Your SlideShare is downloading. ×
0
<ul><li>Congenital Heart Disease </li></ul><ul><li>Most common type of heart disease </li></ul><ul><li>among children </li...
<ul><li>Types: </li></ul><ul><li>a)  L to R shunt </li></ul><ul><li>b)  R to L shunt </li></ul><ul><li>c)  obstructions </...
c)  Over time PVR    to that of SVR i) reverses shunt (cyanosis) 2.-   R to L shunt a)     pulmonary blood flow i)  Cyan...
c)  long standing cyanosis is        associated with “clubbing” of the    tips of the fingers and toes www.freelivedoctor....
3.-   Obstructions (of flow) a)  coarctation of the aorta b)  valvular stenosis i)  aortic ii)  pulmonary  c)  complete ob...
<ul><li>Left to Right Shunts </li></ul><ul><li>Most common: </li></ul><ul><li>a) VSD, ASD, PDA and AVSD </li></ul>www.free...
i)  VSD most common -  close spontaneously (50%)  ii)  ASD usually not symptomatic      before 30 yrs www.freelivedoctor.com
iii)  DA remains open after birth -  ~ 90% occur as isolated    anomaly  -  reversal of flow with    PVR   causes cyanosi...
<ul><li>Right to Left Shunts </li></ul><ul><li>Tetralogy of Fallot </li></ul><ul><li>a)  Most common form of cyanotic     ...
    www.freelivedoctor.com
Tetralogy of Fallot (TOF) Michael P. D'Alessandro, M.D. Peer Review Status: Internally Peer Reviewed   <ul><li>Clinical Pr...
<ul><li>Transposition of the Great Arteries </li></ul><ul><li>(TGA) </li></ul><ul><li>a)  aorta arises from RV </li></ul><...
-  PDA, foramen ovale     unstable shunt (close quickly    after birth)    need surgery <ul><li>the aorta originates fro...
www.freelivedoctor.com
www.freelivedoctor.com
<ul><li>Truncus Arteriosus </li></ul><ul><li>Failure of separation into aorta and  </li></ul><ul><li>pulmonary artery </li...
www.freelivedoctor.com
<ul><li>Tricuspid Atresia </li></ul><ul><li>Clinical Presentation: a)  Cyanosis is almost always    present at birth and i...
ii)  It is classified  by the absence of tricuspid valve, pulmonary stenosis, and VSD.  iii) The most  Common form  is als...
TRICUSPID ATRESIA 1 - atrial septal defect 2 - absent tricuspid valve 3 - ventricular septal defect Blood is shunted throu...
<ul><li>Total Anomalous Pulmonary Venous Connection (TAPVC) </li></ul><ul><li>No pulmonary veins directly join LA </li></u...
1  - superior vena cava 2  - atrial septal defect 3  - left innominate vein 4  - pulmonary veins Oxygenated blood returnin...
<ul><li>Obsttructive Congenital Anomalies </li></ul><ul><li>Coarctation of the Aorta </li></ul><ul><li>Narrowing </li></ul...
<ul><li>b)   adult (without PDA) </li></ul><ul><li>i)  most children asymptomatic  </li></ul><ul><li>  until late in life ...
1  - pinched or coarcted aorta flow patterns are normal but are reduced below the coarctation. Blood pressure is increased...
www.freelivedoctor.com
<ul><li>Pulmonary Stenosis and Atresia </li></ul><ul><li>Obstruction at pulmonary valve </li></ul><ul><li>May occur as iso...
<ul><li>Atresia of valve </li></ul><ul><li>a)  no communication between RV and    lungs </li></ul><ul><li>b)  hypoplastic ...
<ul><li>Valvular (severe) aortic stenosis or </li></ul><ul><li>atresia </li></ul><ul><li>a)  outflow obstruction </li></ul...
Aortic stenosis (valvular) 1 - narrowed aortic valve Flow patterns are normal but blood flow to the aorta is reduced as in...
<ul><li>Subaortic stenosis </li></ul><ul><li>a)  thickened ring below level of cusps </li></ul><ul><li>Supraventricular ao...
Subaortic Stenosis   www.freelivedoctor.com
www.freelivedoctor.com
<ul><li>Heart Failure (i.e.,Congestive) </li></ul><ul><li>Heart cannot eject volume which is </li></ul><ul><li>being retur...
<ul><li>Compensatory mechanisms: </li></ul><ul><li>a)  Frank-Starling mechanism </li></ul><ul><li>i)     stretch       ...
<ul><li>Most frequent causes for failure of </li></ul><ul><li>these compensatory mechanisms are: </li></ul><ul><li>a)  isc...
<ul><li>Cardiac Hypertrophy   </li></ul><ul><li>a)  cardiac myocyte is terminally      differentiated cell </li></ul><ul><...
ii)     wall thickness iii)     cavity diameter c)  volume overload i)  “eccentric hypertrophy” ii)  chamber dilation ii...
<ul><li>Left Sided Heart Failure </li></ul><ul><li>Most often caused by: </li></ul><ul><li>a)  ischemic heart disease </li...
<ul><li>Lungs major problem </li></ul><ul><li>a)     pulmonary vein pressure </li></ul><ul><li>i)  pulmonary congestion a...
<ul><li>d)  orthopnea  </li></ul><ul><li>i)  dyspnea on lying down and    relieved by sitting upright </li></ul><ul><li>e)...
<ul><li>Right Sided Heart Failure </li></ul><ul><li>Major cause is LEFT heart failure </li></ul><ul><li>Occurs as isolated...
ii)  when Left heart failure is    present, hypoxic liver shows    signs of “centrilobular    necrosis” iii)  long standin...
<ul><li>Cardiac Transplantation </li></ul><ul><li>2 major causes: </li></ul><ul><li>a)  DCM </li></ul><ul><li>b)  IHD </li...
b)   infections c)  malignancies i)  lymphomas -  Ebstein-Barr virus www.freelivedoctor.com
Upcoming SlideShare
Loading in...5
×

Congenital heart disease heart failure

4,105

Published on

Transcript of "Congenital heart disease heart failure"

  1. 1. <ul><li>Congenital Heart Disease </li></ul><ul><li>Most common type of heart disease </li></ul><ul><li>among children </li></ul><ul><li>a) ~ 1% of live births </li></ul><ul><li>b) most causes unknown </li></ul><ul><li>i) ~ 10% genetic </li></ul><ul><li>- e.g., trisomy 21 (Down syndrome) </li></ul><ul><li>- congenital defect in parent or sibling is greatest risk factor </li></ul>Tricuspid Atresia Michael P. D'Alessandro, M.D. Peer Review Status: Internally Peer Reviewed www.freelivedoctor.com
  2. 2. <ul><li>Types: </li></ul><ul><li>a) L to R shunt </li></ul><ul><li>b) R to L shunt </li></ul><ul><li>c) obstructions </li></ul><ul><li>1.- L to R shunts </li></ul><ul><li>a) ASD, VSD, PDA </li></ul><ul><li>i)  pulmonary blood flow (ASD) </li></ul><ul><li>- NO cyanosis </li></ul><ul><li>b)  RV pressures and Vol. (VSD,PDA) </li></ul><ul><li>i) hypertrophy </li></ul><ul><li>ii)  PVR (vasoconstriction) </li></ul><ul><li>- to prevent edema </li></ul>www.freelivedoctor.com
  3. 3. c) Over time PVR  to that of SVR i) reverses shunt (cyanosis) 2.- R to L shunt a)  pulmonary blood flow i) Cyanosis “blueness” of skin b) examples: i) tetralogy of Fallot ii) great vessel transposition iii) truncus arteriosus iv) tricuspid atresia v) anomalous pulmonary venous connection www.freelivedoctor.com
  4. 4. c) long standing cyanosis is associated with “clubbing” of the tips of the fingers and toes www.freelivedoctor.com
  5. 5. 3.- Obstructions (of flow) a) coarctation of the aorta b) valvular stenosis i) aortic ii) pulmonary c) complete obstruction is called “Atresia” www.freelivedoctor.com
  6. 6. <ul><li>Left to Right Shunts </li></ul><ul><li>Most common: </li></ul><ul><li>a) VSD, ASD, PDA and AVSD </li></ul>www.freelivedoctor.com
  7. 7. i) VSD most common - close spontaneously (50%) ii) ASD usually not symptomatic before 30 yrs www.freelivedoctor.com
  8. 8. iii) DA remains open after birth - ~ 90% occur as isolated anomaly - reversal of flow with  PVR causes cyanosis - PGE will maintain DA cardiac defects such as obstructive disease iv) complete atrioventricular canal defect - all 4 chambers freely communicate (Down syndrome) www.freelivedoctor.com
  9. 9. <ul><li>Right to Left Shunts </li></ul><ul><li>Tetralogy of Fallot </li></ul><ul><li>a) Most common form of cyanotic congenital heart disease </li></ul><ul><li>Defects: </li></ul><ul><li>a) VSD </li></ul><ul><li>b) Pulmonary artery stenosis </li></ul><ul><li>i) determines clinical outcome </li></ul><ul><li>c) aorta that overrides VSD </li></ul><ul><li>d) RV hypertrophy </li></ul>www.freelivedoctor.com
  10. 10.     www.freelivedoctor.com
  11. 11. Tetralogy of Fallot (TOF) Michael P. D'Alessandro, M.D. Peer Review Status: Internally Peer Reviewed <ul><li>Clinical Presentation: a) The onset and degree of cyanosis depends on: </li></ul><ul><li>i) severity of the pulmonary obstruction </li></ul><ul><li>ii) the size of the shunt. </li></ul><ul><li>b) Cyanosis is usually not seen until 3- 6 months of age. </li></ul><ul><li>c) The cyanosis is due to right ventricle outflow obstruction causing unoxygenated blood through the VSD. </li></ul>www.freelivedoctor.com
  12. 12. <ul><li>Transposition of the Great Arteries </li></ul><ul><li>(TGA) </li></ul><ul><li>a) aorta arises from RV </li></ul><ul><li>i) is anterior and to the right of the pulmonary artery </li></ul><ul><li>b) pulmonary artery arises from LV </li></ul><ul><li>c) total separation of pulmonary and systemic circulations </li></ul><ul><li>i) need a shunt to survive following birth </li></ul><ul><li>- VSD  stable shunt </li></ul>www.freelivedoctor.com
  13. 13. - PDA, foramen ovale  unstable shunt (close quickly after birth)  need surgery <ul><li>the aorta originates from the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs. </li></ul><ul><li>the pulmonary artery originates from the left ventricle, so that most of the blood returning from the lungs goes back to the lungs again </li></ul>www.freelivedoctor.com
  14. 14. www.freelivedoctor.com
  15. 15. www.freelivedoctor.com
  16. 16. <ul><li>Truncus Arteriosus </li></ul><ul><li>Failure of separation into aorta and </li></ul><ul><li>pulmonary artery </li></ul><ul><li>a) results in single great artery </li></ul><ul><li>i) receives blood from both ventricles </li></ul><ul><li>- early systemic cyanosis </li></ul><ul><li>b) accompanying VSD </li></ul><ul><li>c)  pulmonary blood flow </li></ul><ul><li>i) danger of irreversible pulmonary hypertension </li></ul>www.freelivedoctor.com
  17. 17. www.freelivedoctor.com
  18. 18. <ul><li>Tricuspid Atresia </li></ul><ul><li>Clinical Presentation: a) Cyanosis is almost always present at birth and is progressive. </li></ul><ul><li>b) Etiology/Pathophysiology: i) Due to absence of the tricuspid valve. This leads to an interatrial right to left shunt, usually through a patent foramen ovale. </li></ul>www.freelivedoctor.com
  19. 19. ii) It is classified by the absence of tricuspid valve, pulmonary stenosis, and VSD. iii) The most Common form is also associated With a hypoplastic right ventricle www.freelivedoctor.com
  20. 20. TRICUSPID ATRESIA 1 - atrial septal defect 2 - absent tricuspid valve 3 - ventricular septal defect Blood is shunted through an atrial septal defect to the left atrium and through the ventricular septal defect to the pulmonary artery. The shaded arrows indicate mixing of the blood. www.freelivedoctor.com
  21. 21. <ul><li>Total Anomalous Pulmonary Venous Connection (TAPVC) </li></ul><ul><li>No pulmonary veins directly join LA </li></ul><ul><li>a) drain into left innominate vein or coronary sinus </li></ul><ul><li>b) PV drain into RA </li></ul><ul><li>ASD or foramen ovale always present </li></ul><ul><li>a) allows PV blood to enter LA </li></ul><ul><li>b) R to L shunt </li></ul><ul><li>Volume and pressure hypertrophy of RV </li></ul>www.freelivedoctor.com
  22. 22. 1 - superior vena cava 2 - atrial septal defect 3 - left innominate vein 4 - pulmonary veins Oxygenated blood returning from the lungs is routed back into the superior vena cava, rather than the left atrium. The presence of an atrial septal defect is necessary to allow partially oxygenated blood to reach the left side of the heart. Total Anomalous Pulmonary Venous Connection (TAPVC) www.freelivedoctor.com
  23. 23. <ul><li>Obsttructive Congenital Anomalies </li></ul><ul><li>Coarctation of the Aorta </li></ul><ul><li>Narrowing </li></ul><ul><li>Males 2:1 vs. female </li></ul><ul><li>a) females with Turners frequently have coarctation </li></ul><ul><li>2 types: </li></ul><ul><li>a) infantile (with PDA; poor outcome) </li></ul><ul><li>i) prior to PDA </li></ul><ul><li>- symptoms early in life </li></ul><ul><li>- cyanosis of lower body </li></ul>www.freelivedoctor.com
  24. 24. <ul><li>b) adult (without PDA) </li></ul><ul><li>i) most children asymptomatic </li></ul><ul><li> until late in life </li></ul><ul><li>ii) hypertension in upper extremities </li></ul><ul><li>iii) hypotension in lower extremities </li></ul><ul><li>Murmurs </li></ul><ul><li>a) throughout systole </li></ul><ul><li>LV hypertrophy </li></ul>www.freelivedoctor.com
  25. 25. 1 - pinched or coarcted aorta flow patterns are normal but are reduced below the coarctation. Blood pressure is increased in vessels leaving the aorta above the coarctation. The broken white arrow indicates diminished blood flow through the aorta. www.freelivedoctor.com
  26. 26. www.freelivedoctor.com
  27. 27. <ul><li>Pulmonary Stenosis and Atresia </li></ul><ul><li>Obstruction at pulmonary valve </li></ul><ul><li>May occur as isolated defect or with: </li></ul><ul><li>a) Tetralogy of Fallot or </li></ul><ul><li>b) TGA </li></ul><ul><li>RV hypertrophy </li></ul><ul><li>a) usually there is poststenotic dilation </li></ul><ul><li>i) “jet” streaming </li></ul><ul><li>Relatively frequent defect </li></ul>www.freelivedoctor.com
  28. 28. <ul><li>Atresia of valve </li></ul><ul><li>a) no communication between RV and lungs </li></ul><ul><li>b) hypoplastic RV with ASD </li></ul><ul><li>c) flow enters lung through PDA </li></ul><ul><li>Aortic Stenosis and Atresia </li></ul><ul><li>3 types: </li></ul><ul><li>a) valvular </li></ul><ul><li>b) subvalvular </li></ul><ul><li>c) supravalvular </li></ul><ul><li>Systolic murmur </li></ul><ul><li>LV hypertrophy </li></ul>www.freelivedoctor.com
  29. 29. <ul><li>Valvular (severe) aortic stenosis or </li></ul><ul><li>atresia </li></ul><ul><li>a) outflow obstruction </li></ul><ul><li>i) hypoplastic LV and ascending aorta </li></ul><ul><li>b) PDA MUST be present to allow blood flow to: </li></ul><ul><li>i) aorta </li></ul><ul><li>ii) coronary arteries </li></ul><ul><li>iii) always fatal in first week of life </li></ul><ul><li>- when ductus closes !! </li></ul>www.freelivedoctor.com
  30. 30. Aortic stenosis (valvular) 1 - narrowed aortic valve Flow patterns are normal but blood flow to the aorta is reduced as indicated by the broken white arrows www.freelivedoctor.com
  31. 31. <ul><li>Subaortic stenosis </li></ul><ul><li>a) thickened ring below level of cusps </li></ul><ul><li>Supraventricular aortic stenosis </li></ul><ul><li>a) inherited defect </li></ul><ul><li>i) mutation of elastin gene causes this defect </li></ul><ul><li>b) ascending aorta is dysplastic </li></ul><ul><li>i) greatly thickened, causing </li></ul><ul><li>- narrowing of lumen </li></ul>www.freelivedoctor.com
  32. 32. Subaortic Stenosis www.freelivedoctor.com
  33. 33. www.freelivedoctor.com
  34. 34. <ul><li>Heart Failure (i.e.,Congestive) </li></ul><ul><li>Heart cannot eject volume which is </li></ul><ul><li>being returned to it </li></ul><ul><li>a) failure to perfuse tissues effectively </li></ul><ul><li>b) usually slowly progressive </li></ul><ul><li>i) acute forms </li></ul><ul><li>- MI </li></ul><ul><li>- Volume overload </li></ul><ul><li>- valvular dysfunction </li></ul>www.freelivedoctor.com
  35. 35. <ul><li>Compensatory mechanisms: </li></ul><ul><li>a) Frank-Starling mechanism </li></ul><ul><li>i)  stretch   contraction </li></ul><ul><li>b) cardiac structural changes </li></ul><ul><li>i) hypertrophy </li></ul><ul><li>c) activation of neurohumoral system </li></ul><ul><li>i)  sympathetic tone (NE) </li></ul><ul><li>-  HR </li></ul><ul><li>-  contractility </li></ul><ul><li>ii)  renin-angiotensin- aldosterone system </li></ul><ul><li>iii)  ANP </li></ul>www.freelivedoctor.com
  36. 36. <ul><li>Most frequent causes for failure of </li></ul><ul><li>these compensatory mechanisms are: </li></ul><ul><li>a) ischemia </li></ul><ul><li>b) hypertension </li></ul><ul><li>c) whatever the reason for the failure CHF  </li></ul><ul><li>i)  CO (systolic failure) or, </li></ul><ul><li>ii)  CO (diastolic failure) </li></ul>www.freelivedoctor.com
  37. 37. <ul><li>Cardiac Hypertrophy </li></ul><ul><li>a) cardiac myocyte is terminally differentiated cell </li></ul><ul><li>i) no  in cell # (i.e., hyperplasia) </li></ul><ul><li>ii)  load on cells causes cells to  in size (i.e., hypertrophy) </li></ul><ul><li>- pressure or volume overload </li></ul><ul><li>- trophic hormones (e.g., T 4 ) </li></ul><ul><li>b) pressure overload </li></ul><ul><li>i) “concentric hypertrophy” </li></ul>www.freelivedoctor.com
  38. 38. ii)  wall thickness iii)  cavity diameter c) volume overload i) “eccentric hypertrophy” ii) chamber dilation iii)  ventricular diameter iv) wall thickness may not change - owing to simultaneous hypertrophy d)  capillary density with hypertrophy i)  demands  ischemia !! www.freelivedoctor.com
  39. 39. <ul><li>Left Sided Heart Failure </li></ul><ul><li>Most often caused by: </li></ul><ul><li>a) ischemic heart disease </li></ul><ul><li>b) hypertension </li></ul><ul><li>c) aortic and mitral valve disease </li></ul><ul><li>d) nonischemic myocardial disease </li></ul><ul><li>Major effect is “damming” of blood </li></ul><ul><li>within the lungs </li></ul><ul><li>LV hypertrophy and often dilation </li></ul><ul><li>LA secondary dilation </li></ul><ul><li>a) stasis, arrhythmia, thrombus </li></ul>www.freelivedoctor.com
  40. 40. <ul><li>Lungs major problem </li></ul><ul><li>a)  pulmonary vein pressure </li></ul><ul><li>i) pulmonary congestion and edema </li></ul><ul><li>- heavy and wet </li></ul><ul><li>b) iron containing proteins in edema fluid and Hb from RBC </li></ul><ul><li>i) phagocytosed by macrophages </li></ul><ul><li> - converted to “Hemosiderin” </li></ul><ul><li>- “heart failure cells” </li></ul><ul><li>c) dyspnea </li></ul><ul><li>i) early sign </li></ul>www.freelivedoctor.com
  41. 41. <ul><li>d) orthopnea </li></ul><ul><li>i) dyspnea on lying down and relieved by sitting upright </li></ul><ul><li>e) cough is common with Left sided failure </li></ul><ul><li>Kidneys </li></ul><ul><li>a)  CO </li></ul><ul><li>b) renin-angiotensin-aldosterone </li></ul><ul><li>i) retention of salt and water </li></ul><ul><li>- contribute to edema </li></ul><ul><li>- counteracted by ANP </li></ul>www.freelivedoctor.com
  42. 42. <ul><li>Right Sided Heart Failure </li></ul><ul><li>Major cause is LEFT heart failure </li></ul><ul><li>Occurs as isolated failure in only few </li></ul><ul><li>diseases </li></ul><ul><li>a) chronic pulmonary hypertension </li></ul><ul><li>i) “ Cor Pulmonale” </li></ul><ul><li>Minimal pulmonary congestion </li></ul><ul><li>Major areas of edema are systemic, </li></ul><ul><li>peripheral </li></ul><ul><li>a) liver and portal system </li></ul><ul><li>i) “congestive hepatomegaly” </li></ul>www.freelivedoctor.com
  43. 43. ii) when Left heart failure is present, hypoxic liver shows signs of “centrilobular necrosis” iii) long standing Right failure - cardiac cirrhosis iv) portal hypertension - congestive splenomegaly v) ascites vi) pleural and pericardial effusions accompany Right heart failure www.freelivedoctor.com
  44. 44. <ul><li>Cardiac Transplantation </li></ul><ul><li>2 major causes: </li></ul><ul><li>a) DCM </li></ul><ul><li>b) IHD </li></ul><ul><li>Major success due to: </li></ul><ul><li>a) careful selection of patients </li></ul><ul><li>b) drug therapy </li></ul><ul><li>c) sequential biopsies </li></ul><ul><li>Major problems </li></ul><ul><li>a) stenosis (coronary arteries) </li></ul><ul><li>i) silent MI (no pain !) </li></ul><ul><li>- CHF or sudden death </li></ul>www.freelivedoctor.com
  45. 45. b) infections c) malignancies i) lymphomas - Ebstein-Barr virus www.freelivedoctor.com
  1. A particular slide catching your eye?

    Clipping is a handy way to collect important slides you want to go back to later.

×