FALLOT’S TETROLOGY
Transcript of FALLOT’S TETROLOGY
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FALLOTS TETROLOGY
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EMBRYOLOGY:
The HEART develops from splanchnopleuricmesoderm related to the part of intraembryonic
coelom that forms the pericardial cavity. Right and Left endothelial heart tubes fuse to
form one tube.
Series of dilatations form: a) Bulbus cordis, b)
Ventricle, c) Atrium, d) Sinus venosus.
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Bulbus cordis has conus(proximal)
truncus arteriosus(distal)
Sinus venosus- right & left horns.
Atria- Septum primum & Septum secundum
dividing it into right & left atrium.
Foramen ovale is present between both
septa.
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The conus and the primitive ventricle unite
to form one chamber which is divided into
right & left ventricles by: 1) Interventricularseptum, 2) Bulbar septum,
3) Atrioventricular cushions.
The ascending aorta & pulmonary trunkare formed from Truncus arteriosus.
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Truncus arteriosus is continuous with
aortic sac. This sac has right & left horns.
The Arch of Aorta is formed by Aorticsac(left horn) and left 4th arch artery.
Pulmonary artery is derived from 6th arch
artery.
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CONGENITAL HEART DISEASE
Group-I: Left to right shunts.
Group-II: Right to left shunts.
Group-III: Obstructive(Shunts) lesions.
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Group-I(Left to right shunts)
Symptoms & Signs:
Frequent Chest Infections
Soft rib cage + Cardiac dilatation= Precordial bulge.No Cyanosis.
Increased sweating.
On palpation- Hyperkinetic precordium.
Auscultation- Tricuspid or Mitral delayed diastolicmurmur.
X-ray- Plethoric lung fields, Cardiomegaly.
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Group-II(Right to left shunts)
Cyanosis- Present. Polycythemia,Clubbing.
Cyanotic Patients:1. Decreased P.A.P= Reduced P.B.P
due to PS.
2. Increased P.A.P= P.A.H> P.B.FP.B.F.
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Patients may have a mixed picture:
Cyanosis, Cardiomegaly, CCF,
Sweating, Absence of weight gain,Failure to thrive & Plethoric lung fields.
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Group III-Obstructive Lesions
Absence of frequent chest infections & cyanosis.
Absence of precordial bulge.
Presence of forcible or heaving cardiac impulse( dueto concentric hypertrophy of ventricles).
Systolic thrill associated with ejection systolicmurmur.
Absence of tricuspid & mitral delayed diastolicmurmur.
CXR- Normal sized heart & Pulmonary vasculature.
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Left to right shunt:
1.Atrial septal defect.
2. Ventricular septal defect.
3. Patent ductus arteriosus.
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Right to left shunts:
Tetrology of Fallot.
Tricuspid Atresia.
Ebsteins Anomaly.
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Tetrology of Fallot:
4 Anatomical defects
Ventricular Septal Defect.
Pulmonic Stenosis.
Overriding or Dextroposed Aorta.
Right Ventricular Hypertrophy.
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Hemodynamics: Pulmonic stenosis causes > Concentric
Right Ventricular Hypertrophy(withoutcardiac enlargement) & increase in RightVentricular Pressure.
When RV pressure increases as much asLV pressure then > right to left shuntoccurs & right ventricle decompresses.
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Once right & left pressure equalizes, PS
will reduce flow of blood in Pulmonary
artery. Thus, increased pressure in rightventricle causes increased Right to Left
shunt.
Flow from the Right Ventricle toPulmonary Artery occurs across stenosis.
This causes Ejection Systolic Murmur.
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Clinically, the patient may becomesymptomatic anytime after birth.
Neonates & Infants get Anoxic spells(i.e.paroxysmal attacks ofdyspnoea).Commonest symptoms beingDyspnoea on exertion & Exerciseintolerance. Very commonly, patientassumes sitting- squatting position.
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Anoxic Spells:
The child starts crying > becomes
dyspnoeic > becomes blue > may looseconsciousness > convulsions may occur.
Frequency- once every few days OR
numerous attacks /day. Each spell is Life Threatening!!
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GENERAL EXAMINATION:
There is NO Cyanosis, Clubbing,
Prominent a wave in JVP, Mitral
parasternal heave, Lymphadenopathy.
Pallor Present.
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ECHOCARDIOGRAPHY-
Shows a Large Over riding Aorta.
Right Ventricular Hypertrophy.
Pulmonary Stenosis.
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Neurological complications- Anoxic infarction of C.N.S causes
Hemiplegia.Paradoxical Embolism toC.N.S & Venous Thrombosis also maycause hemiplegia.( Due to slowcirculation because of polycythemia).
Brain Abscess- May cause headache,convulsions, vomiting(with or withoutfever) & neurological deficit.
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TREATMENT
1. Medical-
Management of complications & correction
of anaemia.
2.Surgical-
a) Palliative
b) Definitive
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a) Palliative treatment- (It prolongs life &
increases exercise tolerance).
Anastomosing a Systemic Artery withPulmonary Artery to increase the Pulmonary
blood flow & thus, increasing the oxygenated
blood reaching the Systemic circulation.
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The 3 Systemic anastomosis are:
Blalock-Taussig Shunt > Subclavian
Artery-Pulmonary Artery anastomosis. Potts Shunt > Descending Aorta-
Pulmonary Artery.(NOT done nowadays).
Waterstons Shunt > Ascending Aorta-Right Pulmonary Artery.
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b) Definitive Treatment-
This includes > Closing Ventricular Septal
Defect & resecting the InfundibularObstruction. It is done under
Cardiopulmonary Bypass.
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VARIANTS OF TETROLOGY
5% of cases Left Anterior Descending Artery hasan anomalous origin from Right Coronary Arteryor from the Right Sinus of Valsalva.
ASD or Patent Foramen Ovale occurs in 25% ofcases(Pentologyof Fallot).
Aortic Incompetence, secondary to Bicuspid Aorticvalve, Infective Endocarditis or Aortic leaflet
prolapse.
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ANAESTHETIC IMPLICATIONS
Preoperative Preparation- It is very important to avoid
dehydration.Crying associated with IMinjection can lead to hypercyanoticattacks. So, it is better not to use drug bythis route.
-Adrenergic antagonists should becontinued untill the induction of anaesthesia.
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Induction- Inj.Ketamine 3 to 4mg/kg IM or 1 to
2mg/kg IV.( It causes increase in thePulmonary blood flow due to Ketamine-induced increases in systemic vascularresistance, which reduces the Right to Left
shunt. Induction with a volatile anestheticlike Sevoflurane or Halothane is preferredthough it has to be used cautiously.
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Maintenance of Anaesthesia-
This is done with Nitrous oxide & Ketamine.(Advantage-This combination maintainsthe Systemic vascular resistance.Disadvantage-Nitrous causes associated
decrease in inspired oxygen conc & itshould be given only 50%). Use of Opioids& Benzodiazepines should be minimizedto prevent decrease in systemic blood
pressure & vascular resistance.
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CASE PRESENTATION:
A 3 years old male child , a resident ofNalgonda came to the hospital on 25.5.05
withChief complaint of Increasedfrequency of Bluish discoloration of bodyafter crying- since 1 month.
Informant- Mother.
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H/O PRESENT ILLNESS
C/O Bluish discoloration of skin since birthespecially after an episode of crying. Thefrequency of attacks have increased in the
past 1 month. The episode was associated with
respiratory discomfort after crying; whichwas relieved at rest.
The episodes getting more frequenttroubled the mother who got the child tohospital.
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PAST HISTORY
Similar H/O bluish discoloration of body
was seen after a bout of crying once or
twice a month for which no medical helpwas taken.
No H/O Fever, Vomiting, Convulsions,
Asthma, Jaundice, Unconsciousness,Painabdomen, oedema over feet or face,joint
pains, hemoptysis, squatting episodes.
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BIRTH HISTORY
Child had a non-institutional birth.He was
delivered by a midwife at his residence.
He was full term, had a normal delivery &cried immediately after birth.
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IMMUNIZATION HISTORY
The child is well immunized.
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MILESTONES
The child attained appropriate
milestones for his age.
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FAMILY HISTORY
One healthy 5 years old female child with
no similar complaints.
No other significant family history.
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GENERAL PHYSICAL
EXAMINATION
A thinly built and moderately nourished
active male child weighing 8 kgs.
Eyes ~ Conjunctiva- pale; Sclera-Clear. No Cyanosis, Clubbing, Icterus,pedal
edema, Lymphadenopathy or Engorged
veins. Skin & Hair Normal.
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CARDIOVASCULAR SYSTEM
Inspection-
Shape of the chest normal.
Apical impulse seen in 5th intercostal spacejust inside the left midclavicular line.
Pulsation seen in Left Parasternal region.
No precordial bulge.
No visible veins, scars, sinuses.
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Palpation-
Inspectory findings confirmed.
Apex beat palpable in left 5th intercostalspace just inside the midclavicular line.
Systolic Thrill Palpable in left parasternal
region.
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Percussion-
Nothing Significant.
Auscultation-
S1 & S2 heard.
Ejection Systolic Murmur Heard.
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Palpation- Inspectory findings confirmed.
Apical impulse palpable in Left 5th
Intercostal space just inside themidclavicular line.
B/L Chest movements Equal.
Trachea Central.
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Auscultation-
B/L Air entry equal.
B/L Vesicular breath sounds heard.
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CENTRAL NERVOUS SYSTEM
Higher functions Normal.Child is active
and follows verbal command.
Cranial Nerves No abnormality detected. Motor System No Wasting. Power=Gr 5.
Sensory System Superficial, Deep &
Cortical sensations Normal.
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PER ABDOMEN
Inspection
Shape of abdomen Normal.
Centrally situated inverted Umbilicus. Abdomino-thoracic respiratory movement.
No visible pulsations or dilated veins over
the abdomen.
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Palpation
Abdomen Soft, non-tender, No guarding
or rigidity. Liver and Spleen- Just palpable.
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Percussion-
No significant finding on percussion.
Auscultation
Peristalsis Present ; Normal.
No Bruit or Venous hum.
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Provisional Diagnosis:
Congenital heart disease with right to left
shunt(cyanosis). Not in failure, No
Infective Endocarditis.?? Fallots Tetrology.
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THANK YOU