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CONGENITAL HEART DISEASE – RIGHT TO LEFT SHUNT

CONGENITAL HEART DISEASE – RIGHT TO LEFT SHUNT. ADESINA M, Origbemisoye #684. INTRODUCTION. Abnormalities of the heart or great vessels that are present from birth Congenital affections of the heart Faulty embryogenesis – week 3 to week 8 1 st year of life – ½ Discrete region of heart

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CONGENITAL HEART DISEASE – RIGHT TO LEFT SHUNT

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  1. CONGENITAL HEART DISEASE – RIGHT TO LEFT SHUNT ADESINA M, Origbemisoye #684

  2. INTRODUCTION • Abnormalities of the heart or great vessels that are present from birth • Congenital affections of the heart • Faulty embryogenesis – week 3 to week 8 • 1st year of life – ½ • Discrete region of heart • The most common birth defects are cardiovascular in origin

  3. INCIDENCE • Most prevalent malformation and most common type of heart disease among children • 1% of live birth • Incidence is higher in premature infants & in still borns • Coarctation of aorta, VSD, ASD, AVSD, PS, PDA, TOF, TGA, Aortic stenosis, Tricuspid atresia, PTA & TAPVC

  4. PATHOGENESIS GENETICS Many of these genetic defects are autosomal dominant Trisomies -13, 15, 18, 21 (Down’s syndrome) Monosomy x - Turner’s syndrome Microdeletion – DiGeorge syndrome Single gene defects – Noonan’s syndrome, Marfan’s syndrome, Holt oram syndrome, Ellis van creveld syndrome & Alagille syndrome

  5. PATHOGENESIS • MATERNAL DISORDERS – Rubella, SLE, GDM & FAS • DRUGS – warfarin, lithium, phenytoin, sodium valproate & retinoic acid • Multifactorial – environmental factors

  6. CLINICAL FEATURES OF CHD • Malformations causing a left to right shunt • Malformations causing a right to left shunt • Malformations causing an obstruction

  7. RIGHT TO LEFT SHUNTS • Tetralogy of Fallot • Transposition of great arteries • Tricuspid atresia • Persistent TruncusArteriosus • Total anomalous pulmonary venous connection • Hypoplastic left heart syndrome • Ebsteinanormaly

  8. FETAL CIRCULATION & SHUNT

  9. CHANGES AT BIRTH • Umbilical arteries – medial umbilical lig. • Umbilical vein – ligamentumteres of liver • Ductusvenosum – ligamentumvenosum • Ductusarteriosus – ligamentumarteriosus

  10. TETRALOGY OF FALLOT

  11. PRESENTATION OF TOF Wrong positioning of the aorticopulmonary septum • Obstruction to RV outflow (severity) • Aortic override of the VSD • RV hypertrophy • VSD

  12. LAB. WORK FOR TOF • ECG – RV hypertrophy • Chest x-ray – Coeur en sabot • Two dimensional echocardiography – malalignedvsd, overriding aorta & site with PS with severity • Classic contrast angiography – RV outflow & pulmonary valve

  13. TREATMENT FOR TOF • Surgical repair • Endocarditis remains a risk

  14. TRANSPOSITION OF GREAT ARTERIES

  15. TYPES OF TGA Ventriculoarterial discordance with normal arteriovenous concordance • Complete TGA • Congenitally corrected TGA

  16. COMPLETE TGA 2/3 PDA – Exogenous prostaglandin 1/3 VSD Dextro Male > female 10% of CHD Treatment : Surgery - enlargement of intra-atrial communication, arterial switch operation,

  17. CONGENITALLY CORRECTED TGA • Transposition of ascending aorta and pulmonary trunk • Inversion of ventricles

  18. TRICUSPID ATRESIA

  19. Complete occlusion of tricuspid valve orifice • Unequal AV canal • RV underdevelopment • Patent foramen ovale • ECG characteristically shows RA enlargement, left-axis deviation, and LV hypertrophy • Cyanosis dominates the picture • TREATMENT : Atrial septostomy

  20. PERSISTENT TRUNCUS ARTERIOSUS

  21. Failure of aorticopulmonary septum to form • 100% associated vsd • Increase pul. Blood flow • Irreversible pulmonary hypertension • systemic cyanosis

  22. TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION

  23. Pulmonary veins fail to directly join the left veins • Patent foramen ovale /ASD • volume & pressure hypertrophy with dilation of right part of the heart • Dilation of pulmonary trunk • Hypoplastic LA • Normal LV • cyanosis

  24. HYPOPLASTIC LEFT HEART SYNDROME

  25. Left heart of new born fails to develop completely • Small LV & Aorta • Cyanosis & rapid breathing • Norwood procedure • Glenn procedure • Fontan procedure

  26. EBSTEIN’S ANOMALY

  27. Rare • Various presentations • Abnormal muscle of ventricle • Abnormal valve leakage • Progressive cyanosis from right to left atrial shunting • Tricuspid regurgitation & RV dysfunction • Wolff – Parkinson white syndrome • Treatment – repair of native valve.

  28. THANK YOU

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