Cardio/Resp/Renal Flashcards
Most common rheumatic disease of childhood
Juvenile idiopathic arthritis
Most common subtype of juvenile idiopathic arthritis
Oligoarthritis (40-50%), then polyarthritis (25-30) and systemic JIA (5-15)
Hirschsprung disease - histology
Absence of Meissner’s and Auerbach’s plexuses
Hypertrophied nerve bundles
High concentrations of acetylcholinesterase between muscle and submucosa layers
Workup for first presentation T1DM
T1DM antibodies (insulin antibodies, glutamic acid decarboxylase antibodies, zinc transporter 8 antibodies), coeliac screen (total IgA, anti-gliadin and anti-tissue transglutaminase antibodies), TSH and free T4
Leading cause acquired heart disease in developed countries
Kawasaki disease
20-25% untreated develop coronary artery abnormalities - this reduces to 5% if treated with intravenous immunoglobulin (IVIg)
Most common extracranial solid tumour in children
Neuroblastoma
Most commonly diagnosed malignancy in infants
Neuroblastoma
Haemolytic uraemic syndrome - Coombs test
Positive - pneumococci induced (neuraminidase-producing strep pneumoniae)
Otherwise negative
Multiple endocrine neoplasia 1 - affected endocrine glands
Most to least common: Parathyroid glands (hyperparathyroidism) Anterior pituitary (prolactin, GH, ACTH) Endocrine pancreas (insulin, gastrin)
Haemolytic uraemic syndrome - aetiology
Infection - EHEC (Shiga producing) 90%, neuraminidase producing strep pneumo
Genetic - atypical, nondiarrhoeal
Drugs - ciclosporin, tacrolimus
Systemic disease with microvascular injury - malignant HTN, SLE, antiphospholipid syndrome
Multiple endocrine neoplasia 2 - affected endocrine glands
Medullary thyroid carcinoma (ALL patients)
Phaeochromocytoma
2A -> hyperparathyroidism
2B -> not hyperparathyroidism, but with neuromas and specific phenotype (Marfan-like)
Cystic fibrosis mutation classes
I - no protein produced II - retention of misfolded protein at endoplasmic reticulum -> degraded III - impaired channel opening IV - decreased flow of chloride ions V - decreased mRNA/protein/both VI - plasma membrane instability
Thrombotic microangiopathy - most common and triad
Most common is haemolytic uraemic syndrome
Triad of: 1) microangiopathic haemolytic anaemia, 2) thrombocytopaenia (consumptive), 3) renal insufficiency
21-hydroxylase - function
P450 enzyme
Converts progesterone into 11-deoxycortioesterone, eventually leading to aldosterone
Converts 17-hydroxyprogesterone to 11-deoxycortisol, eventually to cortisol
Kawasaki disease - diagnostic criteria
Fever at least 5 days (can be less with typical presentation and experienced consultation), plus 4/5 of (present at any time):
1) Conjunctivitis (bilateral, nonpurulent)
2) Lymphadenopathy (cervical, unilateral, tender, >1.5cm)
3) Rash (polymorphous, no vesicles or bullae)
4) Lips/oral mucosa (hyperaemia, erythema)
5) Extremities (hyperaemia, oedema, desquamation)
Most common life limiting recessive trait among whites
Cystic fibrosis
Cystic fibrosis - most common mutation
F508del - deletion of phenylalanine at amino acid 508
Cystic fibrosis - common airway pathogens
Staph aureus (esp early) Pseudomonas aeruginosa Burkholderia cepaeia
Bronchiectasis - definition
Irreversible abnormal dilation and anatomic distortion of the bronchial tree
CT is usually (?always) used/needed to confirm diagnosis
Most common cause intestinal obstruction 5mo-3yr
Intussusception
Most common abdominal emergency children <2yo
Intussusception
Neuroblastoma metastasis sites
Common: lymph nodes, long bones and skull, bone marrow, liver, skin
Rare: lung and brain (<3%)
Most common bacteria septic arthritis and osteomyelitis
Staphylococcus aureus
Most common cause acquired heart disease worldwide
Rheumatic heart disease
Hypoxaemia - causes (broad)
Hypoventilation
Diffusion limitation
Shunt (R->L)
VQ mismatch
Hypercapnia - causes (broad)
Hypoventilation
VQ mismatch
Restrictive lung disease (definition)
Reduction in functional lung volume - TLC < 80%
WIDENED ANION GAP METABOLIC ACIDOSIS
K = ketoacidosis U = uraemia (renal failure) L = lactic acidosis (ischaemia) T = toxins (ethylene glycol, methanol, aspirin (salicyclates), metformin)
NORMAL ANION GAP METABOLIC ACIDOSIS
C = chloride excess (eg. NaCl) A = acetazolamide, Addison’s G = GIT causes – diarrhoea, vomiting, fistula (pancreatic, ureters, biliary, small bowel, ileostomy) E = extra – RTA
Standard base excess
Reflects the metabolic component of an acid-base disorder
Normal value is 0
If BE <0, have less HCO3- than normal, therefore have metabolic acidosis
If BE >0, have more HCO3- than normal, therefore have metabolic alkalosis
Bronchiectasis
Irreversible abnormal dilatation and anatomic distortion of the bronchial tree and represents a common end stage of many non-specific and unrelated antecedent events
Common thread in the pathogenesis of bronchiectasis consists of difficulty clearing secretions and recurrent infections with a ‘vicious cycle’ of infection and inflammation -> injury + remodelling
Chronic suppurative lung disease
clinical syndrome in children with symptoms and/or signs of bronchiectasis but who lack a radiographic diagnosis of bronchiectasis
Most common cause bronchiectasis (Developed countries)
CF, followed by primary immunodeficiency
Primary ciliary dyskinesia
PCD is an inherited disorder characterised by impaired ciliary function
Includes = ciliary akinesia, dyskinesia and aplasia
Common consequence is impaired mucociliary clearance
Key features
- Chronic sinuopulmonary disease
- Persistent middle ear effusions
- Laterality defects
- Infertility
The common defect in PCD is the absence of dynein arms, the structures necessary for the generation of movement of cilia and sperm tails
Typically autosomal recessive
CF gene carrier rate
1/25
Most common cause croup
Parainfluenza viruses (1,2,3) account for 75%
Most common cause stridor (chronic/noninfective)
- Larygomalacia
- Congenital subglottic stenosis
- Vocal cord paralysis
Most common cause of secondary tracheomalacia
Aberrant innominate artery (AKA brachiocephalic artery)
ii. May be asymptomatic and discovered incidentally, OR cause severe symptoms
iii. Expiratory wheezing and cough occur, and rarely, reflex apnoea or ‘dying spells’
iv. Surgical intervention rarely necessary
v. Infants are treated expectantly as the problem is self-limited
Most common cardiomyopathy
Dilated (60% of childhood cardiomyopathy)
Dilatation -> impaired contraction -> systolic dysfunction
Cause: idiopathic, myocarditis (infective, viral), genetic
Intracavitatory thrombus is common -> PE and systemic emboli
90% present with heart failure
In infants, anomalous coronary origin from a pulmonary artery must be excluded (ALCAPA)
Recent review – 1/3 die, 1/3 recover completely, 1/3 improve with some residual cardiac dysfunction
Doxorubicin cardiomyopathy
a. Becoming one of the most common causes of chronic CHF in children
b. Non-linearly dose related – occurring in 2-5% of patients who have received a cumulative dose of 400-500 mg/m2 and up to 50% of patients who have received more than 1000 mg/m2 of doxorubicin
Patients have a history of receiving doxorubicin, with the onset of symptoms 2-4 months, and rarely years after completion of therapy
ii. Usually asymptomatic until signs of CHF – tachypnoea, and exertional dyspnoea
i. NO effective treatment
a. Symptomatic patients have a high mortality rate
b. 2 year survival rate is about 20%
c. Almost all patients die by 9 years after the onset of illness
Most common cause sudden cardiac death
Hypertrophic cardiomyopathy
30% of childhood cardiomyopathy
e. 10-20% of infants of diabetic mothers develop a transient form of HCM with or without LVOT obstruction
>13mm is abnormal for LVH
Inherited in 30-60% of cases - autosomal dominant
Syndromes (Noonan, Beckwith Weidemann, LEOPARD, Friedreichs ataxia)
Metabolic
ii. Thickening of LV -> reduced diastolic filling -> LA enlargement and pulmonary venous congestion
1. This results congestive symptoms (exertional dyspnoea, orthopnoea, PND)
Restrictive cardiomyopathy
a. Extremely rare – 5% of cases in children
b. Basic defect unknown
c. Diastolic dysfunction with normal wall thickness and systolic dysfunction
d. Relentless downhill course – the worst type of cardiomyopathy (contrasting HOCM which is very variable)
i. Typically require transplantation within years
Mostly idiopathic
b. The ventricles are normal in size and systolic function
c. Only the atria are markedly dilated (biatrial enlargement) – characteristic finding
a. Poor prognosis – not improved by medical management
b. SCD is common
c. High risk of atrial tachycardias and thromboemboli
d. 2 year survival 50%
ECG signs digoxin toxicity
i. First or second degree AV block
ii. Profound sinus bradycardia or sinoatrial block
iii. SVT (atrial or junctional ectopic beats and tachycardias), rarely ventricular arrythmias
iv. Shortening of QTc and reduced amplitude of T wave are signs of digitalis effect
Lymphoproliferative disease
i. Primary post-transplant malignancy
ii. Usually polymorphic: B cell origin, EBV driven
iii. 9% within 7 years, 3 year 70% survival
iv. Management
1. Reduction/ cessation of therapy
2. IV acyclovir
3. Chemotherapy
4. Rituximab has shown some benefit
Most common type ASD
Secundum defect (50-70%) Primum defect (15-30%) Sinus venosus defect (10%) Coronary sinus ASD (rare)
ASD ECG/CXR findings
ECG: • RAD (+90 to +180) with mild RVH or RBB (rsr’ in V1)
• The dilated RV prolongs repolarisation of the RV because of its longer pathway – producing either complete or incomplete RBB (rsr’ in V1) [BBB NOT due to actual block in right bundle)
• Mild RVH may be present
CXR: • Cardiomegaly with enlargement of the RA and RV, increased PBF if large shunt
• Cardiomegaly with enlargement of the RA and RV may be present
• Prominent pulmonary artery segment and increased pulmonary vascular markings – if shunt significant
ASD spontaneous closures
i. ASD <3mm diagnosed <3 months of age – spontaneous closure in 100%
ii. Spontaneous closure occurs >80% of the time with defects 3-8mm by age 1.5 years
iii. Defect >8mm rarely occurs spontaneously; and is not likely to occur after 4 years of age
Most common type VSD
Perimembranous - Membranous defect involves varying amounts of muscular tissue adjacent to membranous septum (Perimembranous VSD)
VSD spontaneous closures
i. Perimembranous + muscular = spontaneous closure can occur
1. Occurs most frequently with small defects during the first 6 months of life
2. 60% of small to moderate muscular VSDs close spontaneously; NOT after 8 years of age
3. 35% of small perimembranous VSDs close spontaneously; NOT after 5 years of age
4. VSDs do not become bigger with age, they decrease in size
ii. Inlet defects and outlet (infundinbular) = defects do NOT become smaller or close spontaneously
VSD shunt haemodynamics
i. Small shunt
1. At < 5mm, the shunt tends to be restrictive (negligible shunting) as there is anatomical to flow
ii. Large shunt
1. >10 mm, minimal anatomical resistance to flow
2. May be called dependent/ non-restrictive, as shunting dependent on level of PVR
3. When PVR drops at ~ 2 months, features of shunting appears
VSD ECG/CXR findings
ECG: • Small VSD = normal
• Moderate VSD = LVH and occasional LAH
• Large VSD = BVH with or without LAH
• If pulmonary obstructive disease = only RVH
CXR: • Degree of cardiomegaly and the increase in pulmonary vascular markings directly relate to the magnitude of the L to R shunt
• Small = pulmonary vascular congestion, no chamber enlargement
• Medium = enlargement of LA, LV and PV, with increased pulmonary markings
• Large = significant cardiomegaly, biventricular enlargement, LA enlargement, greatly increased pulmonary vascularity; also have dilatation/ prominence of main pulmonary artery
• Eisenmenger = the main PA and hilar PAs enlarge, but the peripheral lung fields are ischaemic and heart size normal
AVSD syndromic association
Trisomy 21 - 70% patients with AVSD have Downs
AVSD/ECD classification
i. Complete form of ECD = VSD + ostium primum type of ASD + clefts in the mitral and tricuspid valve (Single valve orifice connecting the atrial and ventricle chambers – usually has 5 leaflets)
ii. Partial form of ECD = ostium primum type of ASD + cleft in the mitral valve (-> mitral regurg)
a. Balanced (the majority): AV orifice equally committed to RV/ LV
b. Unbalanced: orifices committed primarily to one ventricle -> single ventricle physiology (may require Fontan
Partial AVSD ECG/CXR findings
Superior axis = AVSD (pink) or tricuspid atresia (blue)
ECG: • Superior QRS axis with QRS axis between -40 and -150 degrees characteristic
o Different to secundum ASD
o Occurs due to abnormality in development of bundle of His
• RVH or RBBB (rsR’ in V1) as in secundum ASD
• 50% of the patients have a prolonged PR interval (1st degree block)
CXR: • As for secundum ASD – except enlargement of the LA and LV when MR is significant
• Characteristic ‘goose neck’ deformity is seen on a left ventriculogram
AVSD spontaneous closures
Does not occur
Complete AVSD ECG/CXR findings
ECG: • Superior QRS axis with QRS axis between -40 and -150 degrees characteristic
• Prolonged PR interval
• RVH or RBB present in all cases, may have LVH too
• Q waves in lead I and aVL
CXR: • Biatrial and biventricular hypertrophy
o Volume overload of the LA and LV, as in VSD and partially due to MR
o Volume overload of the RA and RV, as in ASD
• Pulmonary vascular markings increased, main PA segment prominent
Bounding peripheral pulses with wide pulse pressure
Characteristic of PDA (not present if small)
Continuous machinery murmur
Characteristic of PDA
Loudest LUSE/infraclavicular
Post stenotic dilatation / dilation of PA/aortic root
Hallmark of stenosis at the semilunar valves/valvular stenosis
o Pulmonary stenosis prominent PA segment visible on CXR
o Aortic stenosis dilated aorta may look like a bulge on the right upper mediastinum, or a prominence of the aortic knob on the left upper mediastinum and chest film
• Post-stenotic dilation is NOT seen with sub-valvular stenosis; it is only MILD or not seen with Supravalvular stenosis
Pulmonary stenosis ECG/CXR findings (valvular)
ECG: • RAD, RVH in moderate PS
• RAH and RVH ‘strain’ may be seen in severe PS (T wave inversion on praecordial leads)
• Neonates with critical PS may show LVH because of a hypoplastic RV and relatively large LV
CXR: • Heart size usually normal on CXR
• Dilatation of the main pulmonary artery may be seen (post stenotic dilatation)
• Pulmonary vascular markings are usually normal but may decrease if severe
• Cardiomegaly only if CHF develops
• In neonates with severe PS – lung fields oligaemic with varying degrees of cardiomegaly
Pulmonary stenosis associations
i. Congenital rubella = pulmonary stenosis AND peripheral pulmonary stenosis
ii. Alagille = peripheral pulmonary stenosis
iii. Williams = peripheral pulmonary stenosis
iv. Noonan = pulmonary stenosis (valvular) with dysplastic pulmonary valves
Most common congenital cardiac defect
Bicuspid aortic valve - most common cause valvular aortic stenosis (valvular is most common type of AS)
2nd is VSD
Aortic stenosis classic triad
Dyspnoea/fatiguability
Exertional syncope
Chest pain
Aortic stenosis ECG/CXR findings
ECG: • LVH with moderate to severe stenosis; may have strain pattern in severe stenosis (poor correlation between ECG and severity)
CXR: • Heart size usually normal in children – can have a dilated ascending aorta or a prominent aortic knob with valvular AS resulting from post-stenotic dilatation
• Significant cardiomegaly does not develop unless CHF occurs later in life or AR becomes substantial
• Newborns with critical AS show generalised cardiomegaly with pulmonary venous congestion
Most common valvular involvement in children with rheumatic heart disease
Mitral valve (regurgitation) - note, still rare - followed by aortic valve
Boot shaped heart on CXR
Tetralogy of Fallot
Most common cyanotic heart defect
Tetralogy of Fallot (10% of cyanotic lesions)
Tetralogy of Fallot ECG/CXR findings
ECG: • RAD (+120 to +150), RVH, RAH
• Acyanotic – may be normal QRS and RVH
CXR: • Heart size is normal or smaller than normal
• Pulmonary vascular markings a decreased – ‘black’ lung fields may be seen in TOF with pulmonary atresia
• Boot shaped heart = concave main PA segment (small pulmonary artery) + upturned apex due to RVH
• RA enlargement (25%) and right aortic ring (25%) may be present
Tricuspid atresia ECG/CXR findings
ECG: • LAD/Superior QRS axis (between 0 and -90) – appears in most patients without TGA (only 50% with TGA)
• LVH is usually present
• RAH or bi-atrial hypertrophy is common
CXR: • Normal or mildly enlarged cardiac silhouette – enlargement of RA and LV
• Pulmonary vascularity decreases in most patients
• Occasionally concave PA segment may produce boot shape heart similar to TOF
Truncus arteriosus syndrome association
DiGeorge in 30% of cases
Key features:
a. Common pulmonary artery/ aorta
b. VSD always present
c. Cyanosis is MILD due to high pulmonary blood flow
d. Eventually leads to increased pulmonary resistance + Eisenmenger’s
Most common cause of death from cardiac defects in the first month of life
Hypoplastic left heart syndrome - Hypoplasia of the LV – completely nonfunctional
a. Unwell in the newborn period
b. Acidosis out of proportion of CO2
c. Complications = inadequate systemic circulation, pulmonary venous hypertension OR pulmonary over circulation
c. Association = Turner, Trisomy 18, Jacobsen’s syndrome
d. Also a prevalence of associated brain anomalies
a. ABG = decreased PO2 and normal PCO2, severe metabolic acidosis out of proportion to the PCO2 (caused by markedly decreased output) characteristic of the condition
10 warning signs primary immunodeficiency
Think about referring your patient to an allergist/immunologist when there is the presence of > 2 of the following warning signs:
- > 8 ear infections in one year
- > 2 severe sinus infections in one year
- > 2 months treatment of antibiotics with little effect
- > 2 pneumonias per year
- Insufficient weight gain or growth delay
- Recurrent deep skin or organ abcesses (ex. :liver, lungs)
- Persistent thrush in mouth or fungal infection on skin
- Need for intravenous antibiotics to clear infections
- > 2 deep seated infections (ex. : septicemia, meningitis)
- Family history of a primary immunodeficiency
Normal rates infection
6-8 resp infections/yr first 10 years
6 otitis media/year first 2-3 years
2 gastroenteritis/yr first 2-3 years
May exceed these in day care or with older siblings
Oligohydramnios tetrad
Altered facies (flattened nose, prominent infraorbital creases, micrognathia, large low set ears)
Aberrant limb positioning
Late growth deficiency
Pulmonary hypoplasia
Subgaleal haemorrhage
Blood from torn emissary veins accumulates within loose connective tissue between galea aponeurotica and periosteum
Subgaleal space extends from orbits to nape of neck and laterally above ears
Can accumulate a large amount of total blood volume
Crosses sutures and covers fontanelles
Shifts to dependent part of head
May push ears forward
Cephalohaematoma
Collection of blood under periosteum
Does not cross sutures, distinct borders
Erb palsy
Brachial plexus injury RFs: macrosomia, shoulder dystocia Injury to C5 and C6 nerve roots +/- C7 Limp arm with internal rotation of forearm and flexion of wrist DDx: clavicular fracture Complete recovery occurs in most cases
Klumpke palsy
Hand is paralysed but infant has full function of elbow and shoulder
Congenital pulmonary airway malformation - key points
a. Congenital pulmonary airway malformation (CPAM) = hamartomatous (noncancerous tumor made of an abnormal mixture of normal tissues and cells from the area in which it grows) or dysplastic lung tissue mixed with more normal lung
b. Due to embryologic abnormalities of branching morphogenesis
c. Connection with tracheobronchial tree
d. Blood supply= pulmonary circulation
e. Prognosis dependent on size
f. Generally confined to one lobe
g. 1-4/100,000 births
Type 1 most common
75% asymptomatic
Surgery (resolve symptoms, malignancy risk)
Pulmonary sequestration - key points
a. Aberrant formation of segmental lung tissue that has NO connection with the bronchial tree or pulmonary arteries
b. Non-functioning
c. Receives its arterial supply from systemic arteries (commonly off the aorta)
d. Classified by how blood returns to the right side of the heart
i. Inferior vena cava = extra-lobar
ii. Pulmonary veins = intra-lobar (75-85%)
e. Sequestration functions as a space-occupying lesion within the chest; does NOT participate in gas exchange
i. Does NOT lead to left to right shunt or alveolar dead space
f. Communication with the airway can occur as a result of a rupture of infected material into an adjacent airway
g. Collateral ventilation within intrapulmonary lesions via pores of Kohn can occur
h. Gastric or pancreatic tissue ay be found within the sequestration
i. Cysts may also be present
Rx: surgical resection
Congenital Lobar Emphysema/ Overinflation - key points
a. Developmental anomaly of the lower respiratory tract characterised by hyperinflation of one or more of the pulmonary lobes 🡪 progressive, massive, uniform dilatation of a lobe
b. Results in compression of the remaining lung tissue and herniation of the affected lobe across the anterior mediastinum into the opposite chest 🡪 displacement of the mediastinum
i. Affected lobe is essentially non-functional due to overdistension - LUL most common
ii. Atelectasis of the ipsilateral normal lung can occur
Almost all present by 1 year of age
Rx: Surgery
Commonest congenital anomaly
Congenital heart disease (1/3 all anomalies)
1% of live births
25% “critical” i.e. needing surgery first year of life
Commonest cause death early infancy
Congenital heart disease
Timing of presentation - duct dependent lesions
24 hours to 2 weeks
Duct dependent PBF -> cyanosis
Duct dependent systemic BF -> shock/hypoperfusion
Causes differential cyanosis
- Occurs in:
a. CHD
i. Critical CoA
ii. Interrupted aortic arch
iii. Critical AS
b. PPHN - Deoxygenated blood flow through the ductus arteriosus supplies the lower half of the body’s circulation
- Oxygenated blood from the left heart supplies the upper body via the vessels proximal to the site of arch obstruction
- Difference of >3% in oxygen saturations measured on the right and (preductal) and either foot (post ductal) identifies differential
Cardiogenic shock - duct dependent lesions
- Hypoplastic left heart syndrome
- Critical AS
- Critical CoA
- Interrupted aortic arch
Cyanosis - duct dependent lesions
R) heart lesions: critical PS, pulmonary atresia with intact ventricular septum, Ebstein anomaly, (TOF and TA depending on RVOT obstruction)
L) heart lesions: HLHS, critical AS
Asplenia - features
a. Spleen absent
b. Bilateral R sidedness characterised malformations of the major organs
i. Bilateral, three lobed lungs with bilateral eparterial bronchi
ii. Various gastrointestinal malformations
iii. Symmetrical midline liver
iv. Malrotation of the intestine
c. Complex cardiac malformations
i. Normal IVC
ii. TGA with pulmonary atresia (85%)
iii. Single ventricle and common AV valve
iv. TAPVR to Extracardiac structures occurs in >75%
> 95% die within first year
Polysplenia - features
Usually female (70%)
a. Multiple splenic tissue
b. Tendency for bilateral left sidedness
i. Bilateral, bilobed lungs (ie two left lungs)
ii. Bilateral, hyperartrial bronchi
iii. Symmetrical liver
iv. Occasional absence of gallbladder
v. Intestinal malrotation
c. Cardiovascular abnormality
iii. Key features
1. Absence of the hepatic segment of IVC with azygous (right side) or hemiazygous (left side) continuation is seen in 85%
2. Two ventricles are usually present
3. TGA, PS or pulmonary atresia, TAPVR occur less often
4. ECG shows superiorly oriented P axis (ectopica atrial rhythm) resulting from absence of the sinus node
5. Occurs more often in females
First year mortality 60%
Vascular ring - classification
Complete = abnormal vascular structures or their remnants form a COMPLETE circle around the trachea and esophagus
- Includes:
a. Double aortic arch (most common, 40%, presents in early infancy with respiratory distress and feeding problems)
b. Tight aortic arch
Incomplete = do not form a complete circle, but do compress the trachea and esophagus
- Includes
a. Anomalous innominate artery
b. Aberrant right subclavian artery (asymptomatic)
c. Anomalous left PA (vascular sling/ pulmonary sling)
Barium swallow diagnostic (EXCEPT for in innominate artery)
Common causes left axis deviation
Common causes = HAT SAND • HOCM • AVSD • Tricuspid atresia • Single ventricle • ASD primum • Noonan’s (especially HCM) • DORV
P wave morphology
- Normal amplitude < 3mm
- Normal duration <0.07 infants, <0.09 children, <0.12 adults
- Amplitude changes with hypertrophy
RAH - tall >3mm
LAH - wide >100msec (2.5 small squares)
Pathologic Q waves
o Pathological = if deep (> 25% R wave amplitude), wide, or unusual location
Appear in the right precordial leads ie V1 (eg severe RVH)
Are absent in the left precordial leads (e.g. LBBB)
Are abnormally deep (ventricular hypertrophy of the volume overload type)
Are abnormally deep and wide (myocardial infarction or fibrosis)
Normal T wave evolution
o For the first 8 days - T waves are upright throughout the precordial leads.
o >8 days the T waves become inverted in V1-3 [right precordium] (= the “juvenile T-wave pattern”)
T wave inversion up to V4 permitted
o Progressive change to upright T waves across precordial leads from L to R with growth
o Juvenile T-wave pattern can persist into adolescence and early adulthood (= “persistent juvenile T waves”)
Particularly common in V1
ECG U waves
• Extra positive deflection at the end of the T wave
• Common causes
o Hypokalaemia
o Normal finding at slower heart rates (sinus bradycardia)
ECG normal intervals
PR <200msec
QRS <120msec
QTc <490msec if <6mo or <440 if >6mo
RVH - ECG features
Summary: upright T waves V1, tall R V1, deep S V6, RAD for age
(Parks)
- RAD for patients age
- Increased right sided QRS voltages
a. Large R wave in V1 (also III, aVR and V2)
b. Large S wave in V6 (also I, V4 and V6)
c. Abnormal R/ S ratio – generally big R waves in R leads, big S waves in L leads - Upright T wave in V1 > 3 days of life if < 7 years (also V3R , V4R)
- Abnormal Q waves in V1 suggestive
- ‘Strain pattern
a. Features more consistent with ‘pressure overload’ – Tall R waves, upright T waves
b. Features more consistent with ‘volume overload’ - rsR’ pattern, prolonged QRS
Normal V1 / V6 voltages for age
Important for determining ventricular hypertrophy
RVH (543, 321) - cm
R in V1: <1mo 5, 1mo-1yr 4, >1yr 3
S in V6: <1mo 3, 1mo-1yr 2, >1yr 1
LVH (123, 345) - squares
R in V6: <1mo 3, 1mo-1yr 4, >1yr 5
S in V1: <1mo 1, 1mo-1yr 2, >1yr 3
LVH - ECG findings
Summary: Tall R V6, deep S V1, T wave inversion V5/V6
Park’s
- (LAD)
- Increase in L sided QRS voltages
a. R wave in V6
b. S wave in V1
c. T axis change = strain
d. R in V5/6 + S in V1 > 30 mm under 1 year, >40 mm after 1 year, > 60mm (?age – Andrew Davies’ lecture/ Harrison)
e. S in V1 > 2x R in V5 - Deep Q waves in L praecordial leads suggestive
- Strain pattern ( ?T waves inverted in lead 1 or aVF)
- Diastolic vs systolic
a. Systolic overload: T wave inversion over L praecordial leads
b. Diastolic overload: Q waves with normal T waves
Biventricular hypertrophy - ECG findings
- +ve criteria for LVH + RVH
- +ve for LVH OR RVH and big voltages in other ventricle (> 50th percentile)
- Large equiphasic QRS complexes in > 2 limb leads mid praecordial leads (V2-5)
RBBB - ECG findings
QRS up in V1
o Diagnostic criteria
Broad QRS >120ms
RSR’ pattern in V1 (+/- V2-3) (M shaped QRS complex) = M
Wide, slurred S wave in lateral leads (I, AVL, V5-6) = W
o Associated features
ST depression and T wave inversion in right precordial leads (V1-3)
o Variations
Sometimes rather than RSR’ pattern, there may be a broad monophasic R wave or qR complex
RBBB causes
o Post-operatively – particularly TOF (90% of repaired TOF have RBBB)
o CHD = AVSD, Ebstein’s anomaly, COA, PAPVR
o Right ventricular hypertrophy / cor pulmonale
o Pulmonary embolus
o Ischaemic heart disease
o Rheumatic heart disease
o Myocarditis or cardiomyopathy
o Degenerative disease of the conduction system
o Congenital heart disease (e.g. atrial septal defect)
Incomplete RBBB - ECG findings
• RSR’ in V1 (R waves the same size)
• QRS normal/ mildly prolonged
• Causes: normal variant, secundum ASD (with RAD), primum ASD (with LAD), Ebstein’s anomaly (with RAH + delta waves)
-> differentials: ASD, Ebsteins anomaly
LBBB - ECG findings
QRS down in V1
o Diagnostic criteria
Broad QRS >120ms
Absence of Q waves in lateral leads (I, V5-6)
QRS down or mostly down in V1 (dominant S wave in V1)
Broad monophasic R wave in lateral leads (I, avL, V5-6)
Prolonged R wave peak time >60ms in left precordial leads (V5-6)
o Associated features
Appropriate discordance: the ST segments and T waves always go in the opposite direction to the main vector of the QRS complex
Poor R wave progression in the chest leads
LAD
LBBB causes
o RARE IN CHILDREN o Cardiac surgery/disease o AV replacement o LVH Other (adults) – aortic stenosis, IHD, HTN, dilated cardiomyopathy, anterior MI, primary degenerative disease (fibrosis) of the conducting system (Lenegre disease), hyperkalaemia, digitoxin toxicity
Hyperkalaemia - ECG changes
• Based on K+ o 5.5-6.5 = tall peaked T waves o 5.5-7.5 = loss of P waves o 7.0-8.0 = widened QRS o 8.0-10.0 = sine wave, ventricular arrythmias, asystole
• Summary of abnormalities
o Tall peaked T waves, best seen in precordial leads
o Prolongation of QRS duration
o Prolongation of PR interval
o Disappearance of P wave
o Wide bizarre biphasic QRS complexes (sine waves).
o Eventual asystole
Hypokalaemia - ECG changes
• K+ <2.5
o Prominent U waves develop with apparent prolongation of the QTc (prolonged “QU” interval)
o Flat or biphasic T waves
o ST segment depression
• K+ falls further
o PR interval prolongs
o Sinoatrial block may occur
Calcium derangements - ECG changes
Hypercalcaemia
• Shortens the ST segment and QTc
Hypocalcaemia
• Prolongs the ST segment with resultant prolongation of the QTc
Pericarditis - ECG changes
- Initial widespread concave ST segment elevation and PR segment depression.
- ST segment returns to normal within 1-3 weeks, along with flattening of the T waves.
- T wave inversion (with isoelectric ST segment) occurs from 2-4 weeks after the onset of pericarditis.
Dextrocardia on ECG
o –ve P wave in I, and +ve in AVF
o V5 and V6 have no wave form – no electrical activity in this part of the chest
P wave axis
P axis is between 0 and +90 degrees (= upright P waves in lead II and inverted P waves in aVR)
Most common symptomatic dysrhythmia in children
Supraventricular tachycardia
Key points
a. Rapid regular, usually narrow (< 80 ms) complex tachy of 220-320bpm (infants) and 150-250 (older children)
b. The P wave is usually invisible, or if visible is abnormal in axis and may precede or follow the QRS (“retrograde P waves”)
c. 90% of paediatric dysrhythmias are SVT; 90% of SVT are of re-entrant type
d. Half of patients with SVT will have no underlying heart disease
e. Almost 1⁄4 will have congenital heart disease and 1⁄4 will have WPW
f. Consider fever or drug exposure (particularly sympathomimetics)
g. SVT may be well tolerated in infants for 12-24 hours congestive heart failure later manifests with irritability, poor perfusion, pallor, poor feeding and then rapid deterioration.
h. Note that > 95% of wide complex tachycardias in paediatrics are NOT VT, but SVT with aberrancy, SVT with BBB (in pre-existing congenital heart disease) or a type of accessory pathway re-entrant SVT
Only broad complex SVT
Antidromic accessory RAVT
Causes SVT
a. No heart disease in 50% of patients
b. WPW pre-excitation present in 10-20% of cases, which is evident only after conversion to sinus rhythm
c. Some CHDs more prone to this arrythmia Ebstein anomaly, hamartoma (eg. in TS), HCM/ DCM, single ventricle, L-TGA (/ congenitally corrected TGA)
d. SVT may occur following cardiac surgeries
ECG features WPW
features in sinus rhythm
a. Short PR (<120ms)
b. Delta wave – slurring slow rise of initial portion of QRS
c. QRS prolongation >110 ms
d. ST segment and T wave discordant changes – ie in the opposite direction to the major component of the QRS complex
e. Absent Q wave in V6
i. Q wave in V6 is due to septal activation L R
ii. There is no Q wave in V6 due to WPW
iii. Absence is a sign of pre-excitation
Premature ventricular contraction - reassuring v non-reassuring features
Arrythmias related to exercise are significant. Induction or exacerbation with exercise may be an indication of underlying heart disease
In children PVCs characteristically are reduced or eliminated by exercise
All children with symptomatic ventricular arrhythmias and those with complex PVCs (multiform PVCs, ventricular couplets, unsustained ventricular tachycardia) should be treated
i. Beta blockers – effective for cardiomyopathy and occasionally for RV dysplasia
ii. Other antiarrhythmic drugs
iii. Anti-arrythmic agents that prolong the QT interval (class IA quinidine + procainamide; class IC encainide + flecainide; class III amiodarone) should be AVOIDED
a. Children with otherwise normal hearts, occasional isolated uniform PVCs that are suppressed by exercise do not require extensive Ix
b. Asymptomatic children with multiform PVCs and ventricular couplets should have 24 hour Holter monitoring, even if they have structurally normal hearts, to detect the severity and extent of ventricular arrythmias
c. Children with uniform PVCs, including bigeminy and trigeminy, do not need to be treated if exercise stress test and echo are normal
e. For patients with symptomatic ventricular arrhythmias or sustained VT and seemingly normal hearts, MRI (preferable) or cardiac cath may be indicated to Ix for RV dysplasia
i. Occasionally invasive EPS or Endomyocardial biopsy indicated
f. Children with multiform PVCs and runs of PVCs (VT) with or without symptoms need to be evaluated by an electrophysiologist
Normal AFI/liquor volume
7-20
Polyhydramnios (1-2%): AFI>24 or single deepest pocket >8
- 1/3 associated anomalies
Oligohydramnios (10%): AFI<5 or single pocket <2
PPHTN - triad
i. Severe hypoxia in FiO2 100%
ii. Evidence of R to L shunt
iii. TTE confirmation structurally normal heart
- Risk factors
a. MAS (41%)
b. Pneumonia (14%), RDS (13%), mixed (14%)
c. CDH (10%)
d. Pulmonary hypoplasia (4%)
e. Idiopathic (17%)
Severe complication whose rate is increased with surfactant treatment
Pulmonary haemorrhage
Key points
a. Relatively uncommon
b. Occurs in 10% of extremely preterm infants
c. Massive pulmonary haemorrhage can be fatal
d. Pulmonary haemorrhage is the only severe complication whose rate is increased with surfactant treatment
i. Seen with all surfactants; incidence ranges from 1-5% of treated infants
ii. Incidence higher with natural surfactant
Neonatal encephalopathy - aetiology
a. 70% of neonatal encephalopathy associated with events prior to onset of labour
b. Antenatal
i. Maternal factors = unemployment, family history of seizures or neurological disorder, infertility treatment, thyroid disease
ii. Placental conditions = severe pre-eclampsia, post-dates, abnormal placenta
iii. Fetal problems = IUGR -> strongest risk factor
c. Intrapartum
i. Persistent occipitoposterior position
ii. Shoulder dystocia
iii. Emergency cesarean delivery
iv. Operative vaginal delivery
v. Acute intrapartum events or sentinel events – uterine rupture, placental abruption, cord prolapse
vi. Inflammatory events – maternal fever, chorioamnionitis
d. NOTE
i. Perinatal stroke is a separate recognized entity in term newborns with encephalopathy
ii. Metabolic and neurodegenerative disorders may underlie neonatal encephalopathy
Neonatal encephalopathy - key points
a. Neonatal encephalopathy = heterogenous, clinically defined syndrome characterised by disturbed neurologic function in infant born >35 weeks gestation
b. Key features
i. Reduced level of consciousness
ii. Difficulty with initiating + maintaining respiration
iii. Depression of tone and reflexes
Therapeutic hypothermia - details
i. Maintained at 72 hours form 33-35 degrees
ii. Started within 6 hours off delivery
iii. Reduces cerebral metabolic rate, reduces apoptosis, lowers production of nitric oxide and free radicals
iv. Must be < 6 hours post birth, and > 35/40
v. Followed by 12 hours of active gradual rewarming after 72 hours of cooling.
vi. Only neuroprotective therapy
vii. Indicated if suspected HIE
viii. Improved survival and outcome at 18 months in infants who meet criteria for HIE
ix. Side effects = thrombocytopenia, reduced HR, SC fat necrosis (can be associated with hypercalcaemia)
Vein of Galean malformation
- Key points
a. Rare, but 30% of paediatric vascular malformations - Pathogenesis
a. Multiple AV shunts draining into a dilated median prosencephalic vein of Markowski (embryonic vein not present in adults)
b. Medial prosencephalic vein usually regresses to form the great cerebral vein (Galen) with formation of vertebral veins
c. If persistently high blood flow, the median prosencephalic vein of Markowski progressively enlarges to form an aneurismal VGM - Clinical features
a. Neonates = high output cardiac failure
b. Infants = hydrocephalus + neurological
c. Older children/adults = headache, seizures, SAH
50% mortality
Perinatal stroke - key points
a. Very common, differs from childhood stroke
b. Defined as acute neurological syndrome with chronic sequelae due to cerebral injury of vascular origin occurring between 20 weeks gestation + 28 days postnatal life
c. Focal cerebral injury due to
i. Arterial ischaemic stroke (majority MCA distribution)
ii. Cerebral venous thrombosis
iii. Primary intracerebral haemorrhage
d. Common cause of neonatal encephalopathy
e. Seizures are most common manifestation
- Outcome
a. Poor outcome
b. Most children have lifelong disability
c. Perinatal stroke accounts for most cases of Hemiparetic CP
d. Additional morbidity seen in 25% - disorders of language, learning, cognition and behaviour, long-term epilepsy
e. Stroke recurrence for both the child and subsequent pregnancies are extremely low
Leading cause perinatal mortality
Congenital anomalies (20-25%)
Most common type intracranial haemorrhage (neonates)
Subdural haemorrhage (subarachnoid second most common)
a. Most common type of intracranial hemorrhage
b. Venous bleed between dural and arachnoid
c. Clinical manifestations
i. 24-48 hrs of life, apnea, respiratory depression & seizures
ii. Irritability, altered GCS
iii. Rarely, increased ICP with increase HC, tense fontanelle, apnea, bradycardia, coma
d. Investigations
i. CT best for diagnosis – blood not bound to suture lines, crescent
e. Treatment
i. Most managed conservatively, surgery in ↑ICP
ii. Serial FBE for ?blood loss and replacement if concern
iii. Think about coagulopathy if birth history not suggestive
iv. Treat seizures with phenobarbitone
Most common neonatal fracture
Clavicle
Humerus is most common long bone fracture
Multiple fractures are rare - suspect osteogenesis imperfecta
Erbs palsy - key points
Erb’s palsy
i. 90% brachial plexus injury
ii. Risk factors – macrosomia, BW >4kg, shoulder dystocia
iii. Injury to C5/6 +/- C7
iv. Excessive traction on upper cords of plexus during delivery
v. Presentation
1. Limp arm in “waiter’s tip” position – arm held in adduction, elbow extended and forearm pronated with wrist flexed
2. Absent biceps jerk
3. +/- phrenic nerve involvement with diaphragmatic palsy in 5%
vi. Prognosis – most resolve spontaneously by 4 months
vii. Management
1. Nothing
2. Physiotherapy (after 1-2weeks)
3. Splinting to prevent contracture if at risk
4. Surgical repair if absent biceps or shoulder function at 6-12weeks (3 months is cut off for intervention) – nerve grafting
Klumpke’s palsy
i. Rare = seen in breech delivery (difficult head extraction)
ii. Injury to C8/T1
iii. Presentation
1. Claw hand deformity
2. Wrist drop
3. Absent grasp reflex
4. May have associated Horner’s
5. May have accompanying bony fracture
iv. Management – as above
Sarcoidosis - general
Sarcoidosis is a multisystem disorder of unknown aetiology characterized by the accumulation of T lymphocytes, mononuclear phagocytes, and noncaseating granulomas in involved tissues.
Key points
a. Idiopathic inflammatory disease involving multiple organ systems
b. Diagnosis of exclusion from other diseases with granuloma formation
c. African American females disproportionately affected
d. Rarely found in children <8 years of age
Clinical manifestations
a. Skin rash, iridocyclitis, and arthritis seen most often WITHOUT pulmonary symptoms in children
b. Pulmonary disease less progressive compared with adults – rarely progresses to fibrosis
c. Ocular disease more likely to be progressive than in adults
d. African American children more likely have LN involvement, nonspecific elevations of gamma globulin, erythema nodosum and hypercalcaemia
e. Usually unremarkable examination
Most common laboratory findings
i. Hypergammaglobulinemia
ii. Hypercalciuria + hypercalcaemia
iii. Elevated ALP when liver disease present
iv. Anaemia of chronic disease
v. Serum ACE elevated in 75% - false positives can occur (diagnostic of sarcoid)
Treatment
a. Pulmonary sarcoidosis spontaneously resolves in 75% of patients without therapy
b. Corticosteroids are mainstay of treatment – usually not started when stage I or II is present without symptoms
c. Absolute indications include stage III disease with symptoms
d. When pulmonary disease is progressive, GCS therapy is aimed at prevention of fibrosis, honey-combing and irreversible lung disease
e. Alternative immunosuppressants are sometimes used
Pneumomediastinum - aetiology
a. Typically caused by alveolar rupture during acute or chronic pulmonary disease
b. LRTI is a common aetiology for pneumomediastinum in children < 7 years of age
c. Acute asthma is more common in older children and adolescents
d. Other causes = following vomiting, dental extractions, T+As, HFNP, normal menses, obstetric delivery, diabetes with ketoacidosis, acupuncture, anorexia and inhalation
e. Can also result from oesophageal perforation (Boerhaave syndrome), penetrating chest trauma or inhaled FB
Chyle - composition
a. TG in the form of chylomicrons
b. T lymphocytes
c. Electrolyte concentration similar to plasma
d. Immunoglobulins and fat soluble vitamins
Chylothorax - aetiology
a. Thoracic duct injury as a complication of cardiac surgery = most common in children
b. Chest injury
c. ECMO
d. Primary or metastatic intrathoracic malignancy – particularly lymphoma
e. Newborns – during delivery
f. LESS COMMON = lymphangiomatosis, restrictive pulmonary disease, thrombosis of the duct, superior vena cava or subclavian vein, TB or histoplasmosis, congenital anomalies of lymphatic system
i. Refractory chylothorax in fetus associated with missense mutation in integrin alaph9 gene
Systemic hypertension - aetiology
- Etiology
a. Primary = essential HTN
b. Secondary
i. Renal (75%) - Renal parenchymal disease = post-infectious GN, chronic GN, obstructive uropathy, reflux nephropathy, haemolytic uraemic syndrome, PCKD
- Renovascular disease = renal artery disorders (stenosis, polyarteritis), renal vein thrombosis
ii. Cardiovascular (15%) = coarctation
iii. Endocrine (5%) = phaeochromocytoma, hyperthyroidism, congenital adrenal hyperplasia, primary hyperaldosteronism, Cushing syndrome
iv. Other (5%) = neuroblastoma, neurofibromatosis, steroid therapy, raised ICP - Most common etiology by age
a. Newborns = renal artery thrombosis or stenosis, congenital renal malformation, CoA, bronchopulmonary dysplasia
b. <6 years = renal parenchymal disease, coarctation, renal artery stenosis
c. 6-10 years = renal artery stenosis, renal parenchymal disease, primary hypertension
d. >10 years = primary hypertension, renal parenchymal disease
HACEK organisms
Haemophilus, Actinobacillus, Cardiobacterium, Eikenella and Kingella
i. Gram Negative bacilli + part of normal oral flora
ii. Often require 5 days to grow
Osler nodes
Tender, pea-size red nodes at the ends of the fingers or toes (rare in children)
- infective endocarditis
Janeway lesions
Small, painless, haemorrhagic areas on the palms or soles (rare)
- infective endocarditis
Roth spots
Oval, retinal hemorrhages with pale centers located near optic disc
- infective endocarditis
