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1. COMMON RESPIRATORY AND
CARDIOVASCULAR
DISEASES IN CHILDREN
Pikria Zhvania, MD Phd

2. CASE 1
A previously healthy 18-month-old girl was brought to the emergency
department with a 2 h history of fever and stridor. On arrival, the patient’s
temperature was 38.5°C, heart rate 200 beats/min, respiratory rate 40
breaths/min and oxygen saturation was 97% on room air. On initial
assessment, the patient had a hoarse cry, inspiratory stridor and mild
suprasternal in drawing but was able to swallow without difficulty. No
drooling was evident. There was no previous history of choking, foreign
body aspiration or sick contact. All routine immunizations, including the
18-month booster, were up to date. The preliminary diagnosis was
laryngotracheobronchitis, and the patient received nebulized budesonide
and epinephrine, followed by intravenous dexamethasone (0.6 mg/kg) and
a fluid bolus of 0.9% normal saline. A complete blood count showed a white
blood cell count of 13.9×109/L (40% polymorphonuclear cells, 2.5% band
forms), hemoglobin 118 g/L and platelets 271×109/L. A blood culture and
viral nasopharyngeal swab were performed.
The child’s stridor diminished, but drooling developed, and further

3. CASE 2
A 1-year-old boy presents with a 2-month history of "wet" coughing.
Over the past 3 to 6 months, he has been treated each month for
acute otitis media. His parents are concerned that, despite a good
appetite, their son has been losing weight and has four to six loose,
foul-smelling stools per day.

4. CASE 3
A previously healthy 2-year-old girl presents with the complaint of
acute-onset wheezing. Her mother denies previous wheezing episodes
and denies a family history of asthma or atopy. The mother says that she
left the child playing in her older brother’s room. Approximately 20
minutes later she heard the child coughing and wheezing.
Differential ds
1. allergy anaphylactic reaction?
2. foreign body obstruct
3. Bronch asthma

5. FUNCTIONS OF THE RESPIRATORY
SYSTEM
Ventilation:
- Movement of air into and out of the lungs
- Inspiratory phase
- Expiratory phase
Diffusion and Perfusion:
- Gas exchange across the alveolar-pulmonary
capillary membranes
Control of Breathing:
- Influenced by neural and chemical factors
- Pons, medulla, chemoreceptors in the carotid
body
- Stimulus for breathing
- Increased carbon dioxide
Hypoventilation
 Slow, shallow breathing
 Causes CO2 to build up in the blood
–Acidosi
Hyperventilation
 Rapid, deep breathing
 Causes CO2 to be blown off -
Alkalosis

6. Airway resistance:
Full term Newborm Airway – 1 mm edema, the
d will be 44% of normal
In adult 1 mm edema - the d will be 81% of
normal

7. ANATOMICAL DIFFERENCES CONT
 Chest
- More rounded at birth, A:P diameter
increases to 2:1 as child growth
- children rely on abd/diaphragmatic
breathing and are obligatory nose
breathers
 Alveoli
-9 times more at the age 12 than at birht
- fewer alveoli = less surface for gas
exchange

8. ASSESSMENT - HISTORY
1. Chief Complaint and HPI
Cough
Shortness of breath/Dyspnea
2. Past Health History
3. disease or breathing problems
4. Frequent severe colds, asthma, emphysema, bronchitis, pneumonia,
tuberculosis
5. Last PPD and/or chest x-ray
6. Allergies, Medication use
7. Family History Alcohol, Drugs, Home environment, Occupational
environment
8. Travel
9. Health Promotional Activities

9. COUGH
Onset – sudden, gradual
Duration
Nature – dry, moist, hacking, barking
Sputum – amount, color, odor
Severity – disrupts activities
Associated symptoms – sneezing, dyspnea, fever, chills, congestion,
gagging
What brings it on? – anxiety, talking, activity
What makes it better?
What has been tried? – medications, treatments
Anything similar in the past?

10. SHORTNESS OF BREATH (SOB) /
DYSPNEA
Onset – sudden, gradual
Duration
Severity – disrupts activities
Associated symptoms – night sweats, pain, chest pressure,
discomfort, ankle edema, diaphoresis, cyanosis
What brings it on? – position, time of day, exercise, allergens,
emotions
What makes it better?
What has been tried? – medications, inhalers, oxygen
Anything similar in the past?

12. DURING A BUSY NIGHT, YOU GET THE FOLLOWING
PAGE:
FYI: Sally, a 2 year old
with PNA had a desat to
88% while on 4L NC.
What do you do next? What initial management steps would you take?

13. HOW DO YOU INITIALLY ASSESS
A PATIENT IN RESPIRATORY
DISTRESS?

14. Rapid assessment
 Quickly determine severity of respiratory condition and stabilize child
 Respiratory distress can quickly lead to cardiac compromise
Airway
 Support or open airway with jaw thrust
 Suction and position patient
Breathing
 Provide high concentration oxygen
 Bag mask ventilation
 Prepare for intubation
 Administer medication ie albuterol, epinephrine
Circulation
 Establish vascular access: IV/IO
INITIAL
ASSESME
NT

15. HISTORY AND PHYSICAL EXAM
History
Trauma
Change in voice
Onset of symptoms
Associated symptoms
Exposures
Underlying medical conditions
Physical Exam
Mental status
Position of comfort
Nasal flaring
Accessory muscle use
Respiratory rate and pattern
Auscultation for abnormal breath
sounds

16. WHAT INITIAL STUDIES WOULD YOU
GET FOR A PATIENT IN
RESPIRATORY DISTRESS?

17. Pulse oximetry
 May be difficult in agitated patient
 May be falsely decreased in very anemic patients
Imaging
 Chest X Ray
 Consider in patients with focal lung findings or respiratory distress
of a unknown etiology
 Soft tissue radiograph of lateral neck
 May identify a retropharyngeal abscess or radiopaque foreign body
Labs
 ABG/VBG
 Chemistry: calculate anion gap
 Urine toxicology and glucose if patient has altered mental status
INITIAL STUDIES

18. WHAT ARE SOME EXAMPLES OF
LIFE THREATENING CONDITIONS?

19. Complete upper airway obstruction
No effective air movement, speech or cough
Respiratory failure
Pallor or cyanosis, altered mental status, tachypnea, bradypnea,
apnea
Tension pneumothorax
Absent breath sounds on affected side, tracheal deviation and
compromised perfusion
Pulmonary embolism
Chest pain, tachycardia, tachypnea
Cardiac tamponade
LIFE THREATENING CONDITIONS

20. SPECIFIC CAUSES OF
RESPIRATORY DISTRESS
Upper airway obstruction
Lower airway obstruction
Lung tissue disease
Disordered control of breathing

21. Upper
Airway
Disease
Croup
Foreign Body
Epiglottitis
Bacterial Tracheitis
Asthma
Bronchiolitis
Pneumonia
Foreign Body
Lower
Airway
Disease
Noise during Inspiration
Proximal to Thoracic Inlet
Nose – Pharynx – Larynx
- Awake/Crying
- it child Improves
- Nose/Pharynx
- If child Deteriorates
- Larynx
Noise during
Exhalation
Distal to Thoracic
Inlet
Trachea, Bronchi,

22. Causes: foreign body, tissue edema,
trauma, infection, intubation, tongue
movement to posterior pharynx with
decreased consciousness
Symptoms
Partial obstruction: noisy inspiration
(stridor), choking, gagging or vocal
changes
Complete obstruction: no audible
speech, cry or cough
UPPER AIRWAY OBSTRUCTION

23. LARYNGOTRACHEOBRONCHITIS -
CROUP
Viral infection (parainfluenza) with Subglottic edema; Air flow
obstruction
Affects larynx, trachea
Incidence: 6 months to 4 years, Males > Females, Fall, early
winter
Signs/Symptoms: Recurs on several nights
Cold” progressing to hoarseness, cough
Low grade fever
Night-time increase in edema with:
 Stridor
 “Seal bark” cough
 Respiratory distress

24. CROUP: MANAGEMENT
Mild Croup
Reassurance
Moist, cool air
Severe Croup
Humidified high concentration oxygen
Monitor EKG
IV corticosteroids per os
Nebulized racemic epinephrine
Anticipate need to intubate, assist ventilations

25. EPIGLOTTITIS: PATHOPHYSIOLOGY
Bacterial infection (Hemophilus influenza)
Affects epiglottis, adjacent pharyngeal tissue
Supraglottic edema
Complete Airway
Obstruction

26. EPIGLOTTITIS: INCIDENCE
Children > 4 years old
Common in ages 4 - 7
Pedi incidence falling due to HiB vaccination
Can occur in adults, particularly elderly
Incidence in adults is increasing

27. EPIGLOTTITIS: SIGNS/SYMPTOMS
Rapid onset, severe distress in hours
High fever
Intense sore throat, difficulty swallowing
Drooling
Stridor
Sits up, leans forward, extends neck slightly
One-third present unconscious, in shock

28. EPIGLOTTITIS: MANAGEMENT
High concentration oxygen, do not examine the throat
IV Antibiotics, if possible cefotaxime 2 mg/kg 6 h
Rapid transport
Do not attempt to visualize airway
Corticosteroids remains controversial
Immediate Life Threat
Possible Complete Airway Obstruction

30. CASE
A previously healthy 18-month-old girl was brought to the emergency
department with a 2 h history of fever and stridor. On arrival, the patient’s
temperature was 38.5°C, heart rate 200 beats/min, respiratory rate 40
breaths/min and oxygen saturation was 97% on room air. On initial
assessment, the patient had a hoarse cry, inspiratory stridor and mild
suprasternal indrawing but was able to swallow without difficulty. No
drooling was evident. There was no previous history of choking, foreign
body aspiration or sick contact. All routine immunizations, including the
18-month booster, were up to date. The preliminary diagnosis was
laryngotracheobronchitis, and the patient received nebulized budesonide
and epinephrine, followed by intravenous dexamethasone (0.6 mg/kg) and
a fluid bolus of 0.9% normal saline. A complete blood count showed a white
blood cell count of 13.9×109/L (40% polymorphonuclear cells, 2.5% band
forms), hemoglobin 118 g/L and platelets 271×109/L. A blood culture and
viral nasopharyngeal swab were performed.
The child’s stridor diminished, but drooling developed, and further

31. RETROPHARYNGEAL ABSCESS
 infection of the space between the pre-tracheal fascia and the alar fascia of
the neck and most common bacteria are :Staphylococcus and streptococcus
 most commonly occurs in children 2-4 years of age.
 Presentation:
-Toxic appearance
-generalized sore throat, fever, and discomfort
-enlarged lymph nodes of the neck and may be neck pain
-dysphasia and odynophagia “ hot potato voice”
-respiratory distress
-can also present much like a patient with peritonsillar abscess or epiglottitis
with changes in voice quality as well as drooling and posturing to maintain a
patent airway.
-Diagnosis: CBC, Blood culture, inflammatory markers, imaging studies

32. head in full extension
Widening

33. CASE
A 10-month-old presented with drooling and
neck swelling. A CT scan revealed a large
retropharyngeal abscess measuring 1.8 cm in the
anterior posterior, 6.8 cm in the lateral, and 7.5
cm in the vertical dimension (Figures 1 and 2).
The low specificity of computed tomography (CT) led investigators to try for a better way of
defining a unique set of criteria that could more effectively determine which patients require
surgical drainage.

34.  Treatment initially should be with broad-spectrum parenteral antibiotics.
 If retropharyngeal abscess is suspected or confirmed by CT scan, patients
should be admitted for IV antibiotics, and ENT consultation for surgical
drainage
 Usually use Ampicillin sulbactam – high doses 50-100 mg/kg +/-
Vancomicin 40 mg/kg/day or Clindamicin 25-40 mg/kg/day
 Some practitioners favor the use of glucocorticoids. However, the
evidence behind such recommendations is limited
RETROPHARYNGEAL ABSCESS
TREATMENT

35. Aspirated Foreign Body
Basics
Common among the 1-3 age
group who like to put everything
in their mouths
Running or falling with objects in
mouth
Inadequate chewing capabilities
Common items - gum, hot dogs,
grapes and peanuts

36. Presentation:
- Patients who present immediately often have severe
symptoms from the product obstructing the trachea or larynx.
- dyspnea and stridor and/or wheezing
- Another group of patients that presents within the first 72
hours are patients who have a witnessed choking episode and
an aspiration event
- 4-7 days after the aspiration might be have cough, fever,
wheezing, or stridor
Complication:
recurrent pneumonia, inflammation of the airway, and
development of atelectasis. Any pediatric patient who
presents for recurring symptoms that do not seem to improve
should be considered to have a foreign body. These foreign
bodies are almost always located in the distal airways of the
bronchi
Aspirated Foreign Body

37. ASPIRATED FOREIGN BODY - MANAGEMENT
if the child can cough and verbalized it is placed inthe position of comfort and
oxygen is given
IV line placement and other interventions which
may agitate
the child in this case are avoided
X-ray evaluation for localization can be
performed
urgently in stable children
The presence of asphyxia indicates the need for immediate
resuscitation and securing the airway

38. ILD – INTESTINAL LUNG DISEASE
Interstitial Lung Disease (ILD), also known as diffuse lung disease, includes large,
heterogeneous group of rare pulmonary disorders that cause derangements of the
alveolar wall and disordered gas exchange
Prevalence in adult – 67-81, and in infants 0.13
ChILD can be very difficult to diagnose and treat, and is associated with significant
morbidity and mortality
ChildRN Classification
• Disorders more prevalent in infancy
• Disorders of the immunocompetent host
• Disorders related to systemic disease
• Disorders of the immunocompromised host
• Disorders masquerading as ILD

39. PEDIATRIC ILD

40. CLASSIFICATION ILD

42. RESPIRATORY SYMPTOMS Breathlessness (most common): Initially, dyspnea on
exertion→ later at rest Nonproductive cough Pleuritic chest pain Wheeing
Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS • Arthritis •
Ocular • Skin and muscle • GERD • Lower GI symptoms • Recurrent sinusitis •
Neurological symptoms • Epilepsy & mental retardation • Diabetes insipidus
INVESTIGATIONS: CHEST X RAY • Typically small lung volumes with Reticular ,
Nodular, or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT) • HRCT is more sensitive •
Combinations of ground glass changes, reticulonodular shadowing, honeycomb
cysts and traction bronchiectasis
PULMONARY FUNCTION TESTING
ARTERIAL BLOOD GAS ANALYSIS • Tuberculin test
Lung biopsy- TRANS BRONCHIAL BIOPSY , OPEN LUNG BIOPSY
Treatment - Prednisone, 1 mg/kg several month, Cytotoxic agents
(Cyclophosphamide)or immunosuppressive agents (Azathioprine)
ILD – INTESTIONAL LUNG DISEASE

43. CYSTIC FIBROSIS- CF
 Multisystem genetic diseases affecting the lung and upper respiratory
systems, Gastrointestinal tract (GI), pancreas, liver, sweat glands.
 CF autosomal recessive disorder caused by mutation in the CF
transmembrane conductance (CFTR) protein - defective ion transport in
exocrine glands – primarily reduced CL- and secondary increased Na
absorbtion.
 Chronic bacterial infection and progressive obstructive lung disease reason
of > 90% death
 Screening: can be identified by newborn screening with elevated
immunoreactive trypsinogen (IRT) and detection of CFTR mutation

44. CF – CLINICAL FEATURES
Chronic recurrent Sino-pulmonary infections with CF pathogens (Staph
aureus, Pseudomonas aeruginosa). Recurrent cough, wheezing, sputum
production, obstructive lung disease
GI – Meconium ileus • Abdominal distention • Intestinal obstruction •
Increased frequency of stools • Failure to thrive (despite adequate appetite) •
Flatulence or foul-smelling flatus, steatorrhea • Recurrent abdominal pain •
Jaundice • GI bleeding
Salt-loss syndrome - hypochloremic metabolic alkalosis
Respiratory system • Cough • Recurrent wheezing • Recurrent pneumonia •
Atypical asthma • Dyspnea on exertion • Chest pain
Genitourinary tract • Undescended testicles or hydrocele • Delayed secondary
sexual development • Amenorrhea

45. PHYSICAL EXAMINATION
Findings related to the pulmonary system may include the following:
• Tachypnea
• Respiratory distress with retractions
• Wheeze or crackles
• Cough (dry or productive of mucoid or purulent sputum)
• Increased anteroposterior diameter of chest
• Clubbing • Cyanosis
• Hyperresonant chest upon percussion (crackles are heard acutely in
associated pneumonitis or bronchitis and chronically with bronchiectasis)

46. DIAGNOSIS
The diagnosis of cystic fibrosis (CF) is based on
• Typical pulmonary manifestations, GI tract
manifestations
• Family history
• Universal newborn screening
• Prenatal screening test
• Sweat test results
Requirements for a CF diagnosis include either
positive genetic testing or positive sweat chloride
test findings (>60 mEq/L) and 1 of the following:
• Typical chronic obstructive pulmonary disease
• Documented exocrine pancreatic insufficiency
• Positive family history (usually affected sibling)
Other examinations • Imaging test (X-ray, US, CT,
MRI) • Genotyping • Pulmonary function test •
Bronchoalveolar lavage and sputum microbiology •
Immunoreactive trypsinogen • Contrast barium
enema

51. CF MANAGEMENT
- Management The primary goals of CF treatment include the
following:
• Maintaining lung function as near to normal as possible by
controlling respiratory infection and clearing airways of mucus
• Administering nutritional therapy (ie, enzyme supplements,
multivitamin and mineral supplements) to maintain adequate growth
• Managing complications

52. CF MANAGEMENT
Mild acute pulmonary exacerbations of cystic fibrosis can be treated
successfully at home with the following measures:
• Increasing the frequency of airway clearance
• Inhaled bronchodilator treatment (especially if bronchial hyper
responsiveness is present or as part of airway clearance [inhaled
bronchodilator followed by chest physical therapy and postural
drainage)
• Chest physical therapy and postural drainage
• Increasing the dose of the mucolytic agent dornase alfa
(Pulmozyme)
• Use of oral antibiotics (eg, oral fluoroquinolones)

53. CF MANAGEMENT CONTINUE
Medications used to treat patients with cystic fibrosis may include the following:
• Pancreatic enzyme supplements
• Multivitamins (including fat-soluble vitamins
• Mucolytics
• Nebulized, inhaled, oral, or intravenous antibiotics
• Bronchodilators • Anti-inflammatory agents
• Agents to treat associated conditions or complications (eg, insulin, bisphosphonates)
• Agents devised to potentially reverse the abnormalities in chloride transport (eg,
ivacaftor)
Pancreatic enzyme supplements • Pancrelipase (Creon, Pancreaze, Ultresa, Zenpep)
Mucolytics • Dornase alpha (Pulmozyme) Bronchodilators • Inhaled beta2-agonist :
Albuterol (AccuNeb, ProAir, Proventil HFA, VoSpire ER, Ventolin HFA) Vaccination •
Vaccination against Pertussis, Haemophilus influenzae, Varicella, Streptococcus
pneumoniae, and measles and annual influenza vaccination.

54. Airway clearance • Postural drainage, percussion, vibration, and assisted
coughing are recommended at the time of diagnosis and should be done on
a regular basis Antibiotics (oral, intravenous, or inhalation) • Aerosolized
form : gentamicin, aztreonam, colistin, and preservative-free high-dose
tobramycin especially formulated for inhalation
Vitamins • Fat soluble vitamins A, D, E, and K and water soluble biotin, folic
acid, niacin, pantothenic acid, B vitamins (ie, B-1, B-2, B- 6, B-12), and
vitamin C. CFTR Potentiators • Cystic fibrosis transmembrane conductance
regulator (CFTR) potentiators are the first available treatment that targets
the defective CFTR protein. • Ivacaftor (Kalydeco) - facilitates increased
chloride transport by potentiating the channel-open probability (or gating)
of certain CFTR gene mutations.
CF MANAGEMENT CONTINUE

55. MOST COMMON CARDIAC
DISEASES IN CHILDREN

56. THE PATIENT
9 year boy previously healthy, football player
1 week of fatigue and dry cough
- Loss of appetite, vague stomach ache
He was seen by his doctor 2 days earlier
- Diagnosed with viral illness
2 days later he came to the ER
- Chest pain, dizzy, short of breath
NO heart problem in the family

57. EPIDEMIOLOGY OF HF IN
CHILDREN
In the United states
- HF related hospitalizations occus in 11.000-14.000
children yearly
- Overall mortality: 7%!!!
Congenital heart disease (69.3%)
Cardiomyopathy (13.6%)
Myocarditis (2.1%)
Arrhythmias (15.2%)

58. DEFINITION OF PEDIATRIC HEART
FAILURE
Inability of the heart to supply sufficient cardiac output to
keep up with metabolic demand

59. HEART FAILURE SYMPTOMS
Quite variable:
Swelling
Dyspnea of exertion
Palpitations
Weight gain or weight loss
Abdominal pain
Syncope
Failure to thrive
Sudden cardiac arrest

60. CARDIOMYOPATHY
A myocardial disorder in which heart muscle is structurally
and functionally abnormal in the absence of coronary artery
disease, hypertension, valvular disease and congenital heart
disease.
3 type:
- Dilated cardiomyopathy DCM
- Hypertrophic cardiomyopathy HCM
- Restrictive cardiomyopathy RCM

61. DILATED CARDIOMYOPATHY DCM
Ventricular dilatation and impaired
systolic function
Most common:>70% of pediatric
cardiomyopathy
Incidence: 0.6-1/100000
Histology: - fibrosis, myocyte
hypertrophy

62. CAUSES OF
DCM IN
CHILDREN

63. DIAGNOSIS DCM
Clinical presentations:
-Symptomatic HF (Syncope, dyspnea, overload)
-Asymptomatic
Physical examination
-Variable degrees of cardiac enlargement
-Pulse pressure is narrow
-JVP raised
-3th and 4 th sounds are common
-Mitral and tricuspid regurgitation are common

64. INITIAL EVALUATION OF NEW
ONSET DCM
Ecg, Echo, cardiac
XRAY
4 limb blood pressure
CBC with differencial
Serum electrolites:
magnesium, calcium,
potassium
BUN, Creatinine
UA
Liver enzymes and albumin
Thyroid tests
Cardiac catheterization
Carnitine and profile
Pyruvate lactate
Genetic evoluation
IEM: Urine for amino serum
acids
Serum free fatty acids

65. ECG - DCM

66. TREATMENT OF DCM
1. therapeutic strategy include preload and afterload reduction therapy:
Diuretics, angiotensin-converting-enzyme inhibitor and β-blockers:
Carvedilol
2. Cardiac transplantation is treatment of choice

67. HYPERTROPHIC CM - HCM
HCM - hypertrophied, nondilated ventricle in
the absence of a hemodynamic cause that is
capable of producing the existent magnitude of
wall thickening, excluding both physiological
hypertrophy (ie, secondary to physical activity)
and pathological hypertrophy (ie, secondary to
hypertension, aortic valvular stenosis, and
other disorders)
The primary diagnostic criterion for HCM is the
maximum diastolic septal or LV free wall
thickness >15mm or >2 standard deviation for
age and sex
Systolic function typically preserved
Abnormal cardiac relaxation
Most common cause of sudden death in
athletes

68. HCM CLINICAL PRESENTATION
Is difficult to diagnosed, commonly asymptomatic
 Clinical presentation
-Dyspnea, fatigue, chest pain, syncope, palpitation
-Dyspnea on effort
-Syncope on effort
-Jerky pulse
-double impulse in apex
-Pansystolic murmur
-Sudden death!!! Usually secondary to abnormal heart rhythm

69. HCM - INITIAL EVALUATION
1. Xray – cardiomegaly
2. ECG: changes are
nonspecific, may be
associated with abnormal
QT
3. Echo:

70. TREATMENT HCM
• B-Blockers and rate limiting calcium antagonists, digitalis and diuretics
are not usually beneficial
•No pharmacological treatment is known to improve prognosis
•Arrhythmia is common and responds well to amiodarone, with low
myocardial contractility
•Outflow tract obstruction can be improved by surgery
•Implantable cardiac defibrillation (ICD) for patient with risk of sudden
death

71. RESTRICTIVE CM
- abnormal relaxation of the
heart muscle with normal
ventricular size and thickness
- 2.5 -5% of total paediatric CM
- may be hereditary, thus
genetic screening is important
- Cause is unknown in children
- Presenting symptoms:
dyspnea, exacerbated by
respiratory illness, syncope.
Xray, Echo, Biopsy, Genetic
testing, MRI

72. OUR CASE
He was diagnosed with myocarditis due to
adenovirus, he had a mild improvement.
… BUT
2month later he could not walk 1 block
without having chest pain
ECHO show enlarged heart with poor
squeeze
He was re admitted and started on IV
Milrinone and diuretics.
He was gene positive, but no family
members
4 month after listing he received a heart
transplantation
He is now doing well and playing football

73. NEW POTENTIAL TREATMENT
1. Stem cell therapy to repair ventricular myocardium
2. Gene Therapy using a virus vector to help with abnormal
remodeling and calcium cycling
3. Surgical – better and smaller VADS

74. SYNCOPE
 Syncope is a sudden, brief loss of consciousness; loss of postural tone &
recovery is spontaneous.
 Up to 15 percent of children experience a syncopal episode prior to the end
of adolescence.
 Preceding symptoms: lightheadedness, nausea, visual disturbances and
palpitation.
Etiology:
- most often benign. can also occur as the result of more serious (usually
cardiac) disease with the potential of sudden death.
- Hypoglycemia (Type 1 DM)
- Supraventricular tachycardia (Rare).
- Bradycardia
- Pregnancy

77. COMPLAINS
fainted…
Following exercise..
Hot weather..
Light-headed..
Seeing black..
Seeing stars..
Nausea..
Abdominal cramps..
Racing heart..
Rapid standing from sitting
down..
Seeing blood..
Blood draws..
Urination..
Turning head rapidly..
Deep emotions..
Forgot what happened..
Headache..
Less common: Palpitation..
Brief convulsions.. Brief
confusion

78. COMMON CONDITION
COMMON CONDITIONS
Vasovagal syncope (neurocardiogenic) is the most common cause of
syncope among children ~50%.
Breath holding spells; typically occur in children between 6 months
and 24 months old; 2 types pallid and cyanotic.
Orthostatic hypotension .
Toxic exposure (opiates, alcohol, carbon monoxide ).
Medications (barbiturates, tricyclic antidepressants, and
phenothiazines)

79. CONDITIONS THAT MIMIC
SYNCOPES
Seizures — a seizure typically includes loss of consciousness and
postural tone.
Migraine syndromes (Basilar migraine)
Hysteria/conversion disorder.
Hyperventilation.
Choking game.

80. RED FLAGS HISTORY
Red flags in history
During exercise (not following exercise)
Preceding chest pain
Resuscitation required (CPR)
Family history of sudden death
Deafness
Progressive symptoms: more frequent fainting, more prolonged episodes.
Preceding palpitation.

85. CONGENITAL HEART DISEASES
DEFINITION
1. obstructive congenital heart lesions , for example congenital mitral
and aortic stenosis
2. Congenital heart lesions that INCREASE pulmonary arterial blood
flow = left to right shunts within the heart , for example atrial septal
defect
3. congenital heart lesions that DECREASE pulmonary arterial blood
flow – can involve right to left shunting of blood leading to systemic
cyonosis - for example tetralogy of Fallot

86. CONGENITAL HEART DISEASES

87. ACYANOTIC CONGENITAL HEART DISEASE
Left-to-Right Shunt Lesions
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Atrioventricular Septal Defect (AV Canal)
Patent Ductus Arteriosus (PDA)

88. Cardiac
case
Interventio
ns
Age
Color
Exa
m
Tests

89. CASE 1 - 9 DAY OLD. POOR FEEDING. CENTRAL
CYANOSIS
9 day
old boy
Interventions
Age
Less than 1 month
Color
Blue – Central
cyanosis
Exam
- Pulmonary N
- Pulse Ox: 75%
- Hr 160’
- Murmurs right
- No pulse delay
Tests
- CXR& ECG
- Oxygen
- Prostaglandi
n
- Fluid
balance
Differential:
 Trauma
 Sepsis
 Infection

90. CASE 2 - 3 WEEK OLD. POOR PERFUSION,
GREY
21 day
old boy
Interventions
Age
Less than 1 month
Color
Grey and
shock
Exam
- Pulmonary N
- Pulse Ox: 75%,
right hand 92%
- Hr 160’
- Murmurs
- pulse delay
Tests
- CXR& ECG
- Oxygen
- Prostaglandi
n
- Fluid
balance
Differential:
 Trauma
 Sepsis
 Infection

91. SHOCK AND LEFT SIDE
OBSTRUCTION (CLUES AND
GOALS)
 shock from cardiac obstruction or not?
Is oxygen safe? Yes mostly
PGE – trials in neonates
Fluids 10 ml/kg aliquots
 CXR – Cardiomegaly
Bedside echo

92. ATRIAL SEPTAL DEFECT
ASD is an opening in the atrial septum permitting free
communication of blood between the atria. Seen in 10% of
all CHD.

93. ATRIAL SEPTAL DEFECT
There are 3 major types:
Secundum ASD – at the Fossa Ovalis, most
common.
• Primum ASD – lower in position & is a
form of ASVD, MV cleft.
• Sinus Venosus ASD – high in the atrial
septum, associated w/partial anomalous
venous return & the least common.

94. ATRIAL SEPTAL DEFECT
Secundum ASD
Sinus Venosus ASD

95. ATRIAL SEPTAL DEFECT
Clinical Signs & Symptoms
Rarely presents with signs of CHF or other
cardiovascular symptoms.
• Most are asymptomatic but may have easy
fatigability or mild growth failure.
• Cyanosis does not occur unless pulmonary
HTN is present.

96. ATRIAL SEPTAL DEFECT
Clinical Signs & Symptoms
• Hyperactive precordium, RV heave, fixed widely
split S2.
• II-III/VI systolic ejection murmur @ LSB.
• Mid-diastolic murmur heard over LLSB.

97. ATRIAL SEPTAL DEFECT
Treatment:
Surgical or catherization laboratory closure is generally
recommended for secundum ASD w/ a Qp:Qs ratio
>2:1.
• Closure is performed electively between ages 2 &
5 yrs to avoid late complications.
• Surgical correction is done earlier in children w/
CHF or significant Pulm HTN.

98. ATRIAL SEPTAL DEFECT
Treatment
• Once pulmonary HTN w/ shunt reversal occurs this is
considered too late.
• Mortality is < 1%.

99. VENTRICULAR SEPTAL DEFECT
VSD – is an abnormal opening in the ventricular
septum, which allows free communication between the
Rt & Lt ventricles. Accounts for 25% of CHD.

100. VENTRICULAR SEPTAL
DEFECT
4 Types
Perimembranous (or membranous) – Most
common.
Infundibular (subpulmonary or supracristal
VSD) – involves the RV outflow tract.
• Muscular VSD – can be single or multiple.
• AVSD – inlet VSD, almost always involves
AV valvular abnormalities.

101. VENTRICULAR SEPTAL DEFECT
Hemodynamics
The left to right shunt occurs secondary to PVR being
< SVR, not the higher pressure in the LV.
This leads to elevated RV & pulmonary pressures &
volume hypertrophy of the LA & LV.

102. VENTRICULAR SEPTAL
DEFECT
Clinical Signs & Symptoms
• Small - moderate VSD, 3-6mm, are
usually
asymptomatic and 50% will close
spontaneously
by age 2yrs.
• Moderate – large VSD, almost always
have

103. VENTRICULAR SEPTAL
DEFECT
Clinical Signs & Symptoms
• II-III/VI harsh holosystolic murmur heard along the
LSB, more prominent with small VSD, maybe absent
with a
very Large VSD.
• Prominent P2, Diastolic murmur.
• CHF, FTT, Respiratory infections, exercise intolerance
hyperactive precordium. Symptoms develop between
1 – 6
months

104. VENTRICULAR SEPTAL DEFECT
Treatment
• Small VSD - no surgical intervention, no
physical restrictions, just reassurance and
periodic follow-up and endocarditis prophylaxis.
• Symptomatic VSD - Medical treatment
initially with afterload reducers & diuretics.

105. VENTRICULAR SEPTAL
DEFECT
Treatment
Indications for Surgical Closure:
Large VSD w/ medically uncontrolled symptomatology &
continued FTT.
Ages 6-12 mo w/ large VSD & Pulm. HTN
Age > 24 mo w/ Qp:Qs ratio > 2:1.
Supracristal VSD of any size, secondary to risk of developing
AV insufficiency.

106. ATRIOVENTRICULAR SEPTAL
DEFECT
AVSD results from incomplete fusion the the endocardial cushions,
which help to form the lower portion of the atrial septum, the
membranous portion of the ventricular septum and the septal
leaflets of the triscupid and mitral valves.
They account for 4% OF ALL CHD.

107. ATRIOVENTRICULAR SEPTAL
DEFECT
Question:
What genetic disease is AVSD more
commonly seen in?
• Answer:
Down’s Syndrome (Trisomy 21), Seen in 20-25% of cases.

108. ATRIOVENTRICULAR
SEPTAL DEFECT
Complete Form
Low primum ASD
continuous with a posterior
VSD.
Cleft in both septal leaflets
of TV/MV.
Results in a large L to R
shunt at both levels.
TR/MR, Pulm HTN w/
increase in PVR.
Incomplete Form
Any one of the
components may be
present.
Most common is
primum ASD, cleft in
the MV & small VSD.
Hemodynamics are
dependent on the
lesions.

109. ATRIOVENTRICULAR SEPTAL
DEFECT
Complete AVSD

110. ATRIOVENTRICULAR SEPTAL
DEFECT Clinical Signs & Symptoms
Incomplete AVSD maybe indistinguishable
from ASD - usually asymptomatic.
Congestive heart failure in infancy.
Recurrent pulmonary infections.
Failure to thrive.
Exercise intolerance, easy fatigability.
Late cyanosis from pulmonary vascular
disease w/ R to L shunt.

111. ATRIOVENTRICULAR
SEPTAL DEFECT
Clinical Signs & Symptoms
Hyperactive precordium
Normal or accentuated 1st hrt sound
Wide, fixed splitting of S2
Pulmonary systolic ejection murmur
w/thrill
Holosystolic murmur @ apex w/radiation to
axilla
Mid-diastolic rumbling murmur @ LSB

112. ATRIOVENTRICULAR SEPTAL
DEFECT
Treatment
Surgery is always required.
Treat congestive symptoms.
Pulmonary banding maybe required in premature
infants or infants < 5 kg.
Correction is done during infancy to avoid irreversible
pulmonary vascular disease.
Mortality low w/incomplete 1-2% & as high as 5% with
complete AVSD.

113. PATENT DUCTUS ARTERIOSUS
PDA – Persistence of the normal fetal vessel
that joins the PA to the Aorta.
Normally closes in the 1st wk of life.
Accounts for 10% of all CHD, seen in 10% of
other congenital hrt lesions and can often play
a critical role in some lesions.
Female : Male ratio of 2:1
Often associated w/ coarctation & VSD.

114. PATENT DUCTUS
ARTERIOSUS
Hemodynamics
As a result of higher aortic pressure, blood
shunts L to R through the ductus from
Aorta to PA.
Extent of the shunt depends on size of the
ductus & PVR:SVR.
Small PDA, pressures in PA, RV, RA are
normal.

115. PATENT DUCTUS ARTERIOSUS
Hemodynamics
Large PDA, PA pressures are equal to systemic pressures. In extreme
cases 70% of CO is shunted through the ductus to pulmonary
circulation.
Leads to increased pulmonary vascular disease.

116. PATENT DUCTUS ARTERIOSUS
Clinical Signs & Symptoms
Small PDA’s are usually asymptomatic
Large PDA’s can result in symptoms of CHF,
growth restriction, FTT.
Bounding arterial pulses
Widened pulse pressure
Enlarged heart, prominent apical impulse
Classic continuous machinary systolic murmur
Mid-diastolic murmur at the apex

117. PATENT DUCTUS ARTERIOSUS
Treatment
Indomethacin, inhibitor of prostaglandin synthesis can
be used in premature infants.
PDA requires surgical or catheter closure.
Closure is required treatment heart failure & to prevent
pulmonary vascular disease.
Usually done by ligation & division or intra vascular
coil.
Mortality is < 1%

118. OBSTRUCTIVE HEART LESIONS
Pulmonary Stenosis
Aortic Stenosis
Coarctation of the Aorta

119. PULMONARY STENOSIS
Pulmonary Stenosis is obstruction in the region of
either the pulmonary valve or the subpulmonary
ventricular outflow tract.
Accounts for 7-10% of all CHD.
Most cases are isolated lesions
Maybe biscuspid or fusion of 2 or more leaflets.
Can present w/or w/o an intact ventricular
septum.

120. PULMONARY STENOSIS
Hemodynamics
RV pressure hypertrophy  RV failure.
RV pressures maybe > systemic pressure.
Post-stenotic dilation of main PA.
W/intact septum & severe stenosis  R-L shunt through PFO 
cyanosis.
Cyanosis is indicative of Critical PS.

121. PULMONARY STENOSIS
Clinical Signs & Symptoms
Depends on the severity of obstruction.
Asymptomatic w/ mild PS < 30mmHg.
Mod-severe: 30-60mmHg, > 60mmHg
Prominent jugular a-wave, RV lift
Split 2nd hrt sound w/ a delay
Ejection click, followed by systolic murmur.
Heart failure & cyanosis seen in severe cases.

122. PULMONARY STENOSIS
Treatment
Mild PS no intervention required, close follow-up.
Mod-severe – require relieve of stenosis.
Balloon valvuloplasty, treatment of choice.
Surgical valvotomy is also a consideration.

123. AORTIC STENOSIS
Aortic Stenosis is an obstruction to the outflow from
the left ventricle at or near the aortic valve that causes
a systolic pressure gradient of more than 10mmHg.
Accounts for 7% of CHD.
3 Types
Valvular – Most common.
Subvalvular(subaortic) – involves the left outflow tract.
Supravalvular – involves the ascending aorta is the
least common.

124. AORTIC STENOSIS
Hemodynamics
Pressure hypertrophy of the LV and LA with obstruction to flow from
the LV.
Mild AS 0-25mmHG
Moderate AS 25-50mmHg
Severe AS 50-75mmHg
Critical AS > 75mmHg

125. AORTIC STENOSIS
Clinical Signs & Symptoms
Mild AS may present with exercise intolerance, easy
fatigabiltity, but usually asymptomatic.
Moderate AS – Chest pain, dypsnea on exertion,
dizziness & syncope.
Severe AS – Weak pulses, left sided heart failure,
Sudden Death.

126. AORTIC STENOSIS
Clinical Signs & Symptoms
LV thrust at the Apex.
Systolic thrill @ rt base/suprasternal notch.
Ejection click, III-IV/VI systolic murmur @ RSB/LSB w/ radiation to the
carotids.

127. AORTIC STENOSIS
Treatment
Because surgery does not offer a cure it is
reserved for patients with symptoms and a
resting gradient of 60-80mmHg.
For subaortic stenosis it is reserved for
gradients of 40-50mmHg because of it’s
rapidly progressive nature.
Balloon valvuloplasty is the standard of
treatment.

128. AORTIC STENOSIS
Treatment
Aortic insufficiency & re-stenosis is likely after surgery
and may require valve replacement.
Activity should not be restricted in Mild AS.
Mod-severe AS, no competitive sports.

129. COARCTATION OF THE AORTA
Coarctation- is narrowing of the aorta at varying
points anywhere from the transverse arch to the iliac
bifurcation.
98% of coarctations are juxtaductal
Male: Female ratio 3:1.
Accounts for 7 % of all CHD.

130. COARCTATION OF THE AORTA
Hemodynamics
Obstruction of left ventricular outflow  pressure hypertrophy of the
LV.

131. COARCTATION OF THE AORTA
Clinical Signs & Symptoms
Classic signs of coarctation are diminution or
absence of femoral pulses.
Higher BP in the upper extremities as compared to
the lower extremities.
90% have systolic hypertension of the upper
extremities.
Pulse discrepancy between rt & lt arms.

132. COARCTATION OF THE AORTA
Clinical Signs & Symptoms
With severe coarc. LE hypoperfusion, acidosis, HF and
shock.
Differential cyanosis if ductus is still open
II/VI systolic ejection murmur @ LSB.
Cardiomegaly, rib notching on X-ray.

133. COARCTATION OF THE AORTA

134. COARCTATION OF THE AORTA
Treatment
With severe coarctation maintaining the ductus with
prostaglandin E is essential.
Surgical intervention, to prevent LV dysfunction.
Angioplasty is used by some centers.
Re-coarctation can occur, balloon angioplasty is the
procedure of choice.

135. TOF – TETRADE FALLOT
4 anatomic malformations:
-Right Ventricular
Hypertrophy
-Pulmonary Valve
Stenosis
-Transposition of
the aorta
-Ventricular Septal
Defect

136. TOF
RVH
-secondary to PA Stenosis
-Increased P on RV leads to RVH
Transposition of Aorta
-aorta is displaced
VSD
-”hole in the heart”
-mixing of oxygenated and unoxygenated blood
-cyanosis
PVS
-more severe, less blood transported to the lungs and
more deoxygenated blood will pass through VSD to aorta
to be circulated throughout the body

137. CLINICAL PRESENTATION TOF
Clinical presentation is directly related to the degree of pulmonary stenosis.
Severe stenosis results in immediate cyanosis following birth. Mild stenosis will not
present until later.
Growth is retarded – insufficient oxygen and nutrients
SOA on exertion
SYMPTOMS:
▫ Severe cyanosis
▫ Hypercyanotic spells
- Associated with irritability or inconsolable crying
because of the hypoxia and Breathlessness and pallor
due to acidosis
▫ Squatting on exercise.
SIGNS: ▫ Clubbing in older groups ▫ Loud harsh ejection systolic murmur which will
shorten as RV outflow increases.

138. “TET SPELL”
“Tet spells” at 2-
3yo, child
becomes cyanotic,
may experience
syncope

140. MYOCARDITIS
Etiology – viral and non viral condition (autoimmune, Lyme/..)
Presentation: nonspecific GI and respiratory symptoms
 Heart failure signs in kids:
-Hepatomegaly
-Dyspnea
-Persistent Tachycardia
 Evaluation:
-ECG - Sinus tachycardia most common
-CXR – abnormal 50%
-Troponin
-BNP

141. MYOCARDITIS
Echocardiography is the most cost-effective test used for evaluation of
myocardial function. It is sensitive but not specific.
Findings include the following:
Global hypokinesis (the most common finding)
Increased left ventricular end diastolic and systolic dimensions
Left ventricular dysfunction, primarily systolic with decreased ejection
fraction and shortening fraction
Segmental wall motion abnormalities
Pericardial effusion
on ECG:
nonspecific ST/T wave changes and low voltages
Pseudoinfarction patterns with pathologic Q waves and poor progression
of R waves in the precordial leads may also be present. T-wave flattening
or inversion is a common finding associated with small or absent Q waves
in V5 and V6.

142. Case
A 15-year-old boy without cardiovascular risk factors or
previous history of cardiovascular disease presented to the
emergency department in our institution for persistent
chest pain with mild fever (<38°C) for the last 3 days. The
patient reported no respiratory tract signs.
The physical examination revealed blood pressure of
100/60 mmHg, heart rate of 75 b.p.m., oxygen saturation
of 98% while breathing ambient air, and body temperature
of 36.9°C. The electrocardiogram showed diffuse ST
elevation without reciprocal changes
Blood tests revealed a slight increase in C-reactive protein
level (41 mg/L, normal <6 mg/L) with normal leucocytes
(6.1 × 109/L, normal 4–10 × 109 cells/L) and elevated
cardiac troponin 6.1 μg/L (99th upper reference limit
0.045 μg/L). N-terminal probrain natriuretic peptide (NT-
proBNP 65 ng/L, normal <300 ng/L) and D-dimer (259
ng/mL, normal <500 ng/mL) remained normal.
Because of systematic suspicion of COVID-19 in patients
with unexplained fever, a PCR was performed on a
nasopharyngeal swab and resulted positive for SARS-CoV-
2. A multiplex real-time PCR was also performed and
resulted negative, allowing the exclusion of viral co-
infections. Chest CT scan showed no lung anomalies
Transthoracic echocardiography showed a mild diffuse
hypokinesia with left ventricular ejection fraction (LVEF) at
50%, preserved cardiac output, normal right ventricular
function, no significant valvular disease, and normal
pulmonary pressure without lower vena cava dilatation.
There was a mild pericardial effusion around the lateral
wall of the left ventricle (maximum, 5 mm) without signs of
tamponade

143. QUESTIONS
Examination of a 3-hr old infant reveals dysmorphic
features and cyanosis. Both the occiput and facial
profile are flat, and the fontanelle is abnormally
enlarged. The space between the great and second
toe is wide, and there is a palmar crease extending
across the left palm. Room air oximetry reveals a
saturation 70%.

144. QUESTIONS
Of the following, the MOST likely lesion to
be found on echocardiography would be
A. Atrioventricular septal defect
B. Coarctation of the aorta
C. Hypoplastic left heart
D. Total anomalous pulmonary venous return
E. Truncus arteriosus

145. QUESTIONS
After a few days of poor feeding and tachypnea, a 3 week old presents
with hypotension, poor central and peripheral pulses, and severe
metabolic acidosis. A
gallop is audible, and the heart appears enlarged on chest radiography.
hepatomegaly
is marked.

146. QUESTIONS
Of the following, the BEST intervention to
produce a sustained improvement is
A. 100% Oxygen administration
B. Dopamine infusion
C. Gamma globulin infusion
D. Phenylephrine infusion
E. Prostaglandin E infusion