Paediatrics 4 Flashcards
(240 cards)
1
Q
What area are most childhood brain tumours located?
A
infratentorial (below the cerebrum)
2
Q
What tumours most commonly spread to the brain?
A
Lung (most common), breast, bowel, skin, kidney
3
Q
What is the most common primary brain tumour in children?
A
Pilocytic astrocytoma (brain tumour originating from the star shaped cells, astrocytes). They are low grade gliomas
4
Q
What is the most common supratentorial tumour in children?
A
Craniopharyngioma
5
Q
What is a craniopharyngioma?
A
Solid/cystic tumour of the sella region (near the pituitary gland and hypothalamus). They are slow growing midline epithelial tumours
6
Q
How do infratentorial tumours typically present in children?
A
Raised ICP, headaches, vomiting, cerebellar ataxia
7
Q
What is the best diagnostic method for brain tumors?
A
CT: quick and available. MRI: generally gives better tumour definition
8
Q
How is hydrocephalus due to brain cancer typically treated?
A
Control swelling with high dose steroids (usually dexamethasone) and CSF drainage via surgery
9
Q
What are the most common sites of pilocytic astrocytomas in children?
A
Cerebellum and optic pathway
10
Q
What condition has high co-morbidity with optic pathway low grade gliomas?
A
Neurofibromatosis type 1
11
Q
What are differential diagnosis of billious vomiting in neonates?
A
Duodenal atresia, malrotation with volvulus, meconium ileus
12
Q
What sign is seen on AXR with duodenal atresia?
A
Double bubble sign
13
Q
What are sanctuary sites in chemotherapy?
A
Areas where the chemotherapy doesn’t reach: cancer can survive. CNS and testes
14
Q
What is the treatment for brain cancer?
A
- excision
- craniospinal radiotherapy
- chemotherapy
15
Q
What is an ependymoma?
A
Tumour of the epndymal cells that line the ventricles
16
Q
What is the most common site of ependymoma in children compared to adults?
A
Children: intracranial
Adults: spinal
17
Q
How do ependymoma typically present?
A
Obstructive hydrocephalus
18
Q
What population are CNS germ cell tumours typically seen in?
A
Teenage males
19
Q
Where do germ cell tumours typically arise?
A
Pineal or suprasellar regions of the brain
20
Q
What is the most common type of CNS germ cell tumour?
A
Germinoma
21
Q
What are secreting tumours characterised by?
A
Raised markers (AFP or hCG) in serum or CSF
22
Q
What are the different types of germ cell tumours?
A
Germinomas (pure germ cell tumours) and non germinomatous tumours (secrete chemicals into CSF + bloodstream). Types of non germinomatous tumours: terotomas, choriocarcinomas, yolk sac tumours, mixed
23
Q
How are CNS germ cell tumours treated?
A
- teratoma: surgery
- all others: radiotherapy and chemotherapy
24
Q
What hormone do teratomas secrete?
A
hCG; however, most do not actually secrete it
25
What is the most common leukaemia in children?
ALL
26
What is the 2nd most common leukaemia in children?
AML
27
What age does ALL most commonly effect?
2-3 years
28
What age does AML typically affect?
Under 2 years
29
What are risk factors for leukaemia in children?
- radiation exposure in pregnancy
- Down's
- Kleinfelter syndrome
- Noonan syndrome
- Fanconi's anaemia
30
How does leukaemia typically present?
Persistent fatigue, unexplained fever, failure to thrive, weight loss, night sweats, pallor, petechiae and abnormal bruising and unexplained bruising (due to thrombocytopenia), abdominal pain, generalised lymphadenopathy, bone/joint pain
31
What should be done for any child presenting with unexplained petechiae or hepatomegly?
Immediate specialist assessment
32
What is the management for a child presenting with queried leukaemia?
Full blood count in 48 hours
33
What might be seen on a blood film in leukaemia?
Blast cells
34
What is the management of leukaemia?
Primarily chemotherapy. Can also do radiotherapy, bone marrow transplant, surgery
35
What are poor prognosis factors for CLL?
- <2 years or >10 years
- WBC >20*10^9/l at dx
- T or B cell surface markers
- non caucasian
- male sex
36
What condition might be associated with gingivial hyperplasia?
AML
37
What is Wilms' tumour?
A tumour affecting the kidney in children, generally under 5
38
What is the presentation of Wilms' tumour?
Consider in any child under 5 presenting with a mass in the abdomen. Also:abdominal pain, haematuria, lethargy, fever, HTN, weight loss
39
What is the first line investigation for Wilms tumour?
Ultrasound. Then CT/MRI to stage, and biopsy for histology
40
What is the management of Wilms' tumour?
Surgical excision of the tumour along with the affected kidney (nephrectomy). May also give adjuvant chemo or radiotherapy
41
What is prognosis of Wilms tumour?
Good
42
If a child presents with an unexplained abdominal mass, what is the next steps?
Urgent paediatric review within 48 hours
43
What is a neuroblastoma?
A tumour arising from the neural crest tissue of the adrenal medulla and sympathetic nervous system
44
What are the features of neuroblastoma?
Abdominal mass, pallor, weight loss, limp, bone pain, hepatomegaly, paraplegia, proptosis
45
What is a retinoblastoma?
Ocular malignancy
46
What is the average age of diagnosis of retinoblastoma?
18 months
47
What is the cause of retinoblastoma?
Can be sporadic, but there are familial forms that are autodomal dominant
48
What is the presentation of retinoblastoma?
absence of red reflex of the eye (leukocoria), strabismus, visual problems
49
What is the management?
Treatment may involve radiotherapy or enucleation (removal of the eye), with chemotherapy
50
What cancer is associated with auer rods?
AML
51
What is the most common malignant bone tumour?
Osteosarcoma
52
Where do osteosarcomas most commonly occur?
In th elong bones prior to epiphyseal closure
53
What is seen on xray for osteosarcomas?
Codman triangle and a sunburst pattern
54
What tumour may be associated with retinoblastoma?
Osteosarcoma: both have mutation of Rb gene
55
Where does Ewing's sarcoma typically occur?
In the pelvic and long bones
56
What is seen on a xray in Ewing's sarcoma?
Onion skin appearance
57
What is the presentation of osteosarcoma?
Localised pain and swelling, pathological fracture
58
What lobe of the liver does a hepatoblastoma typically begin in?
Right
59
What is tumour lysis syndrome?
When chemicals are released when cells are destroyed by chemo. Results in: high uric acid, high potassium, high phosphate, low calcium
60
What can reduce tumour lysis syndrome?
Allopurinol can reduce uric acid. Can also reduce AKI
61
What age is Hodgkin's lymphoma most common?
Bimodal peaks: 20-25 and 80
62
What are risk factors for hodgkins lymphoma?
HIV, EBV, autoimmune conditions (such as rheumatoid arthritis and sarcoidosis), FHx
63
What are three types of non-hodgkins lymphoma?
Diffuse large B cell lymphoma, Burkitt Lymphoma, MALT lymphoma
64
What types of Non-Hdgkins lymphoma are particularly associated with HIV?
Burkitt
65
What are risk factors for non-hodgkins lymphoma?
HIV, EBV, H.pylori infection, Hep B or C, pesticides exposure, FHx
66
What are B symptoms of lymphoma?
Fever, weight loss, night sweats
67
What unique symptom will patient's with Hodgkin's lymphoma experience?
Lymph node pain after drinking alcohol
68
What is a typical finding for Hodgkin's lymphoma on biopsy?
Reed-Sternberg cells: owl cells
69
What is the classification used for lymphoma?
Lugano classification: replacing the old Ann Arbor system
70
What are b symptoms associated with in Hodgkin's lymphoma?
Poor prognosis
71
What is each of the stages of lymphoma?
1: single node/group of nodes
2: more than one group of nodes but on same side of the diaphragm
3: multiple nodes but both sides of the diaphragm
4: widespread involvement and symptoms outside fo the lymph nodes
72
What does rituximab target?
B cells
73
What are the two chemotherapy regimes that are used in Hodgkins lymphoma management?
ABVD (standard). Also BEACOPP.
74
Which type of lymphoma is most common?
Non-hodgkins
75
Which type of lymphoma more commonly has extra nodal disease?
Non hodgkins
76
What is seen on biopsy for Burkitt's lymphoma?
'Starry sky' appearance
77
What are the steps of newborn resuscitation?
1. Dry baby and maintain temperature
2. Assess tone, RR, HR
3. If gasping or not breathing give 5 inflation breaths
4. Reassess chest movements
5. If the heart rate is not improving and <60 BPM start compressions and ventilation breaths at a rate of 3:1
78
What is assessed in an apgar score?
Appearance (skin colour), pulse, grammace (response to stimulation), activity (muscle tone), respiration
79
What is the compression to ventilation ratio in PBLS?
If one person, 30:2, and if two people, 15:2
80
What is the rate of chest compressions in PBLS?
100-120
81
What are risk factors for neonatal sepsis?
Vaginal GBS colonisation, GBS sepsis in a previous baby, maternal sepsis/chorioamnionitis/fever, prematurity (<37 weeks), early rupture of membranes, PROM, low birth weight
82
What are red flags for potential neonatal sepsis?
Confirmed or suspected sepsis in the mother, signs of shock, seizures, term baby needing mechanical ventilation, respiratory distress starting more than 4 hours after birth, presumed sepsis in another baby in multiple pregnancy
83
What is the most common presentation of neonatal sepsis?
Respiratory distress, such as grunting, nasal flaring, use of accessory respiratory muscles, tachypnoea
84
How is diagnosis usually established in neo natal sepsis?
Blood culture
85
What baseline investigations should be done for neonatal sepsis?
FBC and CRP
86
What abx are used in treatment of sepsis/suspected sepsis?
BenPen and gentamycin
87
What can acidosis on the umbilical artery blood gas indicate in a newborn?
Hypoxic Ischaemic Encephalopathy
88
What are the risk factors for necrotising enterocolitis?
Low birth weight/premature, formula feeds, respiratory distress and assisted ventilation, sepsis, patent ductus arteriosus and other congenital heart conditions
89
What is the presentation of necrotising enterocolitis?
Intolerance to feeds, vomiting (particularly with green bile), generally unwell, distended abdomen, absent bowel sounds, blood in stools
90
What are signs seen in xray for necrotising enterocolitis?
Dilated loops of bowel, bowel wall oedema, pneumatosis intestinalis (gas in bowel wall), free gas in the peritoneal cavity
91
What is the management of necrotising enterocolitis?
NBM with IV fluids, total parenteral nutrition and abx. Surgical emergency
92
What population is bronchopulmonary dysplasia seen in?
Premature: often before 28 weeks
93
What is the diagnosis of bronchopulmonary dysplasia based upon?
- chest xray changes
- infants requiring oxygen therapy after 36 weeks
94
What are the features of bronchopulmonary dysplasia?
Low O2 saturations, increased work of breathing, poor feeding and weight gain, crackles and wheezes on chest ausculatation, increased susceptibility to infection
95
What is the preventative method for bronchopulmonary dysplasia?
Give corticosteroids if in premature labour before 36bweeks
96
What can be done when a newborn is born to prevent development of bronchopulmonary dysplasia?
- CPAP rather than intubation and ventilation
- caffeine to stimulate respiratory effort
- not over oxygenating
97
What treatment is given to babies with bronchopulmonary dysplasia?
Montly injections of palivizumab to reduce risk of bronchiolitis
98
What are risk factors for respiratory distress syndrome?
Male sex, diabetic mothers, c-section, second born of premature twins
99
What is seen on chest xray in surfactant deficient lung disease?
Ground glass appearance
100
What population is meconium aspiration most common in?
Term or post term babies
101
What causes meconium aspiration?
In utero peristalsis, triggered by foetal hypoxic stress, or vagal stimulation due to cord compression
102
What can meconium aspiration cause?
- partial or total airway obstruction
- foetal hypoxia
- pulmonary inflammation
- infection
- surfactant inactivation
- persistent pulmonary hypertension
103
What are maternal RF for meconium aspiration syndrome?
maternal HTN, pre-eclampsia, chorioamnionitis, smoking, substance abuse
104
Describe the excretion of bilirubin
When RBC breakdown, they release unconjugated bilirubin into the blood. This is conjugated in the liver. This is excreted via urine/stool, or reabsorbed in the small intestine
105
What causes physiological jaundice in the newborn?
- neonates have v high concentration of RBC, and a reduced liver function
- fetal RBC break down more rapidly than RBC after delivery
- this would normally be excreted via the placenta, but when they are born, it will lead to a normal rise in bilirubin. This causes mild jaundice
106
What days is physiological jaundice seen?
Days 2-7, and typically clears by 10 days
107
When is jaundice pathological?
If within the first 24 hours of life
108
What can cause neonatal jaundice due to increased production of bilirubin?
Haemolytic disease of the newborn, ABO incompatibility, haemorrhage, intraventricular haemorrhage, cephalo-haematoma, polycythaemia, sepsis and DIC, G6PD deficiency
109
What can cause neonatal jaundice due to decreased clearance of bilirubin?
Prematurity, breast milk jaundice, neonatal cholestasis, extrahepaic biliary atresia, endocrine disorders, Gilbert syndrome
110
What is kernicterus?
Brain damage due to high bilirubin levels
111
What can cause exaggerated jaundice in premature babies?
Due to exaggerated response from the immature liver
112
Why can breast milk cause jaundice?
Some components of breast milk inhibit ability of the liver to process bilirubin. If inadequate breastfeeding may cause slow passage of stools, and increasing jaundice
113
When is jaundice considered prolonged?
More than 14 days in full term babies, and more than 21 days in premature babies
114
What should be ruled out if there is persistent jaundice?
Biliary atresia, hypothyroidism, G6PD deficiency
115
What investigations should be done in neonatal jaundice?
- FBC + blood film
- conjugate bilirubin
- blood type testing
- direct coombs test (for haemolysis)
- thyroid function
- blood and urine cultures
116
How is neonatal jaundice monitored?
Treatment threshold charts. This will be plotted
117
What is first line for correcting neonatal jaundice?
Phototherapy
118
What might extreme jaundice need?
Exchange transfusion
119
What is the process of photo therapy?
Phototherapy converts unconjugated bilirubin into isomers that can be excreted in the bile and urine without requiring conjugation in the liver. Phototherapy involves removing clothing down to the nappy to expose the skin and eye patches to protect the eyes. Blue light is the best at breaking down bilirubin. A light-box shines blue light on the baby’s skin.
120
Why can jaundice cause kernicterus?
Bilirubin can cross the blood brain barrier. Excess bilirubin causes direct damage to the CNS, and can lead to long term complications such as cerebral palsy, learning difficulties, deafness
121
What is gastroschisis?
Congenital defect in the anterior abdominal wall lateral to the umbilical cord. Intestines may come through
122
What is the management of gastroschisis?
Vaginal delivery can be attempted, newborns should go straight to theatre
123
What is exomphalos (omphalocoele)?
When the abdominal contents protrude through the anterior abdominal wall
124
What is the management of exomphalos?
C-section to reduce risk of sac rupture, and a staged repair.
125
What can cause intestinal obstruction?
Meconium ileus, Hirschspung's. disease, oesophageal atresia, duodenal atresia, intussusception, imperforate anus, malrotation with a volvulus, strangulated hernia
126
What is found on investigations for biliary atresia?
Conjugated bilirubin is abnormally high
127
What is seen on investigation for duodenal atresia?
Double bubble sign on AXR
128
When does malrotation with volvulus typically present?
Usually around 3-7 days after birth
129
What procedure treats malrotation with volvulus?
Ladd's
130
What is seen on AXR for jejunal/ileal atresia?
Air fluid levels
131
What drug can increase the risk of cleft lip and palate?
Maternal antiepileptic use
132
What lobe of the brain does herpes simplex encephalitis characteristically affect?
The temporal lobes
133
What causes retinopathy of prematurity?
Abnormal blood vessel development and scarring, due to higher oxygen concentrations a preterm baby will be exposed to
134
What group of babies are typically affected by retinopathy of prematurity?
Those born before 32 weeks gestation
135
What does TORCH stand for?
Toxoplasmosis, Other (Syphilis, parvovirus B19, VZV, listeria), rubella, CMV, HSV-2
136
What classically spreads toxoplasmosis?
Contamination with faeces from a cat that is a host of the parasite
137
What is the triad of symptoms in congenital toxoplasmosis?
Intracranial calcification, hydrocephalus, chorioretinitis (can also get blueberry muffin rash)
138
What are the symptoms of congenital syphilis?
Hydrops fetalis, maculopapular rash, bone and facial deformities (saddle nose, big forehead, small chin, Hutchinson teeth, Saber shins, deafness)
139
What occurs in congenital parvovirus B19?
hydrops fetalis, due to suppression of fetal erythropoiesis
140
What symptoms can varicella zoster cause in pregnant for the mother?
More severe case, leading to pneumonitis, hepatitis, or encephalitis
141
When is chickenpox transmission most risky in pregnancy?
In the 1st + 2nd trimester
142
What is the presentation of congential varicella zoster syndrome?
Low birth weight, limb atrophy, microcephaly, eye + neuro defects
143
In pregnancy, when can a woman recieve acyclovir for varicella?
If they present with a rash within 24 hours of development and more than 20 weeks gestation
144
Where might mothers pick up a listeria infection in pregnancy?
Unpasteurised dairy
145
What is the presentation of congenital listeria?
Hydrops fetalis, nodular skin rash, sepsis/menginitis, gastroenteritis
146
When is the risk of congenital rubella syndrome the highest?
First 3 months of pregnancy
147
Can you vaccinate for MMR during pregnany?
NO: it is a live vaccine. Give after giving birth
148
What Ig's are raised in past vs current infection?
IgM: current infection. IgG: past infection
149
What must be done with any suspected cases of rubella in pregnancy?
Discuss with the local health protection unit
150
What are the triad of symptoms of congenital rubella infection?
Deafness, cataracts, heart defects (+blueberry muffin rash)
151
What are the symptoms of congenital CMV?
Fetal growth restriction, seizures, hearing loss, vision loss, microcephaly, cerebral palsy, petechiae +/- blueberry muffin rash
152
What is the treatment for a herpes simplex attack after 28 weeks in pregnancy?
C-section
153
What are the symptoms of congenital HSV-2?
Vesicular skin lesions, meningoencephalitis (lethargy, seizures, irritability)
154
How does fetal blood bypass pulmonary circulation?
The three shunts: ductus venosus, foramen ovaela, ductus arteriosus
155
What does the ductus venosus do?
Shunt that connects the umbilical vein to the IVC and allows bypass of the liver
156
What does the foramen ovale do?
Connects the RA and LA, allows blood to bypass the RV and pulmonary circulation
157
What does the ductus arteriosus do?
Connects the pulmonary artery with the aorta and allows bypass of the pulmonary circulation
158
What occurs with the babies first breaths and the heart?
- alveoli expand, decreased pulmonary vascular resistance
- fall in pressure in the right atrium
- LA has greater pressure, squashes the atrial septum and closes foramen ovale
159
What closes the ductus arteriosus?
Prostaglandins required to keep it open; increased blood oxygenation causes a drop in circulating prostaglandins
160
What causes the closure of ductus venosus?
Umbilical cord is clamped and there is no flow in the umbilical veins
161
What does the foramen ovale become?
fossa ovalis
162
What does the ductus arteriosus become?
Ligamentum arteriosum
163
What does the ductus venosus become?
Ligamentum venosum
164
What are features of innocent murmurs?
Soft, short, systolic, symptomless, situation dependent
165
What investigations should be done for a childhood murmur?
ECG, CXR, ECHO
166
What can cause a pan systolic murmur?
Mitral regurgitation, tricuspid regurgitation, ventricular septal defect
167
What can cause an ejection systolic murmur?
Aortic stenosis, pulmonary stenosis, hypertrophic obstructive cardiomyopathy, Turners syndrome
168
What causes splitting of the second heart sound?
During inspiration, there is negative intra-thoracic pressure. The right side of the heart fills faster, and fills more, which can cause a delay in the pulmonary valve closing. This might make it close slightly after the aortic valve
169
What murmur is heard with atrial septal defects?
Mid systolic, crescendo-decrescendo, and fixed split second heart sound that has no variation with respiration
170
What is heard with a patent ductus arteriosus?
Continous crescendo-descrendo machinery murmur that might mask the second heart sound
171
What causes the murmur in tetraology of fallot?
Pulmonary stenosis
172
What murmur is heard in tetraology of fallot?
Ejection systolic
173
What causes cyanosis?
When deoxygenated blood enters the systemic circulation, thus in a right to left shunt
174
What causes a right to left shunt and therefore cyanotic heart disease?
VSD, ASD, PDA, transposition of the great arteries
175
Why are VSD, ASD and PDA not usually cyanotic?
Pressure in the left side of the heart is much greater than the right, and blood flows in the high pressure direction
176
What is eisenmenger syndrome?
If pulmonary pressure increases beyond the systemic pressure, it will flow right to left across the defect.
177
Do patients with transposition of the greater arteries have cyanosis?
Yes
178
When does the ductus arteriosus usually completely close by?
First 2-3 weeks of life
179
What infection might cause PDA?
Rubella
180
What is a large risk factor for PDA?
Prematurity
181
What shunt occurs in PDA, and what can be a complication?
- left to right shunt (aorta to pulmonary vessels)
- can lead to pulmonary hypertension, and right sided heart strain as the the RV contracts against increased resistance, can lead to RV hypertrophy
- left ventricular hypertrophy can occur as more blood returns to the left side of the heart
182
What is the presentation of PDA?
Dyspnoea, difficulty feeding, poor weight gain, frequent respiratory infections
183
How is PDA dx?
ECHO
184
Up until what age might the PDA be monitored as it might close on its own?
1 year
185
What are the features of patent ductus arteriosus?
- continous machinery murmur
- large volume, bounding, collapsing pulse
- left subclavicular thrill
- wide pulse pressure
186
What medical management can be given for PDA?
Indomethacin or ibuprofen given to the neonate, inhibits prostaglandin synthesis, will close the connection
187
How do atrial septal defects develop?
Two walls (the septum primum and septum secondum) fused with the endocardial cushion to separate. If there is defects can lead to defect
188
What can ASD lead to and why?
Can lead to eisenemngers syndrome due to the pulmonary hypertension. This is because as there is more blood on the right atrium there is overload and right heart strain, which can lead to right heart failure and pulmonary hypertension
189
What is the most common ASD?
Ostium secondum
190
What are potential complications of ASD? (4)
- stroke due to VTE
- AF and atrial flutter
- pulmonary HTN + right sided heart failure
- Eisenmengers syndrome
191
Why can ASDs lead to strokes in patients with DVT?
Clot will not travel to the lungs: will move across defect and up into the brain. Could be the first presentation of asymptomatic ASD
192
What condition might be linked with PFO?
Migraine with aura (woof)
193
What is the management for ASD?
- refer all to paediatric cardioology
- small + asymptomatic: watch and wait
- large: transvenous catheter closure or open heart surgery
194
What conditions is VSD most commonly associated with?
Downs and Turners
195
What is a possible complication of VSD that should have prophylaxis in surgery?
Infective endocarditis
196
What is the most common cause of congenital heart disease?
Ventricular septal defect
197
What are possible complications of VSD?
Aortic regurg, infective endocarditis, Eisenmengers, right heart failure, pulmonary hypertension
198
What are the underlying lesions that can cause Eisenmenger's syndrome?
ASD, VSD, PDA
199
What is the pathophysiology of Eisenmenger's?
- over time, the right side of the heart increases in pressure, can lead to pulmonary hypertension. If this increases over systemic pressure can reverse the shunt
- deoxygenated blood bypasses the lungs and enters the body
200
How does Eisenmenger's lead to polycythyaemia rubra?
Low O2 blood sats leads to bone barrow producing more RBC and haemoglobin which leads to polycythaemia. The blood becomes more viscous and there is a higher risk fo clots
201
What examination findings are associated with pulmonary HTN?
Right ventricular heave, raised JVP, peripheral oedema
202
What findings are associated with a right to left shunt and chronic hypoxia?
Cyanosis, clubbing, dyspnoea, plethoric complexion
203
What is the only definitive management of Eisenmengers?
Heart lung transplant: it is not possible to medically reverse the condition
204
What is a treatment for pulmonary hypertension
Sildenafil
205
What is coartication of the aorta?
When there is a narrowing of the aorta arch usually around the evel of ductus arteriosus
206
What condition is particularly associated with coartication of the aorta?
Turners
207
What is often the only sign of coartication of the aorta in a neonate?
Weak femoral pulses. There may be raised blood pressure in limbs supplied by arteries before the narrowing, and lower in those after
208
What murmur is typically heard in coartication of the aorta?
Systolic, below the left clavicle and left scapula
209
What limbs might poorly develop in children with coartication of the aorta?
Legs and left arm
210
What is the management of severe coartication of the aorta?
Keep the ductus arteriosus open using prostaglandin E, to allow some blood flow through distal to the coartication
211
How does aortic stenosis typically present?
symptoms of fatigue, shortness of breath, dizziness, fainting, worse on exertion, heart failure if very severe
212
What are the features of aortic stenosis on examination?
- ejection systolic murmur, crescendo-descrendo character
- radiates to the carotids
- ejection click
- palpable thrill
- slow rising pulse and narrow pressure pulse
213
How is aortic stenosis diagnosed?
ECHO is gold standard
214
What are the possible treatment options for aortic stenosis?
Percutaneous balloon aortic valvoplasty, surgical aortic valvotomy, valve replacement
215
What is pulmonary stenosis typically associated with?
Tetralogy of fallot, william syndrome, noonan syndrome, congenital rubella
216
What is the presentation of pulmonary valve stenosis?
Fatigue on exertion, SOB, dizziness, fainting
217
What is found on examination for pulmonary stenosis?
Ejection systolic mumur, palpable thrill of the pulmonary area, right ventricular heave, raised JVP
218
What is the management of pulmonary stenosis?
- mild: watch and wait
- severe: balloon valvuloplasty via venous catheter
219
What is the most common cause of congenital cyanotic heart disease?
ToF
220
What are the four features of ToF?
Right ventricular septal defect, right ventricular hypertrophy, pulmonary stenosis due to RV outflow tract obstruction, overrising aorta
221
What determines the severity of ToF?
Degree of pulmonary stenosis
222
What is the pathophysiology of ToF?
Pulmonary stenosis means that more blood is encouraged to flow across the defect. additionally, the over riding aorta pulls more blood up from the right right ventricle. Therefore, more deoxygenated blood moves around the body in the right to left shunt. This movement causes right ventricular hypertrophy.
223
What are risk factors for ToF?
Rubella infection, increased age of mother (over 40), alcohol consumption in pregnancy, diabetic mother
224
What is seen characteristically on a CXR in ToF?
Boot shaped heart due to the right ventricular thickening
225
What is produced during ECHOs in ToF?
Doppler flow studies
226
What are the signs and symptoms of ToF?
Cyanosis, clubbing, poor feeding, poor weight gain, ejection systolic murmur heard loudest at pulmonary area, tet spells
227
What are tet spells?
Intermittent periods where the right to left shunt becomes worsened, causing a cyanotic episode. Typically when pulmonary vascular resistance increases of systemic resistance decreases
228
What can trigger tet spells?
Child exercising and producing CO2 which is a vasodilator and leads to reduced systemic vascular resistance. Also can occur when infant is upset, in pain, or has a fever
229
What is the management of tet spells?
Supplementary oxygen, beta blockers to relax the right ventricle, IV fluids to increase pre load, morphine to decrease respiratory drive
230
What is the management of ToF?
Surgery, and prostaglandin infusion maintains the ductus arteriosus to allow blood flow back to the pulmonary arteries
231
What is Ebsteins anomaly?
Congenital condition where the tricuspid valveis lowered and leads to ventricularisation of the right atrium.
232
What can ebstein's anomaly associated with?
Lithium use in pregnancy, atrial septal defects, wolf parkinson white syndrome
233
What is the presentation of Ebstein's anomaly?
Evidence of heart failure (eg, oedema), gallop rhythm of the heart (addition of S3+S4), cyanosis, SOB and tachypnoea, poor feeding, collapse or cardiac arrest
234
What change might be seen in the JVP in Ebstein's anomaly?
Prominent a wave
235
What is wolff parkinson white syndrome?
congenital accessory conducting pathway between the atria and ventricles leading to atrioventricular re-entry tachycardia (AVRT). As the accessory pathway does not slow conduction AF can degenerate rapidly to VF
236
What ECG is associated with wolff-parkinson white syndrome?
Delta wave
237
What occurs in transposition of the great arteries?
The aorta and the pulmonary trunk are swapped: the RV pumps blood into the aorta, and LV pumps blood into the pulmonary vessels
238
What conditions might transposition of the great arteries be associated with?
VSD, coartication of the aorta, pulmonary stenosis
239
What are the clinical features of TGA?
Cyanosis, tachypnoea, loud single S2, egg on a string appearance on CXR, prominent right ventricular impulse
240
What is the management of TGA?
Maintain ductus arteriosus with prostaglandins, surgical correction is definitive
