Congenital Cyanotic Heart Disease & TOF - Dr. D. Gunasekaran

Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI

Cyanotic Congenital Heart Disease – Tetrology of FallotCYANOTIC CONGENITAL HEART DISEASE

Dr. D. GunasekaranConsultant Paediatrician


Cyanotic Congenital Heart Disease – Tetrology of Fallot

What is the criteria used to identify CongenitalHeart diseases?



NADAS CRITERIA

MAJOR CRITERIA MINOR CRITERIA

SYSTOLIC MURMUR Grade 3 OR MORE IN INTENSITY

SYS. MURMUR Grade 2 or less IN INTENSITY

DIASTOLIC MURMUR Abnormal SECOND SOUND

CYANOSIS Abnormal ECG

CHF Abnormal CxR

Abnormal BP

DIAGNOSIS OF CHD



How do we broadly classify congenital Heartdiseases?

How do you further classify Cyanotic congenital heart diseases?



Congenital heart disease

Cyanotic CHD Acyanotic CHD

PBF PBFVolume Pressure

PSASCoA

ASDVSDAVSDPDA

TOF

Pulmonary atresia

Tricuspid atresia

Ebstein anomaly

TGA

TAPVC

Truncus arteriosus

Single ventricle



What is the most common CCHD?

a) TOFb) Truncus arteriosusc) TGAd) TAPVC



TOF



TOF

What are the four components of TOF?

1. RV Outlet obstruction

2. Non restrictive VSD

3. Right ventricular hypertrophy

4. Right sided aortic arch (25%)



Blood from SVC & IVC

Major amount goes toLV (No murmur)

Oxygenated bloodIn Pulmonary veins

Small amount Goes to PA (turbulent blood flow ESM)P2 soft or absent)

Aorta

Systemic circulation

Right atrium

Right ventricle

Oxygenated blood In Left atrium



What is the most common CCHD, manifesting after 2 months of life?

a) TOFb) Truncus arteriosusc) TGAd) TAPVC



What is the usual time of appearance of cyanosis in TOF?

a) At birth

b) 8-12 weeks

c) 5-6 months

d) 1 year

What are the reasons?


Cyanotic Congenital Heart Disease – Tetrology of FallotWhat is the usual time of appearance of cyanosis in TOF?

8-12 weeks ; Reasons:

1)Foetal Hb starts declining after birth

2)Obstruction to PBF starts increasing after birth

3)O2 req. increases as child grows (activity increases)

4)Ductus closure is delayed.

FOOD



What are the symptoms of TOF?

Bluish extremitiesExertional breathlessnessExercise intoleranceFeeding difficultiesFailure to thrive

But, the most common symptom isCyanotic spell (often with squatting)



Cyanotic spell

What is the cyanotic spell?: Exaggeration of existing cyanosis with or without loss of consciousness and /or seizures.

Usual time of appearance: 2-6 months of age



Sequence of events during a spell

Gotting up from sleep

Strains

Becomes more cyanosed & more breathless


Cyanotic Congenital Heart Disease – Tetrology of FallotPathophysiology during SpellGot up from sleep (vulnerable resp. centre)

Strain (Sympathetic over activity & excess Catecholamine release)

Ionotropic & Chronotropic & Spasm of Infundibulum (RVO part)

More blood pumped out of heart & less blood going in to pulmonary system

Increased venous return & increased RL shunt

More cyanosis

Increased hypoxia & acidosis

More Respiratory Centre stimulation

Hyperpnoea, which again stimutes Resp. centreHyperpnoea which again stimulates respiratory centre



SquattingWhat is squatting?Child who gets spells repeatedly, slowly learns to squat - sits as if

he / she is sitting on the Indian toilet;

By squatting, cyanosis decreases. How?

1. Compression of major veins in LL de-oxygenated blood returning to the heart decreases

2. Compression of major arteries in LL Systemic vascular resistance increases Pulmonary blood flow increases

3. As a result of 1 & 2, Cyanosis decreases



Squatting

What are the Squatting equivalents?

Sitting with legs drawn underneath

Legs crossed while standing / sitting



Vitals in TOF

Pulse: Volume? Rate? Character? Rhythm?

Blood pressure?



General examinationUndernourished (M, K & Vit def)

Suffused conjunctivaCentral cyanosisClubbing

Always look for dental hygiene – Why?



Examination of heart

Inspection:

No Precordial bulge




Palpation: Mild para sternal heave (due to RVH) No thrills




Auscultation: Heart sounds: S2 usually single (P2 soft or absent, depending on severity of PS)

Ejection click may be + (if prominent aortic root dilatation is +)

Murmurs: ESM – in the Pulmonary area(VSD remains silent)



What are the possible Complications for any child with Heart disease?

FTT

Infective Endocarditis

CCF

PHT



ComplicationsSpell (some times, with hypoxic seizures)

Failure to thriveCerebral thrombosis (<2 years of age)

Cerebral abscess (>2 years of age)

Infective Endocarditis – rare

CCF – Never occurs unless there are complications like anemia or Infective endocarditis

PHT- Never occurs



Investigations

What are the three basic investigations

necessary in any child with suspected

congenital heart disease?



Investigations

CXR: NO CUP



Investigations

ECG Chamber hypertrophy

ECHO Anatomical diagnosis



Management

Medical: Anemia prophylaxis & correction Proper nutrition Dental Hygiene Infective Endocarditis Prophylaxis Management of spell & Prevention of spell



Management of spell –

1.Knee-chest position2. Oxygen3. Morphine 0.1-0.2 mg/kg (reduces anxiety, releases infundibular spasm)

4. Sodium bicarbonate 7.5% soln. (1ml/kg + equal amount of D.W) given slow IV

5. IV fluids

Prevention:Propanolol (1mg/kg/day)

KOMSIP



Management - Surgical

Good anatomy (of Pulmonary system and LA+): Complete correction (4-6 months of age)

Poor anatomy: Blalock-Taussig shunt: Subclavian artery PA (Increases the PBF oxygenation improves)Total repair done at 2 years of age



Different types of TOF

Fallot’s Triology----- PS+RVH+PFO

Pentalogy of TOF ----- TOF + ASD

Pink TOF (Acyanotic TOF) ---------- VSD + PS



Points to remember

What is the most common CCHD?It usually presents ----age with ---------------Children with TOF learn to ----- during spellsCardiomegaly?Mild parasternal heave, single S2 & ESM at PACXR: NO CUPMedical: Anemia, dental care, food, IE prophylaxis & KOMSIP

Surgery at 4-6 months, if anatomy is goodIf anatomy is poor, BT shunt; surgery 2 yrs latter

Congenital Cyanotic Heart Disease & TOF - Dr. D. Gunasekaran

Healthcare

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