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Congenital Heart abnormalities (Atrial Septal Defect (Symptoms (Fatigue,…
Congenital Heart abnormalities
Foetal Circulation
Blood travels from the IVC --> RA --> Foramen ovale --> LA --> LV bypasses the lungs by going through the ductus venosus
Aorta --> Internal iliac arteries --> umbilical arteries --> placenta (removed CO2 and waste products)
In the normal fetus there is a shunt at the lungs and the liver. As at these two points the lungs have yet to develop
A decrease in RA pressure then allows the foramen ovale to close
The presence of oxygen on the ductus venous causes it to constrict
Ductous venous --> ligament venous
Umbilical vein --> ligamnetum teres. This is continuous with the falciform ligament
Epidemiology of CHA
Mostly more common in males
Persistant ductus arterioles and Atrial septal defects are more common in women
1% of the population is affected
General symptoms
Central cyanosis
Finger clubbing
Syncope (as O2 cerebral levels fall)
Tetralogy of Fallot and Tricuspid atresia
General signs
Polycythaemia
Pulmonary hypertension
Growth retardness- very common in Children presenting with the condition
Paradoxical embolism (right to left shunt)
General Presentation
Endocarditis
Progression of valvular lesions
Atrial + ventricular arthrymias
RHF and sudden cardiac death
General Causes
Trisomy 21 (Down's)
Drugs: Thalidomise and Lithium
Maternal alcoholic misuse
Diabetes
Prenatal Rubella
These conditions should be noticed as soon as possible so that early treatment can be initiated
Classification of Congenital Heart Disease
Acyanotic without shunts: coarctation of the aorta and congenital aortic stenosis
Cyanotic with shunts: tetralogy of Fallot and Severe Ebstein's anomaly
Acyanotic with shunts: AS defect, VS defect and Patent ductus arteriosus
Cyanotic without shunts: severe pulmonary stenosis, tricuspid atresia and pulmonary atresia
Ventricular Septal Defect. Shunt is Left to Right so patient does not go blue
Types
Small Restrictive VSDs : Large pan systolic murmur. This is a murmur that is heard throughout systole . And a thrill (buzzing sensation)
Moderate Restrictive VSDs: will produce a loud tearing systolic murmur
Large VSDs: will cause there to be pulmonary hypertension and also a soft murmur. Eisenmengers syndrome is produced
What is Eisenmenger's syndrome ?
This is when there is a greater pressure in the RV than the LV
Blood then starts to shunt from the RHS to the LHS causing cyanosis
Causes
Down's/ Edwards syndrome
Foetal Alcohol Syndrome
Diabetes
Epidemiology
Most common 1:500.
Involves a left to right shunt due to left ventricular pressure being higher than in the right ventricle
When the LV contracts it ejects blood into the aorta and means that som e also goes into the ventricular septal defect and thus also in the RV and the Pulmonary Artery
Symptoms
Tachypnoea
Increased respiratory effort
Trouble with feeding
Problems with gaining weight
Problems with exercise capabilities
Signs
Depending on size of the defect determines the murmur that is heard
Diagnosis
ECHO
Doppler
ECG
Treatment
Propthylatic antibiotics to preven t the risk of endocarditis
Moderate size lesion: you will want to reduce the load on the heart by using ACE inhibitors, diuretics and digoxin
Most close up on theirselves
Atrial Septal Defect
Epidemiology
3rd most prevalent congenital abnormality
Higher incidence in women
Risk Factors
Autosomal Dominant
Foetal Alcohol syndrome
Common in Down's syndrome
Diabetes
Maternal Smoking
Shunt is Left to Right. So patient will not be cyanosed. However there will be an increased blood flow to the RHS and also the Lungs. This can lead to right heart overload and dilation.
Definition :
This is when there is communication between the left and the right side of the heart. This is due to the patent fossa ovale not closing
Types
Sinus venosus defect
Ostrium secondum defect
Ostrium primum defect
Increased pulmonary flow can cause: RV hypertrophy, Pulmonary hypertension --> Eisenmenger's reaction ) and increased risk of infective endocarditis
Symptoms
Fatigue
Exercise intolerance
Dyspnoea
Pulmonary hypertension
Syncope
Signs
Wide split second heart sound
Soft systolic ejection mumble
Arrthymia
Diagnosis
Atrial flutter and Atrial fibrillation
CXR is abnormal as shows dilated pulmonary arteries and heart
ECG
Tall P wave and incomplete RBBB and RA deviation
Echo will show hypertrophy and dilation of the RHS of the heart and will show enlarged pulmonary arteries
Treatment
Cardiopulmonary bypass operation
Percutanous - key hole surgery
Patent Ductus arteriosus
Epidemiology:
affects 1 in 2000 births. Is a remnant of the 6th aortic arch that connects the pulmonary artery and the proximal descending aorta. The shunt is normally left to right. However, if it doesn't;t close it overloads the lungs. Affects more girls than boys
ECHO and diuretics
Signs:
are a bounding pulse, machinery murmur (caused by the turbulent pulmonary artery to aortic shunt) and pulmonary hypertension
Symptoms: failure to thrive and recurrent URTIs
and RF are rubella, Downs, asphyxia in pregnancy and also High Altitude
C
oarctation of the Aorta
Narrowing of the aorta distal to the ductus arteriosus. Strong association with Turner's syndrome
Symptoms=
headaches, nose bleeds, hypertension and cold legs
More common in women and white people . This can be diagnosed in utero
Signs=
Increased BP and also delayed pulsation in the legs compared to arms. These pulses are also poor.
Diagnosis:
Echo, CXR, ECG and U&E, creatinine, FBC and blood glucose
Treatment
= Beta Blockers, Ace inhibitors and and increased risk of endocarditis so use prophylaxis
Bicuspid Aortic Valve
Exercise accelerates the complication s
Epidemiology
Most common type of congenital heart disease. Affects more men than women. Happens when a normal aortic valve has 3 cusps. This valve has only 3 cusps. Meaning it is more prone to calcification and degeneration
Very common to take place if there are also coarctation and dilation of the ascending aorta
Patient may develop aortic stenosis or aortic regurgitation
Atrio-ventricular septal defects (AVSD)
Associated with Down's syndrome
Can either be a partial or complete defect. This leads to one big malformed atrio-ventricular valves
Breathlessness, poor weight gain and Eisenmengers
Pulmonary artery banding. Reduces the chance of Eisenmenger's syndrome