Masturbation-induced presyncope: a case report of painless aortic dissection in immunoglobulin G4-related disease

Article information

Clin Exp Emerg Med. 2025;12(1):86-89

Publication date (electronic) : 2024 March 15

doi : https://doi.org/10.15441/ceem.23.182

Charles Sanky, Nidhish Sasi

Department of Emergency Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA

Correspondence to: Charles Sanky Department of Emergency Medicine, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Place, New York, NY 10029, USA Email: charles.sanky@mountsinai.org

Received 2023 December 28; Revised 2024 February 21; Accepted 2024 March 2.

Abstract

INTRODUCTION

Aortic dissections typically present with severe, stabbing chest pain; as such, painless dissection is an atypical presentation associated with increased mortality. Immunoglobulin G4 (IgG4)-related disease is an autoimmune condition with multiple manifestations, including vascular involvement. This is the first case of its kind to document an atypical, painless aortic dissection presentation in a patient with IgG4-related disease.

CASE REPORT

This case concerns a 59-year-old male patient with a medical history of hypertension, chronic renal insufficiency, and autoimmune pancreatitis secondary to IgG4-related disease, who was on chronic steroid treatment. After masturbating, the patient experienced lightheadedness, a tingling sensation in both hands, and jaw tightness. He decided to lie down and experienced an episode of urinary incontinence, after which he called emergency medical services. Upon arrival of emergency medical services, the patient was hypotensive to 84/50 mmHg and bradycardic to 30 beats per minute. He was given 0.5 mg of atropine and 300 mL of normal saline with improvement of vital signs and symptoms. He denied experiencing chest pain, back pain, shortness of breath, confusion, frank numbness, loss of consciousness, nausea, vomiting, fever, or chills. One day prior, the patient went on a 3-mile (4.8 km) jog and admitted to feeling dehydrated. He denied any insect bites, recent travel, drug use, or alcohol intake.

In the emergency department, he was persistently hypotensive to 95/47 mmHg and bradycardic to 58 beats per minute. He received intravenous fluids. His physical exam demonstrated a new systolic murmur. His chest x-ray was normal. He had vasoplegia: an excessive low systemic vascular resistance in the setting of high cardiac output, commonly found following cardiac surgery or in sepsis. Despite the lack of an obvious source, broad-spectrum antibiotics were empirically administered for suspected severe sepsis given leukocytosis to 14.6×10⁹/L, lactate to 2.7 mmol/L, hypotension, immunocompromise, and vasoplegia. His vital signs improved, and he was hemodynamically stable. On gait testing, the patient reported some nonspecific postural dizziness, and he was admitted to the medicine floors on telemetry for further monitoring and cardiac workup because of his new auscultated murmur and constellation of signs and symptoms. Despite an emergency department cardiac ultrasound showing no acute abnormalities, a formal inpatient transthoracic echocardiogram revealed an acute type A aortic dissection and severe aortic regurgitation. This was further confirmed with expedited computed tomography angiography, which revealed a dissection originating from the aortic root with dissection flaps extending to the brachiocephalic, left subclavian, and left common iliac arteries (Figs. 1, 2). The celiac artery, inferior mesenteric artery, and right renal artery arose from the false lumen. After diagnosis, the patient was transferred to the cardiac care unit on an esmolol infusion, and then taken to the operating room by cardiothoracic surgery for aorta graft replacement.

Fig. 1.

Computed tomography angiography (axial view), demonstrating type A ascending aortic dissection.

Fig. 2.

Computed tomography angiography (coronal view), demonstrating type A ascending aortic dissection.

DISCUSSION

This case demonstrates an unusual presentation of aortic dissection without chest pain, shortness of breath, or hypertension. This patient had symptoms that could be consistent with vasoplegia and/or neurovascular sequelae given his episode of subjective paresthesia in both hands, although symptoms resolved completely before presentation. Furthermore, he was hypotensive, not hypertensive, which is classically more consistent with later, more severe manifestations of aortic syndrome, such as tamponade or rupture [1]. Approximately 6.4% of aortic dissections present without chest pain; as such, painless dissection is an atypical presentation [2] associated with increased mortality [3]. This patient’s x-ray showed no mediastinal widening, a pathognomonic finding only in 12% to 15% of patients with acute aortic dissection [4]. Syncope is observed in only 12% of patients with type A dissection [4]. Additionally, review of the International Registry of Acute Aortic Dissection demonstrated a murmur consistent with aortic regurgitation in 44% of type A dissections and 12% of type B dissections [4]. The proposed mechanism is that a type A dissection flap distorts leaflet alignment, leading to valvular pathology. Although a new systolic murmur was detected by the emergency department and internal medicine teams, the cardiac care unit team documented a diastolic murmur. It is possible that the murmur evolved over time; a literature review demonstrates that systolic murmurs are also found in early aortic dissection and are believed to be secondary to aortic deformity in the beginning phase of dissection and intimal tearing [5]. As such, new murmurs of any kind, not just diastolic murmurs, may be found in acute aortic dissection.

IgG4-related disease is a complex autoimmune, fibroinflammatory disease that can cause multiorgan dysfunction [6,7]. One case report demonstrated IgG4 disease resulting in spontaneous coronary artery dissection [8]. Case series have shown that patients with IgG4 may have possible large vessel vasculitis, namely with aortic and periaortic involvement, but rarely in the form of aortic dissection. When it is present, aortic dissection is diagnosed in the late stages of aortic involvement, such as after development of a large aortic aneurysm. In these cases, the dissection is usually associated with chest pain [9]. One patient with atypical chest pain was found to have aortitis with diffuse lymphoid infiltration, fibrous/atheromatous lesions, and interstitial hyperplasia [10]. Another case report discussed a patient with IgG4 disease who was found to have an aortic dissection intraoperatively while attempting aortic root replacement [11]. She was thought to have an ascending aortic aneurysm and dilated root, although histological studies demonstrated active mural inflammation with microabscess formation and the presence of IgG4. Although this patient had a history of chronic renal insufficiency, the mechanism of renal insufficiency is fibrosis within the kidney itself, and is attributed to tubulointerstitial nephritis and membranous nephropathy [12]; as such, it is unlikely that a prior dissection in the right renal artery was related primarily to chronic renal insufficiency. Rather, fibroinflammatory consequences of vascular involvement were the likely etiology.

The patient was on chronic steroids as a treatment for their IgG4-related disease. Chronic steroid use has been demonstrated to result in hypertension and cause vessel fragility due to its impact on connective tissue strength, collagen formation, and mural thickness [13]. This patient’s presentation is likely multifactorial, but we cannot rule out the possible implications of chronic steroid use. Nevertheless, the patient’s hypotension, painless presentation, computed tomography angiography, and operative visualization suggest a mechanism of interstitial hyperplasia, not mural thinning, which is more associated with the classic, tearing chest and back pain [14]. As such, IgG4-related disease is an important risk factor for this patient’s painless dissection.

This case reinforces that aortic dissection can be present without pain, including the classic presentation of tearing chest pain or back pain. The mechanism of fibroinflammatory processes causing aortic vasculitis and ultimately aneurysm and dissection require more investigation; in this patient, the rapid, spontaneous progression without pain demonstrates the potential insidiousness and severity of this condition. When considering presyncope in conjunction with possible temporary paresthesia or vasoplegia, aortic dissection should be included in the differential diagnosis. Additionally, new murmurs in the setting of these symptoms should prompt an echocardiogram and/or cardiology consultation to assess valvular pathology or dissection, such as seen in this case. Furthermore, understanding IgG4-related disease as an autoimmune condition with multiple manifestations, including vascular emergencies, is paramount. It is important for providers to consider a wider differential in this rare subset of patients. In summary, this case reinforces the need for further investigation into the pathophysiology behind IgG4-related manifestations. Emergency physicians should maintain a high index of suspicion for aortic syndrome in all emergency department patients, especially patients with IgG4-related disease, and consider atypical presentations and manifestations of aortic dissection.

Article information Continued

Notes