TOF.pptx

congenital health problems in children is very serios problem in children ,it is major cause of mortality in children .it can prevented by proper care of mothers during pregnancy .

1. TETRALOGY OF FALLOT BY SACHIN DWIVEDI CLINICAL INSTRUCTOR , KGMU CON LUCKNOW

2. INTRODUCTION Tetralogy of Fallot (TOF), first described in 1888 and named after Louis Arthur Fallot . Tetralogy of fallot - the most common congenital heart disorders. TOF is classified as a cyanotic heart disorder because TOF results in an inadequate flow of blood to the lungs for oxygenation.

3. DEFINITION Tetralogy of fallot is a congenital heart defect which is characterised by the combinations of four defects : Pulmonary stenosis (right ventricular outflow tract obstruction). Ventricular Septal Defect. Overriding or dextroposition of the aorta Right ventricular hypertrophy.

4. Incidence Tetralogy of Fallot (TOF) represents approximately 7%-10% of  congenital heart diseases  (CHDs), and it is the most common cyanotic CHD, with 0.23-0.63 cases per 1,000 births .

5. ETIOLOGY Unknown Genetic factor:TBX1 Gene Methylene tetrahydrofolate reductase (MTHFR) gene polymorphism Maternal rubella during pregnancy Poor prenatal nutrition Maternal alcohol use Maternal age older than 40 years Maternal phenylketonuria and diabetes.

6. PATHOPHYSIOLOGY

7. CLINICAL MANIFESTATIONS Cyanosis Tet spells Squatting position Slow weight gain and mental slowness. Difficulty with feeding and failure to thrive. Clubbing Polycythemia Decreased activity tolerance

8. DIAGNOSTIC EVALUATION Auscultation Harsh systolic ejection murmur heard best at the upper left sternal border. During a hypercyanotic spell the murmur disappears.

9. DIAGNOSTIC EVALUATION Chest X-ray Boot shaped because of pulmonary stenosis with an upturned apex resulting from Right Ventricular Hypertrophy

10. DIAGNOSTIC EVALUATION Hematologic studies Haemoglobin and hematocrit values are usually elevated in proportion to the degree of cyanosis. Prolonged cyanosis causes reactive polycythemia that increases the oxygen carrying capacity.

11. Coagulation studies There is diminished coagulation factors and diminished total fibrinogen, which are associated with prolonged prothrombin and coagulation times.

12. DIAGNOSTIC EVALUATION ABG and oximetry ECG

13. DIAGNOSTIC EVALUATION Echocardiogram with Doppler study

14. DIAGNOSTIC EVALUATION Cardiac catheterization

15. MANAGEMENT Supplemental oxygen - to compensate for restricted pulmonary blood flow Analgesics- Most analgesic agents have sedating properties, which are beneficial for patients who are having hypercyanotic episodes

16. MANAGEMENT Morphine sulfate , 0.1-0.2 mg/kg intramuscularly (IM) or subcutaneously (SC), may decrease systemic venous return as well as producing a sedative effect that provides comfort and diminishes anxiety for the patient

17. MANAGEMENT Alpha-adrenergic Agonists Phenylephrine , 0.02 mg/kg IV, is used to increase SVR. This drug produces vasoconstriction of arterioles, thereby increasing peripheral venous return. Improve myocardial contractility Increased heart rate and CO.

18. MANAGEMENT IV propranolol ( Inderal ) may be administered, which relaxes the infundibular muscle spasm causing RVOTO.

19. Management of " tet " spells Infant can be placed in a knee-to-chest position if possible. This provides a calming effect, reduces systemic venous return, and increases Systemic Vascular Resistance (SVR). Morphine sulfate Alpha-agonist phenylephrine

20. SURGICAL MANAGEMENT The timing of complete surgical repair is dependent on numerous variables, including symptoms and any associated lesions ( Eg . Multiple VSD). The trend is to perform a complete surgical procedure before the age of 1 year and preferably by the age of 2 years. Studies have shown, however, that surgery is preferably done at or about 12 months of age.

21. SURGICAL MANAGEMENT PALLIATIVE Blalock- Taussig shunt : use of a Gore-Tex graft between subclavian artery and pulmonary artery.

22. SURGICAL MANAGEMENT CORRECTIVE Closure of the ventricular septal defect utilizing a patch. The infundibular obstruction can be surgically resected to reduce the amount of restriction

23. SURGICAL MANAGEMENT BALLOON PULMONARY VALVULOPLASTY : To increase oxygen saturation

24. COMPLICATIONS Hypoxia. Hypercyanotic spells. Polycythemia . CHF Right ventricular dysfunction. Ventricular arrhythmias. Infective endocarditis .