Cyanotic Congenital Heart Diseases-C1.pptx

Cyanotic Congenital Heart

1. Cyanotic Congenital Heart Diseases Hayat Ahmed, MD Pediatric Cardiologist January , 2023 1/31/2023 1

2. What is cyanosis? Cyanosis is a bluish discoloration of the skin and mucous membranes Desaturation of 5g/dl of hemoglobin in the cutaneous veins Central cyanosis - desaturation of arterial blood Peripheral cyanosis- increased extraction of oxygen by peripheral tissue in the presence of normal arterial saturation 1/31/2023 2

3. What is the influence of hemoglobin l evel on cyanosis? A normal person with hemoglobin of 15 g/dl →3 g of reduced hemoglobin results from 20% desaturation (because 3 is 20% of 15) →80% saturation Cyanosis is recognized at a higher level of oxygen saturation in patients with polycythemia and at a lower level of oxygen saturation in patients with anemia polycythemia hemoglobin of 20 g/dl→ 3 g of reduced hemoglobin results from only 15% desaturation → 85% arterial saturation A nemia hemoglobin of 6 g/dl →3 g of reduced hemoglobin results from 50%desaturation → cyanosis appear when arterial oxygen saturation is reduced to 50% 1/31/2023 3

4. Consequences and Complications of cyanosis Polycythemia Clubbing CNS complications Bleeding disorders Hypoxic spells and squatting Scoliosis Hyperuricemia and gout 1/31/2023 4

5. Classification of cyanotic CHD With increased pulmonary flow TGA TAPVC Truncus arteriosus HLHS With decreased pulmonary flow TOF Pulmonary atresia Tricuspid atresia Ebstein Anomaly 1/31/2023 5

6. Tetralogy of Fallot 1/31/2023 6

7. Tetralogy of Fallot Common cyanotic congenital cardiac anomaly Four anatomical components of TOF: VSD Overriding Aorta Right ventricular outflow obstruction Right ventricular hypertrophy 1/31/2023 7

8. Pathophysiology Severity directly proportional to the degree of RVOT obstruction. Change in pulmonary and systemic vascular resistance and the degree of RVOT obstruction affect degree of R-L shunt. Infundibular stenosis is progressive. 1/31/2023 8

9. Clinical Manifestations Variable depend on RVOT obstruction pink to cyanosis CHF is not a usual manifestation of TOF Squatting Dyspnoea on exertion Hypoxic spells Growth failure 1/31/2023 9

10. Physical Examination Cyanosis variable Clubbing Usually S2 is single Quite precordium Thrill at the pulmonary area(-+) Systolic ejection murmur at the pulmonary area(LUSB) 1/31/2023 10

11. Electrocardiography (TOF) Right axis deviation Right atrial and right ventricular enlargement 1/31/2023 11

12. Chest x ray (TOF) Normal sized, boot shaped heart. Reduced pulmonary vascular marking ( oligemic ) depend on the degree of RVOT obstruction. 1/31/2023 12

13. Echocardiography (TOF) Location and size of the VSD The aortic override The degree of RVOT obstruction The size of pulmonary valve annulus Look for additional pulmonary artery branch stenosis. Look for other associated anomalies Right aortic arch Coronary arteries anatomic variations 1/31/2023 13

14. Complications Cardiovascular accidents : occurs in 4-5% of cases is due to cerebral embolism. Brain abscess: rare in the first two years of life may be due to small cerebral infract which is super infected due to bacteraemia. Infective endocarditis Polycythemia 1/31/2023 14

15. Management Medical management: A) Neonatal period if pulmonary flow is dependent of DA, give prostaglandin to prevent ductal closure and followed by palliative shunt (B-T Shunt) B)Recognition and treatment of hyper cyanotic spell. Knee chest positioning of the patient Administration of oxygen Volume expansion Correction of acidosis Sedation with morphine Propranolol 1/31/2023 15

16. Management cont … C ) Treat infective endocarditis with antibiotic IV for 4-6 weeks D) Anemia should be corrected Check RBC indices if MCV low start with iron despite high HCT E)Correct severe polycythemia Phlebotomy indicated if HCT > 65% or > 60 if patient is symptomatic. volume removed : measured Hct --desired HCtx80/kg BV measured Hct 1/31/2023 16

17. Management Surgical Management: Palliative: Modified Blalock-Taussig shunt between pulmonary artery branch and subclavian artery B) Corrective surgery : closing of the VSD and reliving all possible source of RVOT obstruction. 1/31/2023 17

18. Transposition of The Great Arteries 1/31/2023 18

19. Complete Transposition of the Great Arteries The great arteries arise from morphologically wrong ventricles. (The aorta arise from the RV and Pulmonary arises from LV in the setting of a concordant atrioventricular connection . 1/31/2023 19

20. Transposition of the Great Arteries TGA occurs in 8-9% of CHD Male are affected than Females Different variety exist 1/31/2023 20

21. Hemodynamic The RV pressure is systemic The blood returning from the lung pass to the lung via PA Systemic venous return passes back to the systemic circulation via the aorta. Both ventricles are volume overloaded. The right ventricle also pressure overloads and result in CHF Survival depend on associated Large ASD, VSD,PDA. 1/31/2023 21

22. Clinical features Cyanosis detected 87% of the neonates immediately ; rest later at the age of 1 to 3 month and is progressive. Clubbing Squatting only 20% of patients Dyspnoea Cardiac failure Cough 1/31/2023 22

23. Clinical features cont.. TGA with no VSD Cyanosis intense Precordial lift Loud first sound Murmur may not be heard Large heart with egg - on side appearance. Increased pulmonary vascular marking 1/31/2023 23

24. Echocardiography(TGA) Shows the origin of : pulmonary artery from the LV Aorta from RV Associated lesions like VSD and ASD be identified. Coronary artery anatomy 1/31/2023 24

25. 1/31/2023 25

26. Prognosis Patient die of anoxia and or CHF in the first 6 month of life if there is little communication. 1/31/2023 26

27. Management Medical management : Prostaglandin to keep the DA open until palliation done Give oxygen Treat metabolic acidosis with bicarbonate. Diuretics Surgical : Palliative balloon septostomy creating (increasing) ASD, Rashkind procedure. Corrective surgery has to be done early Arterial Switch ( Jaten Procedure) Atrial switch ( Mustard or senning procedure) 1/31/2023 27

28. Management cont Atrial baffle operations (Mustard and Senning operations) Rastelli operation Arterial switch operation (ASO)/ Jatene -choice of surgery 1/31/2023 28

29. Management cont Rastelli operation ASO/ Jatene 1/31/2023 29

30. Congenitally corrected TGA(L-TGA) It occurs in <1% of all patients with CHD T he atrioventricular relationships are discordant: the right atrium is connected to a left ventricle and the left atrium to a right ventricle ( ventricular inversion ) The great arteries are transposed, with the aorta arising from the right ventricle and the pulmonary artery from the left. T he aorta arises to the left of the pulmonary artery 1/31/2023 30

31. L-TGA cont 1/31/2023 31

32. L-TGA Without other defects ,the hemodynamics almost normal. In most patients associated anomalies coexist VSD Ebstein -like abnormalities of the left-sided atrioventricular (tricuspid) valve pulmonary valvular or subvalvular stenosis (or both) A trioventricular conduction disturbances complete heart block A ccessory pathways such as Wolff-Parkinson-White syndrome Dextrocardia in 50% of patients 1/31/2023 32

33. Further readings Nelson text book of pediatrics. 21 st edition. Park’s pediatrics cardiology for practitioners, 7th edition Moss and Adams’ Heart Disease in Infants, Children,and Adolescents,10 th edition. A guide for pediatrics physical diagnosis for medical students and practitioners 1/31/2023 33