Resp and cardio Flashcards
Effects of scoliosis on respiration
Management options
Respiratory effects = restrictive lung disease
- Rigid thoracic cage not allowing full expansion
- Decr insp muscle strength
- Reduced lung growth
Mx
- Bracing
- Spinal implants/distraction devices (rods allowing for growth)
- Spinal fusion (for severe scoliosis in adolescents)
Cyanosis + scar if <5 or >5
<5 - hypoplastic L heart post BCPC repair
>5 - hypoplastic L heart post Fontan
Cyanosis + no scar
Unrepaired TOF
CF complications
- Lungs
- recurrent infection, inflammation
- reduced lung function
- pulmonary HTN
- OSA
- Cardiac
- R and L heart dysfunctino
- Cor pulmonale
- ENT and sleep
- Nasal polyps
- OSA
- Dental staining
- Liver
- CF related liver disease
- Liver transplant
- Pancreas
- Hyperglycaemia and CF related diabetes
- GI
- Malabsorption, malnutrition
- Mec ileus
- Constipation, DIOS, rectal prolapse
- Infertility
- No vas deferens in 99% of boys
- Female infertility impacted by malnutrition
- Mental Health
- Depression and Anxiety
- Medications
- Chronic steroids
- Chronic/recurrent abx
CF diagnosis
- Antenatal diagnosis - family history
- Newborn screening - top % of IRT + genetics panel +/- sweat test (if additional evidence needed)
- Clinical presentation
- Mec ileus (presents before IRT result back generally)
- FTT
- Chronic resp infections/bronchiectasis
- Infertility in adults
Benefits of modulators in CF
Reduce exacerbations, daily symptoms and improve QOL
Improve FEV1
Reduce treatment burden
- Ivacaftor - stabilises BSLs → reduction of insulin requirement
Side effect - monitor LFTs
Cough
- Characteristics of cough (dry vs wet, how much sputum per day in tsp/TB/cup etc)
- Any haemoptysis?
- What type of airway clearance PT do they do? (chest wall percussion in younger kids to loosen mucus, PEP devices, oscillatory vests)
- Medications for airway clearance?
- Pulmozyme (mucolytics)
- Hypertonic saline and mannitol (mucus thinner)
- Bronchodilators (Ventolin)
- Compliance (BD) and technique
- Exercise
Investigations to ask about in patients with CF
Lungs
- Lung function (FEV1) - know their baseline; big drop from baseline necessitates admission for tune up
- Chest CT (?present of bronchiectasis)
- Sputum micro (?presence of pseudomonas or MRSA)
- If pseudomonas: age at colonisation, previous attempts at eradication (methods), antibiotics for this (inhaled tobramycin, colistin, amikacin)
- NTM (non tb mycobacterium)
- ABPA (treat with steroids)
Antibiotic questions to ask about in CF
What antibiotics do they take, route, when did they start
Any drug allergies and SEs (?resistance ?hearing loss with ahminoglycosides)
Can they take tablets?
Next steps for CF kids if dropping FEV1 despite optimisation of meds/chest PT etc
Screen for causes outside the lung
- CF related diabetes
- Malnutrition
Ventilatory support (O2, bipap etc)
Lung transplant when FEV1 <30%, or life threatening haemoptysis, or resp failure
Palliative care
Signs and mx of pancreatic insufficiency in CF
Diarrhoea, foul smelling
Faecal elastase screening
OGTT screening and HBA1C
Mgmt
- pancreatic enzyme replacement (Creon)
- supplementation of fat soluble vitamins
- endocrinology involvement +/- insulin
Mgmt of malnutrition in CF
- Dietician and food diary
- Salt replacement
- Pancreatic enzyme supplements (creon)
- Fat soluble vitamin supplements
- Appetite stimulants
- High energy shakes
- PEG
- TPN
Screening for CF related liver disease in children
Yearly liver USS and LFTs
psychosocial things to ask about in CF
Children
- Education, CF knowledge
- Compliance and tablet taking
- Developmental concerns (ASD, ADHD)
Teens
- Compliance and treatment burden
- Nutrition and body image
- Sexuality, safe sex, fertility
- Smoking and vaping
- Mental health - depression, anxiety, mortality and life goals
- Transition and the future
Family
- CF knowledge, treatment and OPC burden
- Smoking
- Sibling impacts
- Family planning
- Financials
- Location (rural vs metro, access to healthcare resources)
Environment
Other systems to ask about in CF
- Venous access, bloods (GAs, long lines vs ports)
- Needle phobia
- Immunisations - flu vax, covid >16yo, consider hep A and men B
- Endocrine
- DEXA scans for vit D deficiency
- Delayed puberty
Support groups for CF
CF apps
CHIPS
CF australia
CF organisations can provide funding for CF equipment
Respiratory consequences of NM disease (duchesses, SMA2) and screening/mgmt of these
- Inability to ventilate (hypoventilation +/- apnoeas): first nocturnal, then progression to daytime, then 24/24
- Screen with spirometry and PSG and daytime cap gas
- Mx: BiPAP
- Reduced lung capacity due to scoliosis and also collapsed lung from dependent positioning
- Screen: clinical exam and xrays
- Mx: scoliosis surgery and spinal fusion and aim to maintain ambulation as long as possible
- Inability to cough
- Screen: peak cough flow
- Mx: PT for airway clearance techniques and breathing techniques, BiPAP, cough assist
- Aspiration risk due to upper airway hypotonia
- Screen: clinical history
- Mx: formal swallow assessment from SP, change diet (thickened fluids/puree), PEJ/PEG +/- fundo
What are important considerations when starting a child on Bipap?
- Will the child die if the resp support is ceased?
- If so: contact power companies for power backup, appropriate alarms, home monitoring (spO2)
- TRAINING
- Child - mask removal and communication
- Guardian - first aid (bag mask ventilation), trache, ventilation and suction, trouble shooting for leaks and disconnection
Criteria for obstructive vs restrictive vs mixed picture spirometry
Obstructive - FEV1 (low)/FVC (low/normal) is LOW.
- Bronchodilator response is positive if >20% or >200ml change with BD
- eg: asthma, bronchiectasis (CF etc)
Restrictive
- FEV1/FVC preserved but both FEV1 and FVC are LOW
- TLC low
- eg: NMD
Mixed picture
- Low FEV1/FVC AND low TLC
- Right thoracotomy scar - ddx
CV
- PA banding
- ‘shunt’
Resp
- Pneumothorax
- Pleurectomy
- Pulmonary resections (wedge resection, pneumonectomy, lobectomy)
Left thoracotomy - ddx
CV
- CoA
- PDA ligation
- PA banding
- ‘Shunt’
Resp
- Pneumothorax
- Pleurectomy
- Pulmonary resections (wedge resection, pneumonectomy, lobectomy)
Median sternotomy ddx
Cardiac
- Cardiac transplant
- Valvular surgery/repair
- VSD/septal defects
- Fontan (hypoplastic L heart)
Left parasternal heave = ?
Right heart dilatation or hypertrophy
Palpable P2 (thrill over pulm valve) = ?
Pulmonary HTN
If there is a thrill associated with a murmur, what grade is that murmur?
Grade 4-6
- 4: easily heard murmur + thrill
- 5: thrill + murmur heard with stethoscope partly off chest wall (tilted)
- 6: thrill + murmur heard with stethoscope entirely off chest wall (5-10mm)
Grade 1 vs 2 murmur - define
Soft murmur heard
1: only in quiet surrounds
2: in noisy surrounds
Grade 3 vs 4 murmur - define
Loud/easily heard murmur heard
3: without thrill
4: with a thrill
Grade 4 murmur (murmur + thrill) heard in Suprasternal region = ?
AS
Grade 4 murmur (murmur + thrill) heard at the LUSE = ?
Pulmonary stenosis
Grade 4 murmur (murmur + thrill) heard at LLSE = ?
VSD
What effect does inspiration have on murmurs?
Increases rIght sided murmurs
What effect does expiration have on murmurs?
Increases lEft sided murmurs
Murmur loudest at RUSE - ddx?
Aortic stenosis (Eject systolic)
Pulmonary Stenosis
Murmur loudest at LUSE - ddx?
- ASD
- PS
- AS
- CoA
*all Ejection systolic
Murmur loudest at LLSE - ddx?
- VSD (pan systolic)
- AVSD
- TOF (VSD)
- TR (pan systolic)
- HOCM
Murmur loudest at apex - ddx?
- MR
- MVP
Cardiac tick box spiel / summary
In summary, today I examined XX, a XX year old boy/girl with respect to the cardiovascular system. Of note… XX appeared well/unwell today, with (ab)normal vital signs including XXX
- Growth and development
- Cyanotic/acyanotic
- Heart surgery (any scars as evidence) vs no heart surgery
- Any features of heart failure? Or chronicity ?
- Specific cardiac features
- murmur: grade, ES or pan systolic, loudest at, radiating to
- Dysmorphism
- There are/are no distinctive facial features to suggest an underlying genetic condition (list if present)
- Putting these findings together, this could be consistent with (R or L VOTO)
- In the presence of previous surgery, this murmur is consistent with
- If cyanotic: ‘palliative surgery for complex congenital heart disease’
- If acyanotic: ‘congenital heart lesion with partial correction’
- In the presence of previous surgery, this murmur is consistent with
- I would like to confirm my diagnosis with an ECG, CXR and echocardiogram
Steps of cardiovascular exam
IHUGVIDEP
Inspection
- Growth - Short ?Noonan or turners or T21. Tall marfans
- Scars (thoracotomy or sternotomy, drain scars/pacing wires), groin incisions for catheter
- Cyanosis?
Hands
- Clubbing?
- Peripheral cyanosis
- Peripheral cap refill
- Any stigmata of endocarditis (Janeway lesions, oslers nodes or splinter haemmhorages)
Wrist
- Radial pulse, radial-radial delay
- Offer to check for radial-femoral delay (to maintain report I will come back to this)
Arms
- Blood pressure
- ?Water-hammer pulse
Neck
- Check JVP at 45 degrees if >5yo
- Carotid pulse
- Scars ?central line
Eyes
- Pallor
- Scleral icterus
Mouth
- Cyanosis? (They appear cyanotic but I would like to confirm this with oxygen saturation’s)
- Dentition comment (relevant for IE)
Chest
- Inspection
- Scars
- Symmetry
- Pectus excavatum (Scooped in) or carinatum (pigeon)
- Palpation
- Heaves (palpate over sternum; palms on chest, indicates RV dilation or hypertrophy)
- Thrills (supracalvicularly, suprasternally then over each valve) - if thrill palpable then murmur is at LEAST grade 4
- Apex beat (both sides)
- Auscultation
- Listen in all 4 valve spots with Bell and diaphragm
- Listen for radiation in axilla and carotid and on the back between the scapula (coarctation or peripheral pulmonary stenosis) and supraclavicular
- Maneuvers
- Inspiration - increases R sided murmurs
- Expiration - increases L sided murmurs
- Sitting up
- Rolling to left
- Valvsalva manoeuvres (blow on your thumb)
Lungs
- Listen for creps
Abdomen
- Hepatomegaly and/or ?pulsatile liver (tricuspid regurgitation)
- Sacral oedema and peripheral oedema
Describe the features of the aortic stenosis murmur
- RUSE
- Ejection systolic
- Radiates to carotids (mostly R side)
- Carotid thrill
Describe the features of pulmonary stenosis murmur
- Ejection systolic
- LUSE
- Radiates to the back
- Thrill at LUSE
Describe the features of ASD murmur
- Ejection systolic
- Loudest at LUSE
- Widely split, fixed S2
- Diastolic murmur at apex (from incr flow across mitral valve)