Cyanotic heart disease

1. CYANOTIC HEART DISEASE Dr.B.BALAGOBI

2. 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

3. Tetralogy of Fallot TOF is the most common cyanotic heart disease. 4 features VSD Overriding Aorta Infundibular Pulmonic Stenosis (more severe with advancing age R to L shunt increase) RVH Severity depend on PS. More frequently develops cyanosis in second half of the first year.

4. 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 hypoxia PolycythaemiaThrombosis (CVA) Infective endocarditis  Cerebral abcess  Hemiplegia

5. R/S aortic arch Uplifted apex Pulmonary artery Bay Oligaemic lung field Boot shaped small heart

6. Management of hypercyanotic spell Usually selflimiting If prolonged(>15 min) need Rx Morphine sedatereduce hyperventilationreduce venous return reduce R-L shunt  reduce cyanosis Knee-chest position(y? use ur brain!) IV propranolol  relieve infundibular PS ABG PH,O2

7. 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

8. 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.

9. D-transposition of the Great Arteries

10. 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”

11. 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

12. 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

13. Total Anomalous Pulmonary Venous Connection

14. 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

15. 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

16. Ebsteins Anomaly Atrialization of RV, sail-like TV, TR 50% ASD/PFO 50% ECG evidence of WPW Age at presentation varies from childhood adulthood 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.

17. 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).

18. Adult Congenital Heart Disease Atrial Septal Defect Coarctation of Aorta Tetralogy of Fallot Common Ventricle Ebstiens Anomaly Eisenmenger Syndrome

19. T/F Causes for cyanosis in first 48 hours after birth? Transposition of great vessels VSD Tricuspid atresia Pulmonary atresia PDA Eisenmenger syndrome

20. Which of the following are associated with recurrent LRTI? PDA TOF AS Small VSD Eisenmenger syndrome

21. T/F TOF? Majority will present at Day 1 Overriding of aorta is a feature Cause cardiomegaly Cause plethoric lung fields on CXR Murmur is inaudible during cyanotic episodes

22. T/F regarding TOF? Is the commonest cyanotic congenital heart disease Β blockers are used in cyanotic spells BT shunt improves the saturation Cause cerebral abscess Cause anaemia

23. T/F which of the following cyanotic heart diseases are cause reduced pulmonary blood flow? TGA TOF Tricuspid atresia TAPVD Eisenmenger syndrome

24. T/F Regarding following combinations? Boot shaped heart - TOF Egg on side appearance – TGA Coil embolization – PDA Down syndrome – Atrio ventricular canal defect Murmur radiates to the neck - AS .

25. T/F causes for ejection systolic murmur in upper left sternal edge in 2 year old child.? ASD Large VSD Small PDA Anaemia MS

26. T/F which of the following are normal in child? Parasternal heave S3 Sinus arrhythmia Heart rate of 150/min in a neonate Central cyanosis 

27. T/F features of cardiac failure? Tender hepatomegaly Ankle oedema Sweating of the scalp Gallop rhythm Cyanosis

28. T/F which of the following are useful in a cyanotic spell? IV Morphine IV propranalol IV NaHCO3 IV frusemide Knee chest position

29. T/F which of the following cause weak thread pulse, Cardiac failure PDA Septic shock Hypovolaemic shock AR AV malformation

30. T/F, in tetralogy of fallots ? Usually cyanosis presents at birth Cardiomegaly is commonly seen ECG at birth shows right axis deviation Murmur is due to VSD Recurrent infection is common

31. T/F Cyanotic heart disease with decreased pulmonary blood flow ? Tricuspid atresia Transposition of great Arteries(TGA) Tetralogy of fallot (TOF) Total abnormally in pulmonary venous drainage(TAPVD) Eisenmenger’s syndrome

32. T/F Biventricular hypertrophy is a feature of? Septum secondum ASD Small size VSD PDA TOF PS

33. T/F Causes for loud second heart sound? Large PDA Eisenmenger syndrome Pulmonary stenosis Systemic hypertension Mitral stenosis

34. The treatment of hypercyanotic spells includes which of the following? a) Isoproterenol b) Adenosine c) Digoxin d) Oxygen e) Furosemide f)Morphine

35. Hypercyanotic spells can be provoked by any of the following: a) Pain b) Induction of anesthesia c) Dehydration e) Squatting f)Exercise