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CONGENITAL HEART DISEASE, CYANOTIC TYPE, TETRALOGY OF FALLOT.pptx
1. BAGUIO GENERAL HOSPITAL AND MEDICAL CENTER
Department of Pediatrics
Alexious Marie T. Callueng, M.D
2. OBJECTIVES:
ď§ To present a case of a patient with Diabetic Ketoacidosis
(DKA)
ď§ To recognize its clinical features
ď§ To discuss its pathophysiology and diagnostic work-up
ď§ To identify the classification of DKA
ď§ To discuss the appropriate management of DKA
ď§ To formulate a Discharge Plan for a patient with Diabetes
3. GENERAL DATA
⢠C.C.
⢠11 year-old
⢠Male
⢠Benguet
⢠Filipino
⢠Roman Catholic
⢠Birthday:
⢠2nd admission at
6. History Of Present Illness
⢠(+) cough and colds
⢠(+) Undocumented fever
⢠Frontal headache
⢠(+) Dizziness, loss of consciousness
2 weeks PTA
ď Given paracetamol at 10 mkdose â relief of pain
ď NO CONSULT DONE
7. History Of Present Illness
⢠Resolution of fever
⢠(+) persistence of headache
⢠(+) Right-sided body weakness
⢠(+) loss of sensation, immobility,
right sided
10 days PTA
ď NO CONSULT DONE
⢠Persistence of symptoms prompting
consult
Few hours
PTA
8. REVIEW OF SYSTEMS
General: weight loss
Integumentary System: (-) pruritus, no rashes
HEENT: no blurring of vision, no headache, no photophobia,
no head trauma, (-) ear pain, (-) itchiness and sore throat
Respiratory System: (-)cough or colds
Cardiovascular System: no chest pain, no orthopnea, no easy
fatigability
9. REVIEW OF SYSTEMS
Gastrointestinal System: (-) diarrhea, (-) constipation,
(-) hematochezia/melena
Genitourinary System: (-) discharges on the genital area,
(-) hematuria, (-) dysuria
Musculoskeletal System: (-) limping, (-) joint pains
Nervous System: (-) loss of consciousness, (-) dizziness, (-)seizure
Endocrine system: (-) profuse sweating, denies polyphagia
10. Past Medical History
⢠(+) Brain abscess, CHD TOF â BGHMC 2017
⢠No asthma, heart disease nor had PTB
⢠No known allergies to food or medications
11. Immunization History
ď§1 dose of BCG
ď§3 doses of Hepatitis B
ď§3 doses of OPV
ď§3 doses of DPT
ď§1 dose of Measles
ď§2 doses of COVID 19 vaccine (Pfizer)
13. Personal and Social History
⢠Lives in a bungalow-type of house with her
father and 2 siblings
⢠2nd child in a brood of 3
HOME
14. ⢠Grade 6 pupil
⢠Averagely performing in school
EDUCATION
⢠Most of the time he does household chores
⢠He belongs to a peer of 4 (classmates)
ACTIVITY
⢠Denies cigarette smoking or illicit drug use
⢠Not an alcoholic drinker
DRUGS
Personal and Social History
⢠No food preference
⢠No food allergies
DIET
15. ⢠Roman Catholic
⢠Seldom goes to church
SPIRITUALITY
⢠Admits being attracted to opposite sex
⢠Not in a relationship
⢠Denies history of sexual activity
SEXUALITY
⢠No history of suicidal ideation or depression
SUICIDALITY
Personal and Social History
25. HEENT
ď§ Nose: septum at midline; with alar flaring; no nasal
discharge
ď§ Mouth: (+) circumoral cyanosis, dry lips and oral
mucosa; tongue at midline, uvula at midline; no
tonsillopharyngeal enlargement
ď§ Neck: no lesions, no cervical lymphadenopathies
26. CHEST AND LUNGS
â Symmetrical chest expansion
â No retractions
â Clear breath sounds
27. CARDIOVASCULAR
⢠Adynamic Precordium
⢠Irregular rhythm, (+) holosystolic murmur,
grade 3/6
⢠PMI: 5th Left ICS Left Midclavicular line
⢠No thrills or heaves
⢠No murmur
28. ABDOMEN
⢠Flat
⢠Normoactive bowel sounds
⢠Predominantly tympanitic
⢠Soft, non-tender
⢠No organomegaly
GENITALIA
⢠Grossly normal female; Tanner Stage II
31. NEUROLOGIC
⢠CRANIAL NERVES:
CN 1: ABLE TO SMELL ON BOTH NOSTRILS
CN II: 2-3 ERTL
CN III IV VI: FULL EOMS, PRIMARY GAZE MIDLINE, NO NYSTAGMUS, NO PTOSIS
CN V: INTACT V1-V3 SENSORY (SENSATION)
CN VIII: (+) FACIAL ASSYMETRY
CN VIII: INTACT GROSS HEARING IX: UVULA AT THE MIDLINE, WITH SWALLOW REFLEX
CN XI: CAN TURN HEAD SIDE TO SIDE AND SHRUG SHOULDERS AGAINST RESISTANCE
CN XII: (+) TONGUE DEVIATION, ABLE TO MOVE SIDE TO SIDE, NO DEVIATION, NO
FASCICULATIONS
32. NEUROLOGIC
SENSORY TESTING
⢠100% on all extremities
DEEP TENDON REFLEX RESULT
Biceps Reflex 2+
Triceps Reflex 2+
Patellar Reflex 2+
Ankle Reflex 2+
33. NEUROLOGIC
Motor Testing
⢠No Atrophy
⢠No Spasticity
⢠No Fasciculations
Left Arm 5/5
Right Arm 2/5
Left Leg 5/5
Right Leg 2/5
34. Cerebellar
⢠Cannot be assessed
NEUROLOGIC
Meningeal Signs
No nuchal rigidity
No Kernigâs sign
No Brudzinski sign
35. AT THE EMERGENCY ROOM
⢠(+) severe epigastric pain
⢠With difficulty of breathing
⢠No active vomiting
Vital Signs:
BP: 80/60 mmHg
PR: 138bpm
RR: 31cpm
T: 37.2ËC
O2 sat: 98% at room air
⢠Drowsy, weak looking
⢠In cardiorespiratory distress
⢠Sunken eyeballs
⢠Dry lips and mucosa
⢠Rapid, deep breathing
⢠Fair pulses
⢠Poor skin turgor
⢠Warm extremities
-NPO
-02 Facemask at 5LPM
Diagnostics:
⢠CBC
⢠ABG
⢠HGT
⢠Blood CS
⢠Chest XRAY
⢠12 lead ECG
Therapeutics:
⢠IVF: (20cc/kg)PNSS
⢠(100) Ceftriaxone
⢠(1) Omeprazole
⢠(10mcg/kg/min) Dopamine
***IFC inserted
46. Rapid, deep breathing with sudden
severe epigastric pain
CARDIOVASCULAR
RESPIRATORY
SEPSIS
ENDOCRINE
INFECTIOUS
NON INFECTIOUS
DIABETIC KETOACDIDOSIS
HYPEROSMOLAR HYPERGLYCEMIC STATE
47. Rapid, deep breathing with sudden
severe epigastric pain
CARDIOVASCULAR
RESPIRATORY
SEPSIS
ENDOCRINE
INFECTIOUS
NON INFECTIOUS
DIABETIC KETOACDIDOSIS
HYPEROSMOLAR HYPERGLYCEMIC STATE
48. Rapid, deep breathing with sudden
severe epigastric pain
RESPIRATORY
INFECTIOUS NON INFECTIOUS
ASTHMA
PNEUMONIA
52. Rapid, deep breathing with sudden
severe epigastric pain
ENDOCRINE
DIABETIC KETOACIDOSIS
HYPEROSMOLAR HYPERGLYCEMIC
STATE
53. DIABETIC KETOACIDOSIS
HYPEROSMOLAR
HYPERGLYCEMIC STATE
ONSET HOURS DAYS to WEEKS
AGE Younger age ADULTS
SYMPTOMS
Polyuria
Polydipsia
Dehydration
Abdominal pain
KUSSMAULâS breathing
(deep, rapid breathing)
Polyuria
Polydipsia
Dehydration
Severe form HYPOVOLEMIA HYPOVOLEMIA
54. HYPEROSMOLAR HYPERGLYCEMIC
SYNDROME
Plasma glucose >600
Arterial pH >7.30
Bicarbonate >18
Urine Ketones None-small
Anion Gap Variable (Normal)
Mental Status Stupor/coma
PATIENT G.B.
LABORATORY AND DIAGNOSTIC FINDINGS:
ďź Metabolic Acidosis
CHO3: 3.5
pH:6.98
ďź Initial CBG: HIGH
55. Rapid, deep breathing with sudden
severe epigastric pain
ENDOCRINE
DIABETIC KETOACDIDOSIS
HYPEROSMOLAR HYPERGLYCEMIC
STATE
56. DIABETIC KETOACIDOSIS
ONSET HOURS
AGE Younger age
SYMPTOMS
Polyuria
Polydipsia
Dehydration
Abdominal pain
KUSSMAULâS breathing
(deep, rapid breathing)
Severe form
HYPOVOLEMIA
57. Abdominal pain is a frequent DKA manifestation (present in 40-75%
of the cases) and its prevalence increases as arterial pH and serum
bicarbonate levels decline. Many mechanisms have been suggested
to underlie the abdominal symptoms in DKA, namely acute
hyperglycemia mediated impaired gastrointestinal motility
(esophageal, gastric and gallbladder), rapid expansion of the
hepatic capsule, and mesenteric ischemia precipitated by volume
depletion.
58. Mild DKA Mod. DKA Severe DKA
Plasma glucose >250 >250 >250
Arterial pH 7.25-7.30 7.00-7.24 <7.00
Serum Bicarb 15-18 10 to <15 <10
Urine Ketones Positive Positive Positive
Anion Gap >10 >12 >12
Mental Status Alert Alert/Drowsy Stupor/coma
PATIENT G.B.
LABORATORY AND DIAGNOSTIC
FINDINGS:
ďź Metabolic Acidosis
CHO3: 3.5
pH:6.98
ďź Initial CBG: HIGH
59. In several studies, leukocytosis in DKA has been attributed to various factors. many of researcher
believed lack of insulin can stimulate production of Neutrophil in bone marrow (13) , upon
insulin administration and fluid therapy induced to decrease leukocyte count. Furthermore,
secretion of adrenaline, cortisol and inflammatory mediators can lead to increase of
leukocytes amount.
In general, leukocytosis in DKA can linked to different factors such as infections, insulin
deficiency, dehydration and stress hormones.
LABORATORY FINDINGS:
ďź Leukocytosis
with neutrophilic predominance
64. TETRALOGY OF FALLOT
- conotruncal family of heart lesions - primary
defect is an anterior deviation of the infundibular
septum
65. Nelsonâs Textbook of Pediatrics 21st edition
4 Components
1. obstruction to right ventricular (RV) outflow (pulmonary
stenosis)
2. a malalignment type of ventricular septal defect (VSD)
3. dextroposition of the aorta so that it overrides the ventricular
septum
4. right ventricular hypertrophy
67. Pathophysiology
Physiologically the pulmonary stenosis causes
concentric right ventricular hypertrophy without
cardiac enlargement and an increase in right
ventricular pressure.
When the right ventricular pressure is as high as the
left ventricular or the aortic pressure, a right to left
shunt appears to decompress the right ventricle.
68. Once the right and left ventricular become identical,
increasing severity of pulmonary stenosis reduces
the flow of blood into the pulmonary artery and
increases the right to left shunt.
As the systolic pressures between two ventricle are
identical there is little or no left to right shunt and
the VSD is silent.
69. The flow from the right ventricle into the pulmonary
artery occurs across the pulmonary stenosis
producing an ejection systolic murmur
More severe the pulmonary stenosis, the less the
flow into the pulmonary artery and the bigger the
right to left shunt, more the cyanosis
70. Thus the severity of cyanosis is directly proportional to
the severity of pulmonary stenosis
The VSD of TOF is always large enough to allow free
exit to the right to left shunt
Thus Congestive failure not occurs in TOF.
71. CLINICAL MANIFESTATIONS
Clinical findings are variable & mainly depends on
the degree of right ventricular outflow obstruction.
ďąCyanosis :
ď§ Patient with mild obstruction are minimally cyanotic
or even acyanotic
ď§ Those with maximal obstruction are deeply cyanosed
since birth
ď§ Most have progressive cyanosis by 4 months of age
72. ďą Paroxysmal Hypercyanotic Attacks :
This is the hallmark of sever TOF & usually occurs
during first 2 years of life, most commonly by 4-6
months of age. Spells occur most frequently in the
morning on awakening or after episodes of vigorous
cry. The attacks are due to further reduction of an
already compromized pulmonary blood flow & more
severe systemic hypoxia.
73. Cyanotic Spells are characterized by
ď§ Sudden onset of dyspnoea. Sometimes gasping
respiration & syncope
ď§ Sudden deepening of cyanosis
ď§ Alteration in consciousness, from irritability to
syncope.
ď§ Sometimes convulsion & hemiparesis
ď§ Temporary disappearance or decrease in the intensity
of the systolic murmur at pulmonary area
ď§ Metabolic acidosis
74.
75. ďąEasy fatigability & dysponea on exertion
ďąSquatting is of diagnostic significance & is highly
typical of infants with TOF
ďąFailure to thrive
Squatting
position
76. Physical Examination
⢠Varying degrees of cyanosis
⢠Conjunctiva are congested
⢠Clubbing of the fingers and toes occurs after 3
months of age and is proportional to the level of
cyanosis.
⢠Stunted growth
⢠Precordium may be bulged due to right ventricular
hypertrophy.
78. ⢠Apex beat is tapping in character, but not shifted
⢠A systolic thrill may be palpable at the left sternal
border, with a harsh midsystolic murmur in that
location.
⢠The shorter the murmur, the more severe the
infundibular pulmonary stenosis.
⢠Left parasternal heave may be present
⢠The first heart sound (S1) is usually normal
79. ⢠Second heart sound is characteristically single & loud,
because the pulmonary component is too soft to be
heard.
⢠An ejection systolic murmur is heard at pulmonary
area
⢠A continuous murmur is heard if a PDA or large
collateral vessels are present.
80. ⢠Cardiac findings in cyanotic tetralogy of Fallot:
A long ejection systolic murmur at the upper and mid
left sternal border and a loud, single S2 are
characteristic auscultatory findings of TOF.
82. Investigations
ďąComplete blood counts :
⢠Haemoglobin & haematocrit values are usually
elevated which is proportional to the degree of
cyanosis
⢠PBS shows microcytic hypochromic anaemia
83. ďąChest X-ray
ďźBoot shaped heart (due to ventricular
hypertrophy). It means apex is lifted up.
ďźConcavity in the region of pulmonary artery
ďźRight sided aortic arch.
ďźOligaemic lung fields.
ďź Hilar vessels are few,
ďź Lung vessels also few.
86. ⢠Echocardiography
Provides information about the extent of aortic
override of the septum
Location and degree of the right ventricular outflow
tract obstruction
Size of the pulmonary valve annulus and main and
proximal branch pulmonary arteries and the side of
the aortic arch.
87. ⢠PLAX view showing the overriding aorta and a large
subaortic VSD (star).
⢠RVH is also present
89. ďąCardiac catheterization
ďźTo assess the anatomy of RVOT and main PA
branches, RV and LV function, site and size of VSD
and competence of aortic valve.
ďźIdentical systolic pressure in RV and LV and low
systolic pressure in PA (diagnostic of TOF)
90. ďą Aortography or coronary arteriography
outlines the course of the coronary arteries. In 5-10%
of patients with the tetralogy of Fallot, coronary artery
abnormalities may be present
91. COMPLICATIONS
⢠Hypercyanotic Spells
⢠Sever polycythaemia
⢠Cerebral thrombo-embolism & stroke: usually
occurring in the cerebral veins or dural sinuses and
occasionally in the cerebral arteries. Thromboses
occur most often in patients younger than 2 years.
92. ⢠Brain abscess : As deoxygenated blood enters the
systemic circulation & brain , bypassing lungs without
clearing the germs by pulmonary scavenger cells.
Patients with a brain abscess are usually older than 2
years. The onset of the illness is often insidious and
consists of low-grade fever or a gradual change in
behavior, or both.
93. Some patients have an acute onset of symptoms that
may develop after a recent history of headache,
nausea, and vomiting. Seizures may occur; localized
neurologic signs depend on the site and size of the
abscess and the presence of increased intracranial
pressure. CT or MRI confirms the diagnosis. Antibiotic
therapy may help keep the infection localized, but
surgical drainage of the abscess is usually necessary.
94. ⢠Bacterial endocarditis may occur in the right
ventricular infundibulum or on the pulmonic, aortic,
or, rarely, tricuspid valves. Endocarditis may
complicate palliative shunts or, in patients with
corrective surgery, any residual pulmonic stenosis or
VSD.
⢠Delayed growth, development & puberty
⢠Others : Hyperuricemia & gout
Relative IDA
Bleeding disorders
95. ASSOCIATED ANOMALIES
Tetralogy of Fallot may present with other anatomical
anomalies, including:
⢠Stenosis of the left pulmonary artery, in 40%
⢠A bicuspid pulmonary valve, in 60%
⢠Right-sided aortic arch, in 25%
⢠Coronary artery anomalies, in 10%
⢠PDA may be present
⢠Congenital absence of the pulmonary valve
96. ⢠Absence of a branch pulmonary artery
⢠A patent foramen ovale or atrial septal defect, in
which case the syndrome is sometimes called a
pentalogy of Fallot
⢠An atrioventricular septal defect partially or
totally anomalous pulmonary venous return.
97. Treatment
Counsel the parents about disease, treatment option
& prognosis
Treatment options :
⢠Medical
⢠Surgical
98. Medical
⢠Neonate with severe cyanosis is treated with IV
infusion of prostaglandin E1(0.05 - .1 mcgm/kg/min)
to keep the ductus arteriosus patent & thereby to
improve pulmonary circulation and is life saving.
99. ⢠Treatment of cyanotic spells ( in hospital)
ďźPlace the infant in a knee chest position
ďźGive O2 3-5 L/min
ďźEstablish a calm environment by isolating the
patient
100. If the spell persists , give the following :
ďź Intravenous fluids : 10ml/kg bolus normal saline
followed by maintenance fluids
ďź Morphine : 0.1 -0.2 mg/kg SC for keeping the child
calm and for muscle relaxation
ďź NaHCO3 : 1 mEq/kg IV to correct acidosis
ďź Propranolol : 0.1 mg/kg IV which relaxes the
infundibular muscle and thereby reduce spasm
101. If these measures do not control the spell, then
arrange to transfer the child to ICU
ďź Phenylephrine :10-20mcgm/kg bolus IM or SC
followed by 0.1-0.5mcgm/kg/min IV infusion titrated
according to heart rate and blood pressure.
Phenylephrine raise systemic BP as well as systemic
vascular resistance. This will reduce right to left shunt
and ultimately promote pulmonary blood flow.
102. ⢠If the preceding steps do not relieve the spell or if the
infant is rapidly deteriorating , intubation and
ventilatory support should be given.
103. ⢠Treatment at home
ďźEducate parents to recognize the spells and know
what to do.
ďźPropranolol : 0.25-1mg/kg/day orally to be continued
to prevent cyanotic spell
ďźFluid & Nutrition
⢠Provide high calorie diets to ensure growth
⢠Supplement Iron 3-6 mg/kg/day elemental iron orally
to promote maturation of RBC
⢠Supplement vitamins & minerals
104. ⢠Counsel parents to pay special attention to fluid
intake so as to prevent dehydration.
⢠Dehydration of any child with TOF should be referred
immediately for prompt rehydration.
105. Surgical
⢠Total surgical repair
Total surgical repair of TOF is a curative surgery.
Different techniques can be used in performing TOF
repair. However, a transatrial, transpulmonary artery
approach is used for most cases. The repair consists of
two main steps: closure of the VSD with a patch and
reconstruction of the right ventricular outflow tract.
106. Diagrammatic representation of surgical repair of tetralogy of Fallot.
1. Patch closure of a VSD
2. Right ventricular outflow/main pulmonary artery outflow
patch (transannular patch)
107. ⢠This open-heart surgery is designed to relieve the right
ventricular outflow tract stenosis by careful resection of
muscle and to repair the VSD. Additional reconstructive
surgery may be done on patients as required by their
particular cardiac anatomy.
108. ⢠Timing of surgery in asymptomatic patients is usually
between the ages of 2 months to one year. However,
in symptomatic patients showing worsening blood
oxygen levels, severe tet-spells (cyanotic spells), or
dependence on prostaglandins from early neonatal
period (to keep the ductus arteriosus open) need to
be planned fairly urgently.
109. ⢠Surgery is now often carried out in infants one year
of age or younger with less than 5% perioperative
mortality. Post surgery, most patients enjoy an active
life free of symptoms. Currently, long term survival is
close to 90%.
110. Palliative surgery
⢠Classic Blalock-Taussing shunt: anastomosed between
the subclavian artery and the ipsilateral PA, is usually
performed for infants older than 3 months because
the shunt is often thrombosed in young infants.
Classic Blalock-
Taussing shunt
111. ⢠Modified Blalock-Taussing shunt : a Gore-Tex
interposition shunt is placed between the subclavian
artery and the ipsilateral PA.
This is the most popular procedure for any age,
especially for infants younger than 3 months of age.
Modified Blalock-
Taussing shunt
112. ⢠The Waterston shunt : anastomosis between the
ascending aorta and the right PA, is no longer
performed because of a high incidence of surgical
complications.
⢠The Potts operation : anastomosed between the
descending aorta and the left PA, is no longer
performed either.
It may result in heart failure or pulmonary
hypertension, as in the Waterston operation.
113.
114. PROGNOSIS
After successful total correction, patients are
generally asymptomatic and are able to lead
unrestricted lives. If left untreated, TOF carries a 35%
mortality rate in the first year of life, and a 50%
mortality rate in the first three years of life. Patients
with untreated TOF rarely progress to adulthood.
115. Notable cases
⢠Shaun White, American professional snowboarder and musician.
⢠Beau Casson , Australian cricketer
⢠Dennis McEldowney, New Zealand author and publisher
116. Take home message
⢠Tetralogy of Fallot is the most common form of
cyanotic congenital heart disease, and one of the first
to be successfully repaired by congenital heart
surgeons.
⢠The care of children with tetralogy of Fallot and their
transition to adult life has been a success of modern
medicine.
⢠Most of them now survive early repair and have an
essentially normal childhood.
117.
118. DIAGNOSIS
⢠CHEST X-RAY:
⢠Boot-shaped heart
⢠hilar areas and lung fields are relatively clear because of diminished
pulmonary blood flow
⢠Aorta is large
⢠ECG:
⢠right axis deviation and RVH
⢠dominant R wave, RSRⲠpattern: right precordial chest leads (V1, V2)
⢠RVH: a positive T wave in leads V3R and V1
⢠RAE: P wave may be tall and peaked
119. DIAGNOSIS
⢠ECG:
⢠right axis deviation and RVH
⢠dominant R wave, RSRⲠpattern: right precordial chest leads (V1, V2)
⢠RVH: a positive T wave in leads V3R and V1
⢠RAE: P wave may be tall and peaked
120. DIAGNOSIS
⢠2D ECHO W/ DOPPLER:
⢠extent of aortic override of the septum
⢠location and degree of the RVOT obstruction
⢠size of the pulmonary valve annulus
⢠main and proximal branch pulmonary arteries
⢠side of the aortic arch
⢠obviates the need for catheterization before surgical repair