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Congenital Heart Disease from the Block (as in J-Lo from the block, pun definitely intended!)

Congenital Heart Disease from the Block (as in J-Lo from the block, pun definitely intended!). Premchand Anne, MD, MPH PGY IV 9/1/2005. Objectives. Fetal and neonatal circulation DA Pathophysiology Left to Right shunts Obstructive Valvular regurgitation Cyanotic congenital heart disease.

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Congenital Heart Disease from the Block (as in J-Lo from the block, pun definitely intended!)

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  1. Congenital Heart Disease from the Block(as in J-Lo from the block, pun definitely intended!) Premchand Anne, MD, MPH PGY IV 9/1/2005

  2. Objectives • Fetal and neonatal circulation • DA • Pathophysiology • Left to Right shunts • Obstructive • Valvular regurgitation • Cyanotic congenital heart disease

  3. Fetal Circulation Fetal Circulation: four shunts • Placenta • Receives 55% of total CO • Lowest vascular resistance • Ductus venosus • From placenta: has 70% sats • Highest PO2: umbilical vein (30) • Foramen ovale • 1/3 of RA return goes to LA • Oxygenate brain and coronaries better (PO2=28) • Lower body: PO2=24 • Ductus arteriosus • PA => Descending Aorta => placenta

  4. Neonatal Circulation • Change in gas exchange from placenta to lungs • Increase in systemic vascular resistance due to absence of placenta and closure of DA due to presence of increased PO2 • Reduction of PVR after lung expansion due to oxygen, increased LA return, fall in RA pressure due to DA closure and increase in systemic pressure => closure of foramen ovale • Rapid initial fall, slower fall by 6-8 weeks and then after 2 years

  5. Objectives • Fetal and neonatal circulation • DA • Pathophysiology • Left to Right shunts • Obstructive lesions • Valvular regurgitation • Cyanotic congenital heart disease

  6. DA closure • Within 10-15 hours of birth • Postnatal increase in Oxygen is the strongest stimulus for closure; premies don’t respond as well to oxygen. • PGE2 decreases after birth due to loss of placenta and increased pulmonary blood flow to wash it off. • Indomethacin closes PDAs • Maternal ingestion of ASA can cause Persistent Pulmonary Hypertension of the Newborn (premature closure of DA and poor development of arterioles) • PGE1 keeps DA open.

  7. Pulmonary arteries and DA respond in opposite manner

  8. Objectives • Fetal and neonatal circulation • DA • Pathophysiology • Left to Right shunts • Obstructive lesions • Valvular regurgitation • Cyanotic congenital heart disease

  9. Left to Right Shunts • ASD • VSD • PDA • ECD

  10. Left to Right Shunts-ASD

  11. ASD • RA and RV dilated • RV dilation => increased time for repolarization => RBBB on EKG • NO CHF until 6-8 weeks when PVR drops considerably.

  12. ASD

  13. Left to Right Shunts-VSD

  14. VSD • LA and LV dilated in a small to moderate VSD: does this make sense? • Shunt occurs only during systole, where the blood goes from LV to pulmonary artery • There is increased pulmonary return to LA and then to LV => dilation • RV, LA, LV are all dilated in a large size VSD • Complication of large VSD=Eisenmenger’s => generalized cyanosis

  15. VSD

  16. Left to Right Shunts-PDA

  17. PDA • Onset of CHF is similar to that of a VSD • Complication of a large PDA=> Eisenmenger’s => differential cyanosis (lower body cyanosis): WHY?

  18. PDA

  19. ECD • Endocardial cushion is responsible for upper part of ventricular septum and lower part of atrial septum • Absence leads to VSD, primum ASD, clefts in mitral and tricuspid valves

  20. Left to Right Shunts-ECD

  21. ECD • Primum ASD = Secundum ASD; RA and RV are dilated with widely split and fixed S2 and systolic ejection murmur at left upper sternal border. • RBBB due to prolonged repolarization • Obligatory shunt with LV -> RA lesion

  22. ECD

  23. Objectives • Fetal and neonatal circulation • DA • Pathophysiology • Left to Right shunts • Obstructive lesions • Valvular regurgitation • Cyanotic congenital heart disease

  24. Obstructive Lesions • Ventricular Outflow obstruction • Aortic stenosis • Pulmonary stenosis • Coarctation of the aorta • Stenosis of AV valves • Mitral stenosis • Tricuspid stenosis

  25. Ventricular Outflow ObstructionAS, PS, COA • All three lesions produce the following: • Ejection systolic murmur • Hypertrophy of the respective ventricle • Post-stenotic dilatation is present with the obstruction at the valvular level; absent in subvalvular stenosis

  26. Aortic Stenosis • Murmur is loudest in RUSB • Loudness is proportional to severity • LVH

  27. Pulmonary Stenosis • Murmur is loudest at LUSB • Loudness is proportional to severity • RVH

  28. Coarctation of Aorta • Ejection type SEM over the descending aorta, distal to COA • Often see Bicuspid aortic valves • Delayed or absent pulses in LE • Post stenotic dilation => figure-of-3 sign on xray • Lesion is juxtaductal • Symptomatic patients have a VSD and may see RVH and RBBB rather than LVH

  29. A-V valve obstructionMitral Stenosis • More often rheumatic than congenital • Diastolic murmur due to pressure gradient between LA and LV; subsequently LA, Pulmonary veins, RV dilation; best at apex • Pulmonary edema if hydrostatic > osmotic pressure; dyspnea with or without exertion. • Loud S1 due to widely parted MV leaflets at onset of systole due to prolongation of diastole • Dilated LA leads to A-fib

  30. A-V valve obstructionTricuspid Stenosis • Rare and usually congenital • Dilation and hypertrophy of RA • If severe, can lead to hepatomegaly and JVD. • (+) mid diastolic murmur

  31. Objectives • Fetal and neonatal circulation • DA • Pathophysiology • Left to Right shunts • Obstructive lesions • Valvular regurgitation • Cyanotic congenital heart disease

  32. Valvular Regurgitation • Mitral regurgitation • Tricuspid regurgitation • Aortic regurgitation • Pulmonary regurgitation

  33. Mitral Regurgitation • Volume overload of the LA and LV with LVH and LAH on EKG • Regurgitant systolic murmur at the apex • (+) S3 due to rapid early diastole due to fluid overload of LA • Pulmonary hypertension occurs occasionally due to dampening of pressure by dilated LA

  34. Mitral Regurgitation

  35. Tricuspid Regurgitation • RV and RA enlarge • RAH and RVH with RBBB on EKG • Systolic regurgitant murmur with S3 in tricuspid area. • Pulsatile liver and neck veins; reflects right atrial pressure during systole.

  36. Aortic Regurgitation • Overload of LV • LV enlargement on xray and LVH on ECG • Wide pulse pressure and bounding peripheral pulse due to rapid drop in BP in the aorta due to leakage • High pitched decrescendo murmur at the apex • AUSTIN FLINT MURMUR due to mitral valve flutter during diastole (blood in opposite directions)

  37. Aortic Regurgitation

  38. Pulmonary Regurgitation • RV enlargement and prominent PA segment • The direction of regurgitation is to the RV, aka along Left sternal border and diastolic.

  39. Objectives • Fetal and neonatal circulation • DA • Pathophysiology • Left to Right shunts • Obstructive lesions • Valvular regurgitation • Cyanotic congenital heart disease

  40. Cyanotic Congenital Heart Lesions

  41. Cyanotic Congenital Heart Lesions • Complete Transposition of the Great Arteries (TGA) • Persistent Truncus Arteriosus and single ventricle • Tetralogy of Fallot • Tricuspid atresia • Pulmonary Atresia • Total Anomalous Pulmonary Venous Return

  42. Complete Transposition of the Great Arteries • D-TGA is the most common cyanotic lesion • Aorta from RV and Pulmonary artery from LV • Normally, aorta is under and over the RPA • In TGA, aorta is to the right of the RPA because of opening to the RV, hence D-TGA • In L-TGA, aorta is to the left of the PA and congenitally corrected TGA • A PFO is normally present in D-TGA

  43. TGA • Newborn is cyanotic, with metabolic acidosis, detrimental to myocardium • Leads to CHF in the first week of life. • Associated with hypoglycemia • Consider TGA if CYANOSIS, CHF WITH CXR FINDINGS, AND NO MURMUR • Must do BALLOON ATRIAL SEPTOSTOMY (RASHKIND PROCEDURE) to increase mixing, if only PFO.

  44. TGA • Small PFO: rashkind procedure • Large VSD: RVH with LV and LA dilation • Corrective procedures • Mustard or Senning Procedure • Jatene Switch

  45. Persistent Truncus Arteriosus and Single Ventricle • Single arterial blood vessel arises from heart in TA, along with a large VSD; PAs come off the TA • In single ventricle, both AV valves empty into the single ventricle, with the aorta or PA coming off the rudimentary ventricle. • Similarities • Complete mixing of systemic pulmonary venous blood in the ventricle • Pressures in both ventricles are identical • Level of oxygen saturation in systemic circulation is dependent on the magnitude of pulmonary blood flow

  46. Tetralogy of Fallot • 4 main findings: • VSD • Overriding Aorta (not always present) • Pulmonic stenosis • RVH (secondary PS) • With mild PS, shunt is left to right, leading to PINK TOF, LV and RV pressures are same • With severe PS, the shunt is right to left, with PBF from PDA

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