Article, Cardiology

Pseudocoarctation of the aorta presented as chest discomfort with a pulsatile left supraclavicular mass and left arm coldness

Case Report

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American Journal of Emergency Medicine

journal homepage: www. elsevier. com/ locate/ajem

American Journal of Emergency Medicine 35 (2017) 523.e1-523.e3

Pseudocoarctation of the aorta presented as chest discomfort with a pulsatile left supraclavicular mass and left arm coldness?,??,?

Pseudocoarctation of the aorta is a rare congenital anomaly of the aortic arch, and it has been described as an elongation of the aortic arch with kinking at the level of the ligamentum arteriosum. It is usually asymptomatic or presents with hypertension; therefore, it is often diag- nosed late despite the recognition of hypertension in young patients. It is also occasionally misdiagnosed as a mediastinal mass or a true coarc- tation of the aorta, for which surgical correction is often needed. We present an unusual case of pseudocoarctation of the aorta associated with multiple saccular aneurysms and a hypoplastic left subclavian ar- tery in a 19-year-old man who was admitted in the emergency depart- ment for chest discomfort with a pulsatile left supraclavicular mass and left arm coldness. The patient was free from any major cardiovascular events for a follow-up period of 12 months with conservative treatment.

A 19-year-old man was admitted in the emergency department for chest discomfort. He reported that the chest discomfort wors- ened during hard exercise or emotional stress and was often accom- panied by a pulsatile left supraclavicular mass and left arm coldness. He had a medical history of hypertension without medication diag- nosed at 2 years ago. On initial examination, auscultation of the heart revealed a 3/6 harsh pansystolic murmur at the left upper ster- nal border with a 2.0 x 2.0-cm pulsating left supraclavicular mass. Review of vital signs revealed pressures of 150/91 (right brachial), 112/74 (left brachial), 154/96 (right ankle), and 145/87 mm Hg (left ankle); and his pulse was 71 beats/min. He had weaker left ra- dial pulsation, and the left arm was cold compared with the opposite arm. Radial-femoral delay of the right extremities was palpated within the normal range but was palpated relatively weak on the left side.

Twelve-lead electrocardiogram on admission showed sinus rhythm. Posteroanterior Chest x-ray revealed left superior mediastinal widening with a positive cervicothoracic sign (Fig. 1), presumed to be a posterior superior mediastinal mass without cardiomegaly. The subse- quent 3-dimensional thoracic computed tomographic angiography revealed pseudocoarctation of the aorta (PCoA) with multiple saccular aneurysms (Fig. 2A-D). Seven saccular aortic aneurysms were observed, with a maximal aortic diameter of 34 mm. The left subclavian artery was even more hypoplastic than the right (maximal diameter 4 mm vs 8 mm), but collateral circulation was not detected (Fig. 2E). The pulsatile left supraclavicular mass was likely caused by the

? Grant or other financial support: None.

?? Previous presentation: None.

? Conflicts of interest: None declared.

elongated and kinked aorta with saccular aneurysms (Fig. 2F), and the left arm coldness likely resulted from hypoplastic left subclavian artery (Fig. 2E). To relieve peripheral vascular resistance, we prescribed ramipril 5 mg daily, which was gradually increased to 10 mg, and added bisoprolol 2.5 mg daily for heart Rate control.

Transthoracic echocardiography was performed in the outpatient clinic and showed normal cardiac function (ejection fraction, 62%) without evidence of other associated congenital cardiac anomalies. The patient was followed up with a CT scan every 6 months for follow-up of multiple saccular aneurysms, as described in the rele- vant guidelines [1]. The patient’s symptoms were relieved, and he was free from any major cardiovascular events for a total follow-up period of 12 months.

Pseudocoarctation of the aorta is a rare anomaly of the thoracic aorta that occurs when the third to seventh embryonic dorsal seg- ments fail to fuse properly to form the aortic arch. An elongation of the aortic arch and the first portion of the descending aorta occurs; as it is fixed by the ligamentum arteriosum, an abnormal kink de- velops [2]. Although cardiac catheterization and interventional angi- ography provide a definitive diagnosis for this condition, CXR with thoracic CTA is very helpful, and the following findings are consid- ered diagnostic of this congenital anomaly according to previous case reports: (1) demonstration of the abnormal mass in the superior mediastinum; (2) an unusually high aortic arch in the mediastinum;

(3) visualization of the isthmic portion of the descending thoracic aorta not adjacent to the spine but rather ventral to it and surrounded by aerated lung; and (4) a more caudal origin of the left subclavian artery [3,4]. Unlike true coarctation of the aorta, PCoA has no critical isthmic lesion and has been described in the lit- erature as a “benign” entity that warrants no interventional therapy, but surgical treatment is considered in cases of aneurysm formation, which seems to increase the risk of rupture [5,6].

Our case was unusual in terms of Patient symptoms and associated findings. To the best of our knowledge, there have been no previous re- ports of PCoA associated with multiple saccular aneurysms and hypo- plastic left subclavian artery. Despite the abnormal tortuosity and morphologic aspects, similar to those of aortic coarctation, a significant gradient rarely develops through the kinking, and it is usually asymp- tomatic, even if hypertension, chest pain, or dysphagia may occasionally be present [2]. Moreover, although a few case reports [5,7,8] demon- strated PCoA complicated by an aortic aneurysm, PCoA with 3 or more aneurysms has rarely been reported [6,9].

Our case showed an exceptional association of PCoA with a hypo- plastic left subclavian artery. Embryologically, the left subclavian artery is derived entirely from the left seventh intersegmental artery, whereas the portions of the right are derived from the right fourth arch and the

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523.e2 I.-S. Kim et al. / American Journal of Emergency Medicine 35 (2017) 523.e1523.e3

Seong Jong Yun, MD

Department of Radiology, Aerospace Medical Center, Republic of Korea Air Force, PO Box 335-21, 635 Danjae-ro, Namil-myeon, Cheongwon-gun

Chungcheongbuk-do 363-849, Republic of Korea Corresponding author at: Department of Radiology, Aerospace Medical Center, Republic of Korea Air Force, PO Box 335-21, 635

Danjae-ro, Namil-myeon, Cheongwon-gun Chungcheongbuk-do 363-849, Republic of Korea Tel.: +82 43 290 5691; fax: +82 43 290 5692

E-mail address: [email protected]

Sun Hwa Lee, MD, PhD Department of Emergency Medicine, Sanggye Paik Hospital, Inje University College of Medicine, 1342 Dongil-ro, Seoul, Nowon-gu 139-707

Republic of Korea

Dong Hyeon Kim, MD Hyeon Hwan Cho, MD

Department of Radiology, Aerospace Medical Center, Republic of Korea Air Force, PO Box 335-21, 635 Danjae-ro, Namil-myeon, Cheongwon-gun

Chungcheongbuk-do 363-849, Republic of Korea

Fig. 1. Posteroanterior CXR demonstrates left superior mediastinal widening (arrows). This silhouette extends to the upper border above the level of the clavicle (cervicothoracic sign positive).

right dorsal aorta [10]. We believe that the hypoplastic subclavian artery impacted the pseudocoarctation. The hypoplastic left subclavian artery was responsible for the difference in pulsations and blood pressures be- tween the upper limbs.

In summary, PCoA with multiple saccular aneurysms and a hypo- plastic left subclavian artery is a rare clinical condition, but emergency physicians should remember this clinical entity and related clinical pre- sentation, especially in young patients with hypertension, because PCoA may mimic other diseases and may present as aortic rupture in cases of late diagnosis.

In-Soo Kim, MD Yonsei University College of Medicine, Yonsei University Health System, 250 Seungsanno, Seodaemun-gu, Seoul 120-752, Republic of Korea

Division of Cardiology, Department of Internal Medicine, Aerospace Medical Center, Republic of Korea Air Force, PO Box 335-21, 635 Danjae-ro Namil-myeon, Cheongwon-gun, Chungcheongbuk-do 363-849

Republic of Korea

Minji Kim, MD Yonsei University College of Medicine, Yonsei University Health System, 250 Seungsanno, Seodaemun-gu, Seoul 120-752, Republic of Korea

http://dx.doi.org/10.1016/j.ajem.2016.10.025

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    Fig. 2. A, Three-dimensional thoracic CTA reveals a high-riding aortic arch located just below the left thyroid gland (asterisk). B, The descending aorta is kinked and elongated, and the narrowest lumen (arrows) measured approximately 13 mm immediately distal to the origin of the left subclavian artery, at the level of the ligamentum arteriosum. C and D, Volume- rendering images show 5 saccular aneurysms in the Ascending aorta (C) and 2 saccular aneurysms in the descending aorta near the more caudal originated left subclavian artery (arrow) (D). E, The diameter of the left subclavian artery (arrows) is even smaller than that of the right (arrowheads), but collateral circulation is not detected. F, The location of the pulsatile left supraclavicular mass is consistent with saccular aneurysms (arrows) at the left supraclavicular level.

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