Coarctation of the Aorta – Causes, Symptoms, Diagnosis, Treatment and Ongoing care

Basics

Description

A narrowing (discrete or of varying lengths) of the aorta at any point, but usually located just distal to the left subclavian artery at the junction of the ligamentum arteriosum; primary effects are cardiovascular

Pediatric Considerations

  • Condition is noted more commonly in infants than in adults
  • A greater risk of complications is possible if correction is delayed beyond early childhood. Often, the diagnosis itself is delayed.
  • Lowest long-term mortality rates are observed in patients having surgery between 1 and 5 years of age.

Pregnancy Considerations

  • Uncorrected coarctation or restenosis carries a high risk of aortic rupture or dissection and cerebral hemorrhage (ruptured aneurysm, circle of Willis) but a smaller risk of preeclampsia than with other forms of HTN.
  • Even with correction, higher incidence of miscarriage and HTN occurs in pregnancy.

Epidemiology

Incidence

  • 64/100,000 in patients < 1 year of age
  • More common in fall and winter births
  • Predominant age: Infants and children
  • Predominant sex: Male > Female (1.7–5:1)
  • 7 times more common in whites than in Asians

Risk Factors

5–10% of all congenital heart conditions show coarctation.

Genetics

No mendelian inheritance, but is seen commonly in association with Turner syndrome; family history of left ventricular outflow tract obstruction is a risk factor.

Etiology

  • Turner syndrome
  • Congenital left-sided heart abnormalities
  • Takayasu arteritis

Commonly Associated Conditions

  • Bicuspid aortic valve: 85%
  • Patent ductus arteriosus: 65%
  • Ventricular septal defect: 30–35%
  • Mitral valve abnormalities
  • Aortic stenosis and insufficiency
  • Subvalvular aortic stenosis
  • Transposition of great vessels or double outlet right ventricle
  • Aneurysm of circle of Willis
  • Neurofibromatosis I
  • Acquired intercostal aneurysms

Patent ductus arteriosus, Mitral valve, Cardiovascular Disorders, congenital heart conditions, cerebral hemorrhage,

Diagnosis

History

  • Headaches
  • Leg fatigue and pain on exertion
  • Chest pain, syncope, or dyspnea on exertion
  • Epistaxis
  • Cyanosis, rarely
  • Infant irritability or diaphoresis

Physical Exam

  • Cyanosis, rarely
  • In infancy, also may have heart failure, failure to thrive, irritability, tachypnea, and dyspnea
  • Prominent neck pulsations
  • Epistaxis
  • HTN
  • Pulse disparity: Pulse delay in femoral vs radial artery and increased amplitude in brachial vs femoral pulse
  • Delayed, weak, or absent lower extremity pulse
  • Prominent left ventricular impulse
  • Murmur (aortic stenosis or insufficiency, ventricular septal defect, rarely mitral valve)
  • S4 systolic ejection click
  • Bruit (coarctation, collaterals, patent ductus arteriosus)
  • Funduscopy: Corkscrew tortuosity of retinal arterioles
  • Extensive collaterals develop from branches of the subclavian, internal mammary, superior intercostal, and axillary arteries.
  • BP: Evaluation shows upper limb systemic HTN and a differential ≥ 10 mm Hg compared with lower extremity.
  • Left arm diameter may be smaller than the right.

Diagnostic Tests & Interpretation

Age of diagnosis depends on the severity of condition.

Imaging

  • Doppler exam of pulses reveals disparity.
  • CXR may show rib notching, “3” sign, rarely cardiomegaly
  • The adult type of coarctation is more commonly distal to the left subclavian artery than proximal (1).
  • ECG may show right ventricular hypertrophy in neonates but left ventricular hypertrophy in older patients.
  • Echocardiography for coexisting cardiac anomalies
  • Transesophageal echocardiography
  • MRA/MRI

Diagnostic Procedures/Surgery

Cardiac catheterization and angiography: Poststenotic dilation

Pathological Findings

  • Segmental tubular hypoplasia
  • Discrete obstruction with medial thickening
  • Distal aneurysm

Differential Diagnosis

  • Takayasu arteritis
  • Neurofibromatosis
  • Pseudocoarctation (±HTN, peripheral vascular disease)
  • Hypoplastic left heart syndrome
  • Dilated or hypertrophic cardiomyopathy

Treatment

Treatment options and timing depend on age diagnosed and severity of narrowing.

Medication

  • Alprostadil (prostaglandin E1) to maintain patency of ductus arteriosus in infancy
  • Antibiotic prophylaxis (for dental and/or invasive procedures); see Complications.
  • Postoperative antihypertensives may be needed, including esmolol or nitoprusside.
  • Long-term antihypertensives include β-blockers and ACE inhibitors.
  • Preload and afterload reduction if heart failure develops
  • Precautions:
    • Lowering BP in upper extremity may cause hypoperfusion of lower extremities.
    • Lowering BP is not advised in pregnancy unless mandated by emergency.

Surgery/Other Procedures

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  • Inpatient surgery is required.
  • Surgical correction or balloon angioplasty can be done in infancy if urgently needed. Best results when performed in patients aged 1–2 years (2).
  • Surgery should be done in childhood and adulthood as soon as coarctation is diagnosed to prevent later complications.
  • Common operative procedures:
    • End-to-end anastomosis
    • Patch aortoplasty (insertion of Dacron patch)
    • Subclavian flap procedure
  • Common endovascular procedures:
    • Balloon angioplasty of coarctation offers good results for primary treatment and for postoperative restenosis. Good long-term results as primary treatment out to 22 years.
    • Stent placement (3):
      • May prevent unnecessary dilation of coarctation during angioplasty
      • May better prevent late recoarctation than angioplasty alone

Ongoing Care

Follow-Up Recommendations

  • Exercise may exacerbate HTN, but normal activity is nonetheless recommended after correction.
  • Isometric activities such as heavy lifting should be avoided.
  • Consider exercise stress test to gauge BP response to exercise.

Patient Monitoring

Frequent postoperative follow-up for evidence of restenosis and late complications. Clinical evaluation (check for HTN and pulse disparities) combined with MRI has been shown to be cost-effective screening strategy. Need for antihypertensives may decrease with time (45).

Diet

Heart-healthy and low-sodium diet is recommended for HTN reasons.

Patient Education

  • Discuss postcoarctation syndrome with patient.
  • Patients should be encouraged to lead normal lives and pursue usual activities after coarctation correction.
  • For favorably reviewed patient education materials on this topic, contact American Heart Association, 7320 Greenville Avenue, Dallas, TX 75231, (214) 373-6300, www.americanheart.org.

Prognosis

  • Depends on age of repair and presence of other cardiac abnormalities
  • Survival after surgery: 10 years (91%), 20 years (84%), 30 years (72%)
  • Uncorrected, 80% mortality before age 50 years

Complications

Most common with late or no correction:

  • Residual or restenosis: 6–33%
  • HTN: 25%
  • Subsequent cardiac surgery: 11%
  • Heart failure
  • Aneurysm of circle of Willis, rupture possible
  • Rupture or dissection of aortic aneurysm
  • Endarteritis or endocarditis (need antibiotic prophylaxis for 6 months following complete repairs, indefinite for residual defects SOR B) (6)
  • Aortic valve disease (stenosis or insufficiency)
  • Postcoarctectomy syndrome: Recurrence, HTN, atherosclerotic heart disease, aneurysm at site of coarctectomy, progressive aortic stenosis, and/or regurgitation
  • In the abscence of CAD, postrepair myocardial perfusion defects (7)
  • Fistula formation between aorta and airways leading to hemoptysis (8)

References

1. Grainger RG. TAM. In: Adam A, Dixon AK, Grainger RG, Allison DJ, eds. Grainger & Allison’s diagnostic radiology. A textbook of medical imaging, 5th ed. New York, NY: Churchill Livingstone; 2008 Chapter 23.

2. Tabbutt S, Nicolson SC, Dominguez TE, et al. Perioperative course in 118 infants and children undergoing coarctation repair via a thoracotomy: a prospective, multicenter experience. J Thorac Cardiovasc Surg. 2008;136:1229–36.

3. Ledesma M, Alva C, Gómez FD, et al. Results of stenting for aortic coarctation. Am J Cardiol. 2001;88:460–2.

4. Therrien J, Thorne SA, Wright A, et al. Repaired coarctation: a “cost-effective” approach to identify complications in adults. J Am Coll Cardiol. 2000;35:997–1002.

5. Toro-Salazar OH, Steinberger J, Thomas W, et al. Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol. 2002;89:541–7.

6. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1736–54.

7. Cook SC, Ferketich AK, Raman SV. Myocardial ischemia in asymptomatic adults with repaired aortic coarctation. Int J Cardiol. 2008;:

8. Heikkinen LO, Järvinen AA. Aortopulmonary fistula after coarctation repair with Dacron patch aortoplasty. Ann Thorac Surg. 2002;73:1634–6.

Additional Reading

Fawzy ME, Fathala A, Osman A, et al. Twenty-two years of follow-up results of balloon angioplasty for discreet native coarctation of the aorta in adolescents and adults. Am Heart J. 2008;156:910–7.

Manganas C, et al. Reoperation and coarctation of the aorta: The need for lifelong surveillance. Ann Thorac Surg. 2001;7:1222–1224.

Rosenthal E. Stent Implantation of aortic coarctation: The treatment of choice in adults? J Am Cardiol. 2001;38:1524–1527.

Vriend JW, Drenthen W, Pieper PG, et al. Outcome of pregnancy in patients after repair of aortic coarctation. Eur Heart J.2005;26:2173–8.

Codes

ICD9

  • 747.10 Coarctation of aorta (preductal) (postductal)
  • V17.49 Family history of other cardiovascular diseases

Snomed

7305005 coarctation of aorta (disorder)

Clinical Pearls

  • Screening for upper and lower extremity blood pressure differences may help to identify the diagnosis.
  • Delayed, weak, or absent lower extremity pulse should suggest the possibility of the diagnosis.
  • 7 times more common in whites than in Asians

About the author

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