out of left ventricle
need persistent ductus arteriosus or ventricular septal defect to be compatible with life - to get some oxygentated blood to the systemic circulation
- **medical emergency - cyanotic at birth
- give prostaglandins or baloon catheter to keep ductus arteriosus open
- surgery (arterial switch)
What defect is shown in the provided image?
Transposition of the great arteries
Describe the defect that occurs with Truncus Arteriosus.
When the truncus arteriosus in fetal heart development does not split into pulmonary trunk & aorta - mixing of oxygenated & deoxygentated blood & presents as a right-to-left shunt
What defect is shown in the provided image?
Truncus Arteriosus
Describe the defect that occurs with Total Anomalous Pulmonary Venous Return
Treatment?
venous return from pulmonary vein inserts into the superior vena cava
no pulmonary veins join the left atrium
need patent foramen ovale or septal defect to be compatable with life
** medical emergency & need to be taken to surgery quickly
Describe the defect associated with Tricuspid Atesia
Tricuspid Atresia– or missing tricuspid valve
difficult for blood to go through the normal route
*medical emergency
- Treatment in 3 stages (b/c as baby grows valve will have to grow with them)
- surgery immediately
- again at 4-6 months
- between 18 months & 4 years
What pathology is shwon in the provide image?
Congenital bicuspid aortic valve
can be a stenotic valve at birth, or can develop calcifications in childhood or early adolescence
Identify the 4 types of aortic stenosis
- Degerative calcific aortic stenosis
- Rheumatic aortic stenosis
- Bicuspid arotic stenosis
- unicuspid aortic stenosis
- looks similar to rheumati aortic stenosis, except without calcifications & scarring
What pathology is shown in the provided image?
Congenital Pulmonary Stenosis
can be the valve itself (most cases) or the artery above the valve that is stenotic
What pathology is shown in the provided image?
it is most common in what populations?
Coarctation ofthe aorta – discrete narrowing of the aorta
more common in people with Turner’s Syndrome - but can be seen as a solitary defect
with full patent ductus arteriosus or without
What pathlogy is shown in the provided image?
It can lead to what other problems?
Treatment?
Coarctation of the Aorta
Systemic Hypertension - resulting hypertrophy of LV
Blood flow to head & neck is preserved
So, hypertension in upper body & hypotension in lower
Resection & grafting are curative
What is a unique presentation in older children & adults with coarctation of the Aorta?
enlarged intercostal or internal mammary arteries b/c blood is finding alternative routes
can cause notches in the ribs
What conditions require emergent surgery?
