Updated: 2/26/2018

Transposition of Great Vessels

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Snapshot
  • A neonatal boy is born to a 37-year-old mother via normal spontaneous vaginal delivery. His mother has type 2 diabetes and hypertension. Since birth, he has had trouble feeding, seems to be constantly tachypneic, and has increasingly blue lips. He is immediately given prostaglandin E, and an echocardiogram is obtained, revealing transposition of the great vessels. He is scheduled for surgical repair.
Introduction
  • Clinical definition
    • a congenital heart defect in which the aorta leaves the right ventricle and the pulmonary trunk leaves the left ventricle, resulting in the complete separation of the systemic and pulmonary circulations
  • Epidemiology
    • demographics
      • male > female
      • present at birth
      • most common cyanotic lesion presenting immediately after birth
    • risk factors
      • maternal diabetes
      • maternal smoking
      • advanced maternal age
  • Etiology
    • failure of neural crest cells to migrate
  • Pathogenesis
    • failure of the aorticopulmonary septum to spiral results in complete separation of the systemic and pulmonary circulations
    • infants survive only if a shunt between the two circulations exist, to mix oxygenated blood into the systemic circulation
      • patent ductus arteriosus (PDA)
      • ventricular septal defect (VSD)
      • atrial septal defect (ASD)
      • patent foramen ovale (PFO)
  • Associated conditions
    • VSD
    • coarctation of the aorta
  • Prognosis
    • infants experience severe cyanosis and tachypnea as the ductus arteriosus closes in the newborn period
Presentation
  • Symptoms
    • early and progressive cyanosis that does not correct with oxygen
    • signs of heart failure
      • tachypnea
      • sweating
      • poor feeding
  • Physical exam
    • cardiac
      • loud and single S2 heart sound
      • may have a soft systolic ejection murmur
      • may have harsh holosystolic murmur if patients have VSD
    • central cyanosis
Imaging
  • Radiography
    • indication
      • for all patients
    • recommend views
      • chest
    • findings
      • cardiomegaly
      • egg-on-a-string appearance
        • narrowed mediastinum
  • Echocardiography
    • indication
      • performed as a diagnostic test
      • most specific test
    • findings
      • pulmonary trunk arising from the left ventricle
      • aorta arising from the right ventricle
Studies
  • Making the diagnosis
    • based on clinical presentation and echocardiography
    • in some cases, patients may be diagnosed prenatally with fetal echocardiography
Differential
  • Tetralogy of Fallot
    • distinguishing factor
      • tet spells (cyanosis) that are resolved when placed in knee-chest position
Treatment
  • Medical
    • prostaglandin E1
      • indication 
        • for all neonates
      • mechanism of action
        • maintains a patent ductus arteriosus for adequate lower extremity perfusion
  • Operative
    • balloon atrial septostomy (Rashkind procedure)
      • indication
        • for all patients
        • performed for temporary mixing prior to definitive surgical repair
    • arterial switch surgical repair
      • indication
        • for all patients as definitive treatment
        • commonly performed in the first month of life
Complications
  • Heart failure
  • Sudden cardiac death
 

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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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