coarctation of the aorta
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Introduction
Constriction of the aorta near its isthmus, usually just beyond the origin of the left subclavian artery.
Etiology
- congenital malformation
- frequently associated with bicuspid aortic valve
- atherosclerosis
- thrombosis
- aneurysm
- compression
Epidemiology
- more common in males
- 2nd most common cardiac anomaly in Turner's syndrome (18%)
- generally detected in childhood or as young adult
Pathology
- generally occurs around the ductus arteriosus just distal to the origin of the left subclavian artery
- volume expansion
- inappropriate renin secretion
- atherosclerosis may occur in vessels proximal to the coarctation
- collateral vessels may reduce trans-coarctation gradient
Clinical manifestations
- may be asymptomatic - 20% diagnosed in adulthood
- epistaxis
- intermittent (exercise-induced) claudication
- dyspnea on exertion
- dizziness
- headaches
- tinnitus
- upper body hypertension (75%)
- brachial blood pressure may be elevated (hypertension)
- blood pressure may be higher in left arm than right arm
- systolic blood pressure difference of 20 mm Hg between upper & lower extremities
- brachial blood pressure may be elevated (hypertension)
- diminished femoral pulses, radial to femoral pulse delay
- cold feet, diminished pedal pulses
- palpable pulsating arteries in axilla & around scapula
- arterial collateral vessels visible widely over the thorax
- systolic murmur may be heard over left sternal edge & over mid-thoracic spine (back); over the left infraclavicular region; continous murmur over back
- left ventricular systolic dysfunction
- S4 heart sound
Diagnostic procedures
- electrocardiogram:
- left ventricular hypertrophy
- ST segment & T-wave abnormalities
- echocardiography may be normal
- angiography, aortography
Radiology
- chest X-ray
- dilated ascending aorta
- notching of lower rib borders from collateral vessels
- displacement of esophageal shadow rightward on PA view
- a '3' configuration of the aortic knob (figure 3 sign)
- computed tomography (CT)
- magnetic resonance imaging (MRI)
- better than CT[3]
- screening for recurrent aortic coarctation or aortic aneurysm[3]
Complications
- left ventricular systolic dysfunction (CHF)
- aortic valve disease
- bicuspid aortic valve (50%)
- aortic valvular stenosis &/or aortic regurgitation requiring surgery in 70%[3]
- bicuspid aortic valve (50%)
- aneurysm proximal to the coarctation
- thoracic aortic aneurysm
- aortic rupture or aortic dissection
- rupture of an aneurysm of the circle of Willis secondary to hypertension proximal to the coarctation
- recurrent coarctation of the aorta[3]
- endarteritis
- hypertension (75%)
Management
- balloon angioplasty
- discreet narrowing, proximal hypertension & pressure gradient > 20 mm Hg
- has been associated with formation of aneurysms
- surgery
- indications:
- systolic pressure gradient > 20 mm Hg[3]
- radiologic evidence of severe coarctation with collateral flow[3]
- resection with end to end reanastomosis
- 75% incidence of hypertension after surgical repair
- surgically treated patients often die prematurely of:
- coronary artery disease
- heart failure
- stroke
- ruptured aorta
- earlier age at surgery associated with better prognosis
- lifelong follow-up[7]
- indications:
- medical mangagement of hypertension
- patients with unrepaired or residual aortic diseease should avoid pregnancy, contact sports & isometric exercise[3]
- antibiotic prophylaxis for endocartitis[3]
- no longer indicated[4]
More general terms
Additional terms
References
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 865
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 47-48, 484
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
- ↑ 4.0 4.1 Wilson W et al, Prevention of infective endocardititis: guidelines from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committe, Council on Cardiovascular Disease in the Young, and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. J AM Dent Assoc 2008, 139:3S PMID: https://www.ncbi.nlm.nih.gov/pubmed/18167394S
- ↑ Tanous D, Benson LN, Horlick EM. Coarctation of the aorta: evaluation and management. Curr Opin Cardiol. 2009 Nov;24(6):509-15. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19667980
- ↑ Tsai SF, Trivedi M, Boettner B, Daniels CJ. Usefulness of screening cardiovascular magnetic resonance imaging to detect aortic abnormalities after repair of coarctation of the aorta. Am J Cardiol. 2011 Jan 15;107(2):297-301 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21211607
- ↑ 7.0 7.1 Brown ML, Burkhart HM, Connolly HM et al Coarctation of the aorta: lifelong surveillance is mandatory following surgical repair. J Am Coll Cardiol. 2013 Sep 10;62(11):1020-5 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23850909