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