PEDBASE.org - The Pediatric Database - Detailed information of COARCTATION OF THE AORTA

COARCTATION OF THE AORTA

DEFINITION:

An acyanotic congenital heart disease characterized by constriction of the aorta.

EPIDEMIOLOGY:

incidence: 5-7% of all congenital cardiac lesions
age of first detection:
- newborn -> childhood
risk factors:
- Trisomy-18, -13, Turner Syndrome, Fetal Hydantoin Syndrome, Fetal Valproate Effect, Maternal PKU, Crouzon Syndrome
associated anomalies:

1. Major

bicuspid aortic valve (in 70%)
mitral valve anomalies (MI)

2. Minor

subaortic stenosis, VSD, PDA, hypoplastic LH

PATHOGENESIS

1. Background

collaterals provide flow around the obstructed segment of the aorta
branches provided from the subclavian, superior intercostal, internal mammary, and axillary arteries
for BP differential between upper and lower limbs must have at least a 50% reduction in the size of the aorta

CLINICAL FEATURES:

1. Symptoms

1. Asymptomatic

present in later childhood or adult
found as part of work up for hypertension and/or murmur

2. Symptomatic

1. Congestive Heart Failure

presents at 2 weeks, 4-6 months, or as adults
dyspnea, lethargy, difficulty feeding, organomegaly
2 weeks
- usually associated with a L->R shunt (VSD, PDA)
- poor response to medical therapy
4-6 months
- may be associated with additional lesions
- good response to medical therapy

2. Signs

1. Periphery

weak/absent femoral pulses
BP differential between upper & lower limbs (SBP >30)

2. Palpation

quiet precordium

3. Auscultation

1. Heart Sounds

S1 - loud (due to hypertension)
S2 - narrow split

2. Murmurs

1. Systolic

1. Collateral Flow Murmur

systolic bruit @ back between scapulae, axillae, and latissimus dorsi muscles

2. Coartation Murmur

soft @ back and left infraclavicular area

3. Associated Anomalies

bicuspid aortic valve, VSD, MI

INVESTIGATIONS:

1. Chest X-Ray

cardiomegaly with increased pulmonary vascular markings
hypoplastic aortic knob with dilated poststenotic segment of the aorta ("reverse 3" sign)
rib notching

2. ECG

LVH

3. 2D-Echo

coarctation
associated anomalies

MANAGEMENT:

1. Supportive

although mild coarctations may be well tolerated during childhood, patients should avoid vigorous or competitive sports
1. Medical Management
1. Congestive Heart Failure
- digoxin, lasix

2. Surgery

1. Indications for:

greater than 1 year of age
- to avoid recoartation due to poor growth of suture and/or remaining ductal tissue
- late repair of coarct does not correct the hypertension which can lead to later coronary artery disease
gradient across coarctation >30mmHg
- increased surgical risk of poor peripheral perfusion post-op if gradient <30mmHg
difference in systolic BP >60mmHg between upper and lower limbs during a stress (exercise) test

2. Types

1. Resection

resection of ductal tissue with end-to-end anastomosies

2. Subclavian Flap Repair

3. Complications

30% incidence of recoarctation

Pediatric Database - COARCTATION OF THE AORTA

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