CYANOTIC DISEASE• TOF(Tetralogy of fallot)• TGV(Transposition of great vessels)• Tricuspid atresia• Truncus arteriosus• Total anomalous of pulmonary venous drainage• Hypoplastic left heart syndrome• Pulmonary atresia• Ebstein anomaly
Tetralogy of Fallot• TOF is the most common cyanotic heart disease.• 4 features – VSD – Overriding Aorta – Infundibular Pulmonic Stenosis(more severe with advancing ageR to L shunt increase) – RVH• Severity depend on PS.• More frequently develops cyanosis in second half of the first year.
Clinical features• Hyper cyanotic spells after exercise/cry and squatting position.• clubbing.• Long loud ejection systolic murmur(Due to PS) – No PSM! – During cyanotic episodes murmur is inaudible• Chronic hypoxiaPolycythaemiaThrombosis(CVA)• Infective endocarditis Cerebral abcess Hemiplegia
R/S aortic arch Pulmonary artery BayOligaemic lung field Uplifted apex Boot shaped small heart
Management of hypercyanotic spell• Usually selflimiting• If prolonged(>15 min) need Rx – Morphinesedatereduce hyperventilationreduce venous return reduce R-L shunt reduce cyanosis – Knee-chest position(y? use ur brain!) – IV propranolol relieve infundibular PS – ABG PH,O2
Tetralogy: Surgical Treatment• Systemic – Pulmonary Shunt[palliative surgery] to increase pulmonary flow. – Blalock-Taussig(BT shunt)• Complete Repair at age of 6 months – takedown of prior shunt – patch VSD – resection of subpulmonic obstruction
Transposition of the Great Arteries• Aorta from right ventricle, pulmonary artery from left ventricle.• Cyanosis from birth, hypoxic spells sometimes present.• Heart failure often present.• corrected initially with prostaglandin to keep ductus open and balloon atrial septostomy to improve systemic saturation• repair via “atrial switch” Mustard procedure• Cardiac enlargement and diminished pulmonary artery segment on x-ray.
TGA• The most common cyanotic defect presents with cyanosis at neonatal period.• The degree of saturation will depend on the degree of mixing of the 2 “parallel” circuits.• The mixing sites are: ASD, PDA, and VSD.• The more mixing, the higher the “effective pulmonary blood flow”
CLINICAL Findings TGA• Cyanosis,Clubbing• Possibly CHF• Closely split/single S2• If pulmonary stenosis present, may cause systolic murmur.• CXR: egg on side appearance – Narrow mediastinum – RVH increased pulmonary vascular markings
Total Anomalous Pulmonary Venous Drainage• Pulmonary veins do not make a direct connection with the left atrium.• Blood reaches the left atrium only through an atrial septal defect or patent foramen ovale.• Pulmonary congestion, tachypnea, cardiac failure, and variable cyanosis.• Operative repair in all cases• Cyanosis variable and largely dependant on degree of pulmonary venous obstruction.• Snowman on CXR
Truncus Arteriosus• A SINGLE GREAT VESSEL ARISES FROM THE HEART AND GIVES OFF THE CA’S,PA’S AND AORTA• embryological structure known as the truncus arteriosus not properly divides into the pulmonary artery and aorta.• Large VSD is present.• Bounding pulses(it’s like a big PDA), There may be a continous murmur if the PA’s are tight
TRUNCUS:Tx• Decongestive tx pending surgery• Surgery consists of VSD closure and a graft to the PA’s.• Early surgery essential.The average age of death untreated is 5 wks.• Sequelae:depends on degree of truncal valve insufficiency and pulm artery obstruction
Ebsteins Anomaly• Atrialization of RV, sail-like TV, TR• 50% ASD/PFO• 50% ECG evidence of WPW• Age at presentation varies from childhoodadulthood and depends on factors such as severity of TR, Pulm Vascular resistance in newborn, and associated abnormalities such as ASD• Cyanosis and arrhythmias in infancy are common.• Right heart failure in half of patients.• Operative repair with tricuspid valve replacement.
Tricuspid Atresia• Tricuspid valve is completely absent in about 2% of newborns with congenital heart disease.• Blood flows from right atrium to left atrium through foramen ovale.• Early cyanosis.DDs – TGV,TA,PA,Severe TOF,Ebstein’s anomaly.• Repair consists of shunt from right atrium to pulmonary artery or rudimentary right ventricle (Fontan procedure).
Adult Congenital Heart Disease• Atrial Septal Defect• Coarctation of Aorta• Tetralogy of Fallot• Common Ventricle• Ebstiens Anomaly• Eisenmenger Syndrome
T/F Causes for cyanosis in first 48 hours after birth?A. Transposition of great vesselsB. VSDC. Tricuspid atresiaD. Pulmonary atresiaE. PDAF. Eisenmenger syndrome
Which of the following are associated with recurrent LRTI?A. PDAB. TOFC. ASD. Small VSDE. Eisenmenger syndrome
T/F TOF?A.Majority will present at Day 1B.Overriding of aorta is a featureC.Cause cardiomegalyD.Cause plethoric lung fields on CXRE. Murmur is inaudible during cyanotic episodes
T/F regarding TOF?A. Is the commonest cyanotic congenital heart diseaseB. Β blockers are used in cyanotic spellsC. BT shunt improves the saturationD. Cause cerebral abscessE. Cause anaemia
T/F which of the following cyanotic heart diseases are cause reduced pulmonary blood flow? A. TGA B. TOF C. Tricuspid atresia D. TAPVD E. Eisenmenger syndrome
T/F Regarding following combinations?A. Boot shaped heart - TOFB. Egg on side appearance – TGAC. Coil embolization – PDAD. Down syndrome – Atrio ventricular canal defectE. Murmur radiates to the neck - AS .
T/F causes for ejection systolic murmur inupper left sternal edge in 2 year old child.? A. ASD B. Large VSD C. Small PDA D. Anaemia E. MS
T/F which of the following are normal in child?A. Parasternal heaveB. S3C. Sinus arrhythmiaD. Heart rate of 150/min in a neonateE. Central cyanosis
T/F features of cardiac failure?A. Tender hepatomegalyB. Ankle oedemaC. Sweating of the scalpD. Gallop rhythmE. Cyanosis
T/F which of the following are useful in a cyanotic spell?A. IV MorphineB. IV propranalolC. IV NaHCO3D. IV frusemideE. Knee chest position
T/F which of the following cause weak thread pulse,A. Cardiac failureB. PDAC. Septic shockD. Hypovolaemic shockE. ARF. AV malformation
T/F, in tetralogy of fallots?A. Usually cyanosis presents at birthB. Cardiomegaly is commonly seenC. ECG at birth shows right axis deviationD. Murmur is due to VSDE. Recurrent infection is common
T/F Cyanotic heart disease withdecreased pulmonary blood flow?A. Tricuspid atresiaB. Transposition of great Arteries(TGA)C. Tetralogy of fallot(TOF)D. Total abnormally in pulmonary venous drainage(TAPVD)E. Eisenmenger’s syndrome
T/F Biventricular hypertrophy is a feature of?A.Septum secondum ASDB. Small size VSDC. PDAD.TOFE. PS
T/F Causes for loud second heart sound?A.Large PDAB. Eisenmenger syndromeC. Pulmonary stenosisD.Systemic hypertensionE. Mitral stenosis
• The treatment of hypercyanotic spells includes which of the following?a) Isoproterenolb) Adenosinec) Digoxind) Oxygene) Furosemide f)Morphine
• Hypercyanotic spells can be provoked by any of the following:• a) Pain• b) Induction of anesthesia• c) Dehydration• e) Squatting• f)Exercise