C.CHDBy:

Abdullah Taskin Abdulabri

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Non‐cyanotic CHD (L R)

• Atrial septal defects (ASD)

• Ventricular septal defects (VSD)

• Patent ductus arteriosus (PDA)

• Obstruction to blood flow

• Pulmonic stenosis (PS)

• Aortic stenosis (AS)

• Aortic coarctation

Cyanotic CHD (R L)

• Tetralogy of Fallot (TOF)

• Transposition of the great vessels

• Tricuspid atresia (TA)

• Total anomalous pulmonary venous return (TAPVR)

• Truncus arteriosus

• Hypoplastic left heart syndrome

• Pulmonary atresia (PA)

• Double outlet right ventricleIntr

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cyan

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s• Cyanosis is a bluish or purplish tinge to the skin and mucous

membranes.

• Peripheral cyanosis (blueness of the hands and feet) .

• Central cyanosis, seen on the tongue as a slate blue colour, isassociated with a fall in arterial blood oxygen tension.

• It can only be recognized clinically if the concentration of reduced haemoglobin in the blood exceeds 5 g/dl.

• Persistent cyanosis in infant is nearly always CHD .

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cyan

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cyan

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sHyperoxia (nitrogen washout) test• cardiac VS respiratory causes of cyanosis.

• Infant placed in 100% oxygen for ten minutes.

• It works on the assumption that if there is right to left shunting in cyanotic heart disease, no amount of oxygenation in the pulmonary circulation will alter the desaturation effect of the shunt.

• However, if there is a pulmonary defect causing cyanosis this may be corrected by increasing the inspired oxygen.

cyan

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sHyperoxia (nitrogen washout) test• If the infant remains cyanotic after this period, the cyanosis

is said to be secondary to cyanotic heart disease.

• This can be defined in blood gases as follows:

TOF

•Most common cause of C.CHD .

• Characterized by :

Problem Of Small Heart

TOF

TOF

• Key problem : RV outflow obstruction

• VR > RH > LH > body , without going to lung !

• Degree of PS > severity of disease .

• Presentation : •Murmur in 1st 2 month of life . ( inc. severity > shorter)• Cyanosis & softer • Typically : ( Single S.2 + RV impulses at Left sternal border )

TOF

• Key problem : RV outflow obstruction

• VR > RH > LH > body , without going to lung !

• Degree of PS > severity of disease .

• Presentation : •Murmur in 1st 2 month of life . ( inc. severity > shorter)• Cyanosis & softer • Typically : ( Single S.2 + RV impulses at Left sternal border )

• Investigation : • ECG : R‐axis deviation + RV hypertrophy • CX‐ray : boot shaped heart & ABG • Echo : PS , coronary A > LAD arise from R‐coronary A( 5%)

TOF

TOF

Tet spells• hypercyanotic episodes precipitated by a sudden increase

in righ‐to‐left shunting of blood.

• lf sever > loss of murmur , unconsciousness , convulsion , hemiparesis & death .

• They can be elicited by activity (e.g. eeding, crying), or theymay occur without warning.

• The classic description is of a patient who becomes cyanotic and then assumes a squatting position to relieve thecyanosis and hypoxia.

TOF

• Complication : • cerebral thromboembolism & cerebral abscess

• Treatment : • Initially is medical • Definitive surgical repair at 5 month . • If sever cyanosed > artificial tube btw subclavian art. & PA

or balloon dilatation of RV outflow .• Spells : mostly self limiting & followed by period of sleep• If > 15 min , need treatment :

TOF

• If > 15 min , need treatment :

1. sedation and pain relief ( morphine ) 2. IV propranolol or a‐adreno‐receptor agonist 3. IV volume 4. Bicarbonate for acidosis 5. Muscle paralysis 6. Ventilation

TGA

• Transposition of the Great Arteries (TGA)

• the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, creating two parallel circuits.

• body to body circulation without lung .

• Lung to lung circulation without body .

• Fortunately : naturally ASD , VSD & PDA or therputicshort term .

TGA

TGA

• Clinically : • cyanosis “ductal closure” • S2 loud and single • Usually No murmur “but may +”

• Investigation : • CX‐ray : classic CXR presentation features a heart with an “egg on a string” appearance• ECG : normal • Echo : essential for Dx .

TGA

TGA

•Management :

• in sever key is > mixing ( ASD , VSD , PDA )

•Maintain PDA by prostaglandin

• Balloon atrial septostomy

• Surgery of arteries switching in neonate period

• Coronary .A transferred to new aorta .

TGA

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Ref

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s : • Nelson essential 7th E.

•Illustrated pediatric 4E