Fetal Circulation

Normal Heart

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

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

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

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

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?

Physiologic Categories of Congenital Heart Disease

• Left-to-right shunt

• Right-to-left shunt

• Admixture lesions

• Obstructive lesions

Imaging CHD

• Echocardiography

• Cardiac Catheterization

• CT

• MRI

• CXR

Small Muscular Ventricular Septal Defect

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

• 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

• Antibiotic Prophylaxis Against Bacterial Endocarditis During Times Of Risk (e.g. with dental work)

• Surgical Or Transcatheter Closure Not Indicated

Small VSD – Treatment Options

Large Perimembranous Ventricular Septal Defect

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

• 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

• Diuretics

• Afterload Reduction

• Inotropes

• Prophylaxis Against Endocarditis

• Surgical Closure

• Transcatheter Occlusion (Experimental)

Large VSD – Treatment Options

Secundum Atrial Septal Defect

• Right Heart Failure

• Pulmonary Hypertension

• Atrial Arrhythmias

• Premature Death

ASD – Subsequent Course/Complications

• Surgical Closure

• Transcatheter Occlusion

ASD – Treatment Options

Amplatz Atrial Septal Defect Occluder

Patent Ductus Arteriosus

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

• Prolonged Ventilator Course• Intraventricular Hemorrhage• Necrotizing Enterocolitis• Contributor To Neonatal Mortality & Morbidity

PDA in the Premature Neonate –Subsequent Course/Complications

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

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

• 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

Amplatz Ductal Occluder Device

Atrioventricular Septal Defect

• 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

Tetralogy of Fallot

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

• Chronic Progressive Cyanosis• Polycythemia, Stroke, Brain Abscess• Exercise Intolerance• Hypercyanotic Episodes• Endocarditis Risk• Premature Death

Tetralogy of Fallot – Subsequent Course/Complications

• 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

Transposition of the Great Arteries

• Progressive Hypoxemia

• Acidosis

• Death in Infancy

Transposition of the Great Arteries – Subsequent Course/Complications

• Prostaglandin E1

• Balloon Atrial Septostomy

• Arterial Switch Operation

• Atrial Baffle Operations (e.g. Senning, Mustard) – Historical Interest

Transposition of the Great Arteries – Treatment Options

• 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

Pulmonary Valve Stenosis

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

• Endocarditis Risk

• Mild Cases Often Remain Mild, Asx, And Have Normal Longevity

• Progressive Right Heart Failure If Severe

Pulmonary Valve Stenosis – Subsequent Course/Complications

• Prophylaxis Against Endocarditis

• No Definitive Intervention If Mild

• Balloon Pulmonary Valvuloplasty

• Surgical Valvotomy Or Valvectomy

Pulmonary Valve Stenosis – Treatment Options

Aortic Valve Stenosis

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

• 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

• 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

Coarctation of the Aorta

“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

• 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

• Inotropes, Diuretics, Antihypertensives, Prostaglandin E1 (Neonates) For Stabilization Of CHF

• Prophylaxis Against Endocarditis

• Surgical Repair

• Balloon Aortoplasty (Debatable)

Coarctation of the Aorta – Treatment Options

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)

• 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

Total Anomalous Pulmonary Venous Connection –Without Obstruction

Total Anomalous Pulmonary Venous Connection - Obstructed

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

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

Medical Stabilization

• Diuretics

• Positive Pressure Ventilation (if veins obstructed)

Surgical Repair

TAPVC – Treatment Options

Hypoplastic Left Heart Syndrome

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

• Shock• Acidosis• Neonatal Death (When Ductus Closes)

Hypoplastic Left Heart Syndrome – Subsequent Course/Complications

• Comfort Measures Only

• Prostaglandin E1

• Palliative Reconstruction (Norwood)

• Heart Transplant

Hypoplastic Left Heart Syndrome – Treatment Options

Hypoplastic Left Heart Syndrome After Stage I Palliation

Hypoplastic Left Heart Syndrome After Stage II Palliation

Hypoplastic Left Heart Syndrome After Stage III Palliation

Pulmonary Atresia - Intact Ventricular Septum

Pulmonary Atresia - Intact Ventricular Septum With RV-Coronary Sinusoids

Polysplenia:TAPVRInterrupted IVCAz ContinuationMitral AtresiaLV HypoplasiaDORV

Mitral AtresiaTransposition of the Great ArteriesDouble Outlet Right VentriclePulmonary StenosisLeft SVC to LA

Helex Device

CardioSEAL