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Almeselmani, Alsoufi, Elwan, Albawab, and Musa: Severe aortic coarctation incidentally discovered in a young university student

Introduction

Coarctation of the aorta accounts for 5–10% of all congenital heart defects. Most cases are sporadic, with a reported prevalence of about 1 in 10 000 live births.1,2 It occurs more frequently in men than in women (59% vs. 41%, respectively). There are two theories regarding its cause: one attributes coarctation of the aorta to reduced anterograde intrauterine blood flow, causing underdevelopment of the fetal aortic arch,3 the other to migration or extension of the ductal tissue into the wall of the fetal thoracic aorta.4 The defect results in left ventricular overload, arterial hypertension, heart failure, neurovascular complications such as stroke and intracranial aneurysms.

Case report

A 19-year-old female university student presented with headaches, tiredness and incidentally discovered arterial hypertension which had been ongoing for almost 1 year. Examination revealed a well-built female with tachycardia (with a heart rate of 115 bpm). Her blood pressure was 160/88 mmHg in the left arm and 185/95 mmHg in the right arm. Pulses in the lower extremities were weak and delayed, with a blood pressure of 110/70 mmHg. Cardiac auscultation was unremarkable. Electrocardiography revealed signs of left ventricular hypertrophy. Chest radiography was normal.

Echocardiography revealed signs of mild left ventricular hypertrophy. However, aortic isthmus imaging was inconclusive. As a consequence, computerized tomography (CT) aortography was performed, showing aortic isthmic hypoplasia, rich periscapular and intercostal collaterals, coarctation and the classic reverse-figure 3 (Figure 1).3

FIGURE 1

Aortogram showing abnormal tapering of the aortic arch (white arrow) and an area of coarctation (blue arrow). The diameter of the isthmus is < 25% of the diameter of the ascending aorta; the classic reverse-figure 3 is clearly visible.

HMJ-680-fig1.jpg

Diagnostic and therapeutic cardiac catheterization was performed; it confirmed the diagnosis of severe aortic coarctation in the thoracic aorta, distal to the origin of the left subclavian artery, with multiple collaterals (Figure 2). Haemodynamics revealed a systolic gradient of about 50 mmHg. The coarctation area was dilated by balloon, which led to a significant improvement in the haemodynamic results. However, after balloon dilatation, pre-stenotic pressure was 143/70 mmHg, whereas post-stenotic pressure was 123/68 mmHg. Therefore, a 4.5 × 16 mm stent was deployed over the balloon, achieving maximum dilatation and producing very good results (Figure 3).

FIGURE 2

Aortography showing severe coarctation.

HMJ-680-fig2.jpg
FIGURE 3

Balloon dilatation with stent.

HMJ-680-fig3.jpg

Discussion

First-time presentation of aortic coarctation in adults is rare. The average life expectancy of individuals with unaerated coarctation is approximately 35 years; 75% of affected individuals are dead by the age of 46 years.5 In previously undiagnosed adults, the classic symptom is arterial hypertension; most patients are asymptomatic unless hypertension is present. Aortic coarctation may cause headaches, epistaxis, heart failure or aortic dissection. Our patient presented with a history of hypertension and headaches lasting almost 1 year. The diagnosis was based on systolic hypertension in the upper extremities, radiofemoral delay (delayed femoral pulses) and low blood pressure in the lower extremities.

Electrocardiography in adults may be normal or show signs of left ventricular hypertrophy,6 as it did in our reported case. In older children and adults, abnormal radiography results are not uncommon, including signs of indentation of the aortic wall at the site of lesion with pre- and post-coarctation dilatation, and notching of the posterior one-third of the third to eighth ribs due to erosion by collateral arteries.7 However, chest radiography may be normal, as it was in our patient.

Transthoracic echocardiography can establish the diagnosis, assess the gradient across the coarctation and detect associated cardiac defects in most patients. However, the presence of collateral blood flow may diminish the gradient across the coarctation, and it may be less severe than expected considering the degree of obstruction.8 Adult echocardiography can be technically demanding and both false-negative and false-positive results are frequent.8 In our case, echocardiography of the cardiac isthmus was difficult and inconclusive. Therefore, the diagnosis was confirmed by multislice chest CT aortography. Furthermore, the 2008 American College of Cardiology and American Heart Association (ACC/AHA) guidelines recommend that every patient with aortic coarctation, repaired or not, should undergo CT at least once (or magnetic resonance imaging) for complete evaluation of the thoracic aorta.9

Although correction of coarctation can sometimes be delayed in children, it should be performed in adults at the time of diagnosis. The 2010 European Society of Cardiology guidelines for management of adult congenital heart disease state that all patients with a non-invasive pressure difference of > 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 left ventricular hypertrophy should undergo the intervention.10 On the other hand, the 2008 ACC/AHA guidelines for congenital heart disease recommend intervention if the peak-to-peak coarctation gradient is ≥ 20 mmHg, or if the peak-to-peak coarctation gradient is < 20 mmHg but there is anatomical imaging evidence of significant coarctation and radiological evidence of significant collateral flow.9 Stent placement after balloon angioplasty has been reported to reduce complications associated with angioplasty or surgery, minimize residual gradient, improve luminal diameter and sustain haemodynamic benefits.11 Our patient met both European and ACC/AHA criteria for intervention, so she underwent a successful stent placement after balloon angioplasty. Her blood pressure returned to normal and follow-up after 1 year confirmed clinical stability.

Conclusion

Although uncommonly diagnosed in adults, aortic coarctation should not be overlooked as a potential cause of secondary systemic hypertension; careful clinical examination is crucial. All patients with systemic arterial hypertension should undergo clinical examination that includes radial, brachial and femoral pulse palpation. Examining blood pressure in the upper and lower limbs is an essential part of the primary evaluation of patients with systemic hypertension. Diagnostic tests including echocardiography, CT aortography and angiography should be performed to confirm and guide diagnosis and management. Balloon angioplasty and stent placement for aortic coarctation is an evolving procedure, with significant improvements being observed over the past two decades.

References

1. 

Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998–2005. J Pediatr 2008; 153:807–13. https://doi.org/10.1016/j.jpeds.2008.05.059

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Rudolph AM, Heymann MA, Spitznas U. Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol 1972; 30:514–25. https://doi.org/10.1016/0002-9149(72)90042-2

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Forbes TJ, Moore P, Pedra CAC, et al. Intermediate follow-up following intravascular stenting for treatment of coarctation of the aorta. Catheter Cardiovasc Interv 2007; 70:569–77. https://doi.org/10.1002/ccd.21191

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Allen HD. Moss & Adams’ Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 8th edn. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2015.

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Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. First of two parts. N Engl J Med 2000; 342:256–63. https://doi.org/10.1056/NEJM200001273420407

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Armstrong WF, Ryan T, Feigenbaum H. Feigenbaum’s Echocardiography. 7th edn. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2010.

9. 

Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease): developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118:e833. https://doi.org/10.1161/CIRCULATIONAHA.108.190811

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Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE. Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC). Guidelines for the management of grown-up congenital heart disease. The Task Force on the Management of Grown–up Congenital Heart Disease of the European Society of Cardiology (ESC) endorsed by the European Pediatric Cardiology (AEPC). Euro Heart J 2010; 31:2915–57. https://doi.org/10.1093/eurheartj/ehq249

11. 

Forbes TJ, Kim DW, Du W, et al. Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). J Am Coll Cardiol 2011; 58:2664–74. https://doi.org/10.1016/j.jacc.2011.08.053




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