Cyanotic heart diseaseCyanotic heart disease

Oleagamic lung Oleagamic lung

tetralogy of fallottetralogy of fallot

tricuspid atresiatricuspid atresia

pulmonary atresiapulmonary atresia

Plethoric lung Plethoric lung

transposition of transposition of great vesselsgreat vessels

total anomelies total anomelies pulmonary venous pulmonary venous returnreturn

double outlet RT Vdouble outlet RT V

CYANOTIC HEART CYANOTIC HEART DISEASEDISEASE

FALLOT TETROLOGYFALLOT TETROLOGY

CHARACTARISED BY ;CHARACTARISED BY ;

1-obstruction to RT V out flow –pul 1-obstruction to RT V out flow –pul stenosis stenosis

2-ventricular septal defect 2-ventricular septal defect

3-Dextroposition of aorta with septal 3-Dextroposition of aorta with septal overriding overriding

4-RTv hypertrophy 4-RTv hypertrophy

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

Pulmonary valve annulus varies in size Pulmonary valve annulus varies in size .the valve is bicuspid . The valve is .the valve is bicuspid . The valve is stenotic+hypertrophy of sub pulmonary stenotic+hypertrophy of sub pulmonary muscles this stenosis may extend to the muscles this stenosis may extend to the branches or complete pul atresia (extreme branches or complete pul atresia (extreme Fallot)Fallot)

VSD nonrestrictive - large- located just VSD nonrestrictive - large- located just below aortic valve below aortic valve

Aorta with normal continuity to Aorta with normal continuity to mitral valve with large root mitral valve with large root

Rt side aortic arch in 20% of pt Rt side aortic arch in 20% of pt

If more than 50% of aorta overriding call it If more than 50% of aorta overriding call it DORV DORV

Systemic veins return are normal Systemic veins return are normal

When RTV contract in the presence of pul When RTV contract in the presence of pul stenosis then the blood will shunted stenosis then the blood will shunted across VSD to the aorta across VSD to the aorta

This will cause mixing of the blood and This will cause mixing of the blood and appearance of cyanosis appearance of cyanosis

Both ventricles pressure is similar Both ventricles pressure is similar

CLINICAL FEATURESCLINICAL FEATURES

Mild degree of RTV out flow obst may present by Mild degree of RTV out flow obst may present by congestive heart failure due to ventricular septal congestive heart failure due to ventricular septal defect defect By time RTV hypertrophy increase and out flow By time RTV hypertrophy increase and out flow become more narrow and cyanosis will appear become more narrow and cyanosis will appear in mucous membrane +lips + mouth +fingernails in mucous membrane +lips + mouth +fingernails +toenails +toenails NB cyanosis delayed due to PDA NB cyanosis delayed due to PDA Older children came with extreme cyanosis –Older children came with extreme cyanosis –dusky lips +blue sclerae +finger clubbing dusky lips +blue sclerae +finger clubbing FTT +dyspnea FTT +dyspnea

EXAMINATION EXAMINATION

Normal pulse Normal pulse

Lt ant hemi thorax bulging Lt ant hemi thorax bulging

Substernal RTV impulse Substernal RTV impulse

Systolic thrill in lt sternal border Systolic thrill in lt sternal border

Systolic murmur –ejection or holosystolic Systolic murmur –ejection or holosystolic preceded by click preceded by click

S2 may be single S2 may be single

DIAGNOSIS DIAGNOSIS

Chest x ray –narrow base cardiac shape Chest x ray –narrow base cardiac shape like wooden shoe (Coeur en sabot )like wooden shoe (Coeur en sabot )

Large aorta oligaemic lungs Large aorta oligaemic lungs

ECG RTaxis RTV H P tall or ECG RTaxis RTV H P tall or peaked or bifid peaked or bifid

ECHO overriding aorta RTVH degree ECHO overriding aorta RTVH degree of RTV out flow obst pul valve and of RTV out flow obst pul valve and branches PDA other lesions branches PDA other lesions

CATHETARIZATION CATHETARIZATION

To evaluate the entire heart To evaluate the entire heart

To measure the pressure in different To measure the pressure in different cardiac chambers cardiac chambers

To visualized the pul branches To visualized the pul branches

To see if there is collaterals To see if there is collaterals

COMPLICATION COMPLICATION

1- Cerebral thrombosis due to 1- Cerebral thrombosis due to polycythemia or dehydration polycythemia or dehydration

2- brain abscess headache+ nausea 2- brain abscess headache+ nausea +vomiting +convulsion+vomiting +convulsion

3-bact endocarditis 3-bact endocarditis

4- congestive heart failure 14- congestive heart failure 1 stst few months few months of life of life

TREATMENTTREATMENT

MEDICAL + SURGICAL MEDICAL + SURGICAL

NB PGE1 to keep the ducts open it will NB PGE1 to keep the ducts open it will cause relaxation of the smooth muscles of cause relaxation of the smooth muscles of the ducts the ducts

Surgical put shunt (BTshunt) to increase Surgical put shunt (BTshunt) to increase pul blood flow pul blood flow

Total correction close VSD enlarge RTV Total correction close VSD enlarge RTV out flow out flow

PAROXYSMAL HYPERCYANOTIC PAROXYSMAL HYPERCYANOTIC ATTACKSATTACKS

Known as hypoxic or TET spellsKnown as hypoxic or TET spellsIt is a problem in 1It is a problem in 1stst 2 ys of life the child become hyperpnenic and 2 ys of life the child become hyperpnenic and restless increased cyanosis gasping ---shockrestless increased cyanosis gasping ---shockOccur most frequently in the morning or with vigorous crying Occur most frequently in the morning or with vigorous crying The murmur disappear due to diminished blood flow to the lungs The murmur disappear due to diminished blood flow to the lungs The spells lasts from few min to hours rarely fatalThe spells lasts from few min to hours rarely fatalShort spells followed by generalized weakness and sleepShort spells followed by generalized weakness and sleepSevere spells progress to unconsciousness convulsion +hemi Severe spells progress to unconsciousness convulsion +hemi paresis paresis Decrease in pul blood flow will lead to hypoxia and acidosis Decrease in pul blood flow will lead to hypoxia and acidosis

FOLLOW UP FOLLOW UP

Antibiotic prophylaxis Antibiotic prophylaxis

Post operative the pt liable to get Post operative the pt liable to get arrhythmias residual pul stenosis pul arrhythmias residual pul stenosis pul regurg aortic stenosis regurg aortic stenosis