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Genetic birth defect
- Single gene
Chrosomal birth defect
- Abnormal normal
Congential birth defect
Unknown cause or abnormal developement
What are the characteristics often seen in children with chromosomal abnormalities?
- Obvious malformations
- Odd facies
- Abnormal ears
- Heart and or kidney problems
- Abnormal hands and or feet
- Low birth weight
- Unexplained mental retardation or changes in health
List three diseases that are autosomal recessive!
What are the three symptoms of CHF?
- Pulmonary edema
- Increased WOB/ dyspnea
- Coughing/ Wheezing
What are the three signs of heart defects?
- Congestive heart failure
- Poor perfusion
- Probably cardiac
- tissue hypoxecimia
- Arterial hypoxecmia
Central cyanotic breath sounds cause by pulmonary disease
- Crackles- atelectasis or pneumonia
- Wheezes- airway diseas
Central cyanotic CXR caused by pulmonary disease
- lung expansion
- Totally white lung color
Central cyanotic ABG's caused by pulmonary disease
- PaCO2 and PaO2 both abnormal
- PaO2 changes with hyperventilation then pulmonary vasodilation
- Low PaCO2 causes pulmonary vasodilation which would increase the PBF and the PaO2
Central cyanotic breath sounds cause by cardiac disease
- Normal breath sounds
- Crackles- pulmonary edema
- Wheeze- CHF
Central cyanotic heart sounds cause by cardiac disease
- Pulse strength abnormalities increase the chances that it is cardiac
Central cyanotic CXR cause by cardiac disease
- Normal lungs
- Bloow flow into the lungs- too much or too little
Central cyanotic ABG's cause by cardiac disease
- PaO2 different between upper and lower body
- PaO2 gets worse with O2 therapy
What is the normal RR for infants?
40 to 60 breaths/minute
What is the normal heart rate for infants?
120 to 170 beats/minute
What is the normal blood pressure for infants?
- Adequate MBP = GA + 5
- 30 weeks = MBP = 30
What are the signs of respiratory distress in babies?
- Nasal flaring
What are the differences between adults and babies?
- Poor stability to the chest wall
- Smaller and more reactive airways
- Larger heart in proportion to cheast area
- Easily distended abdomen
- Fewer and smaller alveoli
- Respiratory muscle fiber type
What does the difference in adults and babies mean for a baby with a lung disease?
- Only gonna see signs of respiratory distress in a sick baby
- The soft ribs cave in when they have to create a extra large drop in pulmonary pressure.
- The more negative they pull, the more the chest sucks in, the sicker the baby
- The extra distended abdomen limits the effectiness of the abdomen
- Babies retract because they have soft ribs and if they have lung disease they need to pull more negative in order to breath, which sucks in the chest
Explain why babies must increase their RR and not their TV?
- Weak diaphgram, 2 way its weak
The more premature the baby,
The more weaker the diaphgram, it tired more easily
- No nasal opening in the back of the nasopharynx
- Babies are obligate nasal breathers untill 6 weeks of age
- If their nose is obstructed they cant breathe
- This problem may be uni or bi lateral
- It is more common in boys
Symptoms of unilateral choanal atresia!
Secretions and sinus infection
Symptoms of bilateral choanal atresia!
- Cyanosis (periods of dyspnea) interrupted by periods of crying
Diagnosis of choanal atresia!
- Symptoms and inability to pass a suction catheter through the nose
- Obtain an ENT consult
Treatment for bilateral choanal atresia!
Most common tracheoesophageal fistula...
- Is esophageal pouch and lower connection above the carina
- Esophageal atresia with a distal tracheoesophageal fistula
The complication for TE fistula!
Is tonce you repair the fistula the patient will frequently have tracheal malacia or tracheal stenosis
Symptoms of esophageal atresia with a distal tracheoesophageal fistula!
- Can't swallow
- Bubbly spit
- Can't pass a feeding tube
- Aspirates on feeding
- Air in the stomach
- History of polyhydramios
Tracheosophageal fistula without esophageal atresia
Symptoms of H-type fistula!
- Can swallow
- Aspirates very little
- Presents with occasional wheeze and recurrent infections (long term micro aspirations)
What are critical to the diagnosis of tracheoesophageal fistula!
Cheast and abdominal radiographs
The course and endpoint of the nasogastric tube confirms...
Obstruction of the proximal esophagus by atresia and determines the relative position of the upper esophageal pouch
Proximal atresia with evedience of air in the gastrointestinal tract indicates...
A distal tracheoesophageal fistula, the most common form of the anomaly
A proximal obstruction without evedience of gas distally most likely indicates...
The presence of esophageal atresia with or without smaller fistulas
Treatment of tracheoesophageal fistula!
- surgical repair
- Be careful with ETT placement and suctioning after surgery
- This effect occurs early in pregnancy (8 - 10 weeks).
- 85% of the defects are on the left
- The amount of lung tissue is reduced on both sides, mostly on the affected side (intestine covered side)
Survival of diaphragmatic hernia depends on...
- Amount of lung tissue
- The amount of pulmonary hypertension
Why does pulmonary hypertension develope in diaphragmatic hernia!
- Reduced number of pulmonary vessels
- Increased pulmonary muscle mass
- Hypoxic vasoconstriction
Diagnosis of diaphragmatic hernia!
- Physical exam
- scaphoid (hollowed out) abdomen
- Shifted away mediastium
Treatment of diaphragmatic hernia!
- Do not ventilate with a bag and mask it makes it worse
- NG tube to suction
- Intubate and ventilate with low pressure- maybe oscillator- Pneumothorax occurs easily
- Give O2 and nitric oxide to reverse pulmonary hypertension caused by spasm
- ECMO is an alternative when NO and ventilator fail. It only aloows the lungs to rest and repair while gas exchange happens in a machine outside the body
What is the problem with bag mask ventilation in patients with diaphragmatic hernia?
It is avoided in order to keep the gut in the chest from becoming full of air and pushing against the lungs and causing tension pneumothorax physiology
Why does pulmonary hypertension develope in CDH?
- Hypoxic vasoconstriction
- Which increases PVR
Acyanotic cardiac defects
- Left to right shunts
- Obstructive lesions
- Increased PBF
- Decreased systemic flow
Left to right shunts
- Arterial blood is directed back to the lungs
- increases pulmonary blood flow
- Decreased systemic flow
- Increased risk for pulmonary edema
- Decreased systemic flow/pressure
- Poor peripheral perfusion
- Increased afterload
What is the most common cardiac defect?
Ventricular septal defect
Anatomy of Ventricular septal defect
- It is the most common defect accounting for approximately 20-30% of all defects
- Hole in the ventricular septum
- Blood flows from left ventricle to right ventricle
- Treatment is pulmonary banding
Ventricular septal defect
- Shunt- Left to Right
- PBF- Increased
- Color- Acyanotic
- Name- VSD
Symptoms of Ventricular septal defect!
- Are generally delayed untill 4 to 6 weeks of age when the left ventricular pressures exceed the right ventricular pressure
- Systolic murmur
- Pulmonary edema
Hemodynamics of Ventricular septal defect!
Depends on the size of the defect and the amount of PVR
Increases PVR which increases RV pressures and decreases the left to right shunt through the defect
Anatomy of Coarctation of the aorta!
- This is a norrowing of the aorta
- It usually occurs high in the aorta usually near the insertion of the DA
- Perepheral cyanosis
- No shunt
Coarctation of the aorta!
- Shunt- No shunt
- SBF- decreased
- Color- Acyanotic
- Name- Co Arc
Symptoms of coarctation of the aorta!
- Differential blood pressure is very diaganositc: High in the arms and low in the legs
- Occasional hypertension
What is prostin?
- Minimizes ductal construction
- Keep DA open for a patient who is ductal dependent
What is Indocin?
Closes the DA
Cyanotic cardiac defects
- Right to left shunts
- Venous admixture shunts
- Decreases in pulmonary blood flow
- Blue baby
Right to left shunt
- Vena caval return is directed to the systemic side of circulation
- Decreased PBF
- Severity of the cyanosis depends on the degree of shunting
Venous admixture shunts
- Vena caval return and pulmonary venous return mix
- The pulmonary and systemic circulations receive the same PaO2
- PBF is varaible
Ballock taussiq shunt!
- Creates an artificial ductus arteriousus to increase PBF
- Temporary to increase PBF usually works for 2 to 4 years then defenative repair
Anatomy of Tetralogy of Fallot!
- Right ventricle to left ventricle
- Hypoxia induced SOB and Squatting to increase SVR
- Treatment is Blalock- taussiq shunt
Tetralogy of fallot!
- Shunt- Right to left shunt
- PBF- decreased
- Color- Cyanotic (blue patient)
- Name- Tet
CXR of Tetralogy of fallot!
Boot shaped heart
What are the four defects in Tetralogy of fallot!
- Pulmonary stenosis
- Right ventricular hypertrophy
- Abnormal (malpositioned) aorta
- This procedure includes advancing a balloon tipped catheter from the RA through the foramen ovale into the LA. This balloon is inflated and rapidly and forcefully withdrawn to create a large ASD
- This ensures adequate mixing between the two atria
Anatomy of Transpositions of the great arteries!
- Venous admixture
- shunts occur threw: ASD, VSD, PDA
- Treatment is Ballon atrial switch
- More common in males and LGA
Transpositions of the great arteries!
- Shunt- Bidirectional
- PBF- Varies
- Color- Acyanotic (blue)
CXR of Transpositions of the great arteries!
Egg on the side
Anatomy of Hypoplastc left heart syndrome
- Ductal dependent for systemic flow
- Keep PaO2 low This Increases PVR, increases right to left shunt through DA, and keeps systemic flow going
- Give prostin
- Pulmonary to systemic
Hypoplastc left heart syndrome
- Shunt-Right to left
- PBF- Varies
- Color- Cyanotic (blue)
- Path- Pulmonary to systemic
Anatomical changes of Hypoplastc left heart syndrome
- Small left ventricle
- Abnormal aortic valve
- Abnormal mitral valve
- Abnormal aorta
What cardiac defect can be made worse with O2?
- Hypoplastic Left Heart Syndrome
- Tetralogy of Fallot
Laryngomalacia is the most common cause of...
Stridor in children
Vocal cord paralysis is the second most common...
- Laryngeal abnormality in the newborn
- Accounts for about 10% of all congential laryngeal abnormalies
Obstructs when the airways
Obstructs on inspiration and expiration
Extra-thoracic (above the clavicals) on flow volume loop with Tracheal stenosis/malacia!
- Narrows on inspiration because it is in the upper airway (Stridor)
- Flow volume loop is limited on inspiration
- No respond to vapo
Intra-thoracis (below the clavical) on flow volume loop with Tracheal stenosis/malacia!
- Narrows on inspiration because it is in the upper airway (wheeze)
- Flow volume loop is limited on expiration
- No response to B2 agonist
- Variable upper airway obstruction
- Obstruction occurs on inspiration
- Stridor does not respond to vaponephrine
- Flow volume loop demonstrates limitation on inspiration with normal expiration
- No treatment they will grow out of it
- Fixed obstruction
- Onstructs on both inspiration and expiration
- Usually occurs 3 to 4 weeks after intubation
- May also occur as a congential malformation
- Stridor does not respond to vapo, often biphasic
Red flags in cardiac for neonatal patients!
Red flags in gastrointestinal for neonatal patients!
- Abdominal distension
- Bloody stools
Red flags in Metabolic for neonatal patients!
Red flags in general for neonatal patients!
The idea time to asses the gestational age of a newborn is...
Within the first 12 hours
Evaluating gestational age requires consideration of gestational duration based on
- The last menstrual cycle
- Prenatal ultrasound evaluation
- Postnatal findings based on physical and neurological examinations
Jaundice on the first day of life...
is always an indication for immediate evaluation
A delayed or weak femoral pulse in relation to the brachial pulse can indicate...
coarctation of the aorta
Placing pulse oximeters on preductal and postductal sites allows for assessing...
right-to-left ductal level shunting through a patent ductus arteriosus, as seen with persistent pulmonary hypertension of the newborn
A scaphoid, hollowed, or unusually flattened abdomen may be associated with...
congenital diaphragmatic hernia
The umbilical cord normally has...
2 arteries and 1 vein
The minimal platelate count for a newborn 24 hours old should be?
If an 8 French (8F) catheter fails to pass through the nose into the oropharynx, what should be expected?
Chronic upper airway obstruction from anatomic malformations results in...
- Chronic hypoxia
- CO2 retention
- Related sequelae such as cor pulmonale
- Pulmonary hypertension
Drooling is the first symptom in the majority of newborns with
A 3200g term infant male is born to a healthy mother. Apgar scores are 7 and 5 with the infant gasping and heart rates decreasing to 90. Physical exam reveals cyanosis, a scaphoid abdomen, and visible tracheal deviation to the right. Considering this information the only appropiate action would be to?
Insert an oral suction catheter to vent the stomach
Considering a likely diagnosis of congential diaphragmatic hernia, which would be appropriate decision concerning ventilator strategy?
Rapid rate and low pressure
Normal transition to extauterine life depends on the pulmonary vascular system...
Changing from a high PVR to a low PVR
What affects PVR?
- Changes in PaO2
- Changes in PaCO2
- Changes in pH
What is the therapeutic goal of subambient oxygen therapy?
The purpose of managing PVR in the presence of cardiuac defects is to...
Ensure the desired balance between systemic and pulmonary blood flow
In complete transposition of the great arteries...
The aorta and pulmonary artery circulation run in parallel.
For which condition is a ventilator strategy utilizing larger tidal volumes, lower rates, and shorter I times typically used?
Increasing gradients between ETCO2 and PaCO2 in patients with congential cardiac defects are often the results of?
Loss of calibration