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PEDIATRIC AND NEONATAL DISEASES
Upper Airway Diseases
Inc.
Pediatric Sleep-Disordered Breathing
Introduction
Sleep deprivation causes serious neurocognitive
deficits and may be lethal (indicated in studies with
animals).
Copyright © 2015, 2010, 2003, 1995 by Saunders, an imprint of
Elsevier Inc.
2
Normal Sleep Development
Copyright © 2015, 2010, 2003, 1995 by Saunders, an imprint of
Elsevier Inc.
3
Normal newborn spends 70% of day asleep
3-4 hours of sleep
1-2 hours awake
Nonrapid eye movement (NREM)
Rapid eye movement (REM)
By 6 months of age
13-14 hours of sleep
6 to 8 hours in duration
Preschool age
11-12 hours of sleep
Disordered Breathing in Infants
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Elsevier Inc.
4
Periodic breathing
Common in infants
Rapid breathing followed by pauses > 3 seconds
Apnea
Respiratory pause > 15-20 seconds
Central apnea
No chest movement and no airflow
Obstructive apnea
Apnea of prematurity
Common in infants < 32 weeks’ gestation
Sudden Infant Death Syndrome (SIDS)
Leading cause of death in first 12 months of life
Third leading cause of death in infants
70% of victims found in early morning hours
Peak incidence―2 to 4 months of life
Uncommon after 6 months of life
Associated with prone position
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Elsevier Inc.
5
Acute Life-Threatening Event (ALTE)
Combination of
Apnea
Color change
Marked change in muscle tone
Choking or gagging
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Elsevier Inc.
6
Obstructive Sleep Apnea (OSA)
Complete or partial obstruction of the upper airway
Gas exchange and/or sleep are compromised
Major cause of OSA in children is adenotonsillar
hypertrophy
Peak prevalence: 28 years of age
Chronic obstruction = substantial morbidity
Daytime symptoms may include poor school
performance, hyperactivity
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Elsevier Inc.
7
Obstructive Sleep Apnea (Cont.)
Treatment options
Adenotonsillectomy
CPAP
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Elsevier Inc.
8
Laboratory Assessment of Breathing in
Sleep
Overnight polysomnography
ECG
Inductive plethysmography
Continuous esophageal pH measurement
Video recording with sound
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Elsevier Inc.
9
Upper Airway Disorders
Periglottic obstruction
Croup (laryngotracheobronchitis)
Epiglottitis
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10
Croup--Etiology
Viral Infection:
Parainfluenza
Influenza
RSV
Adenovirus
Gradual onset
Affects children 6 months to 3 years-old
Croup--Pathophysiology
Swelling and inflammation of subglottic structures.
Larynx
Trachea
Larger Bronchi
Can affect mid-sized and smaller airways
Croup--Clinical Manifestations
Slow onset, like a “cold”
Brassy/barking cough
Horseness & Audible stridor
Neck X-Ray: Steeple Sign
If Severe:
Tachycardia/tachypnea
Retractions
Decrease in SPO2
ABG: Hypoxemia & Respiratory Acidosis
Steeple Sign-Often Found in Croup
Croup--Treatment
Cool Mist w/oxygen via tent or face mask
Reassurance--Parental presence
Racemic Epinephrine via SVN or IPPB
6 Y.O. or less: 0.25 mls of 2.25% w/NSS
More than 6 Y.O. 0.5mls w/NSS
Systemic Steroids: Dextramethasone
0.3 to 0.6 mg/KG
Intubation: Mainly if respiratory failure present: e.g., muscle fatigue, change in sensorium, cyanosis,
ABG results.
Epiglottitis--Etiology
Bacterial infection
Most common microorganisms:
Staphylococcus Aureus
Group A & B Streptococci
Strep Pneumoniae
Other causes:
thermal injury
caustic ingestion
radiation exposure
Epiglottitis--Pathophysiology
Supra-glottic swelling
Epigottis turns bright, cherry red & swollen
Inflamation leads to a/w narrowing and dysphagia
If severe, a/w can become completely obstructed
Epiglottitis--Clinical Manifestations
Patient appears acutely ill
Rapid Onset
Affects mainly children 1 - 5 years old
Drooling, sore throat, dysphagia
Stridor & hoarseness w/diminished breath sounds in lung regions
High fever
Lateral neck x-ray: Balloon-shaped epiglottis/”thumb sign”
Lateral Neck X-Ray—Thumb Sign
Epiglottitis-Treatment
Minimal patient stimulation-keep patient calm!
Cool mist aerosol w/supp’l O2
Antibiotics and fluids (steroids generally not effective)
If severe obstruction, intubation shouldn’t be
attempted in ER
Intubate patient in OR as trach may be necessary
and patient may need to be paralyzed
Epiglottitis Vs. Croup
Epiglottitis
Bacterial
Rapid On-set
Profound illness
Hospitalization common required.
Pt. may be drooling and leaning forward with compromised speech
May need emergent care and airway management.
Croup
Viral
More gradual onset
Mild to moderate illness
Occasionally requires hospitalization
Mainly supportive care
FBAO
March
18
PEDIATRIC RESUSCITATION I 22
Signs:
SUDDEN onset of respiratory distress
Weak or silent coughing
Inability to speak
Stridor
Increasing respiratory difficulty
Cyanosis
FBAO (cont.)
March
18
PEDIATRIC RESUSCITATION I 23
Infant: Back blows-chest thrust (5:5)
Child: Abdominal thrust and Heimlich maneuver
DO NOT perform finger sweep in a responsive
victim
Blind finger sweeps should not be perform in
infants
Pneumonia
Viral
Respiratory syncytial virus (RSV)
Parainfluenza virus types 1, 2, and 3
Influenza viruses
Adenovirus
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Elsevier Inc.
24
Pneumonia (Cont.)
Bacterial
Etiology
Viral or bacterial
Signs and symptoms
Resp. Distress: Retractions, nasal flaring, Inc RR
Diagnosis
CXR
Sputum C&S
Treatment
Supportive, ABX
Copyright © 2015, 2010, 2003, 1995 by Saunders, an imprint of
Elsevier Inc.
25
Asthma
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Pathogenesis of Asthma
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Definition
PathophysiologyChronic airway inflammation
Bronchial hyperresponsiveness
Hypersecretion of mucus
Risk Factors for Developing
Asthma
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Allergic response Atopy—strongest predisposing factor for
developing asthma
Atopic dermatitis
Environmental triggers Tobacco smoke
Cockroach antigens
Molds
House dust mites
Diagnosis
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Medical history
Physical examination
Pulmonary function testing
Exhaled nitric oxide
Bronchoprovocational challengesMethacholine
Exercise
Differential diagnosis
Management of Asthma
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Pharmacologic therapy Long-term control medications
Quick-relief medications
Delivery systems
Control of asthma triggers Identification of allergens
Avoidance and control measures
Role of immunotherapy
Management of Asthma (Cont.)
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31
Peak flow monitoringPeak flow meter
Peak flow diary
Personal best reading
Peak flow zone system
Management of Asthma (Cont.)
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Asthma action planPlan when symptomatic
Based on current peak flow reading and personal
best number
Actions Begin quick-relief medications.
Increase daily medications.
Contact the physician.
Seek emergency care.
Management of Asthma (Cont.)
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33
Patient and family education Asthma disease process
Medication skills
Identification and control of triggers
Self-monitoring techniques
Managing Asthma Exacerbations in
the ED
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34
Assessment
Beta2 agonists
Corticosteroids
Hospitalization and Respiratory
Failure
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Intubation
Semielective vs. emergent
Mechanical ventilation
VT = 6-8 mL/kg
Low to moderate PEEP
Permissive hypercapnia
Exercise-Induced Bronchospasm
(EIB)
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Peaks 5 to 10 minutes after cessation of
activity
May resolve spontaneously in 20 to 30 minutes
Hyperventilation of cool and dry air
Inhalation of beta2 agonist, cromolyn,
nedocromil, or salmeterol 5 to 60 minutes
before exercise
5- to 10-minute warm-up
Asthma at School
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37
Leading cause of absenteeismMore than 10 days/year
Poor academic achievement
Inability to participate in school activities
Low self-esteem
Peak Performance USA
Cystic Fibrosis
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Introduction
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39
Genetic disorder
Cystic fibrosis transmembrane conductance
regulator (CFTR) dysfunction
Complications
Thick, viscous secretions
Chronic obstruction, infection, inflammation of airways
Exocrine pancreatic insufficiency
Infertility in males
Elevated sweat chloride levels
Epidemiology
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Most common genetic disease among whites
One in 28 are carriers.
Median age at diagnosis: 5 months
Genetics and Molecular Biology
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Chromosome 7
Alteration of sodium and chloride transport
Autosomal recessive
Each parent is an obligate carrier.
Diagnosis
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Sweat chloride testing
Gold standard for diagnosis
>60 mmol/L—confirmatory
40-59 mmol/L—intermediate
CFTR mutation analysis
Newborn screening
Pulmonary Disease
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Mucus production and airway obstruction
Bacterial infection
Pseudomonas aeruginosa
Burkholderia cepacia
Airway inflammation
Clinical manifestations
Treatment of Pulmonary Disease
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44
Aerosol therapy
Airway clearance therapy
Antibiotics
Anti-inflammatory agents
Macrolides
Corticosteroids
Ibuprofen
Lung transplantation
Small-molecule CFTR modulators
Nonpulmonary Manifestations
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45
Upper airway disorders
Gastrointestinal disorders
Pancreatic insufficiency
Meconium ileus and distal intestinal obstruction
syndrome
Rectal prolapse
Gastroesophageal reflux disease
Hepatobiliary disorders
Prognosis
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46
Improving outcomes
Median predicted age of survival: 38.3 years
Acute Respiratory Distress Syndrome
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Inc.
Acute Respiratory Distress Syndrome
(ARDS)
AECC definition
Acute onset of respiratory symptoms
Frontal chest radiograph with bilateral infiltrates
Pao2/Fio2 ≤ 200 mm Hg
No clinical evidence of left atrial hypertension as
defined by a pulmonary artery wedge pressure ≤ 18
mm Hg, if measured
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Elsevier Inc.
48
ARDS (Cont.)
Etiology
Usually direct resp. insult
Distant site
Iatrogenic
Sepsis
Clinical course
Stages of ARDS
Mortality: < 20% with early intervention
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Elsevier Inc.
49
Pathology and Role of Inflammatory
Mediators
Pathology and pathophysiology
Exudative stage
Proliferative stage
Fibrotic stage
Inflammatory mediators
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Elsevier Inc.
50
Treatment and Ventilatory Support
Goals
Achieve adequate tissue oxygenation.
Avoid complications.
PEEP
Low tidal volume ventilation
Gas exchange goals
Permissive hypercapnia
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Elsevier Inc.
51
Adjunctive Therapies
HFV
ECMO
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Elsevier Inc.
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Shock
Disruption of natural homeostasis
Inability to maintain sufficient supply of oxygen
Potentially fatal when untreated
Cardiac output is impaired
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Definition and Classification
Hypovolemic
Most common
Cardiogenic
Obstructive
Physical obstruction to blood flow
Cardiac tamponade, tension pneumothorax, pulmonary embolism
Distributive
Sepsis
Anaphylaxis
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54
Pathophysiology
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Cardiac output
Stroke volume
Nutrients and oxygen in blood
Oxygen delivery
Delivery of blood to the tissues
Metabolic response
Management and Treatment
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Elsevier Inc.
56
General considerations
Assessment and evaluation
Monitoring
Central venous pressure (CVP)
Pulmonary artery (PA) catheter
Management and Treatment (Cont.)
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57
System-based treatment approach
Respiratory
Vascular volume
Myocardial function
Hematologic
Endocrine
Immunologic
Nutritional
ECMO
Pediatric Trauma
Inc.
Overview
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Elsevier Inc.
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Epidemiology
Anatomic considerations
Size and shape differences
Mechanisms of injury
Initial assessment and diagnosis
Airway patency
Breathing support
Circulation
Disability
Thoracic Trauma
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60
Birth trauma
Nerve injury
Congenital chylothorax
Pneumothorax
Blunt thoracic trauma
Pulmonary contusion
Flail chest
Penetrating thoracic trauma
Iatrogenic thoracic trauma
Thoracic Trauma (Cont.)
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61
Penetrating thoracic trauma
Incidence
Resuscitation
Imaging
Iatrogenic thoracic trauma
Pneumomediastinum
Pneumothorax
Esophageal-pharyngeal injuries
Ventilator-induced injuries
Thermal Injury
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62
Epidemiology
Scald injuries: most common (0-4 years)
High-mortality risk factors
Pathophysiology
Classification of burn injury
Percent of body surface area burn
Management
Long-term outcomes
Inhalation Injury
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63
Pathophysiology
Upper airway injury
Lung parenchyma injury
CO poisoning
Evaluation of injury
Clinical manifestations
Bronchoscopy
Xenon scan
Spirometry
Thermal and dye dilution
Inhalation Injury (Cont.)
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64
Management
Oxygen therapy
Airway maintenance
Bronchial hygiene therapy
Pharmacologic management
Mechanical ventilatory support
Conventional mechanical ventilation
High-frequency percussive ventilation
Complications
Long-term outcomes
Drowning
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Incidence
One in four fatal drowning victims <14 years of age
Open water is most common site
Witnessed in < 20% of younger children drowning
Pathophysiology
Central nervous system effects
Pulmonary effects
Cardiovascular effects
Drowning (Cont.)
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66
Treatment
At the scene
Emergency department
Inpatient
Outcome
Prevention
Neurological and Neuromuscular
Disorders
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Inc.
Neuromuscular Control of Ventilation
Central nervous system
Peripheral nervous system
Respiratory muscles
Respiratory control system
Bulbar muscles
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68
Neuromuscular Diseases That Affect the
Respiratory System
Central nervous system
Disorders of the brain
Disorders affecting the spinal cord
Trauma
Chiari malformations
Other conditions
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69
Neuromuscular Diseases That Affect the
Respiratory System (Cont.)
Peripheral nervous system
Disorders of the motor nerves
Spinal muscle atrophy
Poliomyelitis
Guillain-Barré syndrome
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70
Neuromuscular Diseases That Affect the
Respiratory System (Cont.)
Peripheral nervous system (Cont.)
Disorders of the neuromuscular junction
Infantile botulism
Myasthenia gravis
Other
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71
Neuromuscular Diseases That Affect the
Respiratory System (Cont.)
Myopathies
Duchenne and Becker muscular dystrophy
Myotonic dystrophies
Glycogen storage diseases
Electrolyte abnormalities
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72
Respiratory Evaluation of Children
with Neuromuscular Disease
PFT (serial)
Spirometry
CO2 and O2 analysis
Cough effectiveness and lung volume
ETCO2
Sleep studies
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73
Respiratory Care of Children with
Neuromuscular Disease
General considerations
Airway clearance mechanisms
Facilitating mucus clearance
Assisted coughing
Glossopharyngeal breathing
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74
Respiratory Care of Children with
Neuromuscular Disease (Cont.)
Mechanical ventilatory support
Noninvasive ventilation
Nocturnal ventilation
Tracheostomy
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75
Neurologic and Neuromuscular
Disorders
Nonrespiratory care
Nutritional
Cardiac status
Extent of scoliosis
Surgical corrections
End-of-life issues
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76
Neonatal Pulmonary Disorders
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Respiratory Distress Syndrome
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78
Incidence– 1% of pregnancies– 60%-80% in infants < 28 weeks
Cause and pathophysiology– Role of surfactant
Clinical presentation and diagnosis– Tachypnea– Grunt– Nasal flaring– Retractions – Cyanosis – L/S ratio (>2.0)
Respiratory Distress Syndrome
(Cont.)
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79
Prevention Avoid poorly timed cesarean section.
Antenatal and intrapartum fetal monitoring
Antenatal steroid therapy
Treatment Surfactant
Oxygen therapy
CPAP
Mechanical ventilation
Complications and prognosis
Bronchopulmonary Dysplasia
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Definition
Incidence
Pathophysiology and risk factors Prematurity
Ventilator-induced lung injury
Hypoxia- or hyperoxia-induced lung injury
Inflammation
Nutrition
PDA
Genetics
Vascular
Bronchopulmonary Dysplasia
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81
Clinical presentation and diagnosis
Differential diagnosis
Prevention Lung protective ventilator strategies
Oxygen therapy
Antioxidants
Inhaled NO
Nutrition
Corticosteroids
Methylxanthines
Bronchopulmonary Dysplasia
(Cont.)
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Treatment
Oxygenation
Mechanical ventilation
Fluid management
Drug therapies
Nutrition
Social issues
Complications and prognosis
Surgical Disorders in Childhood
that Affect Respiratory Care
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Upper Airway Conditions
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84
Choanal atresiaObligate nasal breathers
Unilateral is most common
Bilateral is neonatal emergency
Oral airway
Macroglossia Beckwith-Wiedemann and Down syndrome
Mandibular hypoplasia Pierre Robin or Treacher Collins syndrome
Esophageal Atresia and
Tracheoesophageal Fistula
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Symptoms
Diagnosis
Treatment
Complications Tracheomalacia
Management Preoperative
Perioperative
Postoperative
Chest Wall Malformations
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Pectus excavatum
Pectus carinatum
Scoliosis and kyphoscoliosis
Congenital Cardiac Defects
Shunt Lesions
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88
Patent ductus arteriosus (PDA)
48 hours after birth
Decrease in PgE2 + increase in Pao2 = closure
Predisposing factors for PDA
Prematurity (most common)
Persistent pulmonary hypertension
Respiratory distress syndrome (RDS)
Clinical signs
Management
Patent ductus arteriosus (PDA)
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Shunt Lesions (Cont.)
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Atrial septal defect (ASD)
Left-to-right shunting
Only 8% of children with ASD develop symptoms
before 2 years of age.
Surgical repair
Shunt Lesions (Cont.)
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91
Ventricular septal defect (VSD)
Most common congenital heart defect
Left-to-right shunting
Eisenmenger’s syndrome
Occurs when pulmonary vascular resistance nears that of the
systemic vascular resistance
Left-to-right shunt may reverse to right-to-left shunt
Therapy
Shunt Lesions (Cont.)
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92
Atrioventricular septal defect (AVSD)
AV canal or endocardial cushion defect
Left-to-right shunting
Most common congenital heart lesion in infants with
Down syndrome (trisomy 21)
Clinical manifestations
Surgical repair
Left Ventricular Outflow Tract
Obstruction
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93
Aortic stenosis (AS)
Valvular is most common type
Clinical presentation
Emergency therapy
Prostaglandin E1
Surgical repair
Left Ventricular Outflow Tract
Obstruction (Cont.)
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Coarctation of the aorta
Increase LV afterload
Clinical manifestations
Treatment
Coarctation of the aorta
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Coarctation of the aorta
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Other Cyanotic Congenital Heart
Lesions
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Tetralogy of Fallot (TOF)
Truncus arteriosus
Transposition of the great arteries (TGA)
Tetralogy of Fallot (TOF)
98
Transposition of the great arteries
(TGA)
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Clinical Monitoring of Patients with
Cardiac Anomalies
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Hemodynamic monitoring
Pulse oximetry
Capnography
Cardiac output monitoring