II. Epidemiology

  1. Incidence: 6% of Congenital Heart Disease
  2. More common in Males by a ratio of 1.7 to 1

III. Associated Conditions

  1. Turner's Syndrome
  2. Bicuspid aortic valves (50%)
  3. Aortic Coarctation Syndrome
    1. Patent Ductus Arteriosus (PDA)
    2. Tubular hypoplasia the aortic isthmus
    3. Ventricular Septal Defect (VSD)
    4. Congestive Heart Failure

IV. Pathophysiology

  1. Constriction of aorta near isthmus
    1. Ductus arteriosus insertion
    2. Occurs below origin of left subclavian

V. Etiology

  1. Congenital malformation
  2. Atherosclerosis
  3. Thrombosis
  4. Aortic aneurysm
  5. External compression

VI. Symptoms (May be asymptomatic)

VII. Signs

  1. Arterial Hypertension
  2. Upper extremity Hypertension
    1. Arm to leg systolic Blood Pressure difference >20 mmHg
  3. Diminished or delayed femoral pulses
    1. Femoral pulses lag brachial pulses (Brachial-femoral delay)
    2. Low or unobtainable Blood Pressure in the lower extremities
  4. Palpable pulsating arteries about Scapula and axilla

VIII. Diagnostics: Electrocardiogram

  1. Right Ventricular Hypertrophy (Infants)
  2. Left Ventricular Hypertrophy (older child and adults)

IX. Imaging

  1. Chest XRay
    1. First-line initial screening study
    2. Older Children, adolescents or young adults
      1. Notching of the lower rib borders (figure 3 sign)
      2. Displaced esophageal shadow rightward
    3. Infants with Congestive Heart Failure signs
      1. Cardiomegaly
      2. Pulmonary venous congestion
  2. Transthoracic Echocardiogram
    1. Preferred diagnostic test in children
    2. Findings
      1. Dilated Right Ventricle
      2. Hypoplastic Left Ventricle (Infants)
      3. Coarctation site
  3. MR Angiogram Chest
    1. Preferred diagnostic test in adults

X. Management

  1. Infant with Congestive Heart Failure
    1. PGE1 to open ductus arteriosus (Neonates)
    2. Surgical Repair

XI. Management: Surgical Repair

  1. Indications
    1. Transcoarctation pressure gradient >30 mmHg
    2. Routine repair between ages 2 and 4
    3. Urgent repair
      1. Circulatory shock
      2. Cardiomegaly
      3. Severe Hypertension
      4. Severe Congestive Heart Failure
  2. Techniques
    1. Subclavian flap repair
    2. End to end anastomosis with resection
    3. Balloon Angioplasty (for recoarctation)

XII. References

  1. Cyran (1998) PREP review lecture, October, Phoenix
  2. Merenstein (1994) Pediatrics, Lange
  3. Charles (2017) Am Fam Physician 96(7): 453-61 [PubMed]
  4. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]

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Ontology: Aortic coarctation (C0003492)

Definition (MSHCZE) Koarktace aorty je vrozené zúžení aorty, nejčastěji distálně od odstupu levé a. subclavia v blízkosti ductus arteriosus. Toto zúžení vede k poklesu tlaku za obstrukcí a k vzestupu tlaku před ní.
Definition (NCI) Congenital narrowing of a segment of the aorta. Signs and symptoms include hypertension, muscle weakness, shortness of breath, headaches and leg cramps.
Definition (CSP) congenital constriction of the aorta just distal to the left subclavian artery; causes upper body hypertension but few other symptoms; may present as headache or dyspnea on exertion.
Definition (MSH) A birth defect characterized by the narrowing of the AORTA that can be of varying degree and at any point from the transverse arch to the iliac bifurcation. Aortic coarctation causes arterial HYPERTENSION before the point of narrowing and arterial HYPOTENSION beyond the narrowed portion.
Concepts Congenital Abnormality (T019)
MSH D001017
ICD9 747.10, 747.1
ICD10 Q25.1
SnomedCT 156929001, 204419005, 7305005
English Aortic Coarctation, Aortic Coarctations, Coarctation, Aortic, Coarctations, Aortic, aorta coarctation, AORTIC COARCTATION, Coarctation of aorta NOS, COARCTATION OF AORTA, coarctation of aorta, coarctation of aorta (diagnosis), aortic coarctation, Coarctation of the aorta, Aortic Coarctation [Disease/Finding], Coarctation;aorta, coarctation aortic, coarctation, aorta coarctation of, coarctation aorta, Coarctation of the Aorta, Aorta Coarctation, Coarctation of Aorta, Aorta Coarctations, Aortic coarctation (disorder), Coarctation of aorta NOS (disorder), Coarctation of aorta, Coarctation, Coarctation of aorta (disorder), Aortic coarctation, Coarctation of aorta (preductal) (postductal), coarctation of the aorta
Spanish COARTACION AORTICA, Coartación de aorta (preductal) (postductal), Coartación de aorta, Coartación aórtica, coartación de aorta, SAI (trastorno), coartación de aorta, SAI, coartación aórtica, coartación de aorta (trastorno), coartación de aorta, Coartación de la aorta, Coartación Aórtica
German AORTENISTHMUSSTENOSE, Isthmusstenose der Aorta, Isthmusstenose der Aorta (praeduktal) (postduktal), Koarktation der Aorta, Aortenisthmusstenose, Aortenkoarktation, Coarctatio aortae
Dutch aortacoarctatie, aortastrictuur (voor en achter ductus arteriosus), coarctatie van aorta, Coarctatio aortae
French Coarctation de l'aorte (précanalaire) (postcanalaire), COARCTATION DE L'AORTE, Coarctation aortique, Coarctation de l'aorte
Italian Coartazione aortica (preduttale) (postduttale), Coartazione aortica, Coartazione dell'aorta, Restringimento aortico
Portuguese Coarctação da aorta (pré-canalicular) (pós-canalicular), COARTACAO DA AORTA, Coarctação da aorta, Coartação Aórtica
Japanese 大動脈縮窄, ダイドウミャクシュクサク
Swedish Aortaförträngning
Finnish Aortan koarktaatio
Russian AORTY KOARKTATSIIA, АОРТЫ КОАРКТАЦИЯ
Czech Aortální koarktace, Koarktace aorty (preduktální) (postduktální), Koarktace aorty, koarktace aorty, coartatio aortae, aorta - koarktace
Korean 대동맥 축착
Polish Zwężenie aorty wrodzone, Koarktacja aorty, Zwężenie światła aorty, Zwężenie cieśni aorty
Hungarian Aorta coarctatio, Aorta coarctatiója (preductalis) (postductalis), Coarctatio aortae, Aorta coarctatiója
Norwegian Koarktasjon av aorta, Aortakoarktasjon