Cardiology Flashcards
What CHD is Rubella infection linked to?
peripheral pulmonary stenosis and PDA
What CHD is SLE linked to?
Complete heart block - Anti-ro and Anti-La antibody
What CHD is Down syndrome related to?
ASD, VSD
What heart abnormality is FAS related to ?
ASD, VSD, Tetralogy of Fallot
The umbilical vein and inferior vena cava transfer oxygenated blood from the placenta to the fetal …
Right Atrium!
Most of the blood in the RA is shunted through the …
Foramen Ovale!
Where is the Ductus Arteriosus connected to ?
From the pulmonary trunk to the Aorta
What happens at birth to convert fetal circulation into adult circulation?
At birth the lungs expand, fetal lung fluid is expelled, and the right to left shunts terminate due to a change in the systemic and pulmonary resistance once the placenta is disconnected
What is the ductus venosum?
Takes blood from the Umbilical vein to the IVC?
When does the PDA normally close?
Around 1-2 days of age
What is a Grade III murmur?
Easily Audible
What is a Grade IV murmur?
Easily audible and associated with a thrill
What is a grade VI murmur?
Easily audible, with thrill and can be heard without steth!
What are causes of heart failure in a neonate?
Obstructed (duct dependant) systemic circulation: Hypoplastic left heart syndrome Critical aortic valve stenosis Severe coarction of the aorta Interruption of the aortic arch
What are causes of heart failure in infants?
High pulmonary blood flow:
- VSD
- AVSD
- Large and persistent PDA
What are causes of HF in children and adolescents?
Right or Left heart failure:
- Eisenmenger’s syndrome (RHF only)
Rheumatic disease
Cardiomyopathy
Are Left to right shunts cyanotic?
No. mainly present as breathless of asymptomatic.
Name 4 left to right shunts:
ASD, VSD, PDA, AVSD
Is obstruction of the heart cyanotic or acyanotic? And list examples!
Acyanotic: CoA, Atrial stenosis, Pulmonary stenosis
Examples of right to left shunt:
ToF, Ebstein’s anomaly. These are cyanotic!
Example of other Cyanotic diseases?
TGA, Truncus arteriosus, TAPVC, Tricuspid atresia, pulmonary atresia, HLHS, IAA
What are signs of Cyanosis?
Blue mucous membranes, nail beds and skin.
What is secondary ASD and is it more or less common?
Ostium secundum located within the fossa ovalis and arises from arrested growth of the septum secundum or excessive absorption of the septum primum.
Accounts for 80% of ASD
What genetic syndrome is associated with Ostium Secundum?
Holoram syndrome
What is primary ASD and association?
Arises if the septum primum does not fuse with the endocardial cushions, leaving a defect at the base of the interartrial septum that is usually large.
Seen more commonly in down syndrome!
What is a sinus venosus in ASD?
Defect located at the entry of the superior vena cava into right atrium
What is the natural history of ASD?
80-90% spontaneous closure rate if diammeter less than 8mm
What murmur is heard with ASD?
o An ejection systolic murmur best heard at the upper left sternal edge – due to increased flow across the pulmonary valve because of the left to right shunt.
o A fixed and widely split second heart sound (often difficult to hear) – due to the right ventricular stroke volume being equal in both inspiration and expiration
o With a partial AVSD, an apical pansystolic murmur from AV valve regurgitation
What are ECG changes seen in secundum ASD?
A Right bundle branch block is common, RAD due to right ventricular enlargement
Is ASD, L–> R or R–> L?
Left to R
What does a partial AVSD show on ECG?
a ‘superior’ QRS complex (mainly negative). This occurs because there is a defect in the middle part of the heart where the AV node is – the displaced node then conducts to the ventricles superiorly.
What is the most common CHD?
VSD! (30-50%)
What is Roger’s disease?
Small VSD
What sounds are associated with VSD?
Pansystolic murmur, Mid diastolic murmur (due to tubulent flow over mitral valve)
What is the management of a large VSD (CHF):
High calorie milk via NG tube, Frusemide and Ace-i
What is a complication of VSD and how can it be prevented?
– Eisenmenger syndrome. Surgery at 3-6mo can prevent this.
What is PDA associated with?
Congenital rubella syndrome
What are four features seen on physical exam in PDA?
Murmur, Pulse, Pulse and ____precordium?
Heavy machinery murmur (at infraclvicular area), High pulse rate, Wide pulse pressure (as pulmonary artery pressure less than aortic pressure) and hyperactive precordium
What is the link between PDA and RDS?
They can co-exist! And when the RDS resolves after 3-5 days, there is a decrease in pulmonary circulation which suddenly increases the left to right shunting resulting in an increase in ventilator requirement?
What is the management of PDA?
Indomethacin: PGE2 antagonist (PGE2 maintains ductus arteriosus Patency)).
In term infants catheter for surgical closure if PDA causes respiratory compromise or FTT or continues after 3rd month of life.
What is also termed endocardial cushion defect?
Atrioventricular septal defect - CHD whereby there is a large defect involving both atrial and ventricular septum’s which allows blood to freely flow between the two ventricles and atriums as a left to right shunt
What is AVSD associated with? And what murmur?
Down syndrome! Pansystolic.
Is coarctation of the aorta duct dependent/independant
dependant!
What is the pathophys behind CoA?
Due to the arterial duct tissue encircling the aorta just at the point of insertion of the duct. When the duct closes, the aorta also constricts, causing severe obstruction to the left ventricular outflow. This is the commonest cause of collapse due to left outflow obstruction
What is coarctation of the aorta associated with?
It’s associated with Turner’s Syndrome and bicuspid aortic valve. Rarely - Takasayu’s arteritis
When does CoA usually present?
on about day 2 once the PDA begins to close!
Signs/Sx of CoA?
Sick neonate with severe heart failure!
Radio-femoral delay or absent/weak femoral pulse
May have metabolic acidosis, proximal hypertension
What murmur is associated with CoA?
Continuous or late systolic murmur over thoracic spine.
What are two signs seen on CXR with CoA?
Rib notching - posterior one third of the third to eighth ribs (due to erosion by the large collateral intercostal arteries running posteriorly to the ribs)
Figure 2 sign - due to pre and post-coarctation dilation.
What is the management of the CoA? (3 surgical tech)
Keep duct open with prostaglandin infusion?
Removal of constricted section or use of subclavian arty to enlarge constricted part or balloon angioplasty?
What are the two most common types of Aortic stenosis?
Valvular (75%) and subvalvular (20%)
What Aortic stenosis is associated with William’s syndrome?
Supravalvular
What is AS associated with and should be checked for?
Mitral valve stenosis and CoA.
What is AS?
Aortic valve leaflets are partly fused together, giving a restrictive exit from the left ventricle
What is the presentation of AS?
and clinical features?
CHF in neonate! May hear Ejection systolic murmur at RUSB, Small volume with slow rising pulse and carotid thrills (ALWAYS)
Most common Mx of AS?
Balloon valvuloplasty!
Most common type of Pulmonary stenosis?
Valvular (90%)
Definition of critical pulmonary stenosis?
Inadequate pulmonary blood flow, dependent on ductus for oxygenation, progressive hypoxia and cyanosis
Which one of AS or PS is duct dependent?
PS
Signs of Pulmonary Stenosis?
Wide split S2 on expiration, SEM at LUSB, Pulmonary ejection click, Heave
What does CXR show in Pulmonary Stenosis?
Post-Stenotic dilatation of the main pulmonary artery
What is the pathophysiological reasoning for Acute Rheumatic fever?
Inflammatory disease due to antibody cross-reactivity following Group A streptococcus infection (pyogenes). Affected only by the sore throat
For Acute RF: Initial episode- _ Major or _ major plus _ minor + evidence of recent GAS infection
2 Major or 1 Major and 2 minor
Major manifestations of Acute RF (STREP):
Syndhems Chorea Transient Migratory polyarthritis Rare Subcutaneous nodules Erythema Marginatum (small lumps) Pancarditis
What is the pathophysiology in Eisenmenger’s Syndrome?
Combination of increased pressure in the (due to endurance) in the pulmonary vasculature + reduction in the size of the shunt. This in turn creates a right to left shunt – which is Acyanotic
What are the 5 T’s of cyanotic heart disease?
- Truncus arteriosus
- Transposition of the great arteries
- Tricuspid atresia
- Tetralogy of Fallot
- Total Anomalous pulmonary venous return
What syndrome is ToF associated with?
Digeorges syndrome
What are the four cardinal features of Tetralogy of Fallot? (DROP)
(Defect -VSD, RVH, Overriding aorta, Pulmonary stenosis)
- Large VSD
- Overriding Aorta - it is placed over the VSD so connected to both right and left ventricle
- Sub-pulmonary stenosis causing right ventricular outflow tract obstruction
- Right ventricular hypertrophy
What determines the shunt direction in ToF?
Degree of RVOTO determines the direction and change of shunt. If the right ventricular obstruction is significant enough to increase resistance, it will be easier for blood to cross the VSD into the left ventricle and go out the aorta, which now becomes the path of least resistance.
What condition are “tet” spells associated with?
ToF!
Hypoxic “tet” spells (increased cyanosis followed by syncope)
During exertional states the increasing pulmonary vascular resistance and decrease in systemic resistance causes an increase in right-to-left shunting -
Clinical features include paroxysm of rapid and deep breathing, irritability and crying, increasing cyanosis, decreased intensity of murmur )
What signs are present in ToF?
Single loud S2 due to severe pulmonary stenosis
Loud harsh Systolic ejection murmur (LSB). With increasing RVOTO which is predominantly muscular and below the pulmonary valve,
Clubbing - associated with cyanosis!
What CXR findings are seen with ToF?
Boot shaped heart, decreased pulmonary vasculature, right aortic arch
How are the “tet” spells managed in ToF?
O2, knee-chest position
Can also give fluid bolus and morphine sulphate with propanalol (peripheral vascoconstrictor and relieving the subpulmonary muscular obstruction)
A modified Blalock Taussig shunt is used in ToF - what is it?
surgical placement of an artificial tube between the subclavian artery and the pulmonary artery to increase pulmonary blood flow.
(it is also used in tricuspid atresia)
Is ToF Duct dependent?
May or may not be depending on the size of the VSD. But a duct may be needed if ROVFO is severe?
What is Ebstein’s anomaly?
The septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle which divide the right ventricle into two chambers and lead to regurgitation (atrialisation of the RV)
What is a possible cause of Ebstein’s Anomaly?
Possibly caused by maternal lithium and benzodiazepine use in the 1st trimester
Is Ebstein’s Anomaly associated with WPW?
YES
What is Transposition of the great arteries?
And what is survival dependent on?
Aorta and pulmonary artery switch places. Survival is dependent on PDA.
Technically two parallel circuits!
on CXR: Egg shaped heart with narrow mediastinum (egg on a string)
Transposition of the great arteries.
Mx of Transposition of the great arteries?
Prostaglandin infusion then Septostomy with balloon inserted to open the foramen ovale!
What is Truncus arteriosus?
Single great vessel gives rise to the aorta, pulmonary and coronary arteries
What is Truncus Arteriosus associated with?
Associated with VSD where the truncal valve overlies a large VSD -
Potential for coronary ischaemia with fall in pulmonary resistance
What is tricuspid atresia?
No Tricuspid valve making the right side small and non-functional. Creates a hypoplastic right ventricle.
What is needed to maintain flow from right atrium in Tricuspid atresia?
ASD and VSD/PDA hence pansystolic murmur.
What is a Glen or hemifontan operation?
Connecting the superior vena cava to the pulmonary artery
What is Pulmonary atresia?
Cyanotic, other, Duct dependent CHD whereby there is an absence of pulmonary valve
There are two types of Pulmonary Atresia:
- PA with intact ______– causing hypoplastic right ventricle
- PA with VSD – form of ______
- PA with intact interventricular septum – causing hypoplastic right ventricle
- PA with VSD – form of ToT
What is hypoplastic left heart syndrome?
There is under-development of the entire left side of the heart. Left ventricle hypoplasia may include atretic or stenotic mitral and/or aortic valve, small ascending aorta, and coarctation of the aorta with resultant systemic hypoperfusion
How do hypoplastic left heart syndromes present?
Very Sick after duct closes and with weakness or absence of all peripheral pulses
What is total anomalous pulmonary venous connection?
All pulmonary veins drain into right sided circulation (systemic veins, RA) drain into the SVC
No direct oxygenated pulmonary venous return to left atrium
Often associated with obstruction at connection sites
ASD must be present for oxygenated blood to shunt into the LA and systemic circulation
A snowman heart seen on CXR:
Total anomalous Pulmonary venous connection
What is interrupted aortic arch?
A part of the aortic arch is absent leading to severe obstruction to blood flow to the lower part of the body. No connection between the proximal and distal duct, so that the cardiac output is dependent on right to left shunt via the duct
To maintain circulation, the ductus arteriosus connects to the descending aorta, and there is a VSD
What are some clinical signs seen in an infant with heart failure?
feeding difficulties, early fatigability, diaphoresis while sleeping or eating, respiratory distress, lethargy, FTT
What are commoner causes of HF in an older child?
Eisenmenger syndrome, Rheumatic heart disease, cardiomyopathy
Kawasaki disease is a ____ vessel vasculitis with predilection _________
Medium sized vasculitis with predilection for coronary arteries
What is the most common age group affected by kawasaki?
Peak age 3mo – 5yr
What is the diagnostic criteria for Kawasaki?
Fever of unknown origin persisting for 5 days +
4/5 of CREAM:
1. Bilateral CONJUNCTIVITIS - non exudative
2. Polymorphous RASH - truncal Exanthema
3. Peripheral Erythema/Edema: Peeling from tips of fingers and toes
4. Cervical lymphADENOPATHY
5. Mucosal changes: Strawberry tongue, Red-infected fissured lips and injected pharyxn
In Kawazaki disease, ____ increase in the ____ week of illness
Platelets increase in the 2nd week of illness
What Ix would you do in suspected Kawasaki disease?
Check inflammatory markers (CRP, ESR, WCC) and platelets. .
ECHO - looking for mycardian disease, pericardial effusion and coronary aneurysm formation (consider angiography or MRI)
Mx of kawasaki disease?
- High (anti-inflammatory) dose of Aspirin when febrile. Low dose of aspirin in subacute phase until platelets normalise or longer if there is coronary artery invlvement to reduce the risk of thrombosis.
- IV immunoglobulin (IVIG) (2g/kg) within 10d of onset reduces the risk of coronary aneurysm formation. All future vaccines delayed by 6mo following IVIG
- Follow up with ECHO’s
- Some kids may need warfarin or another dose of IVIG if recurring fever.
Persistent inflammation and fever may require treatment with infliximab
Complications of Kawasaki disease?
Coronary artery vasculitis with aneurysm formation occurs in 20-25% of untreated children,
Acute Rheumatic fever results from…
Antibody cross-reactivity following Group A streptococcus infection (pyogenes). Affected only by the sore throat
treatment of Acute RF:
- Analgesia (paracetamol or codeine)
- Oral penicillin - Phenoxymethicillin?
- Carbamazepine or Sodium Valproate for Syndenham’s Chorea
- Frusemide or Spironolactone for pancarditis
Where are aschoff nodule/bodies seen?
Granulomatous nodules found in the heart in Rheumatic heart disease
Most common Arrhythmia in childhood?
SVT
Presence of anti-Ro or Anti-La antibodies in the mother’s serum can lead to…
Congenital complete heart block
In congenital complete heart block the antibodies …
Antibody prevent normal development of the electrical conduction system in the developing heart, with atrophy and fibrosis of the AV node.
Four S’s of a physological (flow murmur)?
aSymptomatic murmur
Soft blowing murmur
Systolic murmur
Left Sternal edge
FIXED wide splitting of S2
ASD
Most common cardiac abnormality associated with Down Syndrome?
AVSD
Usually asymptomatic but large PDA may result in ____
Pulmonary Hypertension
Absent femoral pulses on baby check
CoA
Baby with murmur presents with acute circulatory collapse about 2 days of age when the duct closes
CoA
Noonan syndrome is related to which heart defect?
Pulmonary stenosis
How do you differentiate Pulmonary stenosis murmur to Aortic stenosis murmur?
AS radiates to the carotids, PS doesn’t.
How do you treat a TeT spell?
DRSABCD Sedation and pain relief Propanolol to relieve the muscular obstruction UV fluids Bicarbonate correct acidosis
Boot shaped heart?
ToF
How do kids with Transposition of the great arteries survive?
Via either an ASD, VSD or PDA
CXR: Egg on string appearance, egg shaped heart with narrow mediastinum
Transposition of the great arteries
Pulmonary veins are connected to the the right side and drain into the SVC - hence no direct oxygenated pulmonary venous return to the left atrium
Total Anomalous pulmonary venous connection
Banage a kid that presents with a murmur and then becomes cyanotic on day 2 of life?
PGEF infusion
In tricuspid atresia the PDA is needed for the blood to go from the _______ to the ______. Whereas in hypoplastic left heart syndrome - the opposite happens
In tricuspid atresia the PDA is needed for the blood to go from the Aorta to the pulmonary artery. Whereas in hypoplastic left heart syndrome - the opposite happens
SVT is any arrhythmia that originates above _____
the bundle of HIS
WPW occurs because there’s an accessory pathway called ________
Bundle of Kent
Which heart problem is associated with Congenital sensorineural deafness - the Kervell and Lange-Neilsen Syndrome
Long Qt syndrome
How do you manage congenital Long QT syndrome?
- BETA BLOCKERS
- Avoid triggers and prolonging drugs
- Genetic testing?
