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Congenital Heart Disease Part I. By Katrice L. Herndon, M.D. Acyanotic Congenital Heart Disease. Left-to-Right Shunt Lesions Atrial Septal Defect (ASD) Ventricular Septal Defect (VSD) Atrioventricular Septal Defect (AV Canal) Patent Ductus Arteriosus (PDA). Atrial Septal Defect.

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congenital heart disease part i

Congenital Heart DiseasePart I


Katrice L. Herndon, M.D.

acyanotic congenital heart disease
Acyanotic Congenital Heart Disease

Left-to-Right Shunt Lesions

  • Atrial Septal Defect (ASD)
  • Ventricular Septal Defect (VSD)
  • Atrioventricular Septal Defect (AV Canal)
  • Patent Ductus Arteriosus (PDA)
atrial septal defect
Atrial Septal Defect
  • ASD is an opening in the atrial septum permitting free communication of blood between the atria. Seen in 10% of all CHD.
atrial septal defect1
Atrial Septal Defect
  • There are 3 major types:
  • Secundum ASD – at the Fossa Ovalis, most common.

•Primum ASD – lower in position & is a form of ASVD, MV cleft.

•Sinus VenosusASD – high in the atrial septum, associated w/partial anomalous venous return & the least common.

atrial septal defect3
Atrial Septal Defect

Clinical Signs & Symptoms

  • Rarely presents with signs of CHF or other cardiovascular symptoms.

• Most are asymptomatic but may have easy fatigability or mild growth failure.

• Cyanosis does not occur unless pulmonary HTN is present.

atrial septal defect4
Atrial Septal Defect

Clinical Signs & Symptoms

• Hyperactive precordium, RV heave, fixed widely split S2.

• II-III/VI systolic ejection murmur @ LSB.

• Mid-diastolic murmur heard over LLSB.

atrial septal defect5
Atrial Septal Defect
  • Question:

What causes the systolic & diastolic murmurs of ASD?

• Answer:

Systolic murmur is caused by increased flow across the pulmonary valve, NOT THE ASD.

Diastolic murmur is caused by increased flow across the tricupsid valve & this suggest high flow Qp:Qs is 2:1.

atrial septal defect6
Atrial Septal Defect


  • Surgical or catherization laboratory closure is generally recommended for secundum ASD w/ a Qp:Qs ratio >2:1.

• Closure is performed electively between ages 2 & 5 yrs to avoid late complications.

• Surgical correction is done earlier in children w/ CHF or significant Pulm HTN.

atrial septal defect7
Atrial Septal Defect


• Once pulmonary HTN w/ shunt reversal occurs this is considered too late.

• Mortality is < 1%.

atrial septal defect8
Atrial Septal Defect
  • Question:

Is endocarditis prophylaxis required for




ventricular septal defect
Ventricular Septal Defect
  • VSD – is an abnormal opening in the ventricular septum, which allows free communication between the Rt & Lt ventricles. Accounts for 25% of CHD.
ventricular septal defect1
Ventricular Septal Defect
  • 4 Types
  • Perimembranous (or membranous) – Most common.
  • Infundibular (subpulmonary or supracristal VSD) – involves the RV outflow tract.

•Muscular VSD – can be single or multiple.

• AVSD – inlet VSD, almost always involves AV valvular abnormalities.

ventricular septal defect2
Ventricular Septal Defect


  • The left to right shunt occurs secondary to PVR being < SVR, not the higher pressure in the LV.
  • This leads to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV.
ventricular septal defect3
Ventricular Septal Defect

Clinical Signs & Symptoms

• Small - moderate VSD, 3-6mm, are usually

asymptomatic and 50% will close spontaneously

by age 2yrs.

• Moderate – large VSD, almost always have

symptoms and will require surgical repair.

ventricular septal defect4
Ventricular Septal Defect

Clinical Signs & Symptoms

• II-III/VI harsh holosystolic murmur heard along the LSB, more prominent with small VSD, maybe absent with a

very Large VSD.

• Prominent P2, Diastolic murmur.

• CHF, FTT, Respiratory infections, exercise intolerance

hyperactive precordium. Symptoms develop between 1 – 6


ventricular septal defect5
Ventricular Septal Defect


• Small VSD - no surgical intervention, no

physical restrictions, just reassurance and

periodic follow-up and endocarditis prophylaxis.

• Symptomatic VSD - Medical treatment

initially with afterload reducers & diuretics.

ventricular septal defect6
Ventricular Septal Defect


  • Indications for Surgical Closure:
  • Large VSD w/ medically uncontrolled symptomatology & continued FTT.
  • Ages 6-12 mo w/ large VSD & Pulm. HTN
  • Age > 24 mo w/ Qp:Qs ratio > 2:1.
  • Supracristal VSD of any size, secondary to risk of developing AV insufficiency.
atrioventricular septal defect
Atrioventricular Septal Defect
  • AVSD results from incomplete fusion the the endocardial cushions, which help to form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the triscupid and mitral valves.
  • They account for 4% OF ALL CHD.
atrioventricular septal defect1
Atrioventricular Septal Defect
  • Question:

What genetic disease is AVSD more

commonly seen in?


Down’s Syndrome (Trisomy 21), Seen in 20-25% of cases.

atrioventricular septal defect2
Complete Form

Low primum ASD continuous with a posterior VSD.

Cleft in both septal leaflets of TV/MV.

Results in a large L to R shunt at both levels.

TR/MR, Pulm HTN w/ increase in PVR.

Incomplete Form

Any one of the components may be present.

Most common is primum ASD, cleft in the MV & small VSD.

Hemodynamics are dependent on the lesions.

Atrioventricular Septal Defect
atrioventricular septal defect4
Atrioventricular Septal Defect

Clinical Signs & Symptoms

  • Incomplete AVSD maybe indistinguishable from ASD - usually asymptomatic.
  • Congestive heart failure in infancy.
  • Recurrent pulmonary infections.
  • Failure to thrive.
  • Exercise intolerance, easy fatigability.
  • Late cyanosis from pulmonary vascular disease w/ R to L shunt.
atrioventricular septal defect5
Atrioventricular Septal Defect

Clinical Signs & Symptoms

  • Hyperactive precordium
  • Normal or accentuated 1st hrt sound
  • Wide, fixed splitting of S2
  • Pulmonary systolic ejection murmur w/thrill
  • Holosystolic murmur @ apex w/radiation to axilla
  • Mid-diastolic rumbling murmur @ LSB
  • Marked cardiac enlargement on CX-Ray
atrioventricular septal defect6
Atrioventricular Septal Defect


  • Surgery is always required.
  • Treat congestive symptoms.
  • Pulmonary banding maybe required in premature infants or infants < 5 kg.
  • Correction is done during infancy to avoid irreversible pulmonary vascular disease.
  • Mortality low w/incomplete 1-2% & as high as 5% with complete AVSD.
patent ductus arteriosus
Patent Ductus Arteriosus
  • PDA – Persistence of the normal fetal vessel that joins the PA to the Aorta.
  • Normally closes in the 1st wk of life.
  • Accounts for 10% of all CHD, seen in 10% of other congenital hrt lesions and can often play a critical role in some lesions.
  • Female : Male ratio of 2:1
  • Often associated w/ coarctation & VSD.
patent ductus arteriosus1
Patent Ductus Arteriosus
  • Question:

What TORCH infection is PDA associated with?



patent ductus arteriosus2
Patent Ductus Arteriosus


  • As a result of higher aortic pressure, blood shunts L to R through the ductus from Aorta to PA.
  • Extent of the shunt depends on size of the ductus & PVR:SVR.
  • Small PDA, pressures in PA, RV, RA are normal.
patent ductus arteriosus3
Patent Ductus Arteriosus


  • Large PDA, PA pressures are equal to systemic pressures. In extreme cases 70% of CO is shunted through the ductus to pulmonary circulation.
  • Leads to increased pulmonary vascular disease.
patent ductus arteriosus4
Patent Ductus Arteriosus

Clinical Signs & Symptoms

  • Small PDA’s are usually asymptomatic
  • Large PDA’s can result in symptoms of CHF, growth restriction, FTT.
  • Bounding arterial pulses
  • Widened pulse pressure
  • Enlarged heart, prominent apical impulse
  • Classic continuous machinary systolic murmur
  • Mid-diastolic murmur at the apex
patent ductus arteriosus5
Patent Ductus Arteriosus


  • Indomethacin, inhibitor of prostaglandin synthesis can be used in premature infants.
  • PDA requires surgical or catheter closure.
  • Closure is required treatment heart failure & to prevent pulmonary vascular disease.
  • Usually done by ligation & division or intra vascular coil.
  • Mortality is < 1%
obstructive heart lesions
Obstructive Heart Lesions
  • Pulmonary Stenosis
  • Aortic Stenosis
  • Coarctation of the Aorta
pulmonary stenosis
Pulmonary Stenosis
  • Pulmonary Stenosis is obstruction in the region of either the pulmonary valve or the subpulmonary ventricular outflow tract.
  • Accounts for 7-10% of all CHD.
  • Most cases are isolated lesions
  • Maybe biscuspid or fusion of 2 or more leaflets.
  • Can present w/or w/o an intact ventricular septum.
pulmonary stenosis1
Pulmonary Stenosis
  • Question:

What syndrome is PS associated with?

  • Answer:

Noonan’s Syndrome, secondary to valve dysplasia.

pulmonary stenosis2
Pulmonary Stenosis


  • RV pressure hypertrophy RV failure.
  • RV pressures maybe > systemic pressure.
  • Post-stenotic dilation of main PA.
  • W/intact septum & severe stenosis  R-L shunt through PFO  cyanosis.
  • Cyanosis is indicative of Critical PS.
pulmonary stenosis3
Pulmonary Stenosis

Clinical Signs & Symptoms

  • Depends on the severity of obstruction.
  • Asymptomatic w/ mild PS < 30mmHg.
  • Mod-severe: 30-60mmHg, > 60mmHg
  • Prominent jugular a-wave, RV lift
  • Split 2nd hrt sound w/ a delay
  • Ejection click, followed by systolic murmur.
  • Heart failure & cyanosis seen in severe cases.
pulmonary stenosis4
Pulmonary Stenosis


  • Mild PS no intervention required, close follow-up.
  • Mod-severe – require relieve of stenosis.
  • Balloon valvuloplasty, treatment of choice.
  • Surgical valvotomy is also a consideration.
aortic stenosis
Aortic Stenosis
  • Aortic Stenosis is an obstruction to the outflow from the left ventricle at or near the aortic valve that causes a systolic pressure gradient of more than 10mmHg. Accounts for 7% of CHD.
  • 3 Types
  • Valvular – Most common.
  • Subvalvular(subaortic) – involves the left outflow tract.
  • Supravalvular – involves the ascending aorta is the least common.
aortic stenosis1
Aortic Stenosis
  • Question:

Which syndrome is supravalvular stenosis found in?

  • Answer:

Williams Syndrome

aortic stenosis2
Aortic Stenosis


  • Pressure hypertrophy of the LV and LA with obstruction to flow from the LV.
  • Mild AS 0-25mmHG
  • Moderate AS 25-50mmHg
  • Severe AS 50-75mmHg
  • Critical AS > 75mmHg
aortic stenosis3
Aortic Stenosis

Clinical Signs & Symptoms

  • Mild AS may present with exercise intolerance, easy fatigabiltity, but usually asymptomatic.
  • Moderate AS – Chest pain, dypsnea on exertion, dizziness & syncope.
  • Severe AS – Weak pulses, left sided heart failure, Sudden Death.
aortic stenosis4
Aortic Stenosis

Clinical Signs & Symptoms

  • LV thrust at the Apex.
  • Systolic thrill @ rt base/suprasternal notch.
  • Ejection click, III-IV/VI systolic murmur @ RSB/LSB w/ radiation to the carotids.
aortic stenosis5
Aortic Stenosis


  • Because surgery does not offer a cure it is reserved for patients with symptoms and a resting gradient of 60-80mmHg.
  • For subaortic stenosis it is reserved for gradients of 40-50mmHg because of it’s rapidly progressive nature.
  • Balloon valvuloplasty is the standard of treatment.
aortic stenosis6
Aortic Stenosis


  • Aortic insufficiency & re-stenosis is likely after surgery and may require valve replacement.
  • Activity should not be restricted in Mild AS.
  • Mod-severe AS, no competitive sports.
coarctation of the aorta
Coarctation of the Aorta
  • Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation.
  • 98% of coarctations are juxtaductal
  • Male: Female ratio 3:1.
  • Accounts for 7 % of all CHD.
coarctation of the aorta1
Coarctation of the Aorta
  • Question:

What other heart anomaly is coarctation associated with?

  • Answer:

Bicuspid aortic valve, seen in > 70% of cases.

coarctation of the aorta2
Coarctation of the Aorta
  • Question:

What genetic syndrome is coarctation seen in?

  • Answer:

Turner’s Syndrome

coarctation of the aorta3
Coarctation of the Aorta


  • Obstruction of left ventricular outflow  pressure hypertrophy of the LV.
coarctation of the aorta4
Coarctation of the Aorta

Clinical Signs & Symptoms

  • Classic signs of coarctation are diminution or absence of femoral pulses.
  • Higher BP in the upper extremities as compared to the lower extremities.
  • 90% have systolic hypertension of the upper extremities.
  • Pulse discrepancy between rt & lt arms.
coarctation of the aorta5
Coarctation of the Aorta

Clinical Signs & Symptoms

  • With severe coarc. LE hypoperfusion, acidosis, HF and shock.
  • Differential cyanosis if ductus is still open
  • II/VI systolic ejection murmur @ LSB.
  • Cardiomegaly, rib notching on X-ray.
coarctation of the aorta7
Coarctation of the Aorta


  • With severe coarctation maintaining the ductus with prostaglandin E is essential.
  • Surgical intervention, to prevent LV dysfunction.
  • Angioplasty is used by some centers.
  • Re-coarctation can occur, balloon angioplasty is the procedure of choice.

Examination of a 3-hr old infant reveals

dysmorphic features and cyanosis. Both the

occiput and facial profile are flat, and the

fontanelle is abnormally enlarged. The space

between the great and second toe is wide, and

there is a palmar crease extending across the

left palm. Room air oximetry reveals a saturation



Of the following, the MOST likely lesion to

be found on echocardiography would be

  • Atrioventricular septal defect
  • Coarctation of the aorta
  • Hypoplastic left heart
  • Total anomalous pulmonary venous return
  • Truncus arteriosus

After a few days of poor feeding and

tachypnea, a 3 week old presents with

hypotension, poor central and peripheral

pulses, and severe metabolic acidosis. A

gallop is audible, and the heart appears

enlarged on chest radiography. Hepatomegaly

is marked.


Of the following, the BEST intervention to

produce a sustained improvement is

  • 100% Oxygen administration
  • Dopamine infusion
  • Gamma globulin infusion
  • Phenylephrine infusion
  • Prostaglandin E infusion

A term infant is born with a large ventricular septal

defect. At what age is the infant most likely to first

demonstrate clinical findings of CHF

  • 2 days
  • 2 weeks
  • 2 months
  • 6 months
  • 12 months