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Fetal Circulation
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Normal Heart
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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;10:205-211.
* Ref - Ferencz et al. Am J Epidemiol 1985;121:31-36
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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
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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
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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
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Cardiovascular Exam in the Child with Heart Murmur
The H&P Beyond Auscultation• HistoryDyspnea, cough, “asthma”
Exercise Intolerance (child)
Feeding Difficulties (infant)
DIzziness, syncope
Palpitations
Chest pain
Cyanosis (infant)
• Physical ExamHeight, weight, growth chartBP (upper and lower)Pulses, perfusionColorLiver, spleenBreath soundsPrecordial palpationRR, grunt? flare? retract?HR, regular?
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Physiologic Categories of Congenital Heart Disease
• Left-to-right shunt
• Right-to-left shunt
• Admixture lesions
• Obstructive lesions
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Imaging CHD
• Echocardiography
• Cardiac Catheterization
• CT
• MRI
• CXR
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Small Muscular Ventricular Septal Defect
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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
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• 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
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• Antibiotic Prophylaxis Against Bacterial Endocarditis During Times Of Risk (e.g. with dental work)
• Surgical Or Transcatheter Closure Not Indicated
Small VSD – Treatment Options
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Large Perimembranous Ventricular Septal Defect
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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 rumble @
apex• Increased precordial
activity
Laboratory testing• X-ray – cardiomegaly
with increased pulmonary vascularity
• EKG – LAE, LVH, BVH• Echo for anatomic dx
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• 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
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• Diuretics
• Afterload Reduction
• Inotropes
• Prophylaxis Against Endocarditis
• Surgical Closure
• Transcatheter Occlusion (Experimental)
Large VSD – Treatment Options
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Secundum Atrial Septal Defect
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• Right Heart Failure
• Pulmonary Hypertension
• Atrial Arrhythmias
• Premature Death
ASD – Subsequent Course/Complications
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• Surgical Closure
• Transcatheter Occlusion
ASD – Treatment Options
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Amplatz Atrial Septal Defect Occluder
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Patent Ductus Arteriosus
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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
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• Prolonged Ventilator Course• Intraventricular Hemorrhage• Necrotizing Enterocolitis• Contributor To Neonatal Mortality & Morbidity
PDA in the Premature Neonate –Subsequent Course/Complications
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Large PDA in the Older Child – Clinical Presentation
H & P• Respiratory sx, exercise
intolerance• Continuous murmur @
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
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Small PDA in the Older Child – Clinical Presentation
H & P• Asymptomatic• Continuous murmur @
left upper sternal border
Laboratory testing• X-ray – usually normal• EKG – usually normal• Echo for anatomic dx
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• 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
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Amplatz Ductal Occluder Device
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Atrioventricular Septal Defect
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• 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
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Tetralogy of Fallot
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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
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• Chronic Progressive Cyanosis• Polycythemia, Stroke, Brain Abscess• Exercise Intolerance• Hypercyanotic Episodes• Endocarditis Risk• Premature Death
Tetralogy of Fallot – Subsequent Course/Complications
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• 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
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Transposition of the Great Arteries
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• Progressive Hypoxemia
• Acidosis
• Death in Infancy
Transposition of the Great Arteries – Subsequent Course/Complications
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• 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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• 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
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Pulmonary Valve Stenosis
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Pulmonary Valve Stenosis – Clinical Presentation
H & P• Asymptomatic if
mild/moderate• Exercise intolerance if
severe• Systolic ejection
murmur @ 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
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• Endocarditis Risk
• Mild Cases Often Remain Mild, Asx, And Have Normal Longevity
• Progressive Right Heart Failure If Severe
Pulmonary Valve Stenosis – Subsequent Course/Complications
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• Prophylaxis Against Endocarditis
• No Definitive Intervention If Mild
• Balloon Pulmonary Valvuloplasty
• Surgical Valvotomy Or Valvectomy
Pulmonary Valve Stenosis – Treatment Options
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Aortic Valve Stenosis
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Aortic Valve Stenosis – Clinical Presentation
H & P• Asx if mild/moderate• Exercise intolerance,
angina, syncope, sudden death if severe
• Systolic ejection murmur @ 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
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• 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
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• 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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Coarctation of the Aorta
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“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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• 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
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• Inotropes, Diuretics, Antihypertensives, Prostaglandin E1 (Neonates) For Stabilization Of CHF
• Prophylaxis Against Endocarditis
• Surgical Repair
• Balloon Aortoplasty (Debatable)
Coarctation of the Aorta – Treatment Options
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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)
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• 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
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Total Anomalous Pulmonary Venous Connection –Without Obstruction
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Total Anomalous Pulmonary Venous Connection - Obstructed
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TAPVC – Clinical Presentation
H & P• Respiratory distress
(severe and early if obstructed veins)
• Cyanosis (can be quite mild)
• Systolic murmur @ LUSB
• Diastolic flow rumble @ LLSB
• Increased RV impulse• Poor growth
Laboratory testing• X-ray – cardiomegaly
with increased pulmonary vascularity
• EKG – RVH, RAE, RAD• Echo for anatomic dx
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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
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Medical Stabilization
• Diuretics
• Positive Pressure Ventilation (if veins obstructed)
Surgical Repair
TAPVC – Treatment Options
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Hypoplastic Left Heart Syndrome
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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
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• Shock• Acidosis• Neonatal Death (When Ductus Closes)
Hypoplastic Left Heart Syndrome – Subsequent Course/Complications
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• Comfort Measures Only
• Prostaglandin E1
• Palliative Reconstruction (Norwood)
• Heart Transplant
Hypoplastic Left Heart Syndrome – Treatment Options
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Hypoplastic Left Heart Syndrome After Stage I Palliation
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Hypoplastic Left Heart Syndrome After Stage II Palliation
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Hypoplastic Left Heart Syndrome After Stage III Palliation
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Pulmonary Atresia - Intact Ventricular Septum
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Pulmonary Atresia - Intact Ventricular Septum With RV-Coronary Sinusoids
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Polysplenia:TAPVRInterrupted IVCAz ContinuationMitral AtresiaLV HypoplasiaDORV
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Mitral AtresiaTransposition of the Great ArteriesDouble Outlet Right VentriclePulmonary StenosisLeft SVC to LA
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Helex Device
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CardioSEAL