Updated: 2/26/2018

Coarctation of the Aorta

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Snapshot
  • A 4-year-old girl presents to her pediatrician’s office for a well-child visit. She and her parents report no concerns except for occasional headaches. During evaluation of her vital signs, her lower extremity systolic blood pressure is found to be more than 20 mmHg less than her upper extremity systolic blood pressure.
Introduction
  • Clinical definition
    • narrowing of the aorta causing hypertension in the upper extremities relative to the lower extremities
      • post-ductal type (adult type)
        • narrowing occurs distal to the ductus arteriosum
      • pre-ductal (infantile type)
        • narrowing occurs proximal to the ductus arteriosum
  • Epidemiology
    • demographics
      • male > female
      • < 10% of all congenital heart defects
    • location
      • aorta
    • risk factors
      • family history
  • Pathogenesis
    • mechanism of coarctation is unknown but thought to be due to
      • decreased blood flow across aorta in utero, which can lead to defects in the aorta
      • ectopic ductal tissue in the aorta, which can cause the aorta to be pulled inwards into a coarctation
    • because of the aortic narrowing, there is hypertension in the upper extremities
      • pulse is delayed in the lower extremities
    • collateral circulation causes intercostal arteries to enlarge
      • these arteries then erode the ribs, causing a notched appearance on radiography
  • Associated conditions
    • Turner syndrome
    • bicuspid aortic valve
    • Williams syndrome
  • Prognosis
    • some may present early in life, but others may remain asymptomatic until adulthood
    • infants may be asymptomatic until the ductus arteriosus closes
Presentation
  • Symptoms
    • headache
    • shortness of breath with exertion
    • exercise intolerance
    • fatigue
    • poor feeding in infants
  • Physical exam
    • cardiac
      • harsh systolic murmur along the left sternal border
      • may also have systolic murmur along left and right side of the chest with thrills
        • from collateral circulation over time
    • pulses
      • femoral < brachial pulses
      • bounding pulses in upper extremities and carotids
      • delay in femoral pulse compared to the radial pulse
    • differential cyanosis
      • cyanotic lower extremities
    • hypertension in upper extremities
      • systolic blood pressure in the arms are > 20 mmHg higher than the legs
    • underdeveloped legs compared with arms
Imaging
  • Radiography
    • indication
      • to rule out any pulmonary pathologies
    • views
      • chest
    • findings
      • rib notching on the inferior surface
        • due to collateral circulation through the intercostals
      • cardiomegaly
      • increased pulmonary markings
  • Echocardiography
    • indication
      • performed as a diagnostic test
      • most specific test
    • findings
      • coarctation of aorta visualized
Studies
  • Electrocardiogram
    • findings
      • left ventricular hypertrophy
  • Making the diagnosis
    • based on clinical presentation and echocardiography
Differential
  •  Tetralogy of Fallot
    • distinguishing factor
      • tet spells (cyanosis) that are resolved when placed in knee-chest position
Treatment
  • Medical
    • prostaglandin E
      • indication
        • neonates with coarctation of the aorta
      • mechanism of action
        • maintains a patent ductus arteriosus for adequate lower extremity perfusion
    • diuretics
      • indications
        • heart failure
  • Operative
    • surgical or transcatheter repair
      • indication
        • adults and neonates after stabilization of heart failure or shock
        • children with hypertension
      • modalities
        • balloon angioplasty
        • stent placement
Complications
  • Heart failure
  • Systemic hypertension
  • Berry aneurysms leading to cerebral hemorrhage
  • Aortic rupture
  • Endocarditis

 

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Questions (2)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.CV.158) A 5-year-old girl presents to the physician with increased muscle cramping in her lower extremities after walking extended distances. The young girl is in the 10th percentile for height. Her past medical history is notable only for a cystic hygroma detected shortly after birth. Which of the following findings is most likely in this patient? Review Topic

QID: 105556
1

Decreased blood pressure in the upper and lower extremities

7%

(1/15)

2

Barr bodies on buccal smear

0%

(0/15)

3

Endocardial cushion defect

27%

(4/15)

4

Inferior erosion of the ribs

53%

(8/15)

5

Apparent hypertrophy of the calves

7%

(1/15)

M1

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