Paeds - CVS Flashcards
(58 cards)
1
Q
What are the 3 foetal shunts?
A
Ductus venosus = connects umbilical vein to inferior vena cava (allows blood to bypass liver)
Foramen ovale = connects RA to LA (allows blood to bypass RV and Pulmonary circulation)
Ductus Arteriosus = connects pulmonary artery with aorta (allows blood to bypass Pulmonary circulation)
2
Q
What happens to the foetal shunts and circulation at birth?
A
- first baby breath expands alveoli
- this decreases pulmonary vascular resistance, causing a fall in pressure in the RA
- When LA pressure becomes greater than RA pressure, this squashes the atrial septum and causes functional closure of the foramen ovale
- Foramen ovale gets sealed shut structurally after a few weeks and becomes the fossa ovalis
- Increased blood oxygenation causes a drop in circulating prostaglandins
- prostaglandins are required to keep the ductus arteriosus open
- Therefore this drop in P causes the ductus arteriosus to close and become the ligamentum arteriosum
- ductus venosus stops functioning immediately after birth as umbilical cord is clamped so there is no flow in the umbilical veins
- the DV structurally closes a few days later and becomes the ligamentum venosum
3
Q
Sometimes the DA doesn’t close for unknown reasons.
(potentially Rubella maternal infection or Prematurity)
What is the pathophysiology of a Patent Ductus Arteriosus.
A
- pressure in the aorta is higher than pressure in the pulmonary vessels so blood flows from the aorta to the pulmonary artery
- Blood crossing from the L to the R circulation = L to R shunt
- This causes increased pressure in the pulmonary vessels = pulmonary HTN
- this leads to R sided Heart Strain as the RV is having to work harder to contract against this higher resistance
- Pulmonary HTN + R Heart Strain = RV Hypertrophy
- The increased blood flowing through the pulmonary vessels and returning to the L side of the heart = L ventricular hypertrophy
4
Q
How would a patent ductus arteriosus present clinically?
A
- usually picked up during the newborn examination with a murmur
- small PDA may not have abnormal heart sounds
- significant PDA causes a normal S1 with a continuous crescendo-decrescendo “machinery” murmur that may continue into S2 (makes S2 difficult to hear)
- SOB
- Difficulty feeding
- Poor weight gain
- Lower Resp Tract Infections
5
Q
How would you deal with a suspected PDA?
A
- confirm PDA with echocardiogram and doppler flow studies to assess the size and characteristics of L to R shunt
- Monitor until 1 years old with Echocardiogram
- If it has not closed in 1 year, it is unlikely it will close spontaneously
- trans-catheter or surgical closure can be performed
- If PDA is symptomatic or there is evidence of heart failure, treat the PDA sooner
6
Q
What is the pathophysiology of an atrial septal defect?
A
- an ASD is a hole in the septum between the two atria
Embryology
- two walls grow down from the top of the heart and fuse to farm the endocardial cushion in the middle of the heart to separate the atria (septum primum and septum secondum)
- defects with these two walls can cause ASD
- the Foramen Ovale is a small hole in the septum secondum that closes at birth
Pathophysiology
- ASD leads to a shunt of blood from the LA to the RA (LA pressure is higher)
- so blood continues to flow through pulmonary vessels to lungs to be oxygenated, so the patient does not become cyanotic
- However increased flow to the R side of the heart = R sided overload and R heart strain.
- this eventually leads to R heart failure and Pulmonary HTN
- Eventually pulmonary HTN can lead to Eisenmenger syndrome (where pulmonary pressure gets greater than systemic pressure so the shunt reverses to be a R to L shunt across the ASD)
- the blood then just bypasses the lungs
- patient becomes cyanotic
7
Q
Name some atrial septal defects (commonest to least common)
A
- Commonest = ostium secondum (septum secondum fails to fully close)
- patent foramen ovale
- ostium primum (septum primum fails to fully close)
- typically results in atrioventircular valve defects though, so is classified as an atrioventricular septal defect
8
Q
What are some complications of an ASD?
A
-
Stroke!!!
- typically when patients have a DVT the clot travels to the R side of the heart and becomes stuck in the lungs as a PE
- in an ASD, the clot can travel across from the RA to the LA and can go through the LV, aorta, and up to the brain to cause a large stroke
- Atrial fibrillation and Atrial flutter
- Pulmonary HTN and R sided HF
- Eisenmenger syndrome
9
Q
How would an ASD present?
A
- usually either picked up on antenatal scans or newborn examinations
- Murmur = ejection-systolic, crescendo-decrescendo murmur
- loudest at the upper left sternal border with a fixed split S2
- the splitting of the S2 is where you hear the closure of the aortic and pulmonary valves at slightly different times
Some people are asymptomatic in childhood, then present in adulthood with dyspnoea, HF or stroke.
- Childhood symptoms:
- SOB
- difficulty feeding
- poor weight gain
- Lower Resp Tract Infections
10
Q
How would you manage an ASD?
A
- refer to a paediatric cardiologist
- if ASD is small and asymptomatic = watching and waiting
- Surgery = transvenous catheter closure via femoral vein
- or open heart surgery
- Anticoagulants (aspirin, warfarin, NOACs) to reduce risk of clots and stroke in adults
11
Q
What is the pathophysiology of ventricular septal defects?
A
- Can be tiny or involve the entire septum to form one large ventricle
- Often associated with Down’s Syndrome and Turners Syndrome
Pathophysiology:
-
Blood shunts from LV to RV (higher pressures in LV)
- blood is still flowing around the lungs and being oxygenated before going round the body so the patient is not cyanotic
- L to R shunt results in R Sided Overload, R HF and increased flow to Pulmonary vessels, leading to Pulmonary HTN
- Eventually pulmonary HTN can result in the R sided Heart pressures to be greater than the L, resulting in a R to L shunt
- this shunt means that blood avoids the lungs, resulting in cyanosis
- This is Eisenmenger Syndrome
12
Q
How would ventricular septal defects present?
A
- can present on antenatal scans or as a murmur on newborn baby check
- pansystolic murmur
- most prominently heard at Left Lower Sternal Border in the 3rd and 4th ICS
- Palpation may feel a systolic thrill
- initially symptomless and can present as late as adulthood
- Typical symptoms:
- poor feeding
- dyspnoea
- tachypnoea
- poor feeding
- failure to thrive
13
Q
What are other causes of a pansystolic murmur?
A
Think ventricular septal defect,
mitral regurgitation,
Tricuspid regurgitation
14
Q
How would you manage a ventricular septal defect?
A
- asymptomatic small VSD with no evidence of pulmonary htn or HF = watched as they can close spontaneously
- Surgery = transvenous catheter closure via femoral vein
- or open heart surgery
- Recommend antibiotic prophylaxis in surgical procedures as VSD patients are at a greater risk of infective endocarditis
15
Q
What 3 lesions commonly result in Eisenmenger syndrome?
A
- Atrial Septal Defect
- Ventricular Septal Defect
- Patent Ductus Arteriosus
16
Q
How would Eisenmenger syndrome present?
(findings associated with pulmonary htn, r to l shunt and chronic hypoxia, the different septal defects)
A
- typically presents in 1-2 years in those with large shunts, or in adulthood with small shunts
- can develop more quickly in pregnancy so monitor women with defects closely with echo
Examination findings associated with pulmonary htn…
- Right ventricular heave (RV pushing forcefully against higher pressure in the lungs)
- Loud S2 due to forceful shutting of pulmonary valve
- Raised JVP
- Peripheral Oedema
Examination findings related to R to L shunt and chronic hypoxia…
- cyanosis
- clubbing
- dyspnoea
- plethoric complexion (red complexion related to polycythaemia)
Examination findings associated with septal defect…
- ASD = ejection-systolic, crescendo-decrescendo murmur
- loudest at upper L sternal border
- VSD = pansystolic murmur
- loudest at L lower sternal border
- PDA = continuous crescendo-decrescendo “machinery” murmur
- Arrhythmias
17
Q
How would you manage Eisenmenger syndrome?
A
- the underlying defect should have been managed optimally or corrected surgically to avoid Eisenmenger syndrome
- Once the pulmonary htn gets high enough to cause Eisenmenger, the only definitive treatment is a heart-lung transplant
- Medical management:
- O2 can manage symptoms but does not affect outcomes
- treatment of pulmonary htn with sildenafil
- treatment of arrhythmias
- treatment of polycythaemia with venesection
- prevention and treatment of thrombosis with anticoagulation
- prevention of infective endocarditis using prophylactic antibiotics
18
Q
What is the pathophysiology of Coarctation of the aorta?
A
- a congenital condition where there is narrowing in the aortic arch, usually around the ductus arteriosus.
- narrowing reduces the pressure of blood flowing to the arteries distal to the narrowing
- it increases the pressure in areas proximal to the narrowing eg. the heart and the first 3 branches of the aorta
- associated with Turner’s Syndrome
19
Q
How would coarctation of the aorta present?
A
Initially in a neonate…
- weak femoral pulses
- when you do a 4 limb blood pressure, there will be high BP in limbs supplied from arteries that come before the narrowing and lower BP in limbs that are supplied from arteries that come after
- Ejection Systolic murmur at L upper sternal edge
- Tachypnoea and increased work of breathing
- poor feeding
- grey and floppy baby
- Rib notching on CXR = “3 sign”
Over time…
- Left ventricular heave due to LV Hypertrophy
- underdeveloped left arm (due to reduced flow from Left Subclavian artery)
- underdevelopment of the legs
20
Q
How would you manage coarctation of the aorta?
A
- In mild cases the patient won’t have symptoms or require surgery until adulthood
- In severe cases, they may need emergency surgery at birth
- Critical cases where there is a risk of HF and death shortly after birth:
- Prostaglandin E is used to keep the ductus arteriosus open while awaiting surgery shortly after birth
- this allows some blood to flow through the DA into systemic circulation distal to the coarctation
- surgery is then performed to correct coarctation and ligate ductus arteriosus
21
Q
Describe the pathophysiology of congenital aortic valve stenosis
A
- narrow aortic valve that restricts blood flow from the LV into the aorta
- the aortic valve is usually made up of 3 leaflets (the aortic sinuses of Valsalva) which prevent blood from backflowing back into the LV
- patients with Aortic Stenosis may have one, two, three or 4 leaflets.
22
Q
How would aortic stenosis present?
A
- can be totally asymptomatic and accidentally discovered as a murmur and other signs during routine examination:
- Ejection systolic murmur heard loudest at aortic area (2nd ICS, R sternal border)
- Crescendo-decrescendo murmur that radiates to carotids
- Ejection click before murmur
- Palpable thrill during systole
- Slow rising pulse and narrow pulse pressure
- severe aortic stenosis = fatigue, SOB, dizziness, fainting
- symptoms are typically worse on exertion as outflow from LV cannot keep up with demand
- Really severe aortic stenosis can present with HF within months of birth
23
Q
How would you manage aortic stenosis?
A
- diagnose with an echocardiogram
- follow up with echocardiograms, ECGs and exercise testing to monitor, as congenital AS worsens over time
- patients with significant stenosis may need to restrict physical activities
- Monitor for Left Ventricular Outflow Tract Obstruction, HF, Ventricular arrhythmia, Bacterial Endocarditis
Management of stenosis:
- percutaneous balloon aortic valvoplasty
- surgical aortic valvotomy
- valve replacement
24
Q
Describe the pathophysiology of pulmonary valve stenosis?
A
- pulmonary valve usually consists of 3 leaflets that prevent blood returning to the heart
- the leaflets can develop abnormally, thickened, or fused
- this can result in a narrow opening between the right ventricle and the pulmonary artery
Can be associated with…
- Tetralogy of Fallot
- William Syndrome
- Noonan syndrome
- Congenital Rubella Syndrome
25
How would congenital pulmonary stenosis present?
* often completely asymptomatic and discovered as an incidental finding of a murmur during routine baby checks
* more significant PVS = fatigue on exertion, SOB, dizziness, fainting
Signs...
* *_Ejection systolic murmur_*
* heard loudest in pulmonary area (*_2nd ICS, L sternal border_*)
* **Palpable thrill** in pulmonary area
* **Right ventricular heave** due to RV hypertrophy
* **Raised JVP** with **giant a waves**
26
How would you manage congenital pulmonary stenosis?
* diagnose with an **echocardiogram**
* Mild without any symptoms generally just watching and wait
* symptomatic or significant stenosis:
* **balloon valvuloplasty** via a venous catheter (done through the femoral vein, through the inferior vena cava and R side of the heart to the pulmonary valve, using **Xray Guidance**)
* open heart surgery if necessary
27
What are the 4 conditions of the tetralogy of fallot?
4 congenital conditions..
Ventricular Septal Defect
Overriding Aorta
Pulmonary Valve Stenosis
Right Ventricular Hypertrophy
28
How do the 4 conditions of the tetralogy of fallot result in the pathology?
VSD
* allows blood to flow between ventricles
Overriding aorta
* means the entrance to the aorta _(aortic valve) is further to the right than normal, above the VSD_
* this means that when the RV contracts and sends blood upwards, the aorta is in that vicinity and a greater proportion of _deoxygenated blood is likely to enter the aorta_ from the R side of the heart
Stenosis of the Pulmonary Valve
* there is a _greater resistance_ against blood flow out of the RV
* this _encourages blood to flow through the VSD and into the aorta_, rather than the normal route to the pulmonary vessels
* The pulmonary stenosis + overriding aorta both encourage blood to be shunted from R to L, _bypassing the lungs_ and resulting in **cyanosis**
Right Ventricular Hypertrophy
* this results due to the _RV having to pump harder against the resistance of the LV and pulmonary stenosis_
* the Right heart muscle thickens due to the strain
29
What are some risk factors for the tetralogy of fallot?
* Rubella infection
* increased age of mother \>40 years old
* alcohol consumption in pregnancy
* diabetic mother
30
How would tetralogy of fallot present?
* usually picked up in antenatal scans
* ejection systolic murmur (due to pulmonary stenosis) is heard on newborn baby check
* loudest in pulmonary area (2nd ICS, left sternal border)
* severe cases can present with HF \<1 years old
* milder cases present with symptoms of HF later
Signs and symptoms...
* cyanosis
* clubbing
* poor feeding
* poor weight gain
* Tet spells
31
How would you investigate tetralogy of fallot?
* **Echocardiogram** for diagnosis
* **doppler flow studies** show direction of bloodflow to assess severity of abnormality and shunt
* CXR can show **"boot shaped" heart** due to RV thickening
32
What is a Tet spell?
Intermittent symptomatic periods where the R to L shunt becomes temporarily worse, causing a **cyanotic episode.**
* this occurs when pulmonary vascular resistance increases or systemic resistance decreases
* Eg. in physical exertion, lots of CO2 is generated. CO2 causes systemic vasodilation and reduces systemic vascular resistance. Blood flows down the path of least resistance so it will flow from RV to aorta instead of through pulmonary vessels.
33
How would you manage a Tet spell?
* squat or position their knees to their chest
* **squatting** increases systemic vascular resistance to encourage blood to enter pulmonary vessels
* **Supplementary O2** in hypoxic children
* **Beta blockers** can relax RV and improve blood flow to pulmonary vessels
* **IV fluids** increase pre-load, so increase the vol of blood flowing to pulmonary vessels
* **Morphine** can decrease resp drive and cause more effective breathing
* **Sodium bicarbonate** can buffer metabolic acidosis if it happens
* **Phenylephrine infusion** increases systemic vascular resistance
34
How would you manage tetralogy of fallot in the long term?
* **prostaglandin infusion can be used in neonates** to maintain the ductus arteriosus
* allows blood to flow from aorta back into pulmonary arteries
* **Total surgical repair by open heart surgery** is the definitive treatment
35
Describe the pathophysiology of Ebstein's anomaly
* a congenital heart condition where the **tricuspid valve** is set lower in the R side of the heart (towards apex)
* this results in a _bigger RA and smaller RV_
* This leads to _poor flow from the RA to RV_, and therefore _poor flow to the pulmonary vessels_
* Often associated with **R to L shunt** across the atria **via an atrial septal defect**
* when this happens, blood bypasses the lungs = **cyanosis**
* Also associated with **Wolff-Parkinson-White syndrome**
36
How would Ebsteins anomaly present?
Symptoms in patients with an associated ASD often present shortly after birth when the DA closes.
* The DA allows blood to flow from the aorta into the pulmonary vessels to be oxygenated and minimses cyanosis
* when the Ductus arteriosus closes, the patient becomes cyanotic and symptomatic
Typical symptoms...
* evidence of HF (eg. oedema)
* *_gallop rhythm heard on auscultation_* characterised by addition of *_3rd and 4th heart sounds_*
* cyanosis
* SOB and tachypnoea
* poor feeding
* collapse or cardiac arrest
37
How would you investigate ebsteins anomaly?
ECG:
* arrhythmias
* RA enlargement
* RBBB
* Left Axis deviation
CXR
* Cardiomegaly
* RA enlargement
Echocardiogram confirms diagnosis and severity
38
How would you manage ebsteins anomaly?
Management =
* treat arrhythmias and HF
* Prophylactic antibiotics to prevent infective endocarditis.
* surgical correction of underlying defect is definitive management
39
Describe the pathophysiology of transposition of the great arteries
* This is where the attachments of the _aorta and the pulmonary trun_k to the heart, are swapped
* Ie RV pumps blood into the aorta and LV pumps blood into the pulmonary vessels
* Systemic circulation is on the Right and Pulmonary system is on the Left and there is no connection between the two
* This condition becomes life threatening at birth, as in the womb gas exchange happens across the placenta so blood flow to the lungs doesn't matter
* There is _no connection between systemic and pulmonary circulation_, so baby becomes **cyanosed**
* **immediate survival depends on if there is a shunt** between the systemic and pulmonary circulation (eg. PDA, ASD, VSD)
40
How would transposition of the great arteries present?
* often diagnosed with antenatal US scans
* or presents as **cyanosis** at or within a few days of birth
* a _PDA or VSD_ can initially compensate by allowing blood to mix between systemic circulation and lungs
* however within a few weeks they present with **resp distress, tachycardia, poor feeding, poor weight gain, sweating**
41
How would you manage transposition of the great arteries?
* **VSD** will alow some mixing of blood between the two systems and allow some time to decide a definitive treatment
* **Prostaglandin infusion** can be used to _maintain DA_ to allow blood from aorta to flow to pulmonary arteries for oxygenation
* **Balloon septostomy** = insert a catheter into foramen ovale via umbilicus and inflate a balloon to _create a large ASD_
* this allows blood returning from lungs on the left, to flow to the Right side of the heart and out through the aorta to the body
* **Open heart surgery** is definitive management
* **Cardiopulmonary bypass machine** is used to perform an "arterial switch" procedure within days of birth
* also correct VSD and ASD at the same time
42
What are the most common underlying congenital heart diseases at risk of infective endocarditis?
* Ventricular septal defects
* Patent Ductus Arteriosus
* Aortic Valve abnormalities eg. bicuspid AV
* Tetralogy of Fallot
43
What is the pathophysiology of Infective Endocarditis?
* in structural abnormalities, there is a significant pressure gradient or turbulent flow, causing **endothelial damage** through sheer stress forces
* prosthetic material can also cause endothelial damage, promoting a sterile **platelet-fibrin deposition** on this endothelium
* there then needs to be **bacteraemia** which adheres to the lesion and invades underlying tissue
* once attached to the lesion, the bacteria are protected within the vegetation from phagocytes and they can easily proliferate
44
What are some common microbes that are involved in IE
these organisms have specific surface receptors to fibronectin, that allow the microbe to adhere to the thrombus
* Staph aureus
* Strep Viridans (a-haem strep that is associated with _dental procedures_)
* Strep pneumonia
* HACEK organisms (Haemophillus, Actinobacillus, Cardiobacterium, Eikenella, Kingella)
* Group A/C/G Streptococci
* Candida albicans
45
What would make you suspect infective endocarditis?
* **persistent low grade fever** should make you consider IE (especially with hx of CHD, prev cardiac surgery, prosthetic material)
* **Change in murmur** (prev murmur usually present from turbulent flow around underlying heart disease etc)
Splenomegally
* **Petechiae**
* **Osler's nodes** on fingers/toes
* **Janeway lesions** on palms/soles
* **Splinter haemorrhages** on nail bed
* a sign of embolic phenomena. other signs are PE, haematuria from glomerulonephritis, seizures/hemiparesis due to cerebral emboli, **Roth spots** due to retinal haemorrhages
46
What are some features of a sub-acute or non-acute presentation of IE?
* fatigue
* malaise
* weight loss
* myalgia
* asymptomatic sometimes
* cardiac failure due to vavular destruction
47
How would you investigate suspected IE?
* **blood cultures**
* multiple samples over 48-72 hours from multiple sites
* **Echocardiography**
* to identify vegetations. however +ve does not confirm IE and -ve does not rule it out.
* **Bloods** (anaemia, leukocytosis, raised ESR suggest IE)
* Urine dip for **microscopic haematuria**
* **Modified Duke's Criteria**
* 2 major OR 1 major+3 minor OR 5 minor
48
How would you manage infective endocarditis?
* **empirical IV antibiotics** determined by sensitivities from cultures
* highly sensitive strep = _IV pen or IV ceftriaxone_ for 4 weeks
* pen resistent strep = _IV pen or IV ceft for 4 weeks + gentamicin for first 2 weeks_
* methicillin-resistant = _vancomycin_ for 6 weeks
* enterococcus = _IV pen + gentamicin_ for 4-6 weeks
* pen allergic = _vancomycin + gentamicin_ for 6 weeks
* HACEK = _ceftriaxone + gentamicin_ for 4 weeks
* fungal = _amphotericin B_
* prosthetic valve in situ = atleast 6 weeks antibiotics
Sometimes surgery is required
49
What is the pathophysiology of Rheumatic Fever?
* Acute Rheumatic Fever occurs 2-4 weeks after **pharyngitis** due to cross-reactivity to **Group A B-haem Strep** (GAS) (**Streptococcus pyogenes**)
* Strep pyogenes is a G+ve cocci
* produces 2 cytolytic toxins = streptolysin O and S
* Rheumatic strains of GAS have M proteins in their cell wall
* our B cells produce anti-M protein antibodies against the infection that cross react with other tissues eg. heart (rheumatic heart disease), brain, joints, skin
50
What are some clinical features of rheumatic fever?
(think about the diagnostic criteria)
Jones Diagnostic Criteria!!!
* **Positive throat culture for Group A b-haem streptococcus** OR **elevated anti-streptolysin O titres** OR **anti-deoxyribonuclease B titre**
_AND_
* 2 major OR 1major+2minor criteria
Major = SPECS
* **Sydenham's chorea**
* **Polyarthritis**
* **Erythema marginatum**
* **Carditis**
* **Subcutaneous nodules**
Minor = CAPE
* **CRP/ESR raised**
* **Arthralgia**
* **Pyrexia/Fever**
* **ECG - prolonged PR interval**
Patient may also present with a _recent hx of a sore throat or scarlet fever_.
In sever disease you may hear a _mitral diastolic murmur._
51
What are some differentials for an Acute Rheumatic Fever presentation and how would you differentiate them?
Septic arthritis = only one joint, +ve gram stain, culture, elevated WBC of aspirated synovial fluid
Reactive arthropathy = associated with urethritis and conjunctivitis, commonly males
IE = +ve blood culture, Echo shows vegetation on valves, Signs like janeway lesions, osler nodes, splinter haemorrhages
Myocarditis = troponin and CK elevated, ECG shows saddle ST or T wave changes
52
What investigations would you do for Rheumatic fever?
* Bloods (ESR, CRP, FBC, WBC)
* Blood cultures to exclude sepsis
* Rapid Antigen Detection Test
* Throat culture
* Anti-streptococcal serology (ASO and anti-DNASE B titres)
* ECG = prolonged PR interval
* CXR if carditis suspected
* Echocardiography
53
How would you manage Rheumatic Fever?
Initial
* antibiotics eg. **Benzathine Benzylpenicillin** 1st line
* pen allergy = cephalosporins, macrolides, clindamycin
* Aspirin or NSAIDs eg. naproxen or ibuprofen
* Assess for **Emergency Valve Replacement**
* Sever carditis = glucocorticoids and diuretics
Long term
* **secondary prophylaxis** with IM Benzathine Benzylpenacillin every 3-4 weeks
* OR Oral phenoxymethylpenicillin 2x daily
* OR Oral sulfadiazine daily
* OR Oral azithromycin in pen allergy
54
How would you recognise an innocent murmur?
7's inoSSents
* soft murmur
* S1 and S2 are normal (heart sounds are normal)
* Symptomless
* systolic
* short
* standing/sitting noises can change (typically louder supine)
* Special tests normal (ECG/CXR/ECHO)
also commonly at left sternal edge and don't radiate
55
AS, PS and Coarctation are some examples of outflow obstructions. How would you detect them?
Aortic Stenosis = ejection systolic murmur
Pulmonary stenosis = ejection systolic murmur
Coarctation = ejection systolic murmur at L upper sternal edge, high BP in the right arm, RadioFemoral delay
56
What would you suspect in a blue baby?
Tetralogy of Fallot (2+ days of life, cyanotic on crying)
Transposition of the great arteries (can present from birth, may not have a murmur)
AVSD (cyanosis + downs)
Eisenmenger's syndrome (acquired cyanotic heart disease)
57
What are the defects associated with certain congenital conditions?
* Downs
* Turners
* Trisomy 13 (Pataus) + 18 (Edwards)
* Williams
* Noonans
Downs = AVSD, VSD, ASD
Turners = coarctation, AS
Trisomy 13/18 = complex septal defects
Williams = supravalvular aortic stenosis
Noonans = tricuspid stenosis, septal defects
58
Kawasaki disease is a generalised vasculitis that involves small to medium sized vessels.
In 1/3 of these cases there are risks of coronary artery aneurysms so you must do follow up echos.
When would you suspect Kawasakis disease?
* Fever for **\>5 days**
OR 4 of...
* **conjunctivitis**
* **red mucous membranes** (strawberry tongue)
* cervical lymphadenopathy
* generalised **rash**
* palm changes (redenning or oedema of palms and soles, membranous desquamation of finger and toe tips)
Manage with IVIGs and Aspirin for 6 months
