Aortic Pseudoaneurysm-Related Acute Ortner’s Syndrome Presenting as Sudden Hoarseness: A Case Series

Introduction

Ortner syndrome was originally described as a left recurrent laryngeal nerve palsy caused by left atrial enlargement due to mitral valve stenosis [1]. Currently, the term cardiovocal syndrome is more frequently used, to denote that left recurrent laryngeal nerve palsy can be secondary to other cardiovascular causes [2]. From 1% to 3% of cases of extra-laryngeal hoarseness have been attributed to Ortner syndrome. Several cardiovascular pathologies have been described as causes of left recurrent laryngeal nerve palsy, including pulmonary hypertension, recurrent pulmonary emboli, and some congenital heart diseases [3].

Aortic pseudoaneurysm is a rare life-threatening condition resulting from a weakening of the aortic wall [4]. Clinical presentation is highly variable, from no symptoms to severe chest pain, dysphagia, hemoptysis, or a pulsatile suprasternal mass. The main cause of aortic pseudoaneurysms is trauma (either penetrating or non-penetrating). However, aortic pseudoaneurysms can result from non-traumatic pathologies such as penetrating aortic ulcer (PAU) [5].

Case Reports

CASE 1:

A 76-year-old man was admitted to the Emergency Department with sudden hoarseness, which had started a week earlier. His medical history included hypertension, smoking habit, and asymptomatic hyperuricemia. He did not report chest pain, dyspnea, or syncope. A transnasal laryngoscopy revealed left vocal cord palsy (Video 1). The patient did not have fever, and acute phase and infection biomarkers (C-reactive protein and procalcitonin) were negative. Chest radiography showed a widened mediastinum (Figure 1A), and laboratory test results revealed high D-dimer levels (1505 ng/mL). A computed tomography (CT) scan showed a thoracic aortic pseudoaneurysm, with a maximum diameter of 52 mm (Figure 1B–1D) located at the aortic arch (zone 3), immediately distal to the exit of the left subclavian artery origin, compressing the left recurrent laryngeal nerve. Urgent thoracic endovascular aortic repair was performed, with exclusion of the sacular pseudoaneurysm, with a Zenith TX2 40-30-167 (Cook Medical) endoprosthesis (Figure 1E). The proximal landing zone was located immediately distal to the left carotid artery (zone 2). Previously, a carotid-subclavian bypass was performed. Finally, the origin of the left subclavian artery was closed with an Amplatzer vascular plug device (Figure 1E) to prevent future leaks. The patient was discharged a week later with persistent hoarseness. He partially recovered his voice at the third month of follow-up.

CASE 2:

A 60-year-old male patient with a past medical history of HIV infection, chronic obstructive pulmonary disease, and hepatitis C virus-related cirrhosis was admitted to the Emergency Department for lumbar pain and urinary tract infection. He had experienced sudden hoarseness, appearing 3 weeks before admission. Laryngoscopy showed a left vocal cord palsy (Video 2). The urinary infection was successfully treated with antibiotics. Due to the persistence of lumbar pain with no further signs, symptoms, or biomarkers of active infection, a CT scan was conducted, and a large pseudoaneurysm (30×23.5 mm) was observed on the left side of the aortic arch (Figure 2A–2D). “Aorta code” was activated, and the patient was transferred to our hospital for urgent surgery [6]. First, a selective arteriography of vertebral arteries confirmed a right dominancy. Then, the subclavian artery was occluded with an Amplatzer (14 mm) device, and a Gore TAG active control endoprosthesis was implanted. The proximal landing zone was immediately distal to the left carotid artery. Finally, sacular pseudoaneurysm embolization with 3 coils was performed through a directed micro-catheter. A control CT scan showed adequate isolation of the pseudoaneurysm, absence of leaks, left carotid artery permeability, and retrograde filling of the subclavian artery through the vertebral artery (Figure 2E). After an initial favorable evolution, the patient had several severe complications: hydropic decompensation, respiratory infection, severe sepsis, and hepatic encephalopathy and finally died.

Discussion

Ortner syndrome is a rare presentation of an acute aortic pseudoaneurysm. Infrequently, some cases of aortic pseudoaneurysm are asymptomatic and incidentally diagnosed on imaging studies. Active search of symptoms is essential to avoid mis-diagnosis. Hoarseness is often caused by benign conditions. However, as in these 2 cases, it can be the first symptom of an underlying serious condition, requiring immediate diagnosis and management. There are a few cases in the literature of aortic-related hoarseness [7], most of them caused by compression of the left recurrent laryngeal nerve by an enlarging aneurysm [2]. Here, we present 2 cases of sudden hoarseness, so-called Ortner syndrome, due to PAU, with acute progression to aortic pseudoaneurysm.

PAU is an entity within the spectrum of acute aortic syndrome [8]. PAU refers to an ulceration of an aortic atherosclerotic lesion that penetrates the internal elastic lamina into the media. PAUs are usually associated with a variable amount of intramedial hemorrhage, which can extend proximally or distally. Aortic ulcers can break through into the adventitia to form a pseudoaneurysm. In this particular situation, the over-lying adventitia contains the hemorrhage, and, therefore, this type of lesion can be considered a contained aortic rupture. The natural history and prognosis of PAU is still a matter of debate. Risk of rupture is higher than that of aortic dissection and has been reported as high as 30% to 40% [8]. A larger ulcer diameter, greater ulcer depth, and increasing pleural effusion are associated with progressive disease [8]. Most PAUs and pseudoaneurysms are diagnosed by chance in a patient undergoing CT scan for another reason. In the present case series, the infectious etiology was ruled out based on the absence of signs of infection and negative biomarkers. In addition, the CT scan images showed signs of aortic atherosclerosis on the edges of the ulcer (Figures 1B, 2A, 2B), supporting the diagnosis of PAU. A PET-CT scan could be useful in the differential diagnosis of aortic pseudoaneurysm [9].

Hoarseness is not included as an alarm symptom in patients with chest pain. However, considering these 2 cases and those reported in the literature, aortic origin should be considered in the differential diagnosis of hoarseness, particularly when it appears suddenly.

Thoracic endovascular aortic repair is a feasible option for those patients with PAUs or pseudoaneurysm located in the aortic arch. As far as we know, there are no formal recommendations for surgical intervention based on the diameter of the pseudo-aneurysm. In these cases, the presence of symptoms and the speed of their appearance are key markers of rapid progression and potential risk of rupture. In patients under follow-up with serial CT scans, a fast growth in the diameter is also a risk factor for aortic rupture, and the aortic repair should be considered.

Currently, thoracic endovascular aortic repair is the preferred approach for enlarging or symptomatic PAUs and thoracic aortic pseudoaneurysm in the descending aorta [8]. Open surgery is the treatment of choice for those located in the ascending aorta. The aortic arch represents a therapeutic challenge. The surgical approach can be either open or endovascular, depending on the PAU location and the surgical team’s experience. The treatment of patients with infective pseudoaneurysm should be individualized. Open surgery is the treatment of choice for patients with infective pseudoaneurysm, and antibiotic treatment with subsequent endovascular repair is usually considered as a secondary option. In the present cases, pseudoaneurysms were located at the distal portion of the aortic arch, and the endovascular treatment was the preferred option. Both patients required subclavian artery occlusion. In this situation, exploring the vertebral artery dominance and checking for potential left arm ischemic signs after the occlusion is mandatory.

Conclusions

Ortner syndrome is a rare presentation of acute aortic pseudoaneurysm. Hoarseness is often caused by benign conditions. However, aortic origin should be considered in the differential diagnosis of hoarseness, particularly when it appears suddenly. Thoracic endovascular aortic repair is a feasible option for those patients with PAUs or pseudoaneurysm located at the aortic arch.