CONGENITAL HEART DISEASE :CONGENITAL HEART DISEASE Dr S Sen Specialist Registrar Paediatrics North Western Deanery


INCIDENCE :INCIDENCE CONGENITAL HEART DISEASE -8/1000 births CYANOTIC HEART DISEASE - 1/3rd of above


CASE HISTORY 1 : CASE HISTORY 1 10 day old ,male infant presents to A & E dept. H/O off feeds x 2 days Lethargic Pale


: Pallor, dyspnoeic , grunting Irritable , weak cry, poor pulses RR 60/min , Liver 3 cm. 2/6 Pan Systolic murmur Left lower sternal border Bilateral basal crepitations. CASE HISTORY 1


OTHER SIGNS / SYMPTOMS :OTHER SIGNS / SYMPTOMS Tachycardia Gallop rhythm Sweating Wt. Loss


DD :DD Coarctation of aorta Severe Aortic Stenosis Interrupted Aortic Arch Hypoplastic Left Heart Myocarditis / Cardiomyopathy SVT A-V fistula : Vein of Galen Anaemia


MANAGEMENT :MANAGEMENT Airways , Oxygenation ! Volume / Diuretics Correct acidosis Blood sugar / Calcium / Potassium Sepsis / anaemia Prostaglandin Inotropes


COARCTATION OF AORTA :COARCTATION OF AORTA Balloon angioplasty End to end repair Subclavian flap repair Stent


CASE HISTORY 2 : CASE HISTORY 2 10 Yrs , male Asymptomatic Incidental murmur 2 yrs. Age O/E : HS normal , click 2nd. 3/6 ESM URSE, Carotid radiation


Slide 11:AORTIC STENOSIS Valvar Supra-valvar Sub-valvar Balloon dilatation Surgery-valvotomy replacement - mechanical / Autograft resection


CASE 3 :CASE 3 5 day old baby Respiratory distress 3/6 systolic murmur Normal saturations Enlarged heart on Chest X Ray


Slide 13:HYPOPLASTIC LEFT HEART SYNDROME Under development of L heart structures Mitral Valve / LV / Aorta Duct dependant Norwood’s procedure Stage 1 & 2 Fontan’s Heart transplant


Slide 15:NORWOOD’S STAGE 1 Within 2 wks. Mortality 30% Accept Sats. 70-85 in air Oxygen only with greatest caution Treat resp. infections promptly Very low threshold for hospitalization


Slide 16:CYANOTIC HEART DISEASE


CASE HISTORY 3 :2 Yrs. Old, Asian female Cyanosis noted 2 wks. Age Asymptomatic , feeding well O/E : 1st.HS –N, 2nd.HS – single 4/6 ESM ULSE, 3/6 CM bilat. Infraclavicular CASE HISTORY 3


Slide 18:CENTRAL CYANOSIS Generalised Blueness Occurs in 30% of congenital heart disease patients TGA Complete AVSD


DIAGNOSIS :DIAGNOSIS Cyanosis Tachycardia Tachypnoea Acidosis Poor capillary refill time Hyopotension


DIFFERENTIAL DIAGNOSIS :DIFFERENTIAL DIAGNOSIS Respiratory Distress Syndrome Persistent Foetal Circulation Methaemoglobinaemia


PERSISTENT FOETAL CIRCULATION :PERSISTENT FOETAL CIRCULATION Causes: Meconium aspiration Asphyxia Polycythaemia Maternal drugs- Aspirin,Indomethacin etc Hypoplastic lung


PERSISTENT FOETAL CIRCULATION :PERSISTENT FOETAL CIRCULATION Marked cyanosis Respiratory distress B.P. normal Loud pansystolic murmur CXR Echocardiography


CYANOTIC CONGENITAL HEART DISEASE :CYANOTIC CONGENITAL HEART DISEASE Fallot’s Tetralogy Transposition of Great arteries Tricuspid Atresia Aortic/Mitral Atresia Total anomalous Pulmonary Venous drainage Pulmonary Atresia / intact septum Double outlet right ventricle Single Ventricle Anomalous systemic venous drainage Pulmonary Arterio-venous aneurysms


MURMURS :MURMURS Systolic Diastolic Fallot’s tetralogy Truncus arteriosus Tricuspid atresia Pulmonary atresia None Truncus arteriosus TGA DORV Ao /Mi. atresia TAPVD TAPVD Single ventricle


CHEST X-RAY ( Heart Size ) :CHEST X-RAY ( Heart Size ) Large TGA Tricuspid Atresia Ao/Mi. Atresia Single Ventricle Truncus Atreriosus Ebstein’s Anomaly Lt. -> Rt. Shunts Small Fallot’s tetralogy TAPVD-Infradiaphragmatic Normal TGA Pulmonary Atresia


CHEST X-RAY (LUNGS) :CHEST X-RAY (LUNGS) Oligaemic Plethoric Fallot’s Tetralogy Truncus Arteriosus Pulmonary Atresia TGA Tricuspid Atresia TAPVD Single ventricle Left -> Right Shunts


Fallot’s Tetralogy :Fallot’s Tetralogy Start Prostaglandin E1 5 Nanograms/Kg/min. Correct Acidosis Accept Sats. 75-85 in air Treat infection Maintain adequate hydration Hypercyanotic spell: IV Propranolol/ morphine oxygen / phenylephrine


FALLOT’S TETRALOGY :FALLOT’S TETRALOGY Balloon Pulmonary valvuloplasty Blalock Taussig shunt Correction


Slide 34:TRANSPOSITION OF GREAT ARTERIES


Slide 35:TGA Prostaglandin E1 Balloon atrial septostomy Arterial switch Atrial switch - Mustard’s / Senning’s Complex TGA - Fontan’s


Slide 36:PULMONARY HYPERTENSION Primary / Secondary Reversible or not ? Cardiac catheterisation / Lung biopsy Active treatment of infections / immunisation hydration / diet Oxygen / Prostacyclin / Sildenafil Heart - lung transplant


Slide 37:ACQUIRED HEART DISEASE Myocarditis Rheumatic heart disease Kawasaki’s disease Endocarditis


Slide 38:INFECTIVE ENDOCARDITIS Symptoms Signs Investigations Treatment - 2 Antibiotics I.V. x 6 wks. Repair lesion Prevention


Slide 39:KAWASAKI’S DISEASE Mortality related to cardiac involvement Myocarditis / Pericarditis / Effusion Prox. Coronary dilatation Infarction Late coronary stenosis / thrombus / infarct. Immunoglobulin / Aspirin


Slide 40:COMMON CARDIAC SYNDROMES Down’s syndr.: AVSD / ASD / VSD / Fallot’s Turner’s : Coarct. Of Ao./ Ao. St. DiGeorge / VCF : Truncus / TGA / Fallot’s / VSD William’s : Supra valvar AS / PS / Coarct. Noonan’s : PS / Cardiomyopathy Alagille’s : peripheral PS


Slide 41:FOETAL CARDIAC TERATOGENS Alcohol : ASD / VSD / TGA Phenytoin : AS / PS / Coarct. / PDA Valproate : Fallot’s / VSD Lithium : Ebstein’s Warfarin : Foetal loss Viruses : Rubella / CMV


Slide 42:MATERNAL RISK FACTORS Diabetes : HCM / VSD / Coarct. SLE : Congenital complete heart block Phenylketonuria : Fallot’s


THANK YOU :THANK YOU