(I-1B.6) Adult patient with coarctation of the aorta (CoA) Danuta Sorysz MD. PhD, Barbara Zawiślak MD

Background
Coarctation of aorta is a common congenital malformation that is usually treated in childhood. Adult patient with CoA have a important incidence of associated cardiac disorders include bicuspide aortic valve (up to 85%), parachute mitral valve and atrial fibrillation, ischemic heart disease. Clinical features include upper body systolic hypertension, lower body hypotension, a blood pressure gradient between upper and lower extremities (20 mmHg indicates significant CoA), radiofemoral pulse delay. Diagnostic methods are echocardiography, CT, NMR, angiography. In case of appropriate anatomy, stenting has become the treatment of first choice in adults. In more complex cases different operative technique are possible.

Case presentation
51 year old woman has been consulting because of uncontrolled hypertension, dyspnea (NYHA II) and systolic and diastolic murmur. Hypertension were diagnosed during pregnancy 20 years ago. Moderate claudication and exertional leg fatigue were observed in childhood. Echocardiography showed good systolic function of nondilated left ventricule, bicuspid aortic valve with moderate aortic regurgitation and non dilated ascending aorta. In descending aorta 61 mmHg systolic peak gradient were measured and in abdominal aorta flow tipical for CoA was obtained. CT scan of thoracic aorta confirmed 3mm CoA, just below of subclavian artery with well-developed collateral circulation. Good controlled hypertension after modification of treatement (valsartan 160mg, bisoprolol 5mg, amlodipine 5mg, hydrochlorothizyd 25mg) were achieved.

Current guidelines
Nowadays, both surgical and catheter interventional treatment are available. The anatomy of the coarctation as well as coexisting defects are decisive in choosing appropriate technique. The main indications for intervention in coarctation of the aorta are as follows:

  • all patients with a non-invasive pressure difference >20 mmHg between upper and lower limbs, regardless of symptoms but with upper limb hypertension (>140/90 mmHg in adults), pathological blood pressure response during exercise, or significant LVH ( Class I C)
  • Independent of the pressure gradient, hypertensive patients with ≥50% aortic narrowing relative to the aortic diameter at the diaphragm level (on CMR, CT, or invasive angiography) should be considered for intervention ( Class IIa C)
  • Independent of the pressure gradient and presence of hypertension, patients with ≥50% aortic narrowing relative to the aortic diameter at the diaphragm level
    ( on CMR, CT, or invasive angiography ) may be considered for intervention
    (Class IIb C )[1].
    Infective endocarditis prophylaxis: Recommended only for high-risk patients.

References
1. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). The Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC)
2. Gewillig M, Budts W, Boshoff D, Maleux G. Percutaneous interventions of the aorta.
Future Cardiol. 2012 Mar;8(2):251-69.
3. Park JH, Chun KJ, Song SG, Kim JS, Park YH, Kim J, Choo KS, Kim JH, Lee SK..
Severe aortic coarctation in a 75-year-old woman: total simultaneous repair of aortic coarctationand severe aortic stenosis. Korean Circ J. 2012 Jan;42(1):62-4. Epub 2012 Jan 31

Expert’s comments:(Written authorization required from each expert)

1. Andrzej Gackowski, MD, PhD
Invasive pressure measurement above and below CoA were proposed before final decision

2. Prof. Lesław Szydłowski, MD, PhD
Clinical presentation suggests coarctation of the aorta. Therefore, angiography should be performed before final decision regarding further approach – surgical vs percutaneous.

Expert’s conclusions:
Aortography and invasive pressure measurement above and below CoA were proposed before final decision (surgical or pharmacological treatement.

Authors:
Danuta Sorysz MD. PhD1, Barbara Zawiślak MD1

Experts:
Andrzej Gackowski MD, PhD2, Prof. Lesław Szydłowski MD, PhD3

1II Departement of Cardiology University Hospital in Cracow
2Department of Coronary Artery Disease, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow
3Department of Pediatric Cardiology, Medical University of Silesia, Katowice, Poland

 

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