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Case 361

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Female patient, 12 years old, goes to the doctor for a routine medical consultation. She has no complaints and she’s previously healthy. At physical examination, she presented pulses with slightly reduced amplitude in the lower limbs relative to the upper limbs, normorrhythmic and normophonetic heart sounds in two stages, without murmurs or extrasystoles. Her blood pressure of the upper limbs was 110 x 70 mmHg (< 90th percentile) and of the lower limbs was 100 x 80 mmHg (95th percentile). There was no other alterations at the physical examination. A color doppler ultrasound was requested, and, afterwards, a magnetic angioressonance (angioRM), as presented on the section “image analysis”.

Considering the clinical history and the color doppler ultrasound, which is the most likely diagnosis?

a) Supravalvular aortic stenosis

25%

b) Aortic dissection

25%

c) Atherosclerotic disease

25%

d) Coarctation of the aorta

25%
   

Image analysis

 

Image 1 - Analysis:  Color doppler ultrasonography of the descending aorta. Colorful mosaic flow (green arrow) indicating turbulent flow. The maximum speed reached is 2.99m / s (red asterisk and dotted line), translating pressure gradient of 35.7 mmHg; There is also a diastolic tail (yellow highlight).

 

 

Image 2 - Analysis: Magnetic angioressonance of the aorta, gradient echo sequence. Left anterolateral vision. There is a segmental stenosis in the aortic arch (green highlight), prior to the left subclavian artery emergence (red highlight). The ascending and descending aorta are presenting normal calibers (highights in blue and yellow, respectively).

 

 

Image 3 - Analysis: Magnetic angioressonance of the aorta, gradient echo sequence. Right sagittal vision. There is a segmental stenosis in the aortic arch (green highlight), prior to the left subclavian artery emergence (red highlight), which has an anomalous position. The ascending and descending aorta are presenting normal calibers (highights in blue and yellow, respectively).

 

 

Image 4 - Analysis: Magnetic angioressonance of the aorta, gradient echo sequence. Linear reconstruction of the aortic arch. There is a segmental stenosis in the aortic arch (green highlight), with a 0,71 cm diameter (green arrow). Ascending aorta (blue highlight); descending aorta (yellow highlight). Emergence of the left subclavian aorta in anomalous position, after the stenosis - anatomic variation (red highlight). The blue and the yellow arrows indicate the limits of the coarctation. 

Highlights

-Usually, coarctation of the aorta (CoA) is the congenital narrowing of the descending aorta, commonly in the insertion of the arterial duct/ligament, most common in boys (59% versus 41% in girls); 

- The CoA generally doesn’t induce problems in the intrauterine time, due to the current flow through the arterial duct, and it’s closing mode defines the symptoms of the patient.

- These findings suggest the CoA diagnosis: high blood pressure in the upper limb, pulse delay in lower limb or diminished amplitude. It needs confirmation by echocardiography.

- Magnetic angioressonance or computed angiotomography can be requested posteriorly to give additional anatomic information and guide the therapeutic intervention.

- The early treatment of the CoA prevents development of complication and provides improvement of life’s quality.

References

1- Agawala NB, Bacha MDEmile, MD, et al. Clinical manifestations and diagnosis of coarctation of the aorta. UpToDate [Internet] 2019. Available at https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-coarctation-of-the-aorta

2- Agawala NB, Bacha MDEmile, MD, et al. Management of coarctation of the aorta. UpToDate [Internet] 2019. Available at https://www.uptodate.com/contents/management-of-coarctation-of-the-aorta?topicRef=5760&source=see_link

3- Baumgartner H, Bonhoeffer P, De Groot MSN, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). European Heart Journal (2010) 31, 2915–2957.

Author

Leandra Prates Diniz, 4th year medical student at UFMG

E-mail: leandraprates[at]hotmail.com

Supervisors

Dr. Alexandre Henrique Cobucci Santana, cardiologist, coordinator of the Echocardiography Department at Madre Teresa Hospital, Belo Horizonte, MG.

E-mail: alexcobucci[arroba]gmail.com

 

Dra. Danielle Lopes Rocha, pediatric cardiologist at Centrocor, Vitória, ES.

E-mail: dn.lopes[at]hotmail.com

Reviewers

Mirella Diniz, Bruno Jordão, Letícia de Melo, Gabriel de Figueiredo, Raphael Dias, prof. JosÄ— Nelson Mendes Vieira, profa. Viviane Santuari Parisotto Marino.

Translated by

Leandra Prates Diniz, 4th year medical student at UFMG

E-mail: leandraprates[at]hotmail.com

Test question

a)

25%

b)

25%

c)

25%

d)

25%

e)

25%
   

Commentics

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