Cardiovascular and respiratory Flashcards
What are causes of acyanotic congenital heart disease? name 3
1) ventricular septal defect
2) atrial septal defect
3) patent ductus arterosus
what is the likely outcome of a small ventricular septal defect ?
closes spontaneously by the age of 5
How does a large VSD present typically?
infants often present with dyspnea while feeding
what is the consequence of a large VSD?
volume overload and heart failure leading to right ventricular hypertrophy and potentially eisenmenger syndrome (reversal of shunt direction)
what is the definitive management of a VSD?
surgical management
does Atrial septal defect present with a murmur?
no
what can ASD lead to?
HF and chest infections
what is the definitive management of ASD?
closed surgically with transcatheter occlusion
describe the murmur associated with a patent ductus arteriosus?
continuous machinery murmur loudest below left clavicle and radiating to the back
describe the pulse associated with a PDA?
collapsing pulse
what is PDA associated with ? name 2
1) prematurity
2) hypoxia
what is the treatment of PDA?
prostaglandin inhibitors (IV ibuprofen)
what is the management of persistent PDA?
transcatheter occlusion or surgical ligation
Name the 5 cyanotic congenital heart diseases?
1) truncus arteriosus
2) transposition of the great arteries
3) tricuspid atresia
4) tetralogy of fallot
5) total anomalous pulmonary venous return
management of truncus arteriosus??
Needs surgical closure
transposition of great arteries is a….. lesion?
duct dependent lesion
what is the management of transposition of great arteries prior to surgical management ?
Prostaglandin infusion to keep PDA open
operation name for repair of transposition of great arteries ?
switch operation
components of tetraology of fallot? 4
pulmonary stenosis
right ventricular hypertrophy
overriding aorta
ventricular septal defect
murmur associated with tetralogy of fallot?
ejection systolic murmur radiating to the back
what is the tet position ?
squatting position to increase systemic vascular resistance to reverse right to left shunt. becomes left to right shunt across the VSD
what is a complication of all congenital heart diseases ?? and what is the commonest organism
infective endocarditis
strep viridans
what are signs of co-arctation of the aorta? 3
1) systolic murmur radiating to the back
2) blood pressure in the arms > legs
3) radio-femoral pulse delay
what is the management of infective endocarditis ?
IV benzylpenicillin + IV Gentamicin for 6 weeks
how does severe coarctation of the aorta present when the ductus arteriosus closes?
presents with shock
what is a genetic condition that co-arctation of the aorta is associated with ?
Turner syndrome (45 XO)
how does mild coarctation of the aorta present ?
later in life signs of left sided heart failure
what is the definitive management of co-arctation of the aorta?
bypass graft
define Eisenmenger syndrome
occurs when a heart condition cause a left to right shunt (acyanotic conditions) progresses to cause irreverssible pulmonary hypertension which reverses the shunt to become right to left
what is the management of Eisenmenger syndrome?
heart-lung transplantation
what are the features of innocent murmurs (physiology)?
7 S
soft, short, systolic, S1 and S2 normal, symptomless, standing and sitting variation, special tests normal (ECG)
define Wolf-Parkinson-White syndrome?
a type of supraventricular tachycardia caused by an accessory electrical pathway in the heart causing a re-entry tachycardia
ECG findings of WPW?
Management of WPW (acute SVT) and definitive
management of the SVT, then percutaneous radio frequent ablation of the accessory pathway (bundle of Kent)
step wise management of SVT?
1) vagal stimulation: cold water immersion (face) - carotid massage - valsalva maneuver
2) establish IV access and give adenosine (increasing dose / 2min)
3) if non-responsive consider amiodarone/procainamide/DC shock
4) consider long term beta blocker (sotalol) - calcium channel blocker if asthmatic
name 2 infectious causes of stridor ?
Croup
epiglottitis
causes of croup?
viruses: parainfluenza, RSV, influenza, rhinovirus
what age group does group usually present in ?
6 months to 6 years
examination findings of patient with suspected croup? name 4
temperature < 38.5
barking cough
hoarse voice
able to drink and speak
onset of croup
over days
is croup a medical emergency ?
no (except if very severe)
management of croup? not severe
oral steroids (prednisone/dexamethasone ) OR nebulized budenoside
what is the scoring system used for severity of croup?
Westly score
what criteria are incorporated in the Wesley system for determining the severity of croup?
consciousness
cyanosis
stridor
air entry
retractions
management of severe croup ?
nebulized epinephrine with oxygen
what are the causes of epiglottitis?
bacteria: hemophilus influenza type B (HiB)
what age range does epiglottitis most commonly occur in?
1-6 years
what is the onset of epiglottitis?
hours (very rapid deterioration)
examination findings of patient with suspected epiglottitis? 5
temperature > 38.5
sitting upright,
drooling
toxic looking
unable to drink or speak
is epiglottitis is medical emergency ?
yes (life threatening)
management of epiglottitis
- avoid examination
- call anaesthesia/ENT
- intubation
- ICU transfer
- Bloods testing
- IV cefuroxime
what are other causes of stridor other than croup and epiglottitis? 7
- bacterial tracheitis (very similar to epiglottitis but cause by S aureus)
- Diptheria (tonsillar pseudomembrane)
- inhaled foreign body/inhaled smoke
- anaphylaxis
- trauma
- hypocalcemia (laryngospasm)
- chronic stridor: subglottic stenosis, laryngomalacia, pscychological
what age range do most cases of bronchiolitis occur in ?
1-9 months
causes of bronchiolitis ?
mostly RSV, but also influenza, parainfluenza, rhinovirus
classic presentation of bronchiolitis? 3
coryza illness with dry cough
expiratory wheeze
feeding difficulties
examination findings in suspected bronchiolitis? 4
1) late inspiratory crackles
2) wheeze
3) hyper inflated chest and displaced liver
4) signs of respiratory distress: grunting, tachypnea, nasal flaring, use of accessory muscles, subcostal and intercostal recession,
what are examination findings that are seen in severe bronchiolitis? 2
cyanosis
decreased level of consciousness
admission criteria for bronchiolitis ? 5
1) apnea
2) severe respiratory distress
3) oxygen saturation < 90%
4) oral intake <50% usual
5) high risk groups: cystic fibrosis, congenital heart disease, asthmatics, preterm
what are risk factors for severe bronchiolitis? 7
1) premature birth,
2) passive smoke exposure, 3) crowded living conditions, 4) congenital heart disease, 5) cystic fibrosis,
6) immunodeficiency
7) chronic lung disease.
management of bronchiolitis?
Supportive including:
1) fluids, NG if poor oral intake
2) CPAP if low O2 saturation
do steroids and bronchodilators have a role in the management of bronchiolitis?
no evidence for the effectiveness of either
what medication can be used for the prevention of bronchiolitis ? how often is it give ? who is it given to
palivizumab
monthly injections
high risk groups
define asthma? how common is it?
- a chronic illness characterized by a reversible airway obstruction, manifesting as wheeze
- 1 in 11 children
key features of asthma - symptoms (3) and signs (3) and family history (1)
1) characteristic symptoms of cough, dyspnea, and wheeze
2) symptoms worse at night
3) interval symptoms between acute exacerbations
4) personal and family history of atopy: eczema, hay fever, allergies
5) examination findings: hyper inflated chest and Harrison sulcus (chronic severe asthma)
diagnosis of asthma?
1) in very young children: history and examination is diagnostic
2) skin prick testing to identify allergens
3) spirometry for children > 5 years old (superior to peak expiratory flow rate)
criteria for diagnosis of asthma based on spirometry? and why can this not be used to diagnose asthma in a child less than 1
- FEV1 improvement of >12% following the use of bronchodilators (used for diagnosis and severity)
- cant be used for children younger than 1 year old due to the absence of beta2 - adrenoreceptor mechanism
Side of effects of long term inhaled corticosteroids? (6)
hoarse voice (irritation of the upper aero digestive tract)
oral candidiasis
growth deceleration
osteoporosis
cataract
weight gain
side effects of short acting beta adrenergic agonists? 4
tremor
tachycardia
palpitations
dry mouth
define acute exacerbation of asthma
an acute episode characterized by:
1) severe asthma symptoms (dyspnea, cough, wheeze, cyanosis)
2) triggered by: URTI, viruses, exercise, cold, allergens
3) not responding to high dose inhaled bronchodilators
what is critical to determine when a patient presents with an acute exacerbation of asthma?
is it severe or life threatening
criteria for severe exacerbation of asthma? 7
1) 33-55% predicted peak flow
2) <92% oxygen saturation
3) respiratory rate > 30 (over ) or >40 (under 5)
4) too breathless to feed or talk
5) Heart rate > 125 (over 5) or >140 (under 5)
6) use of accessory muscles of respiration
7) audible wheeze
classification for life threatening acute exacerbation of asthma? 8
1) SpO2 < 92%
2) Peak expiratory flow rate (PEFR) < 33% predicted
3) silent chest
4) poor respiratory effort
5) altered consciousness
6) agitation/confusion
7) exhaustion
8) cyanosis
Immediate management of acute asthma exacerbation
- calm and reassurance
- oxygen: SaO2 < 94% should receive high flow oxygen to maintain saturations between 94-98%
- bronchodilators: inhaled SABA (salbutamol) - via nebulizer. Inhaler and spacer device is as effective as nebulizer in children with mild to moderate asthma
- Ipatropium bromide (anti-muscarinic) added in if no or poor response to inhaled SABA
- corticosteroids: a short course (3 days) of steroids should be commenced. oral prednisolone is first line, however if the child vomits or is too unwell to take oral medication IV hydrocortisone should be used
Second line management for acute asthma exacerbation? 2
- intravenous salbutamol can be considered with specialist input if there is no response to inhaled bronchodilators. it is essential to monitor for salbutamol toxicity
- magnesium sulphate can be considered, as it has an effect as a bronchodilator
admission criteria for acute exacerbation of asthma? 4
- exhausted
- not responding clinically to high dose SABA
- oxygen saturation < 92%
- FEV1 < 50 %
define mild acute asthma exacerbation? 4
- SaO2 > 92% on room air
- vocalizing without difficulty
- mild chest wall recession
- moderate tachypnea
define moderate acute exacerbation of asthma? 3
- SaO2 < 92%
- breathless
- moderate chest wall recession
what to look for on examination of a patient with asthma? 7
- finger clubbing (not suggestive of asthma, more suggestive of CF or bronchiectasis)
- chest shape - a hyperinflated chest suggests poorly controlled asthma
- chest symmetry
- breath sounds
- presence of crepitations (not suggestive of asthma)
- presence of wheeze
- examination of throat to assess for tonsillar enlargement: infectious cause
**clinical examination of children with asthma is almost always normal in between acute episodes
common mutations associated with cystic fibrosis ?
deltaF508
G551D
Cystic fibrosis is an autosomal ….. condition caused by mutations on chromosome ….
recessive
7
what is the carrier rate for Cystic fibrosis ?
1 in 25
pathophysiology of cystic fibrosis?
CFTR is either absent or defective resulting in a dysregulated eletrolyte level. the net result is an impaired clearance and retention of mucopurulent secretions
3 ways cystic fibrosis can present ? (lungs, intestines, pancreas)
- lungs: mucus plugging (mucous cough), recurrent infections
- intestines: meconium ileus (15% of patients): distal intestinal obstruction causing bilious vomitting in a newborn, meconium plug syndrome (less severe than ileum)
- Pancreas: malabsorption (steatorrhea and growth failure), CF induced diabetes, pancreatic insufficiency
common examination findings in patient with CF? 3
- clubbing
- nasal polyps
- crackles on auscultation
diagnosis of cystic fibrosis? screening and confirmed diagnosis. how is pancreatic insufficiency diagnosed
- most cases are detected with new born screening using heel prick test (Guthrie test) to look for immunoreactive trypsinogen.
- if heel prick test is positive genetic testing and a sweat test to look for a chloride level > 60 mmol/L
- pancreatic insufficiency is diagnosed by a decreased level of elastase-1 enzyme (which measures the exocrine pancreatic function)
Management of Cystic fibrosis?
- MDT (pediatrics, respiratory, dietician, physiotherapy)
- immunize up to date
- Airway clearance and chest symptom management: chest physiotherapy twice daily (pulmonary toilet). increase airway secretion clearance which should reduce airway obstruction and minimize risk of infection
Mucolytics and DNA:
- DNase is inhaled and reduces the viscosity of mucus by digesting DNA which is abundant in the sputum of patients with CF
- hypertonic saline: can be used at the time of physiotherapy to further aid clearance - Nourishment and exercise
- physical exercise
- for those patents with pancreatic insufficiency they will need to have pancreatic enzyme supplementation (CREON) with meals which contains fats
- fat soluble vitamins. pancreatic enzyme replacement is not enough. A, D, E supplements and debate on vitamin K
- monitor growth: metabolic demands may be greater in patients with CF and so nutrition and growth are closely monitored.
- in extreme cases of malnutrition, poor weight gain it may be necessary to have supplemental of enteral feeding (e.g via gastrostomy - Managing/preventing airway infections
- infections are prolonged and are characterized by host-neutrophil response.
- patients should have continual microbiological assessment to identify organisms colonized. prefer sputum cultures
- infections should be treated with 2 weeks of antibiotics even if the child is asymptomatic. repeat culture should be sent after treatment to ensure the infection is treated
- prophylactic antibiotics are recommended until the age of 3
- regular azithromycin has been shown to reduce exacerbations and improve lung function even in those not chronically infected with pseudomonas
what are the most common organisms that cause infection in patients with CF?
staphylococcus aureus
hemophilus influenzae
pseudomonas aeruginosa
Discuss Pseudomonas infection in CF? effect of chronic infection, phenotype change of chronic pseudomonas, treatment modality
- chronic infection is associated with poor lung function hence its importance to detect and aggressively treat this infection
- it is a common infection, and initially caused by an environmental strain that is eradicable. however, chronic infection results in phenotype changes leading to colonies of pseudomonas forming biofilms where they are protected from both host and antimicrobial attack . hence when detected eradication regimen is employed
- chronic pseudomonas should be treated with inhaled antibiotics
role of regular azithromycin?
regular azithromycin has been shown to reduce exacerbations and improve lung function even in those not chronically infected with pseudomonas aeruginosa
discuss elements of infection control in CF patients.
active segregation serves to reduce cross infection patients are cohort such that ‘pseudomonas naive, patients attend different clinics to those with chronic infection and those with non-tuberculous mycobacteria attend a separate clinci
what does the annual review for CF patients entail? 5
- clinical symptoms
- course of antibiotics
- microbiological assessment
- blood tests (FBC, renal function, LFTs, vitamin A,D,E, levels, clotting profile, HbA1c)
- lung function tests
- chest x-ray
- children aged 12 years or over will normally be investigated for CF related diabetes by oral glucose tolerance test
respiratory complications of CF? 5
- allergic bronchopulmonary aspergillosis
- bronchiectasis
- hemoptysis
- pulmonary hypertension and right heart strain
- pneumothorax. associated with more severe disease
- respiratory failure
- Nasal polyps: can occur in up to 10% of children with CF which may be associated with sinusitis
Gastrointestinal complications of CF?
- rectal prolapse. management is to ensure adequate pancreatic enzyme replacement and a laxative to minimize straining
- distal intestinal obstruction syndrome: obstruction of the distal ileum and affects up to 10% of children with CF. present with colicky abdominal pain and clinical examination would reveal a palpable mass in the RUQ
- CF related liver disease: cholestasis, gallstones, liver cirrhosis
endocrine complications of cystic fibrosis?
- CF related diabetes. clinical presentation can differ to typical type 1 DM and comprises weight loss, anorexia and a fall in lung unction. Ketoacidosis is rare. should be screened at least yearly after the age of 12 at annual review
- delayed puberty: average delay of 2 years. delayed puberty has consequences such as reduced bone mineral density
how may CF present in neonates when not picked up by newborn screening?
- meconium ileus
- failure to thrive
- prolonged neonatal jaundice
how may CF present in infancy?
- failure to thrive
- recurrent chest infections
- pancreatic insufficiency: steatorrhea
how may CF present in childhood?
- rectal prolapse
- nasal polyps (strongly suspect CF in children presenting with nasal polyps
- sinusitis
how may CF present in Adolescence?
- pancreatic insufficiency; diabetes mellitus
- chronic lung disease
- distal intestinal obstruction syndrome
- gallstones
- liver cirrhosis
Examination of child with CF, things to look for? hands, face, chest, abdomen
hands: clubbing
face: nasal polyps
chest: hyper inflated, crepitations, portcath (indwelling vascular access device)
abdomen: fecal mass (if constipated/DIOS), may have scar from ileostomy (meconium ileum)
discuss infertility in men with CF? prevalence and cause
98% of men with CF are infertile due to congenital absence of the vas deferent
features of kartagener syndrome? 3
sinusitis
bronchiectasis
situs inversus (right sided heart and left sided liver)
presentation of primary ciliary dyskinesia when young?
recurrent respiratory tract infections and a persistent cough and rhinitis
presentation of primary ciliary dyskinesia in adolescents and adults?
bronchiectasis and males infertility
management of primary ciliary dyskinesia?
- immunizations: annual flu and pneumococcal vaccine
- chest physiotherapy twice daily
- minimizing complications (ENT) and respiratory
define obstructive sleep apnea?
partial or complete airway obstruction during sleep causing periods of apnea and arousal
what are predisposing factors to obstructive sleep apnea? what is the most common reason for OSA in children
- severe obesity
- craniofacial abnormalities (Pierre-Robin sequence)
- neuromuscular disease (Duchenne muscular dystrophy)
- upper airway dystonia (cerebral palsy)
- in children it is most commonly due to adenotonsillar hypertrophy
presentation of obstructive sleep apnea? 4
- a child who is sleepy, moody, a mouth breather
- hard to arouse from sleep and occasional bed wetting
- memory deterioration and problems at school
- growth decline
what is the gold standard for diagnosis of obstructive sleep apnea?
overnight polysomnograpthy (sleep study)
what does a overnight polysomnography consist of? 5
EEG
pulse oximeter
airflow
chest and abdominal movement monitor
pCO2
what apnea-hypopnea index indicates OSA?
> 1.5 hours
what is the management of OSA in children?
- most cases are managed with adenotonsillectomy
- if symptoms consist post-op: CPAP is required
- others may choose to manage conservatively with: weight loss, intranasal steroids, watchful weighing for spontaneous resolution
