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Fetal Circulation. Normal Heart Cardiovascular Exam in the Child with Heart Murmur Epidemiology Innocent murmur - 12,050 schoolage children from.

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Presentation on theme: "Fetal Circulation. Normal Heart Cardiovascular Exam in the Child with Heart Murmur Epidemiology Innocent murmur - 12,050 schoolage children from."— Presentation transcript:

1 Fetal Circulation

2 Normal Heart

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5 Cardiovascular Exam in the Child with Heart Murmur Epidemiology Innocent murmur - 12,050 schoolage children from South Africa, 72% had innocent systolic murmur * Ref - MacLaren et al. Br Heart J 1980;43:67-73 Heart disease - 0.8% of liveborn babies have congenital heart defect, 0.4% bad enough to detect before 1st birthday * Ref - Samanek et al. Pediatr Cardiol 1989;10: * Ref - Ferencz et al. Am J Epidemiol 1985;121:31-36

6 Cardiovascular Exam in the Child with Heart Murmur Features of Innocent Murmurs Still’s Murmur * Timing: Systolic ejection * Intensity: 1-3/6 * Location: Several cm lateral to LLSB * Pitch: Low * Character: Vibratory * Helpful Maneuvers: Inspiration, standing

7 Cardiovascular Exam in the Child with Heart Murmur Features of Innocent Murmurs Pulmonary Flow Murmur * Timing: Systolic ejection * Intensity: 1-3/6 * Location: LUSB * Pitch: Low to medium * Character: Blowing * Helpful Maneuvers: Inspiration, standing

8 Cardiovascular Exam in the Child with Heart Murmur Features of Innocent Murmurs Pulmonary Branch Murmur of Infancy * Timing: Systolic ejection * Intensity: 1-3/6 * Location: LUSB, RUSB, to axillae and back * Pitch: Medium * Character: Blowing * Helpful Maneuvers: None

9 Cardiovascular Exam in the Child with Heart Murmur The H&P Beyond Auscultation History Dyspnea, cough, “asthma” Exercise Intolerance (child) Feeding Difficulties (infant) DIzziness, syncope Palpitations Chest pain Cyanosis (infant) Physical Exam Height, weight, growth chart BP (upper and lower) Pulses, perfusion Color Liver, spleen Breath sounds Precordial palpation RR, grunt? flare? retract? HR, regular?

10 Physiologic Categories of Congenital Heart Disease Left-to-right shunt Right-to-left shunt Admixture lesions Obstructive lesions

11 Imaging CHD Echocardiography Cardiac Catheterization CT MRI CXR

12 Small Muscular Ventricular Septal Defect

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14 Small VSD – Clinical Presentation H & P Asx throughout life Holosystolic murmur at left mid-to-lower sternal border Laboratory testing X-ray – normal EKG – normal Echo for anatomic dx

15 Many Close Spontaneously Usually No Complications At Risk For Bacterial Endocarditis (e.g. with dental work) When Small VSD Stays Open Normal Life Expectancy Without Limitations Small VSD – Subsequent Course/Complications

16 Antibiotic Prophylaxis Against Bacterial Endocarditis During Times Of Risk (e.g. with dental work) Surgical Or Transcatheter Closure Not Indicated Small VSD – Treatment Options

17 Large Perimembranous Ventricular Septal Defect

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19 Large VSD – Clinical Presentation H & P Respiratory sx Failure to thrive Low pitched holosystolic murmur at left lower sternal border Increased intensity P2 Diastolic flow apex Increased precordial activity Laboratory testing X-ray – cardiomegaly with increased pulmonary vascularity EKG – LAE, LVH, BVH Echo for anatomic dx

20 Can Get Smaller Or Close Spontaneously Recurrent Pneumonia Chronic Respiratory Sx, Exercise Intolerance Failure to Thrive Pulmonary Vascular Obstructive Disease (Eisenmenger’s) Endocarditis Risk Premature Death Large VSD – Subsequent Course/Complications

21 Diuretics Afterload Reduction Inotropes Prophylaxis Against Endocarditis Surgical Closure Transcatheter Occlusion (Experimental) Large VSD – Treatment Options

22 Secundum Atrial Septal Defect

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24 Right Heart Failure Pulmonary Hypertension Atrial Arrhythmias Premature Death ASD – Subsequent Course/Complications

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26 Surgical Closure Transcatheter Occlusion ASD – Treatment Options

27 Amplatz Atrial Septal Defect Occluder

28 Patent Ductus Arteriosus

29 PDA in the Premature Neonate – Clinical Presentation H & P Respiratory sx, exacerbation of RDS Failure to thrive Not much murmur Bounding pulses Increased precordial activity Laboratory testing X-ray – cardiomegaly with increased pulmonary vascularity EKG – Not very helpful Echo for anatomic dx

30 Prolonged Ventilator Course Intraventricular Hemorrhage Necrotizing Enterocolitis Contributor To Neonatal Mortality & Morbidity PDA in the Premature Neonate – Subsequent Course/Complications

31 Large PDA in the Older Child – Clinical Presentation H & P Respiratory sx, exercise intolerance Continuous left upper sternal border Wide pulse pressure, bounding pulses Increased LV impulse Laboratory testing X-ray – cardiomegaly with increased pulmonary vascular marking EKG – LVH, LAE Echo for anatomic dx

32 Small PDA in the Older Child – Clinical Presentation H & P Asymptomatic Continuous left upper sternal border Laboratory testing X-ray – usually normal EKG – usually normal Echo for anatomic dx

33 Antibiotic Prophylaxis Against Bacterial Endocarditis During Times Of Risk (e.g. with dental work) Indomethacin (Premature Neonates Only) Transcatheter Closure (Older Than Neonates Only) Surgical Ligation PDA - Treatment Options

34 Amplatz Ductal Occluder Device

35 Atrioventricular Septal Defect

36 Definition: deoxygenated blood is delivered to the systemic arterial circulation without first passing through the lungs Examples: tetralogy of Fallot; transposition of the great arteries Classification of Congenital Heart Disease – Right-to-Left Shunts

37 Tetralogy of Fallot

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41 Tetralogy of Fallot – Clinical Presentation H & P Cyanosis (may not be evident at birth) Systolic ejection murmur at left upper sternal border Increased precordial activity Digital clubbing (late) Exercise intolerance/ Squatting behavior (late) Laboratory testing X-ray – often normal, can show “coeur en sabot”, upturned apex, narrow mediastinum, decreased pulmonary vascularity, right aortic arch EKG – RVH, RAD, less often RAE Echo for anatomic dx

42 Chronic Progressive Cyanosis Polycythemia, Stroke, Brain Abscess Exercise Intolerance Hypercyanotic Episodes Endocarditis Risk Premature Death Tetralogy of Fallot – Subsequent Course/Complications

43 Prophylaxis Against Endocarditis Surgical Repair Palliative Systemic To Pulmonary Arterial Shunt (Blalock-Taussig) Palliative Balloon Pulmonary Valvuloplasty Occlusion (Experimental) Beta Blockade (Historical Interest) Tetralogy of Fallot – Treatment Options

44 Transposition of the Great Arteries

45 Progressive Hypoxemia Acidosis Death in Infancy Transposition of the Great Arteries – Subsequent Course/Complications

46 Prostaglandin E1 Balloon Atrial Septostomy Arterial Switch Operation Atrial Baffle Operations (e.g. Senning, Mustard) – Historical Interest Transposition of the Great Arteries – Treatment Options

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48 Definition: blood is impeded by narrowed valves, arteries, or veins, anywhere in the systemic or pulmonary circulations Examples: pulmonary valve stenosis; aortic valve stenosis; coarctation of the aorta Classification of Congenital Heart Disease – Obstructive Lesions

49 Pulmonary Valve Stenosis

50 Pulmonary Valve Stenosis – Clinical Presentation H & P Asymptomatic if mild/moderate Exercise intolerance if severe Systolic ejection left upper sternal border Systolic ejection click Increased right ventricular impulse if moderate/severe Laboratory testing X-ray – normal heart size, prominent MPA, normal distal pulmonary vascularity EKG – RVH if more than mild Echo for anatomic dx and assessment of severity

51 Endocarditis Risk Mild Cases Often Remain Mild, Asx, And Have Normal Longevity Progressive Right Heart Failure If Severe Pulmonary Valve Stenosis – Subsequent Course/Complications

52 Prophylaxis Against Endocarditis No Definitive Intervention If Mild Balloon Pulmonary Valvuloplasty Surgical Valvotomy Or Valvectomy Pulmonary Valve Stenosis – Treatment Options

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54 Aortic Valve Stenosis

55 Aortic Valve Stenosis – Clinical Presentation H & P Asx if mild/moderate Exercise intolerance, angina, syncope, sudden death if severe Systolic ejection right upper sternal border Systolic ejection click Increased LV impulse if moderate/severe Laboratory testing X-ray – cardiomegaly if severe, prominent ascending aorta EKG – LVH if more than mild; ST-T wave inversion if severe Echo for anatomic dx and assessment of severity

56 Endocarditis Risk Can Progress From Mild To Severe Stenosis Aortic Regurgitation Can Develop Congestive Heart Failure, Exercise Intolerance, Angina If Severe Stenosis Sudden Death If Severe Stenosis Aortic Valve Stenosis – Subsequent Course/Complications

57 Prophylaxis Against Endocarditis No Definitive Intervention If Mild Balloon Aortic Valvuloplasty Surgical Valvotomy Or Valve Replacement (Ross Procedure, Mechanical Valve) Aortic Valve Stenosis – Treatment Options

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59 Coarctation of the Aorta

60 “Mild” Coarctation of Aorta – Clinical Presentation H & P Older child Often asx, occ exercise intolerance, headache Upper extremity hypertension Differential pulses and BP (upper>lower extr) Systolic murmur anteriorly, continuous murmur posteriorly Laboratory testing X-ray – cardiomegaly, rib notching, “3-sign” on descending aortic contour EKG – LVH Echo for anatomic dx

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62 Neonatal Death From Shock/CHF If Severe Progressive CHF Later If “Mild” Chronic Respiratory Sx, Exercise Intolerance Deterioration of Bicuspid Aortic Valve Atherosclerotic Heart Disease Stroke Endocarditis (Endarteritis) Risk Premature Death Coarctation of the Aorta – Subsequent Course/Complications

63 Inotropes, Diuretics, Antihypertensives, Prostaglandin E1 (Neonates) For Stabilization Of CHF Prophylaxis Against Endocarditis Surgical Repair Balloon Aortoplasty (Debatable) Coarctation of the Aorta – Treatment Options

64 Congenital Heart Defects- Predisposing Conditions Most sporadic, cause unknown, can cluster in families, generally not Menedelian Some recognizable syndromes (VACTERL, Noonan’s, etc) Some chromosomal (Trisomy 21, 13,18; 45 XO; 22q deletions) Fetal cardiac teratogens (alcohol, lithium, anticonvulsants) Maternal conditions (rubella, diabetes, lupus, phenylketonuria)

65 Definition: Oxygenated and deoxygenated blood mix completely before delivery to the aorta and pulmonary arteries Examples: total anomalous pulmonary venous connection; hypoplastic left heart syndrome Classification of Congenital Heart Disease – Admixture Lesions

66 Total Anomalous Pulmonary Venous Connection – Without Obstruction

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68 Total Anomalous Pulmonary Venous Connection - Obstructed

69 TAPVC – Clinical Presentation H & P Respiratory distress (severe and early if obstructed veins) Cyanosis (can be quite mild) Systolic LUSB Diastolic flow LLSB Increased RV impulse Poor growth Laboratory testing X-ray – cardiomegaly with increased pulmonary vascularity EKG – RVH, RAE, RAD Echo for anatomic dx

70 If Obstructed Severe Resp’y Distress/Pulmonary Edema Shock, Acidosis, Neonatal Death If Unobstructed Chronic Resp’y Sx, Pneumonias Failure to Thrive Pulmonary Hypertension Early Death TAPVC – Subsequent Course/Complications

71 Medical Stabilization Diuretics Positive Pressure Ventilation (if veins obstructed) Surgical Repair TAPVC – Treatment Options

72 Hypoplastic Left Heart Syndrome

73 Hypoplastic Left Heart Syndrome – Clinical Presentation H & P Neonatal presentation Shock Acidosis Oliguria Respiratory distress Systolic murmur Cyanosis Laboratory testing X-ray – cardiomegaly with increased pulmonary vascularity EKG – RVH Sometimes appreciated by prenatal ultrasound Echo for anatomic dx

74 Shock Acidosis Neonatal Death (When Ductus Closes) Hypoplastic Left Heart Syndrome – Subsequent Course/Complications

75 Comfort Measures Only Prostaglandin E1 Palliative Reconstruction (Norwood) Heart Transplant Hypoplastic Left Heart Syndrome – Treatment Options

76 Hypoplastic Left Heart Syndrome After Stage I Palliation

77 Hypoplastic Left Heart Syndrome After Stage II Palliation

78 Hypoplastic Left Heart Syndrome After Stage III Palliation

79 Pulmonary Atresia - Intact Ventricular Septum

80 Pulmonary Atresia - Intact Ventricular Septum With RV-Coronary Sinusoids

81 Polysplenia: TAPVR Interrupted IVC Az Continuation Mitral Atresia LV Hypoplasia DORV

82 Mitral Atresia Transposition of the Great Arteries Double Outlet Right Ventricle Pulmonary Stenosis Left SVC to LA

83 Helex Device

84 CardioSEAL


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