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Congenital heart disease


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Congenital heart disease

  1. 1. Presenter Dr Gurulingappa Moderator Dr Ranjan RK
  2. 2. <ul><li>Teratology of Fallot. </li></ul><ul><li>Transposition of Great Vessels. </li></ul>
  3. 3. <ul><li>In 1888 Fallot described congenital heart defect composed of 4 characteristics. </li></ul><ul><ul><li>Large VSD </li></ul></ul><ul><ul><li>Right ventricular outflow obstruction </li></ul></ul><ul><ul><li>Overriding Aorta </li></ul></ul><ul><ul><li>Right ventricular hypertrophy. </li></ul></ul><ul><li>This malformation results from an anterior displacement of the cono septum </li></ul>
  4. 4. <ul><li>Pink tet- is a patient with TOF a source for adequate pulmonary blood flow </li></ul><ul><li>Without treatment ,25% of infants TOF &PS die in first year of life, 40% will die by the age of 4 years,70% by 10 years and 95% by 40 years. </li></ul>
  5. 5. <ul><li>Complex shunt with resistance to right ventricualr outflow directing blood Right to Left across VSD and leading to hypoxia and cyanosis. </li></ul>
  6. 7. <ul><li>Treatment </li></ul><ul><ul><li>100% Oxygen and hyperventilation </li></ul></ul><ul><ul><li>Knee chest postion. </li></ul></ul><ul><ul><li>Intraoperatively open chest, direct compression of aorta </li></ul></ul><ul><ul><li>Morphine sulphate can be given to sedate the patients and diminishing hyper apnoic response </li></ul></ul><ul><ul><li>Adequate maintenance of intravascular volume. </li></ul></ul><ul><ul><li>Beta blockers to reduce the infundibular spasm and heart rate. </li></ul></ul><ul><ul><li>If the child continues to be severely hypoxemic, SVR can be elevated and maintained with an infusion of norepinephrine and phenylephrine. </li></ul></ul><ul><ul><li>Sodiumbicarbonate. </li></ul></ul>
  7. 8. <ul><li>History and Physical Examination </li></ul><ul><ul><li>History of hypercyanotic spells </li></ul></ul><ul><ul><ul><li>Frequency </li></ul></ul></ul><ul><ul><ul><li>Severity </li></ul></ul></ul><ul><ul><li>Medication history </li></ul></ul><ul><ul><li>Investigations </li></ul></ul><ul><ul><ul><li>Hb </li></ul></ul></ul><ul><ul><ul><li>Blood Group </li></ul></ul></ul><ul><ul><ul><li>Haematocrit. </li></ul></ul></ul><ul><ul><ul><li>Echocardiography </li></ul></ul></ul><ul><ul><ul><li>Chest Xray </li></ul></ul></ul><ul><ul><ul><li>Cardiac catheterization Data </li></ul></ul></ul><ul><ul><ul><li>Coronary angiography </li></ul></ul></ul>
  8. 9. <ul><li>Palliative </li></ul><ul><ul><li>Balloon dilatation of pulmonic valve </li></ul></ul><ul><ul><li>Systemic and Pulmonary arterial shunts </li></ul></ul><ul><li>Definitive </li></ul><ul><ul><li>Patch closure of VSD </li></ul></ul><ul><ul><li>Ventriculotomy with reconstructionof right ventricle outflow tract </li></ul></ul><ul><li>NPO and antibiotics </li></ul>
  9. 10. <ul><li>Goals of Management </li></ul><ul><ul><li>Maintenance of SVR </li></ul></ul><ul><ul><li>Minimize PVR </li></ul></ul><ul><ul><li>Avoid Myocardial depression </li></ul></ul><ul><ul><li>Maintain Preloading. </li></ul></ul>
  10. 11. <ul><li>Standard Monitors </li></ul><ul><ul><li>ECG </li></ul></ul><ul><ul><li>SPO2 </li></ul></ul><ul><ul><li>Invasive Blood Pressure </li></ul></ul><ul><ul><li>ETCO2 </li></ul></ul><ul><ul><li>Temperature </li></ul></ul><ul><ul><li>Urine Output </li></ul></ul><ul><ul><li>CVP monitoring </li></ul></ul><ul><ul><li>Trans thoracic lines can be placed by the surgeons before separation form CBP. </li></ul></ul><ul><ul><li>Two large bore IV lines to be secured. </li></ul></ul>
  11. 12. <ul><li>Cardiopulmonary Bypass and Or Deep hypothermic circulatory arrest. </li></ul>
  12. 13. <ul><li>Right Ventricular Failure </li></ul><ul><li>Residual or unrecognized VSD </li></ul><ul><li>Residual or unrepairable right sided obstruction. </li></ul><ul><li>Heart block and ventricular dysarrythmias </li></ul>
  13. 14. <ul><li>TGA is a congenital heart disease, the great arteries are transposed as they relate to the heart. </li></ul><ul><li>The aorta arises form right ventricle and pulmonary artery arises from the left ventricle. </li></ul><ul><li>TGA accounts for only 5-7% of all congenital heart disease and has an incidence of 0.2 cases pre 1000 live births. If uncorrected TGA has 30 % mortality rate in first week of life, 45% in first month and 90% in first year. </li></ul>
  14. 15. <ul><li>Aorta arises form right ventricle, pulmonary artery from left ventricle. </li></ul><ul><li>VSD nearly half of the cases. </li></ul><ul><li>Variable degree of sub pulmonic stenosis. </li></ul>
  15. 16. <ul><li>Parallel right and left circuits, pulmonary blood flows to left ventricle and back to pulmonary artery without reaching systemic circulation. </li></ul><ul><li>Some mixing of pulmonary venous return across ASD, VSD, PDA required for survival. </li></ul>
  16. 17. <ul><li>History and Physical Examination </li></ul><ul><ul><li>Gestational age and birth complications </li></ul></ul><ul><ul><li>Evaluation of the airway </li></ul></ul><ul><ul><li>Arterial and intravenous access </li></ul></ul><ul><li>Investigations </li></ul><ul><ul><li>Complete blood count </li></ul></ul><ul><ul><li>Electrolytes </li></ul></ul><ul><ul><li>Platelet count </li></ul></ul><ul><ul><li>ABG </li></ul></ul><ul><ul><li>Calcium , RFT, Glucose </li></ul></ul><ul><ul><li>ECG </li></ul></ul><ul><ul><li>Chest X ray. </li></ul></ul><ul><ul><li>Blood Grouping and cross matching. </li></ul></ul><ul><ul><li>Echocardiography </li></ul></ul><ul><ul><li>Cardiac catheterization data. </li></ul></ul><ul><ul><li>Coronary angiography </li></ul></ul>
  17. 18. <ul><li>Atrial Baffle repair “ Mustard or Senning” </li></ul><ul><li>Anatomic correction with division of great arteries, reattachment to correct ventricular outflow- reimplantation of coronary arteries “Jatene” </li></ul><ul><li>VSD closure with left ventricle outflow to aorta and right ventricle to pulmonary artery conduit. </li></ul>
  18. 19. <ul><li>In the absence of an ASD or VSD, measures need to be taken to maintain ductus arteriousos </li></ul><ul><li>PGE1 </li></ul><ul><li>Ballon atrail septostomy </li></ul>
  19. 20. <ul><li>Maintain preload, cardiac output and heart rate </li></ul><ul><li>Avoid myocardial depression </li></ul><ul><li>Maintain or decrease PVR </li></ul><ul><li>Avoid reductions in SVR </li></ul>
  20. 21. <ul><li>Standard Monitors </li></ul><ul><ul><li>ECG </li></ul></ul><ul><ul><li>NIBP </li></ul></ul><ul><ul><li>Pulse oxymetry </li></ul></ul><ul><ul><li>ETCO2 </li></ul></ul><ul><ul><li>Temperature </li></ul></ul><ul><ul><li>Urine output </li></ul></ul><ul><ul><li>Arterial Line </li></ul></ul><ul><ul><li>Trans esophageal echocardiography </li></ul></ul><ul><ul><li>Two large bore IV lines. </li></ul></ul>
  21. 22. <ul><li>CPB and or Deep Hypothermic circulatory arrest. </li></ul>
  22. 23. <ul><li>Atrail Baffle </li></ul><ul><ul><li>Systemic or Pulmonary Venous return obstructed </li></ul></ul><ul><ul><li>Residual Intra-arterial shunts </li></ul></ul><ul><ul><li>Systemic ( Right) ventricualr Dysfunction </li></ul></ul><ul><ul><li>Systemic (Tricuspid) valvular dysfunction </li></ul></ul><ul><ul><li>Atrial dysarrythmias. </li></ul></ul><ul><li>Anatomic correction </li></ul><ul><ul><li>Inadequate preparation of left ventricle with left ventricular failure </li></ul></ul><ul><ul><li>Inadequate coronary flow, with myocardial ischemia or infraction </li></ul></ul><ul><ul><li>Stenosis of either great vessel anastomosis </li></ul></ul><ul><ul><li>Aortic regurgitation </li></ul></ul><ul><li>Rastelli Preocedure </li></ul><ul><ul><li>Obstruction to left ventricle </li></ul></ul><ul><ul><li>Conduit obstruction </li></ul></ul><ul><ul><li>Heart Block </li></ul></ul>