Cards, Pulm Flashcards
Upper Respiratory Tract pul infections
ØCroup
ØAcute Epiglottitis
LRTI
• Asthma : Diagnosis and Treatment
ØPertussis
ØBronchiolitis
ØCystic Fibrosis
ØHyaline Membrane Disease – Respiratory Distress Syndrome of the Newborn
sounds assoc w/
croup
epiglottitis
bronchiolitis
pertussis
Croup – cough and stridor
Epiglottitis- stridor
Bronchiolitis- wheeze
Pertussis- whooping cough
infecrtious vs noninfectious causes of Acute Epiglottitis
Infectious Causes:
- Strep Pyogenes – remember this is what causes strep throat
- Strep Pneumonia
- Staph
- less likely H Flu in pedi
Noninfectious causes:
thermal causes–
- crack cocaine & marijuana smoking
- throat burns of bottle-fed infants),
caustic insults–dishwasher soap ingestion
FB ingestion–ingestion & expulsion of a bottle cap
reaction to head & neck radiation therapy
define epiglotitis vs croup
epiglottitis - acute inflammation in the supraglottic region of the oropharynx with inflammation of the epiglottis, vallecula, arytenoids, and aryepiglottic folds
croup - Inflammation of the larynx, trachea- subglottic !
etiology of croup
Viral etiology (Distinguished from Bacterial Tracheitis)
- Parainfluenza 1,2,3
- Influenza A or B (A or B may be more severe depending on the year !)
- Adenovirus, RSV
rare complication of croup
Bacterial Tracheitis- a
•Bacterial infection of the trachea -> complete respiratory failure by blockage of the trachea with swelling and purulent drainage….
si/sx of Bacterial Tracheitis-
develop over 1-3 days
- Thick purulent exudate within trachea – may obstruct upper airway
- ++Fever à pt appears TOXIC
si/sx of acute epiglottiti s
RAPID ONSET = mild s/t, fever = TOXIC appearance (resp. distress)
Muffled voice (“hot potato”)
Drooling, Pain
Labored breathing
TRIPODDING
- •Neck hyperextended
- •Mouth open
- •Chin up- sniffing
- •Leaning forward
- •Outstretched arms
As illness worsens:
- •Air Hunger
- •Stridor is a LATE FINDING
- •Restlessness
- Pre apnea -> coma -> death
dx of acute epiglottitis
lateral neck film and look for THUMB PRINT SIGN
Direct visualization with intubation and endoscopy
tx of epiglottitis
Anesthesia Stat to the ED…
KEEP CHILD CALM – NO CRYING (EMS transport!!)
Use O2 if child will tolerate
Establish 2 lines IF the patient will TOLERATE
- Intubation (2-3 days)
- IV antibiotics
- Ceftriaxone or Cefotaxime x7-10 days
- Supportive care
family tx in epiglottitis
Tx of Family - NOT CONTAGIOUS !
- if unimmunized or immunosuppressed family
- or any child <6 mo without HIB vaccine complete—
- THEN….consider Rifampin for ppx
age of epiglottitis vs croup
epi - <6 mo
croup - 3mo-5yr (2 y)
si/sx of croup
URI Si/Sx: Day 0-2
- Rhinorrhea
- Low grade temp
- +/-Cough
- +/- pharyngitis
Barking Cough: Day 0-5
- +/- stridor insp/expir
- WORSEN Day 2 and 3 of the Barking Cough (inform parents)
progression of croup
Typically occurs: 10p-4a
Resolves by day: 5-7
URI –> Barking cough—>resolution
(Day 1-2) - (2-3) ( 5-7)
tx of croup also define (mild, mod, severe)
mild
mild-mod
severe
Mild (at home)- If not seen in the office for
- •Cold air, Open freezer door, Humidified air
- •No abx
Mild-Moderate- seen in office barking cough w/ NO stridor at rest
- •Decadron (IV solution given orally ) PO: 0.6mg/kg max 10-12+mg once
- •HOME if comfortable
- •Lasts 24-72 hours
- •Remind family this gets worse day 2 and 3 !
Moderate-Severe in (office-clinic-ED): stridor at rest
- •Decadron IV solution given PO:
- •RACEMIC EPI by nebulizer : duration of action approx 2 hours- repeat as/if needed
- •watch 2-3 hours for recurrence ..i
- •if recurrence : call anesthesia = consider admission
no improvement :
- After failed racemic epi –> continuous racemic epi, IM ep -> transfer to PICU
definition & etiology of bronchiolitis
Inflammation of the bronchioles, secretions into the inflamed bronchial tree kids < 2yo (LRI)
- >50% caused by RSV
- • (parainfluenza and adenovirus)
- •bacterial- mycoplasma
si/sx of bronchiolitis
Begins with URI
- •copious clear rhinorrhea
- •congestion
- •low grade fever (101/ 102F rectally)
Then develops WHEEZING +/- crackles (rales)
- ↓ breath sounds – impending doom
Worsens day 2-5 of illness (vs croup day 2-3)
Average course of illness 10-12 days
Bronchiolitids worsens day ____of illness
croup worsens day ____ of illness
bronch Worsens day 2-5 of illness
croup day 2-3
dx and imaging of bronchiolitis
CXR: Findings :↑perihilar markings
- •If first episode of wheezing
- •If pneumonia is a consideration
Nasal Washings : PCR for RSV
tx of bronchiolitis
outpatient vs inpatient
Outpatient:
Bronchodilators: +/- helpful
- Albuterol- can make worse !
- Racemic epinephrine –rarely used…(vaponephrine) falling out of favor
Cool Mist +/- helpful –> Saline nebs
Steroids-PO (NO ICS) - Decadron, prednisolone (orapred)
Antibiotics= only if pneumonia superinfection
Inpatient: above +
Hospitalize if hypoxic :
- •awake <91-93%
- •if asleep <91%
If intubated / risk for intubation: needs a PICU available -> Impending respiratory failure
O2 to keep SpO2 above 94%
High flow 02 if sats <92% on 02
Vaccine pphx for bronchiolitis
Synagis(palivizumab)
Synagis(palivizumab) 0-6 mo indications
- Premie < 35 wks
- Chronic Lung Dz
- Cerebral palsy / other neuro dz
- CHD and/or heart transplant
- Cystic Fibrosis (CF)
- Severe immune compromise
- possibly native American Indians or Alaskans
Synagis(palivizumab) 12-24 mo indications
- Chronic Lung Dz with 02 requirement
- Heart transplant during RSV season
- Severe immune compromise
- CF with certain findings (not all CF’ers)
- Bronchopulmonary Dysplasia w/ hospitalization within 6 mo
Atopic Illnesses assoc. w/ asthma
- Atopic Dermatitis (AD) 80% have asthma or allergic rhinitis
- Food allergies: 30% have asthma
- Allergic Rhinitis
si/sx of asthma
Si/sx by age 5
Cough- dry
- •Nocturnal, cold air, seasonal, exercise, lasts >3 wks
wheeze - Pitch varies, expiratory but can be inspiratory
- Prolonged expiration is abnormal.
Could be: breathlessness, chest tightness, chest pain
Dx of asthma
Demonstration of variable/episodic expiratory airflow limitation that is reversible, w/ pre/post bronchodilators
- •An improvement >8% of FEV1
- •Must be able to perform peak flows
Exclusion of other reasons for this finding: CF, FB, RSV, etc….
first time wheezing differential
•Reactive Airway Disease (RAD)
•Bronchiolitis
tx of asthma 0-4
Mild -intermittent: SABA- Step 1
Persistent- step 2-6
- (2)Low dose ICS or cromolyn/ montelukast
- (3)Medium ICS
- (4)Medium ICS + LABA or montelukast
- (5)High dose ICS + LABA or montelukast
- (6)Add oral steroid
CONSIDER Pedi Pulmonologist at step 2 or step 3 !
tx of asthma 5-11
Mild- intermittent – Step 1: (1) SABA
Persistent Step 2-6
- (2) Low dose ICS or cromolyn, neocrodimil, montelukast or theophylline
- (3) low dose ICS + LABA or LTRA, theo, med ICS
- (4)medium ICS + LABA or Medium ICS + LTRA or theo
- (5) high dose ICS + LABA or high dose ICS + LTRA/theo
- (6) high dose ICS + LABA + oral steroid (or LTRA/theo and oral steroid
STEPS 2-4 consider allergy shots for allergic asthma patients
definition & etiology of pertussis
transmission??
coccobaccilus that colonizes the ciliated epithelium - airborne transmission
Bortadella Pertussis in US
Para pertussis causes Sporadic Pertussis (Europe
3 stages of pertussis
3-12 day incubation
Catarrhal stage: most contagious (common cold sx lasting 1-2 wks
- •runny nose, sneezing, low-grade fever, and a mild cough
Paroxysmal stage: 1-6 weeks, up to 10 wks
- The characteristic symptom is a burst, of numerous, rapid coughs.
- patient suffers from a long inhaling effort characterized by a high-pitched whoop
- Infants and young children often appear very ill and distressed -> may turn blue and vomit.
Convalescent stage: last for months.
- cough usually disappears after 2 – 3 weeks, paroxysms may recur whenever the patient suffers any subsequent respiratory infection
dx of pertusssis
Nasopharyngeal swab - takes days –week to return
- High clinical suspicion= Clinical Diagnosis !
- TREAT and watch for results
si/sx of Children/infants w/ paroxysms in pertussis
Children/infants w/ paroxysms = Respiratory distress
- Tongue protruding
- Face purple
- Eyes bulging & Eyes watery
- Post tussive emesis and exhaustion
tx of pertusssis
Zithromax
Can use Erythromycin but 3 x per day for 10 days
Supportive care
complications of pertussis
Mild :
- •Ear infection, loss of appetite, dehydration. pneumonia (up to 5% of cases)
- •rib fracture from coughing (up to 4% of cases).
- •loss of consciousness
- •female urinary incontinence, hernias, angina, and weight loss
If hypoxic from paroxysm:
- Encephalopathy
- Seizures
Common Aspirated Objects
most common cause of FBA in infants vs older kids
•Peanuts! (approx 50% of all FBA )
•Seeds, nuts, popcorn, hardware, toys, batteries. Coins, hot dogs
- Food most common cause of FBA in i_nfants and toddlers_
- Non-food most common in older kids—> Coins, paper clips, pins, pen caps, coins
common causes of fatal FB aspirations
- BALLOONS, gloves, similar expandable items
- Balls
- Marbles
- toys
- anything strong, round, unbreakable
- Even ice cubes, cheese cubes
- Clumpy, sticky foods, hard candies, lollipop pieces
where do FB aspirations occur in lungs of children vs adults
In adults ==== R mainstem
- •Why: diameter of R mainstem and angle of departure from central mainstem
Kids ….proximal mainstem bronchus
- •no preponderance of occurrence R over L
- •R and L are close in diameter size and close in angle of departure
Then
bronchi Right and Left equally
Can be in larynx if large enough
•Laryngeal fb associated with higher morbidity/mortality –> no air passing
dx and tx tool for FBA
Bronchoscopy is diagnostic tool and treatment
describe 2 types of bronchoscopy for FBA and their uses
Flexible Bronchoscopy: used when FB dx is known
- Done with chronic or recurrent pneumonia
- Chronic cough
Rigid Bronchoscopy: If suspected FBA
- If non emergent
- Typically used but requires anesthesia
- Less risk of dislodgement
definition and etiology of CF
secretions viscous secretions in???
↑ salt content in sweat gland secretions viscous secretions in
- • Lungs
- • Pancreas
- • Liver
- • Intestine
- • Reproductive Tract
Genetically driven disruption of the chloride channel
- Genetic mutation of CFTR *
- affects the transport of chloride
what is PATHGNOMONIC FOR CF
Meconium ileus - Kid doesn’t have their first poop by day 2 of life
CF patients have Colonization with:
in childhood-
young & adult?
•Colonization with
•Staph Aureus (and H flu) in childhood-
•pseudomonas can cause clinical disease in young CF patients!
•Pseudomonas Aeruginosa is ultimately found colonized in lungs of CF pts in adulthood
si/sx of CF:
name body areas affected
resp
sinuses
pancreas
billiary
MSK
psych
si/sx resp in CF
newborn , adults, suspicion raised with??
•Newborn :
- Respiratory symptoms not typical in newborn but if respiratory distress of unclear etiology, keep a suspicion
•Infants and Children:
- •Most likely presenting cc leading to dx of CF are respiratory sxs
- •Persistant or chronic uri
- •Wheezing of unclear etiology or recalcitrant
•Suspicion in setting of no CF dx should be raised with
- •Chronic productive cough
- •Recurrent Upper or Lower Resp Infections
- •Hyperinflation on CXR
- •PFT’s c/w obstructive disease
sinus si/sx in CF
- Panopacification of sinuses by the age of 8 mo
- Nasal polyposis found in approx 20 % of pts
pancreas si/sx in CF
- Exocrine function typically insufficient in all newborns with CF
- Insufficient digestive enzymes -> malabsorption–> failure to thrive, electrolyte abnl, anemia
- May also develop endocrine function abnormality -> Glucose Intolerance or CF related DM
billiary si/sx CF
- Focal biliary cirrhosis casused by inspissated bile
- Hepatomegaly
- Asymptomatic liver dz primarily
- If progressive, in rare instances, can cause periportal fibrosis, cirrhosis, portal htn, variceal bleeding and require liver trnplnt
- May see cholelithiasis (12% of pts with cf)
MSK si/sx in CF
•Reduced Bone Mineral Content
- •Poor bone growth
- •Higher bone loss
- •Use of steroids increases risk of osteoporosis
- •Poor absorption incr risk of osteoporosis
•Hypertrophic osteoarthropathy
•CLUBBING OF FINGERS AND TOES…..
- Abnl proliferation of skin and osseous tissue at distal extremities
dx criteria for CF
Clinical sxs c/w CTF in at least 1 organ systems if older than newborn (newborn no organ involvement neccessary for dx)
AND
Evidence of CFTR *dysfunction by any one of the following tests
- ↑Sweat chloride (over 60mmol/L) on 2 occasions
- Presence of 2 disease causing mutations in the CFTR
- Abnormal nasal potential difference
primary dx test for CF
2 reasons we do it?
Sweat Test
- Dasxs(+) newborn screen, after 2 weeks of life and >2kg
- •meconium ileus after Day Of Life 2 (DOL 2)
Measured and reported as : DX vs Possible CF vs CF unlikely
dx CF
+ Newborn screen –> _____ ?
based on results what other tests
- Newborn Screening (IR)
- Chloride Sweat Test
- Intermediate (possible) = Molecular DNA (>2 mutations = CF)
- INconclusive sweat test or <2 gene mutations -= Nasal potential Difference
dx methods for CF & when they are neccessary
•Newborn Screening - 50% of cases dxd
- Possibly 7% cases dxd over the age of 18
- Done on dried blood sample
- Measuring levels of i_mmunoreactive trypsin (IRT)_
- Must be done <8 weeks of life as IRT levels nlly fall
- High false pos and neg rate, but pos must be confirmed
- Confirmation done with DNA and/or sweat testing
Molecular DX
- •DNA testing done on all pts w/ INTERMEDIATE (possible) sweat test result
- •23 common mutations screened
- T_WO mutations_ to be considered (+) for CF
•Nasal Potential Difference
- Done on pts with <2 gene mutations and inconclusive sweat test
- Measure chloride levels pre and post intranasal perfusions to alter chloride transport.
classic vs non-classic dx of CF
tx of CF
CFTR Modulators – (“older” treatments- 2012-2018)
- Ivacaftor-
- Tezacaftor/Ivacaftor
Triple Therapy: Trikafta, Symdeko, Orkambi
Oral Antibiotics: Azithromycin
Bronchodilators: daily albuterol q 4-6 hours
Inhaled Hypertonic Saline
Dnase
name CTFR modulators & age group they are approved
Ivacaftor- G551D mutation (>6 m)
- Restores function of mutant CF protein
- First treatment approved to treat problem, not sequellae
Tezacaftor/Ivacaftor – F508del mutations (>6yo)
- Improves lung function by 4 %
name 3 types of triple therapy and their approved age groups
Trikafta= elexacaftor -ivacaftor - tezacaftor
- > 12 years and older
Symdeko: tezacaftor and ivacaftor
- >6 years and older
Orkambi: lumacaftor and ivacaftor
- >2 years and older
Ivacaftor has been approved for kids 6 mo and older !
CF tx for Airway secretion clearance enhancement
age group appropriate ??
Dnase
- Cleaves long chains of proteins diminishing mucous viscousity
- May be used daily but considering trials with QOD to cut cost
Inhaled Hypertonic Saline
- •Hydrates inspissated mucous
- •Used in kids OVER THE AGE OF 6
inhaled txs for CF
Immunizations for CF
- Influenza
- Pneumoncoccal
- Palivizumab (SYNAGIS) for RSV ppx if age appropriate !
Remember we talked briefly about Synagis on Friday!
ultimate tx for CF
lung transplant
dx imaging & Labs for ARDS
CXR:
- • air bronchograms
- • low lung volume
- • ground glass appearance
- •Pneumothorax
ABG- hypoxia
Hyponatremia from water retention
ARDS
↑risk in infants born < ____wks
- Formation of alveoli begins at ____wks…thus viability begins
- 93% of RDS infants ____ wks
Also if ______ aspirations => increased risk
↑risk in infants born below age 30 weeks
- Formation of alveoli begins at 24 wks…thus viability begins
- 93% of RDS infants <28 weeks
Also if meconium aspirations => increased risk
etiology of ARDS
surfactant deficency (quality & quantity)
leading to lung damage & pulm edema
ARDS id different from TTN ???
TTN - (transient tachypnea of the newborn)
- Seen in more mature newborns, less severe
Interventions to prevent RDS
Administration of Antenatal Corticosteroids (to mom)
- Given to Pregnant women at risk for delivery before 34 weeks
Administration of Exogenous Surfactant (to neonate)
- Provision of Assisted Ventilation
ARDS positive vs negatives of mechanical ventihlation
describe fetal bF
Umbilical vein – delivers oxygenated blood from the placenta into the portal sinus in the liver and ductus venosus in the IVC
- 2/3 of the blood that enters the _RA –> foramen oval_e to the LA, LV, aorta, –> head and upper extremities
- 1/3 of blood that stays in the RA –> RV then PA –> ductus arteriosus –> aorta
Deoxygenated blood returns to the heart via the SVC –> mixes with oxygenated blood from the umbilical vein
Blood that doesn’t leave the aortic arch –> descending aorta where it leaves the fetus via the umbilical arteries –> placenta
Pulmonary artery pressure is higher than systemic pressure so
- blood flow to the lungs is minimal
- flow to the placenta is high
LA pressure ___ RA pressure keeps the foramen ovale open
LA < RA pressure foramen ovale is open
foramen ovale is
Open when ___< ____
closed when ____>____
foramen ovale is
Open when LA pressure < RA Pressure
closed when LA pressure > RA pressure
fetal circulation 2/3 of the blood that enters the ___-> ___ ___ to the LA, LV, aorta –> head and upper extremities
fetal circulation 2/3 of the blood that enters the RA -> foramen ovale to the LA, LV, aorta --> head and upper extremities
maternal fetal blood circ
PA –> ___ ____–> aorta
•PA –> ductus arteriosus –> aorta
derivatives of fetal vasc structures
foramen ovale
umbilical v
ductus venosus
ductus arteriosus
24-48 hours after birth
The ____ pO2 in the blood along with _____ pulmonary pressures causes the ___ ______ to close
24-48 hours after birth
The increased pO2 in the blood along with increased pulmonary pressures causes the ductus arteriosus to close
after birth
With_____ blood returning to the LA from the lungs the LA pressure_____ and excessed the pressure in the RA, which closes the ____ ______
With increased blood returning to the LA from the lungs the LA pressure increases and excessed the pressure in the RA, which closes the foramen ovale
foramen ovale LA pressure > RA pressure
define Acyanotic Heart Defects
how they shunt
name them
↑ blood flow to lungs (L->R shunt)
- Patent ductus arteriosus**
- Atrial septal defect**
- Ventricular septal defect**
Cyanotic Heart Defects definition
shunts
examples
↓ blood flow to lungs (R->L shunt)
- Tetralogy of Fallot**
- Transposition of great vessels
- Tricuspid atresia
- Total anomalous pulmonary venous connection
- Truncus arteriosus
- Hypoplastic left heart
ex of Obstructive Heart Defects
- Coarcation of aorta**
- Aortic stenosis
VSD causes ??
↑blood pressures in the right ventricle and the pulmonary arteries.
ASD causes
•increasing the volume of blood that flows to the right side of the heart and lungs.
define patent ductus arteriosus
Continued connection b/w the aorta and pulmonary a.
- Allows oxygenated blood to flow from the aorta back to the pulmonary artery
Lungs, LA, LV, and aorta handles a larger volume of blood than normal
a systolic continuous rough “machinery” murmur
dx???
Patent Ductus Arteriosus (PDA):
small grade 1-2
moderate 2-4
si/sx of moderate PDA
Exercise intolerance
Murmur (grade 2-4)- Continuous rough “machinery” murmur (systolic
Hyperdynamic and displaced apical pulse
Wide pulse pressure, with low diastolic pressure
si/sx of large PDA
Failure to thrive in infants
SOB and easy fatigue in older children
Murmur decrease (grade 1-2)
Diastolic apical murmur
Loud, split S2
Thrill
Bounding pulses, wide pulse pressure
LV failure
pHTN
Eisenmenger physiology
define Eisenmenger physiology
dx?
PDA OR ASD
Eisenmenger physiology
- Shunt switches from L-R to R-L
- delivers oxygenated blood to the upper extremities
- deoxygenated blood to the lower extremities
- Hands are normal or less affected
- toes are cyanotic and clubbed
PDA closure
Premature infants
Term infants <6 kg
Term Infants >6kg
Adolescents and Adults
Premature infants
•First line: Inhibitors of prostaglandin synthesis – Indomethacin and Ibuprofen
•Second line: Medical management of HF symptoms
Term infants <6 kg
First line:
- Asymptomatic – monitor and observe until >6 kg
- Symptomatic –mgt of HF with Digoxin and Lasix
Second line: surgical ligation is the preferred, but percutaneous closure can be considered
Term Infants >6kg & Adolescents and Adults
First line: Percutaneous closure (coil vs devices closure)
Second line: Surgical ligation
patent foramen ovale is not ASD bc ??
Technically not a ASD because there is no septal tissue missing
si/sx of ASD
usually no murmur
Fixed split S2
pHTN
Heart failure
Right ventricle heave
Harrison’s grooves –transverse depression along the 6th and 7th costal cartilage due to RA enlargement
Eisenmenger syndrome
types of ASD
Primum ASD (15-20%); Defect at the base, usually large
- associated with another abnormality
Secundum ASD (70%): Located within the fossa ovalis
- presents as isolated lesion
Sinus venosus ASD (5-10%): Malposition of the insertion of the SVC or IVC straddling the atrial septum
Coronary sinus ASD (<1%): Part or the entire common wall between the coronary sinus and the left atrium is absent
- associated with persistent left SVC
dx ECG & CXR ASD
Asymptomatic -> usually incidental finding on exam or echo
EKG- Usually normal
- •Can have P-wave abnormalities or changes in V1 and V2 that look like a RBBB
- •Can have a prolonged QRS
Chest x-ray- Normal
- •Cardiomegaly
- •Enlarged pulmonary artery
Echocardiogram
tx of ASD
Medical management
- Atrial arrhythmias
- pHTN
- Endocarditis prophylaxis
ASD closure
Percutaneous closure –secundum ASDs
Surgical closure
- Secundum ASDs unamenable to percutaneous closure
- Primum ASDs
- Sinus Venosus ASDs
- Coronary sinus ASDs
Harrison’s grooves
define & dx?
• transverse depression along the 6th and 7th costal cartilage due to RA enlargement
ASD
types of VSD
Type 1: Infundibular septum (10%): Asian population
- associated with aortic valve prolapse
Type 2: Membranous septum (80%): Most common
- Can also involve the muscular septum, which is then called a perimembranous VSD
Type 3: Inlet septum: not associated with MR or TR
- associated with Down syndrome
Type 4: Muscular septum (trabecular) (5-20%)
- Can be multiple and resemble swiss cheese
AV VSD (rare): Communication from LV to RA
- acquired lesion after endocarditis or valve replacement
most common type of VSD
Type 2: Membranous septum (80%)
• Can also involve the muscular septum, which is then called a perimembranous VSD
si/sx of small VSD
Small VSDs asymptomatic and only present with a murmur
Murmur usually develops 4-10 days of life due to continued ↓ in PVR
si/sx od mod- large VSD
Tachypnea, tachycardia
Poor feeding – may appear hungry but tires easily, sweats with feeding
Poor weight gain
Hepatomegaly
Pulmonary rales, grunting, and retractions
Pallor
Systolic murmur - Harsh or blowing holosystolic murmur
- Mid-lower left sternal border
- If the VSD is large and the ventricular pressures are equal there might be no murmur
Diastolic murmur
- Diastolic rumble at apex
- Diastolic decrescendo murmur at left sternal border
Thrill in the 3rd or 4th left intercostal space
Fixed split S2
ECG & CXR VSD
EKG
- LVH and RVH
- Right atrial enlargement
Chest X-Ray
- Increase pulmonary vascular marking
- Cardiomegaly
EKG & CXR ASD
EKG
- P-wave abnormalities or changes in V1 and V2 that look like a RBBB
- prolonged QRS
Chest x-ray
- Cardiomegaly
- Enlarged pulmonary artery
CXR & EKG of PDA
Chest x-ray
- Prominent main pulmonary artery
- Prominent aortic knob
- Cardiomegaly
- Enlarge pulmonary markings
EKG
- Biventricular hypertrophy
- Left atrial abnormality
Most ____ VSDs close during childhood and large VSDs tend to become _____.
More common in ____ and ____ defects
Most small VSDs close during childhood and large VSDs tend to become smaller
•More common in muscular and membranous defects
Tx of
Small VSDs with no pHTN
Medium to large VSDs
- No signs of pHTN or heart failure
- signs of pHTN or heart failure
Small VSDs with no pHTN — Observation
Medium to large VSDs
- No signs of pHTN or heart failure – observation vs VSD closure
- signs of pHTN or heart failure – VSD closure
VSD closure options
Surgical closure
Percutaneous closure
- •Isolated, uncomplicated, muscular VSDs
- •Membranous VSDs
compications of VSD
Endocarditis
Aortic regurgitation
Seen in membranous VSDs
- Subaortic stenosis
- RV outflow obstruction
LV to RA shunting
describe 4 malformations of the heart in ToF
RVOT obstruction = caused by multiple factors
- Subvalvar obstruction from the infundibular septum
- Hypertrophy of muscle bands
- PV annulus is usually hypoplastic
- PV itself is usually bicuspid and stenotic
Intraventricular communication (VSD)
- a large, singular, subaortic defect located in the perimembranous region
Deviation of the origin of aorta to the right (over riding aorta)
- Aorta is displaced to the right and is over the VSD
- Leads to blood flow from both the RV and LV in the aorta
Concentric RV hypertrophy
describe murmur in ToF
Systolic, crescendo-decrescendo murmur with a harsh ejection quality
• Also, possibly an early systolic click along the left sternal border can be present
si/sx of ToF
Degree of RV obstruction is progressive so eventually patients will develop symptoms
Cyanosis
Respiratory distress
“Tet spells”
- Hypercyanotic spells during times of excitement or stress
- Usually seen in nail beds or lips
Murmur
- Systolic, crescendo-decrescendo murmur with a harsh ejection quality
- Also, possibly an early systolic click along the left sternal border can be present
si/sx of ToF
Minimal obstruction –
Mild- MOd Obstruction
Severe obstruction –
Minimal obstruction –
- asymptomatic at first
- followed by pulmonary overload and HF symptoms within 4-6 weeks of life
Mild-moderate obstructions –
- can be originally asymptomatic
- can have cyanosis spells with excitement, agitation, or hypovolemia (Tet spells)
Severe obstruction –profound cyanosis at birth
EKG and CXR in ToF
EKG
- RA enlargement
- RVH
- Right axis deviation
Chest X-ray
- Boot shaped heart
- Upturned apex
tx goal of ToF
Surgical repair
Goal is complete repair by 1 year of age, ideally by 6 months
ToF Medical Management prior to surgery
Maintain ductal patency
- Prostaglandin therapy – Alprostadil
- Ductal stenting
Heart failure management
Prophylaxis antibiotics until surgical repair
ToF Tet spell management
Tet spell management (step wise approach)
- Knee-chest position
- Oxygen
- IV fluids bolus (10-20 ml/kg)
- Narcotic (IV morphine or intranasal fentanyl)
- IV beta-blocker (propranolol, esmolol)
- IV phenylephrine
- Emergent surgery
complications fo ToF
- Pulmonary regurgitation
- RV dysfunction
- Residual RVOT
- Aortic root and valve dilation
- Arrhythmia
- Sudden cardiac death
F/u for ToF
echo
ekgs
holter
MRI
exercise
Regular echocardiograms -
- •Yearly until 10 years of age
- • Every other year throughout adulthood
Yearly EKGs
Holter monitor – every 3-4 years
Cardiac MRIs – every 3 years
Exercise testing – every 3 years
define CoA
risk factors
Narrowing of the descending aorta, which is typically located at the insertion of the ductus arteriosus just distal to the subclavian
Genetic factors - Familial risk, Turner syndrome
Acquired CoA – rare (Takayasu arteritis, Severe atherosclerosis)
CoA is usually accompanied by another cardiac lesion
- Complex cardiac defects
- Bicuspid aortic valve (most common in adults)
Si/sx of CoA
Neonates:
Older infants & Children
Adults
Neonates: Asymptomatic
- Absent or delayed femoral pulse compared to brachial
- +/- murmur associated with other cardiac abnormality
- Differential cyanosis (O2 sat in arms might be higher than legs)
-
Heart failure with shock – if PDA is closed
- Pale
- Irritable
- Diaphoretic
- Dyspnea
- Absent femoral pulses
- Hepatomegaly
Older infants & Children: Asymptomatic
- Chest pain
- Cold extremities
- Claudication with physical exertion
- Variability in BP , Variability in pulse
- Hypertension
Adults: Asymptomatic
- Hypertension
- Variability in BP
- Headaches
- Epistaxis
- Aortic dissection
O2 sat in arms might be higher than legs
dx?
neonate CoA
CoA si/sx of
Absent or delayed____ pulse when compared to____ pulse
O2 sat in arms might be_____ than legs
Absent or delayed femoral pulse when compared to brachial pulse
O2 sat in arms might be higher than legs
CXR & EKG CoA
EKG- LVH +/- RVH
Chest X-ray
- Cardiomegaly
- ↑ pulmonary vascular markings
-
Notching of the posterior 1/3 of the 3 rd – 8 th ribs
- apparent between ages 4-12
Intervention for CoA should be considered for any patient with one of the following:
- Critical CoA
- CoA gradient > 20 mmHg
- Radiological evidence of clinical significant collateral flow
- Systemic hypertension
- Heart failure
tx of CoA
Neonates and infants:
Infants ≥ 4 months – children <25 kg
Children >25 kg and adults
Neonates and infants: open heart surgery is tx of choice
- Prostaglandin (Alprostadil) along with inotropes are used to stabilize patient prior to surgery if critically ill
Infants ≥ 4 months – children <25 kg
- either balloon angioplasty
- or open heart surgery
Children >25 kg and adults: Transcatheter stenting
TGA is caused when
Aorta arises from the ____ and PA arises from the ____
Aorta arises from the RV
and PA arises from the LV
for TGA to be compatible w/ life what must occur?
At lest one communication between right and left side must exist to be compatible with life
- VSD
- ASD
- PDA
Characterized by absence of the TV resulting in no direct communication between the RA and RV
Associated lesions ???
tricuspid atresia
Associated lesions = ASD • VSD • TGA
Total Anomalous Pulmonary Venous Connection
Failure of all 4 pulmonary veins to make their normal connection to the LA resulting in drainage of all pulmonary venous return into the systemic venous circulation
- If all 4 pulmonary veins don’t connect to the LA then another abnormality needs to be present for right to left communication
Cyanotic congenital heart defect that has a single truncal valve which then gives rise to the aorta and pulmonary arteries
Truncus Arteriosus
Characterized by a diminutive LV and a small left sided structure incapable of supporting the systemic circulation
what must if have to be compatible w/ life??
Hypoplastic Left Heart
PDA and ASD to be compatible with life
define stills murmur
Common type of benign or “innocent” functional heart murmur
- It is not associated with any cardiac abnormality
- Disappears as the patient moves into adolescence
define sounds of stills murmur
Murmur across the aortic valve from high cardiac output
- Soft or vibratory, systolic ejection murmur
- Usually only grade 1-2/6
- Best heard at the apex of the heart and LLSB
- Heard best with the bell
- Decreases with inspiration, sitting up, or standing
describe murmurs assoc w/
Stills
ToF
VSD
PDA
Stills: Soft or vibratory, systolic ejection murmur
- Best heard at the apex of the heart and LLSB
- Decreases with inspiration, sitting up, or standing
ToF: Systolic, crescendo-decrescendo murmur with a harsh ejection quality
- Also, possibly an early systolic click on LSB
VSD
Systolic murmur: Harsh or blowing holosystolic murmur
- Mid-lower LSB
Diastolic murmur
- rumble at apex
- decrescendo murmur at LSB
PDA: Continuous rough “machinery” murmur (systolic)
- late systole at the time of S2
- Heard best at the Left 1st and 2nd ICS at LSB
