NOTES

NOTES
ACYANOTIC DEFECTS

GENERALLY, WHAT ARE THEY?

Eisenmenger syndrome
PATHOLOGY & CAUSES ▪ At rest: asymptomatic
▪ Heart defects presenting without cyanosis ▪ With exertion: cyanosis, palpitations
(blue-tinged skin) dyspnea, chest pain, syncope
▪ Caused by fetal heart malformation, can
lead to heart failure DIAGNOSIS
▪ ASD, PDA, and VSD
▫ All three cause left-to right shunt → DIAGNOSTIC IMAGING
oxygenated blood flows redundantly ▪ Heart imaging to identify defect type
through pulmonary circulation →
becomes Eisenmenger syndrome over
time TREATMENT
SURGERY
SIGNS & SYMPTOMS ▪ Rarely
▪ Sometimes asymptomatic, but can lead to
heart failure, Eisenmenger syndrome MNEMONIC: P(C)AV
Heart failure Acyanotic defects
▪ Infants: poor feeding/failure to thrive, Patent ductus arteriosus
fluid retention, pulmonary congestion, (Coarctation of the aorta): no
hepatomegaly, respiratory distress, shunt
elevated jugular venous pressure Atrial septal defect
Ventricular septal defect

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Chapter 2 Acyanotic Defects

Figure 2.1 Illustration of blood flow through a ventricular septal defect.

ATRIAL SEPTAL DEFECT (ASD)

osms.it/atrial-septal-defect

PATHOLOGY & CAUSES

▪ A hole in the heart wall dividing left/right
atria (left-to-right shunt)
▪ Blood passes through pulmonary
circulation redundantly

SIGNS & SYMPTOMS

▪ Fixed, split S2 and pulmonic ejection
murmur (louder with age)
▪ Infants and children
▫ Respiratory infections
▫ Failure to thrive
▪ Adults (before 40) Figure 2.2 CT scan in the axial plane showing
▫ Palpitations, exercise intolerance, an atrial septal defect. Note the faint contrast
dyspnea, fatigue plume as blood flows from the high pressure
left system to the low pressure right system.
RA; right atrium. LA; left atrium. RV; right
ventricle. LV; left ventricle.

OSMOSIS.ORG 7
DIAGNOSIS
DIAGNOSTIC IMAGING
Chest X-ray
▪ Right heart dilation
▪ Prominent pulmonary vascularity

Transesophageal echocardiography
▪ Visualize size & location accurately

SURGERY
Figure 2.3 Intraoperative view of multiple,
Right heart catheterization pinhole atrial septal defects.
▪ Increased oxygen saturation in:
▫ Right atrium
▫ Right ventricle TREATMENT
▫ Pulmonary artery
SURGERY
▪ Percutaneous surgical closure
▪ Adults: surgery in cases of
▫ Right ventricular enlargement,
paradoxical embolism, right-to-left
shunt

Figure 2.4 Illustration depecting blood shunting from left to right atrium in atrial septal defect.

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Chapter 2 Acyanotic Defects

COARCTATION OF THE AORTA

(CoA)
osms.it/coarctation-of-the-aorta
compared to lower extremities
PATHOLOGY & CAUSES ▪ Secondary hypertension
▪ Severe heart failure, shock if/when PDA
▪ Narrowed segment of aorta
closes
▪ Upstream issues
▪ Other symptoms may more apparent with
▫ Blood flow increases into aortic age
branches before coarctation → blood
▫ Chest pain, cold extremities, claudication
flow, pressure increases in upper
on exertion
extremities, head
▫ Left ventricular impulse palpable,
▪ Downstream issues
sustained
▫ Decreased blood flow, decreased
▫ Pulsations felt in intercostal spaces
pressure in lower extremities
▫ Kidneys receive less blood → activate Adults
renin-angiotensin-aldosterone system ▪ Hypertension (most common)
(RAAS) → secondary hypertension ▪ Hypotension in lower extremities
▪ Preductal coarctation ▪ Bilateral lower extremity claudication
▫ Associated with Turner syndrome, PDA ▪ Dyspnea on exertion
▫ May go unnoticed unless severe. ▪ Delayed/weak femoral pulses
Presents as postductal coarctation
▪ Postductal coarctation
▫ Distal to ligamentum arteriosum DIAGNOSIS
▫ Presents in adulthood
▫ Blood pressure higher upstream, lower DIAGNOSTIC IMAGING
downstream Angiogram
▫ Autoregulatory vasoconstriction/ ▪ Visualize narrowing in aorta, anatomy &
vasodilation preserves regional blood severity
flow
Chest X-ray
▪ Rib notching: 3-sign (narrowed aorta
SIGNS & SYMPTOMS resembles notch of number 3 due to
prestenotic of aortic arch & postenotic of
▪ Depends on presence/severity of PDA descending aorta dilatation)
▪ Systolic murmur
▫ Systole: diamond-shaped murmur Echocardiograph
▫ Diastole: high-pitched decrescendo ▪ Visualize location, size, blood turbulance
murmur
OTHER DIAGNOSTICS
Infants
▪ Lower extremity cyanosis ECG
▪ Absent or delayed femoral pulse ▪ Left ventricular hypertrophy, left atrial
▪ Failure to thrive/poor feeding enlargement
▪ Blood pressure higher in upper extremities

OSMOSIS.ORG 9
TREATMENT
MEDICATIONS
Prostaglandin E
▪ Increases flow to lower extremities

SURGERY
▪ Resection with end-to-end anastomosis
▫ If unfeasible, bypass graft across area of
coarctation
▪ Long-segment coarctation: subclavian
aortoplasty Figure 2.5 Illustration showing narrowing of
▪ Prosthetic patch aortoplasty (rarely) aorta lumen.
▪ Balloon angioplasty with possible stent

Figure 2.7 A chest radiograph demonstrating

the figure of three sign seen in coarctation of
the aorta.
Figure 2.6 CT scan in the sagittal plane
demonstrating coarctation of the aorta.

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Chapter 2 Acyanotic Defects

PATENT DUCTUS ARTERIOSUS

(PDA)
osms.it/patent-ductus-arteriosus

PATHOLOGY & CAUSES DIAGNOSIS

▪ Ductus arteriosus remains open after birth DIAGNOSTIC IMAGING
▪ Left-to-right shunt between atria
Echocardiograph
▪ Sometimes presents with congenital
▪ 2D suprasternal echocardiogram
defects (congenital rubella syndrome)
Chest X-ray
CAUSES ▪ Normal/cardiomegaly

Congenital rubella
▪ Mother-fetal transmission of rubella in first
OTHER DIAGNOSTICS
trimester → cytopathic damage to blood ECG
vessels, ischemia to organs
▪ Left ventricular hypertrophy, left atrial
▪ Prematurity enlargement
▪ Perinatal distress, hypoxia

TREATMENT
SIGNS & SYMPTOMS
▪ Small asymptomatic PDA: monitor
Depend on size of PDA
▪ Smaller
MEDICATIONS
▫ Usually asymptomatic
▫ Neonates: holosystolic “machine-line” Neonates (10–14 days)
murmur on auscultation ▪ Close PDA using prostaglandin inhibitor
▫ Infants, children, adults: continuous
murmur Symptomatic moderate/large PDA
▪ Moderate ▪ During heart failure
▫ Exercise intolerance ▫ Digoxin, furosemide
▫ Continuous murmur
▫ Wide systemic pulse pressure SURGERY
▫ Displaced ventricular apex Symptomatic moderate/large PDA
▪ Larger ▪ Closure recommended for symptoms of
▫ Infants: leads to heart failure left-to-right shunting, left-sided volume
▫ Children: shortness of breath, overload, reversible pulmonary arterial
fatigability, Eisenmenger syndrome hypertension
▫ Children < 5kg/11lbs: surgical ligation
▫ > 5kg/11lbs (including adolescents/
adults): percutaneous occlusion, surgical
ligation for large PDA

OSMOSIS.ORG 11
Figure 2.8 Illustration depicting location of a patent ductus arteriosus.

Figure 2.9 Volume-rendered CT scan of the

heart and great vessels showing a patent
ductus arteriosus.

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Chapter 2 Acyanotic Defects

VENTRICULAR SEPTAL DEFECT

(VSD)
osms.it/ventricular-septal-defect

PATHOLOGY & CAUSES DIAGNOSIS

▪ Left-to-right shunt between ventricles DIAGNOSTIC IMAGING
▪ Most common congenital heart disease
Chest X-ray
▪ Left-to-right shunt between ventricles
▪ Unreliable; may indicate left atrial
▪ Often presents with other defects (e.g. enlargement, right ventricular hypertrophy,
tetralogy of Fallot) left ventricular hypertrophy, or pulmonary
Size of defect artery enlargement
▪ Small: restrictive Echocardiogram
▫ Normal pressure maintained between ▪ Determines location and size
ventricles
▪ Moderate or large: non-restrictive MRI
▫ No pressure difference between ▪ Use if echo does not diagnose
ventricles
SURGERY
Cardiac catheterization
SIGNS & SYMPTOMS ▪ Used if echo and MRI did not diagnose, but
individual still has pulmonary hypertension
▪ Asymptomatic in utero
▪ Holosystolic murmur (loud, high-pitched)
located at lower left sternal border OTHER INTERVENTIONS

Size of defect ECG

▪ Small: asymptomatic, murmur ▪ Left ventricular hypertrophy
▪ Moderate–large: sweating, poor feeding/ ▫ May see right ventricular hypertrophy;
failure to thrive, respiratory infections. left, right atrial enlargement (may see
Murmur plus thrill, and diastolic rumble in Katz–Wachtel phenomenon)
mitral area
▫ Signs of congestive heart failure
(dyspnea, persistent cough, pulmonary
vascular resistance)
▫ Eisenmenger’s syndrome

OSMOSIS.ORG 13
TREATMENT
▪ Most small VSDs close on their own

SURGERY
▪ Repair larger shunts by age 2 to prevent
pulmonary hypertension

Surgical repair
▪ Patch closure over ventricular septal defect
(preferred treatment)

Transcatheter closure
▪ Mesh to close VSD (higher risk)

Hybrid procedure
Figure 2.10 View of the right side of the heart
showing multiple ventricular septal defects.

Figure 2.11 Doppler ultrasound scan demonstrating flow of blood across the interventricular
septum in a VSD.

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